Apley's System of Orthopaedics and Fractures 9th Ed

Apley’s
System of Orthopaedics
and Fractures
Alan Graham Apley 1914–1996
Inspired teacher, wise mentor and joyful friend
Louis Solomon MD FRCS
Emeritus Professor of Orthopaedics
Bristol
UK
David Warwick MD FRCS FRCSOrth Eur Dip Hand Surg
Consultant Hand Surgeon
Reader in Orthopaedic Surgery
University of Southampton
Southampton
UK
Selvadurai Nayagam BSc MChOrth

FRCSOrth
Consultant Orthopaedic Surgeon
Royal Liverpool Children’s Hospital
and
The Royal Liverpool University
Hospital
Liverpool
UK
Apley’s
System of Orthopaedics
and Fractures
Ninth Edition
First published in Great Britain in 1959 by Butterworths Medical Publications
Second edition 1963
Third edition 1968
Fourth edition 1973
Fifth edition 1977
Sixth edition 1982
Seventh edition published in 1993 by Butterworth Heineman.
Eight edition published in 2001 by Arnold.
This ninth edition published in 2010 by
Hodder Arnold, an imprint of Hodder Education, an Hachette UK Company,
338 Euston Road, London NW1 3BH
http://www.hodderarnold.com
© 2010 Solomon, Warwick, Nayagam
All rights reserved. Apart from any use permitted under UK copyright law, this publica-
tion may only be reproduced, stored or transmitted, in any form, or by any means, with
prior permission in writing of the publishers or in the case of reprographic production, in
accordance with the terms of licences issued by the Copyright Licensing Agency. In the
United Kingdom such licences are issued by the Copyright Licensing Agency:
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Whilst the advice and information in this book are believed to be true and accurate at the
date of going to press, neither the author[s] nor the publisher can accept any legal
responsibility or liability for any errors or omissions that may be made. In particular (but
without limiting the generality of the preceding disclaimer) every effort has been made to
check drug dosages; however it is still possible that errors have been missed. Furthermore,
dosage schedules are constantly being revised and new side-effects recognized. For these
reasons the reader is strongly urged to consult the drug companies’ printed instructions
before administering any of the drugs recommended in this book.
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iv
Dedication
To our students, trainees and patients, all of whom have helped to make our lives
interesting, stimulating and worthwhile; and also to our wives and children (and
grand-children) who have tolerated our absences – both material and spiritual – while
preparing this new edition.
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Contents
Contributors ix
Preface xi
Acknowledgements xiii
List of abbreviations used xv
PART 1: GENERAL ORTHOPAEDICS

1 Diagnosis in orthopaedics 3
Louis Solomon, Charles Wakeley
2 Infection 29
Louis Solomon, H. Srinivasan, Surendar Tuli, Shunmugam Govender
3 Inflammatory rheumatic disorders 59
Christopher Edwards, Louis Solomon
4 Crystal deposition disorders 77
Louis Solomon
5 Osteoarthritis 85
Louis Solomon
6 Osteonecrosis and related disorders 103
Louis Solomon
7 Metabolic and endocrine disorders 117
Louis Solomon
8 Genetic disorders, skeletal dysplasias and malformations 151
Deborah Eastwood, Louis Solomon
9 Tumours 187
Will Aston, Timothy Briggs, Louis Solomon
10 Neuromuscular disorders 225
Deborah Eastwood, Thomas Staunton, Louis Solomon
11 Peripheral nerve injuries 269
David Warwick, H. Srinivasan, Louis Solomon
12 Orthopaedic operations 303
Selvadurai Nyagam, David Warwick
PART 2: REGIONAL ORTHOPAEDICS

13 The shoulder and pectoral girdle 337
Andrew Cole, Paul Pavlou
14 The elbow and forearm 369
David Warwick
15 The wrist 383
David Warwick, Roderick Dunn
16 The hand 413
17 The neck 439
Stephen Eisenstein, Louis Solomon
18 The back 453
Stephen Eisenstein, Surendar Tuli, Shunmugam Govender
19 The hip 493
Louis Solomon, Reinhold Ganz, Michael Leunig, Fergal Monsell,
Ian Learmonth
20 The knee 547
Louis Solomon, Theo Karachalios
21 The ankle and foot 587
Gavin Bowyer
PART 3: FRACTURES AND JOINT INJURIES

22 The management of major injuries 627
David Sutton, Max Jonas
23 Principles of fractures 687
Selvadurai Nayagam
24 Injuries of the shoulder, upper arm and elbow 733
Andrew Cole, Paul Pavlou, David Warwick
25 Injuries of the forearm and wrist 767
David Warwick
26 Hand injuries 787
David Warwick
27 Injuries of the spine 805
Stephen Eistenstein, Wagih El Masry
28 Injuries of the pelvis 829
Louis Solomon
29 Injuries of the hip and femur 843
Selvadurai Nayagam
30 Injuries of the knee and leg 875
Selvadurai Nayagam
31 Injuries of the ankle and foot 907
Gavin Bowyer
Epilogue: Global Orthopaedics 935

Christopher Lavy, Felicity Briggs
Index 939
CONTENTS
viii
Contributors
Principal Authors Timothy William Roy Briggs MD(Res)

MCh(Orth) FRCS FRCS Ed
Professor and Consultant Orthopaedic Surgeon
Louis Solomon MD FRCS Eng FRCS Ed Joint Medical Director
Emeritus Professor of Orthopaedic Surgery Joint Training Programme Director
Royal National Orthopaedic Hospital
Honorary Consultant Orthopaedic Surgeon Stanmore, UK
Bristol Royal Infirmary, Bristol, UK Tumours
Selvadurai Nayagam BSc, MChOrth FRCSOrth

Consultant Orthopaedic Surgeon Andrew Spencer Cole BSc MBBS
Royal Liverpool Children’s Hospital and FRCS(TR&Orth)
The Royal Liverpool University Hospital Consultant Orthopaedic Surgeon
Liverpool, UK Southampton University Hospitals
Southampton, UK
David Warwick MD BM FRCS FRCS (Orth) The Shoulder and Pectoral Girdle
Eur Dip Hand Surg Injuries of the Shoulder and Upper Arm and elbow
Consultant Hand Surgeon
Reader in Orthopaedic Surgery
University of Southampton, Southampton, UK Roderick Dunn MBBS DMCC FRCS(Plast)
Consultant Plastic, Reconstructive and Hand
Surgeon, Odstock Centre for Burns, Plastic and
Maxillofacial Surgery, Salisbury District Hospital
Salisbury, UK
The Wrist and The Hand: Congenital Variations
Contributing Authors
Deborah Eastwood FRCS
Will Aston BSc, MBBS, FRCS Ed(TR&Orth) Consultant Orthopaedic Surgeon and
Consultant Orthopaedic Surgeon Hon Senior Lecturer
Royal National Orthopaedic Hospital University College London;
Stanmore, UK Great Ormond Street Hospital for Children
Tumours London, UK
Genetic Disorders, Dysplasias and Malformations
Gavin William Bowyer MA MChir FRCS(Orth) Neuromuscular Disorders
Consultant Trauma and Orthopaedic Surgeon
and Honorary Senior Lecturer
Southampton University Hospitals Christopher J Edwards BSc MBBS FRCP MD
Southampton, UK Consultant Rheumatologist & Honorary Senior
The Ankle and Foot Lecturer
Injuries of the ankle and foot Associate Director
Wellcome Trust Clinical Research Facility
Felicity Briggs MA(Oxon) UK Southampton University Hospitals NHS Trust
Research Assistant and Graduate Medical Student Southampton General Hospital, UK
Epilogue: Global Orthopaedics Inflammatory Rheumatic Disorders
Stephen Eisenstein PhD FRCS(Ed) Wagih S El Masry FRCS FRCP
Hon Professor, Keele University; Emeritus Director Consultant Surgeon in Spinal Injuries;
Centre for Spinal Studies; Director, Midlands Centre for Spinal Injuries
The Robert Jones and Agnes Hunt Orthopaedic President International Spinal Cord Society (ISCOS)
Hospital, Shropshire, UK RJ & AH Orthopaedic Hospital, Oswestry, UK
The Neck Injuries of the Spine
The Back
Injuries of the Spine Fergal P Monsell MSc FRCS FRCS(Orth)
Consultant Paediatric Orthopaedic Surgeon
Reinhold Ganz MD Bristol Royal Hospital for Children
Professor and Chairman Emeritus Bristol, UK
Orthopaedic Department Inselspital The Hip: Disorders in Children
University of Bern, Switzerland
The Hip: Femoro-acetabular Impingement Paul Pavlou BSc (Hons) MB BS MRCS
Orthopaedic Registrar, Wessex training scheme
Shunmugam Govender MBBS MD FRCS The Shoulder and Pectoral Girdle
FC(Orth) (SA) Injuries of the Shoulder
Professor and Head of Department of Orthopaedics;
Director of Spinal Services King George V Hospital; H. Srinivasan MB BS FRCS FRCS Ed
Nelson R Mandela School of Medicine DSc (Hon)
Durban, South Africa Formerly Senior Orthopaedic Surgeon
Infection Central Leprosy Teaching & Research Institute
The Back: Infections of the Spine Chengalpattu (Tamil Nadu), India;
Director Central JALMA Institute for Leprosy
Max Jonas MBBS FRCA (ICMR), Agra (UP), India; and Editor Indian
Consultant and Senior Lecturer in Critical Care Journal of Leprosy
Southampton University Hospitals NHS Trust Infection and Peripheral Nerve Disorders: Leprosy
Southampton, UK
The Management of Major Injuries Thomas G Staunton MB FRCP(C) FRCP
Consultant Neurologist
Theo Karachalios MD DSc Norfolk and Norwich University Hospital;
Associate Professor in Orthopaedics, Consultant Clinical Neurophysiologist
School of Health Sciences, University of Thessalia Robert Jones and Agnes Hunt Orthopaedic
University General Hospital of Larissa Hospital, Shropshire, UK
Hellenic Republic Neuromuscular Disorders: Neurophysiological Studies
The Knee
David Sutton BM DA FRCA
Christopher Lavy OBE MD MCh FRCS Department of Anaesthetics
Hon Professor and Consultant, Southampton General Hospital
Nuffield Department of Orthopaedic Surgery, Southampton, UK
University of Oxford, UK Management of Major Injuries
Epilogue: Global Orthopaedics
Surendar Mohan Tuli MBBS MS PhD
Ian Douglas Learmonth MB ChB FRCS Ed FRCS Senior Consultant in Spinal Diseases and
FCS(SA)Orth Orthopaedics, Vimhans Hospital, New Delhi, India
Emeritus Professor, ; Infection: Tuberculosis of Bones and Joints
Honorary Consultant, University Hospitals, Bristol; The Back
CONTRIBUTORS
Honorary Consultant, North Bristol Trust, UK

Total Hip Replacement Charles J Wakeley BSc MBBS FRCS FRCS Ed
FRCR
Michael Leunig MD Consultant Radiologist, Department of Radiology
Head of Orthopaedics, Lower Extremities University Hospital Bristol NHS Foundation Trust
Schulthess Klinik, Zurich, Switzerland Bristol, UK
The Hip: Femoro-Acetabular Impingement Diagnosis in Orthopaedics: Imaging
x
Preface
When Alan Apley produced the first edition of his Sys- covering the main types of musculoskeletal disorder;
tem of Orthopaedics and Fractures 50 years ago he Regional Orthopaedics, where we engage with these
saw it as an aid to accompany the courses that he con- disorders in specific parts of the body; and thirdly
ducted for aspiring surgeons who were preparing for Fractures and Joint Injuries. In a major departure
the FRCS exams. With characteristic humour, he from previous editions, we have enlisted the help of
called the book ‘a prophylactic against writer’s colleagues who have particular experience of condi-
cramp’. Pictures were unnecessary: if you had any tions with which we as principal authors are less famil-
sense (and were quick enough to get on the heavily iar. Their contributions are gratefully acknowledged.
oversubscribed Apley Course) you would be treated Even here, though, we have sought their permission
to an unforgettable display of clinical signs by one of to ‘edit’ their material into the Apley mould so that
the most gifted of teachers. the book still has the sound and ‘feel’ of a single
You also learnt how to elicit those signs by using a authorial voice.
methodical clinical approach – the Apley System. The For the second edition of the book, in 1963, Apley
Fellowship exam was heavily weighted towards clinical added a new chapter: ‘The Management of Major
skills. Miss an important sign or stumble over how to Accidents’. Typically frank, he described the current
examine a knee or a finger and you could fail outright. arrangements for dealing with serious accidents as
What Apley taught you was how to order the steps in “woefully inadequate” and offered suggestions based
physical examination in a way that could be applied to on the government’s Interim Report on Accident
every part of the musculoskeletal system. ‘Look, Feel, Services in Great Britain and Ireland (1961). There
Move’ was the mantra. He liked to say that he had a has been a vast improvement since then and the num-
preference for four-letter words. And always in that ber of road accident deaths today is half of what it was
order! Deviate from the System by grasping a in the 1960’s (Department of Transport statistics). So
patient’s leg before you look at it minutely, or by test- important is this subject that the relevant section has
ing the movements in a joint before you feel its con- now been re-written by two highly experienced Emer-
tours and establish the exact site of tenderness and gency and Intensive Care Physicians and is by far the
you risked becoming an unwilling participant in a the- longest chapter in the present edition.
atrical comedy. Elsewhere the text has been brought completely up
Much has changed since then. With each new edi- to date and new pictures have been added. In most
tion the System has been expanded to accommodate cases the illustrations appear as composites – a series
new tests and physical manoeuvres developed in the of images that tell a story rather than a single ‘typical’
tide of super-specialisation. Laboratory investigations picture at one moment in the development of some
have become more important and imaging techniques disorder. At the beginning of each Regional chapter,
have advanced out of all recognition. Clinical classifi- in a run of pictures we show the method of examin-
cations have sprung up and attempts are now made to ing that region: where to stand, how to confront the
find a numerical slot for every imaginable fracture. No patient and where to place our hands. For the experi-
medical textbook is complete without its ‘basic sci- enced reader this may seem like old hat; but then we
ence’ component, and advances are so rapid that have designed this book for orthopaedic surgeons of
changes become necessary within the period of writ- all ages and all levels of experience. We all have some-
ing a single edition. The present volume is no excep- thing to learn from each other.
tion: new bits were still being added right up to the As before, operations are described only in outline,
time of proof-reading. emphasising the principles that govern the choice of
For all that, we have retained the familiar structure treatment, the indications for surgery, the design of the
of the Apley System. As in earlier editions, the book is operation, its known complications and the likely out-
divided into three sections: General Orthopaedics, come. Technical procedures are learnt in simulation
courses and, ultimately, in the operating theatre. Writ- about this now, what with the plethora of ‘search
ten instructions can only ever be a guide. Drawings are engines’ that have come to dominate the internet. We
usually too idealised and ‘in theatre’ photographs are can merely bow our heads and say we still have those
usually intelligible only to someone who has already doubts and have given references only where it seems
performed that operation. Textbooks that grapple with appropriate to acknowledge where an old idea started
these impediments tend to run to several volumes. or where something new is being said that might at
The emphasis throughout is on clinical first sight be questioned.
orthopaedics. We acknowledge the value of a more More than ever we are aware that there is a dwin-
academic approach that starts with embryology, dling number of orthopaedic surgeons who grew up
anatomy, biomechanics, molecular biology, physiol- in the Apley era, even fewer who experienced his
ogy and pathology before introducing any patient to thrilling teaching displays, and fewer still who worked
the reader. Instead we have chosen to present these with him. Wherever they are, we trust that they will
‘basic’ subjects in small portions where they are rele- recognise the Apley flavour in this new edition. Our
vant to the clinical disorder under discussion: bone chief concern, however, is for the new readers who –
growth and metabolism in the chapter on metabolic we hope – will glean something that helps them
bone disease, genetics in the chapter on osteodystro- become the next generation of teachers and mentors.
phies, and so forth.
In the preface to the last edition we admitted our LS
doubts about the value of exhaustive lists of references SN
at the end of each chapter. We are even more divided DJW
PREFACE
xii
Acknowledgements
Fifty years ago Apleys’ System of Orthopaedics and are Fiona Daglish, Colin Duncan, Neeraj Garg,
Fractures was written by one person – the eponymous Nikolaos Giotakis, Jagdeep Nanchahal and Badri
Apley. As the years passed and new editions became Narayan.
ever larger, a second author appeared and then a We have been fortunate in having friends and family
third. Throughout those years we have always been around us who have given us helpful criticism on the
able to get help (and sometimes useful criticism) from presentation of this work. Caryn Solomon, a tireless
willing colleagues who have filled the gaps in our internet traveller, found the picture for the cover and
knowledge. Their words and hints are scattered Joan Solomon gave expert advice on layout and
among the pages of this book and we are forever design. James Crabtree stepped in as a model for
grateful to them. some ‘clinical’ pictures. We are grateful to all of them.
For the present edition we have gone a step further Throughout the long march to completion of this
and enlisted a number of those colleagues as nomi- work we have enjoyed the constant help and collabo-
nated Contributing Authors. In some cases they have ration of Francesca Naish, Gavin Jamieson, Joanna
brought up to date existing chapters; in others they Walker and Helen Townson (our Editorial Manager,
have added entirely new sections to a book that has Commissioning Editor, Production Manager and
now grown beyond the scope of two or three special- Design Manager respectively) at Hodder Arnold. No
ists. Their names are appropriately listed elsewhere problem was too complex and no obstacle too great
but here we wish to thank them again for joining us. to withstand their tireless efforts in driving this work
They have allowed us to mould their words into the forward.
style of the Apley System so that the text continues to Nora Naughton and Aileen Castell (Naughton
carry the flavour of a unified authorial voice. Project Management) were in the background setting
We are also grateful to those colleagues who have up the page copies, patiently enduring the many
supplied new pictures where our own collections have amendments that came in over the internet. Their
fallen short. In particular we want to thank Dr attention to detail has been outstanding.
Santosh Rath and Dr G.N. Malaviya for pictures of Finally, we want to express our deepest thanks to
peripheral deformities in leprosy, Mr Evert Smith for those nearest to us who added not a word to the text
pictures (and helpful descriptions) of modern but through their support and patience made it poss-
implants in hip replacement operations, Dr Peter Bul- ible for us to take so much time beyond the everyday
lough who allowed us to reprint two of the excellent occupations of family life to produce a single book.
illustrations in his book on Orthopaedic Pathology,
and Dr Asif Saifuddin for permission to use some L. S.
images from his book on Musculoskeletal MRI. D.W.
Others who gave us generous assistance with pictures S. N.
List of abbreviations used
ACA angulation correction axis CRP C-reactive protein

ACE angiotensin-converting enzyme CRPS complex regional pain syndrome
ACL anterior cruciate ligament CSF cerebrospinal fluid
ACTH adrenocorticotropic hormone CT computed tomography
AFP alpha-fetoprotein CVP central venous pressure
AIDP acute inflammatory demyelinating DDH developmental dysplasia of the hip
polyneuropathy dGEMRIC delayed gadolinium-enhanced MRI of
AIDS acquired immune deficiency cartilage
syndrome DIC disseminated intravascular coagulation
AL anterolateral DIP distal interphalangeal (joint )
ALI acute lung injury DISH diffuse idiopathic skeletal hyperostosis
AM anteromedial DISI dorsal intercalated segment instability
AMC arthrogryposis multiplex congenita DMARDs disease-modifying antirheumatic
ANA antinuclear antibodies drugs
anti-CCP anti-cyclic citrullinated peptide DRUJ distal radio-ulnar joint
antibodies DTH delayed type hypersensitivity
AP anteroposterior DVT deep vein thrombosis
APC antigen-presenting cell DXA dual-energy x-ray absorptiometry
APC anteroposterior compression (injuries) ECRB extensor carpi radialis brevis
ARCO Association Research Circulation ECRL extensor carpi radialis longus
Osseous EDF elongation-derotation-flexion
ARDS adult respiratory distress syndrome EDG extensor diversion graft
ARDS acute respiratory distress syndrome EEG electroencephalography
ARM awareness, recognition, management EMG electromyography
AS ankylosing spondylitis EMS emergency medical service
ATLS advanced trauma life support ENL erythema nodosum leprosum
AVN avascular necrosis ESR erythrocyte sedimentation rate
BASICS British Association for Immediate ETA estimated time of arrival
Care FAI femoro-acetabular impingement
BCP basic calcium phosphate FAST focussed assessment sonography in
BMD bone mineral density trauma
BMP bone morphogenetic protein FDP flexor digitorum profundus
BSA body surface area FDS flexor digitorum superficialis
BVM bag-valve-mask FFOs functional foot orthoses
CDH congenital dislocation of the hip FPB flexor pollicis brevis
CFD congenital femoral deficiency FPE fatal pulmonary embolism
CMAP compound muscle action potential FPL flexor pollicis longus
CMC carpo-metacarpal GABA gamma-aminobutryic acid
CMI cell-mediated immunity GAGs glycosaminoglycans
CNS central nervous system GCS Glasgow Coma Scale
COMP cartilage oligometric matrix protein GMFCS gross motor function classification
CORA centre of rotation of angulation system
CPM continuous passive motion GPI general paralysis of the insane
CPPD calcium pyrophosphate dihydrate HA hydroxyapatite
HEMS helicopter emergency medical service PCR polymerase chain reaction
HGPRT hypoxanthine-guanine PD proton density
phosphoribosyltransferase PE pulmonary embolism
HHR humeral head replacement PEA pulseless electrical activity
HIV human immunodeficiency virus PEEP positive end-expiratory pressure
HLA human leucocyte antigen PET positron emission tomography
HMSN hereditary motor and sensory PFFD proximal focal femoral deficiency
neuropathy PIP proximal interphalangeal (joint)
HRT hormone replacement therapy PL posterolateral
ICP intracerebral pressure PM posteromedial
ICU intensive care unit PMMA polymethylmethacrylate
IL interleukin PNS peripheral nervous system
INR international normalized ratio PPE personal protective equipment
IP interphalangeal PPS post-polio syndrome
IRMER Ionising Radiation Medical Exposure PTH parathyroid hormone
Regulations PTS post-thrombotic syndrome
ITAP intra-osseous transcutaneous PVNS pigmented villonodular synovitis
amputation prosthesis QCT quantitative computed tomography
IVF in vitro fertilization QUS quantitative ultrasonometry
JIA juvenile idiopathic arthritis RA radiographic absorptiometry and
LCL lateral collateral ligament rheumatoid arthritis
LMA laryngeal mask airway RANKL receptor activator of nuclear factor-
LMN lower motor neuron ligand
LMWH low molecular weight heparin RF rheumatoid factor
MCL medial collateral ligament RR reversal reaction
MCP metacarpo-phalangeal (joint) RSD reflex sympathetic dystrophy
M-CSF macrophage colony-stimulating factor RSI rapid sequence induction
MED multiple epiphyseal dysplasia SACE serum angiotensin converting enzyme
MHC major histocompatibility complex SAMU Services de l’Aide Medical Urgente
MIC minimal inhibitory concentration SAPHO for synovitis, acne, pustulosis,
MIPO minimally invasive percutaneous hyperostosis and osteitis
osteosynthesis SCFE slipped capital femoral epiphysis
MIS minimally invasive surgery SCIWORA spinal cord injury without obvious
MODS multiple organ failure or dysfunction radiographic abnormality
syndrome SDD digestive tract
MPM mortality prediction model SE spin echo
MPS mucopolysaccharidoses SED spondyloepiphyseal dysplasia
MRI magnetic resonance imaging SEMLS single event multi-level surgery
MRSA methicillin-resistant Staphylococcus SIRS systemic inflammatory response
aureus SLAP superior labrum, anterior and posterior
MTP metatarsophalangeal (joint) (tear)
NCV nerve conduction velocity SLE systemic lupus erythematosus
NP nasopharyngeal SMR standardized mortality ratio
NSAIDs non-steroidal anti-inflammatory drugs SMUR Services Mobile d’Urgence et de
OA osteoarthritis Reamination
OI osteogenesis imperfecta SNAP sensory nerve action potential
OP oropharyngeal SNPs single nucleotide polymorphisms
ABBREVIATIONS
OPG osteoprotegerin SONK ‘spontaneous’ osteonecrosis of the

OPLL ossification of the posterior knee
longitudinal ligament SOPs standard operating procedure
PA posteroanterior SPECT single photon emission computed
PACS Picture Archiving and Communication tomography
System SSEP somatosensory evoked responses
PAFC pulmonary artery flotation STIR short-tau inversion recovery
catherization STT scaphoid-trapezium-trapezoid arthritis
PAOP pulmonary artery occlusion pressure SCIWORA spinal cord injury without radiographic
xvi PCL posterior cruciate ligament abnormality
TAR prompts one to remember US ultrasound
thrombocytopaenia with absent radius VACTERLS refers to the systems involved and the
syndrome defects identified: vertebral, anal,
TB tuberculosis cardiac, tracheal, esophageal, renal,
99m 99m
Tc-MDP Tc-methyl diphosphonate limb and single umbilical artery.
TE time to echo VCT voluntary counselling and testing
TFCC triangular fibrocartilage complex VISI volar intercalated segment
TIP terminal interphalangeal (joint) instability
TNF tumour necrosis factor VP ventriculo-peritoneal
TR repetition time VS vertical shear
TSR total shoulder replacement VTE venous thromboembolism
UHMWPE ultra-high molecular weight VQC ventilation-perfusion
polyethylene WBC white blood cell
UMN upper motor neuron XLPE highly cross-linked polyethylene
ABBREVIATIONS
xvii
Section 1
General
Orthopaedics
1 Orthopaedic diagnosis 3
2 Infection 29
3 Inflammatory rheumatic disorders 59
4 Crystal deposition disorders 77
5 Osteoarthritis 85
6 Osteonecrosis and related disorders 103
7 Metabolic and endocrine disorders 117
8 Genetic disorders, skeletal dysplasias and
malformations 151
9 Tumours 187
10 Neuromuscular disorders 225
11 Peripheral nerve injuries 269
12 Orthopaedic operations 303
Orthopaedic
diagnosis 1
Louis Solomon, Charles Wakeley
Orthopaedics is concerned with bones, joints, mus- Each symptom is pursued for more detail: we need
cles, tendons and nerves – the skeletal system and all to know when it began, whether suddenly or gradu-
that makes it move. Conditions that affect these struc- ally, spontaneously or after some specific event; how it
tures fall into seven easily remembered pairs: has changed or progressed; what makes it worse; what
makes it better.
1. Congenital and developmental abnormalities.
While listening, we consider whether the story fits
2. Infection and inflammation.
some pattern that we recognize, for we are already
3. Arthritis and rheumatic disorders.
thinking of a diagnosis. Every piece of information
4. Metabolic and endocrine disorders.
should be thought of as part of a larger picture which
5. Tumours and lesions that mimic them.
gradually unfolds in our understanding. The surgeon-
6. Neurological disorders and muscle weakness.
philosopher Wilfred Trotter (1870–1939) put it well:
7. Injury and mechanical derangement.
‘Disease reveals itself in casual parentheses’.
Diagnosis in orthopaedics, as in all of medicine, is
the identification of disease. It begins from the very
first encounter with the patient and is gradually mod- SYMPTOMS
ified and fine-tuned until we have a picture, not only
Pain
of a pathological process but also of the functional
loss and the disability that goes with it. Understand- Pain is the most common symptom in orthopaedics.
ing evolves from the systematic gathering of informa- It is usually described in metaphors that range from
tion from the history, the physical examination, tissue inexpressively bland to unbelievably bizarre – descrip-
and organ imaging and special investigations. System- tions that tell us more about the patient’s state of
atic, but never mechanical; behind the enquiring mind mind than about the physical disorder. Yet there are
there should also be what D. H. Lawrence has called clearly differences between the throbbing pain of an
‘the intelligent heart’. It must never be forgotten that abscess and the aching pain of chronic arthritis,
the patient has a unique personality, a job and hob- between the ‘burning pain’ of neuralgia and the ‘stab-
bies, a family and a home; all have a bearing upon, and bing pain’ of a ruptured tendon.
are in turn affected by, the disorder and its treatment. Severity is even more subjective. High and low pain
thresholds undoubtedly exist, but to the patient pain
is as bad as it feels, and any system of ‘pain grading’
must take this into account. The main value of esti-
HISTORY mating severity is in assessing the progress of the dis-
order or the response to treatment. The commonest
method is to invite the patient to mark the severity on
‘Taking a history’ is a misnomer. The patient tells a
an analogue scale of 1–10, with 1 being mild and eas-
story; it is we the listeners who construct a history.
ily ignored and 10 being totally unbearable. The
The story may be maddeningly disorganized; the his-
problem about this type of grading is that patients
tory has to be systematic. Carefully and patiently com-
who have never experienced very severe pain simply
piled, it can be every bit as informative as examination
do not know what 8 or 9 or 10 would feel like. The
or laboratory tests.
following is suggested as a simpler system:
As we record it, certain key words and phrases will
inevitably stand out: injury, pain, stiffness, swelling, • Grade I (mild) Pain that can easily be ignored.
deformity, instability, weakness, altered sensibility and • Grade II (moderate) Pain that cannot be ignored,
loss of function or inability to do certain things that interferes with function and needs attention or
were easily accomplished before. treatment from time to time.
1 • Grade III (severe) Pain that is present most of the
time, demanding constant attention or treatment.
• Grade IV (excruciating) Totally incapacitating
pain. (3)
(1)
Identifying the site of pain may be equally vague.
Yet its precise location is important, and in
GENERAL ORTHOPAEDICS
orthopaedics it is useful to ask the patient to point to

(4)
– rather than to say – where it hurts. Even then, do
not assume that the site of pain is necessarily the site
(2) (4)
of pathology; ‘referred’ pain and ‘autonomic’ pain can
be very deceptive.
Referred pain Pain arising in or near the skin is usually
localized accurately. Pain arising in deep structures is
(a) (b)
more diffuse and is sometimes of unexpected distri-
bution; thus, hip disease may manifest with pain in the 1.1 Referred pain Common sites of referred pain:
knee (so might an obturator hernia). This is not (1) from the shoulder; (2) from the hip; (3) from the neck;
(4) from the lumbar spine.
because sensory nerves connect the two sites; it is due
to inability of the cerebral cortex to differentiate
clearly between sensory messages from separate but body or a torn meniscus becoming trapped between
embryologically related sites. A common example is the articular surfaces of the knee. Unfortunately,
‘sciatica’ – pain at various points in the buttock, thigh patients tend to use the term for any painful limitation
and leg, supposedly following the course of the sciatic of movement; much more reliable is a history of
nerve. Such pain is not necessarily due to pressure on ‘unlocking’, when the offending body slips out of the
the sciatic nerve or the lumbar nerve roots; it may be way.
‘referred’ from any one of a number of structures in
the lumbar spine, the pelvis and the posterior capsule
of the hip joint.
Swelling
Swelling may be in the soft tissues, the joint or the
Autonomic pain We are so accustomed to matching
bone; to the patient they are all the same. It is impor-
pain with some discrete anatomical structure and its
tant to establish whether it followed an injury,
known sensory nerve supply that we are apt to dismiss
whether it appeared rapidly (think of a haematoma or
any pain that does not fit the usual pattern as ‘atypical’
a haemarthrosis) or slowly (due to inflammation, a
or ‘inappropriate’ (i.e. psychologically determined).
joint effusion, infection or a tumour), whether it is
But pain can also affect the autonomic nerves that
painful (suggestive of acute inflammation, infection or
accompany the peripheral blood vessels and this is
a tumour), whether it is constant or comes and goes,
much more vague, more widespread and often associ-
and whether it is increasing in size.
ated with vasomotor and trophic changes. It is poorly
understood, often doubted, but nonetheless real.
Deformity
Stiffness The common deformities are described by patients in
terms such as round shoulders, spinal curvature,
Stiffness may be generalized (typically in systemic dis-
knock knees, bow legs, pigeon toes and flat feet.
orders such as rheumatoid arthritis and ankylosing
Deformity of a single bone or joint is less easily
spondylitis) or localized to a particular joint. Patients
described and the patient may simply declare that the
often have difficulty in distinguishing localized stiff-
limb is ‘crooked’.
ness from painful movement; limitation of movement
Some ‘deformities’ are merely variations of the nor-
should never be assumed until verified by examina-
mal (e.g. short stature or wide hips); others disappear
tion.
spontaneously with growth (e.g. flat feet or bandy legs
Ask when it occurs: regular early morning stiffness
in an infant). However, if the deformity is progressive,
of many joints is one of the cardinal symptoms of
or if it affects only one side of the body while the
rheumatoid arthritis, whereas transient stiffness of one
opposite joint or limb is normal, it may be serious.
or two joints after periods of inactivity is typical of
osteoarthritis.
Weakness
Locking ‘Locking’ is the term applied to the sudden
inability to complete a particular movement. It sug- Generalized weakness is a feature of all chronic illness,
4 gests a mechanical block – for example, due to a loose and any prolonged joint dysfunction will inevitably
‘I can’t put my socks on’ rather than ‘My hip is stiff’. 1
Moreover, what to one patient is merely inconvenient
may, to another, be incapacitating. Thus a lawyer or a
teacher may readily tolerate a stiff knee provided it is
painless, but to a plumber or a parson the same disor-
der might spell economic or spiritual disaster. One
Orthopaedic diagnosis
question should elicit the important information:
‘What can’t you do now that you used to be able to
do?’
PAST HISTORY
Patients often forget to mention previous illnesses or
1.2 Deformity This young girl complained of a prominent
accidents, or they may simply not appreciate their rel-
right hip; the real deformity was scoliosis. evance to the present complaint. They should be
asked specifically about childhood disorders, periods
of incapacity and old injuries. A ‘twisted ankle’ many
lead to weakness of the associated muscles. However, years ago may be the clue to the onset of osteoarthri-
pure muscular weakness – especially if it is confined to tis in what is otherwise an unusual site for this condi-
one limb or to a single muscle group – is more specific tion. Gastrointestinal disease, which in the patient’s
and suggests some neurological or muscle disorder. mind has nothing to do with bones, may be important
Patients sometimes say that the limb is ‘dead’ when it in the later development of ankylosing spondylitis or
is actually weak, and this can be a source of confusion. osteoporosis. Similarly, certain rheumatic disorders
Questions should be framed to discover precisely may be suggested by a history of conjunctivitis, iritis,
which movements are affected, for this may give psoriasis or urogenital disease. Metastatic bone disease
important clues, if not to the exact diagnosis at least may erupt many years after a mastectomy for breast
to the site of the lesion. cancer. Patients should also be asked about previous
medication: many drugs, and especially cortico-
steroids, have long-term effects on bone. Alcohol and
Instability drug abuse are important, and we must not be afraid
The patient may complain that the joint ‘gives way’ or to ask about them.
‘jumps out of place’. If this happens repeatedly, it sug-
gests abnormal joint laxity, capsular or ligamentous
deficiency, or some type of internal derangement such FAMILY HISTORY
as a torn meniscus or a loose body in the joint. If there
is a history of injury, its precise nature is important. Patients often wonder (and worry) about inheriting a
disease or passing it on to their children. To the doc-
tor, information about musculoskeletal disorders in
Change in sensibility the patient’s family may help with both diagnosis and
Tingling or numbness signifies interference with counselling.
nerve function – pressure from a neighbouring struc- When dealing with a suspected case of bone or joint
ture (e.g. a prolapsed intervertebral disc), local infection, ask about communicable diseases, such as
ischaemia (e.g. nerve entrapment in a fibro-osseous tuberculosis or sexually transmitted disease, in other
tunnel) or a peripheral neuropathy. It is important to members of the family.
establish its exact distribution; from this we can tell
whether the fault lies in a peripheral nerve or in a
nerve root. We should also ask what makes it worse or SOCIAL BACKGROUND
better; a change in posture might be the trigger, thus
focussing attention on a particular site. No history is complete without enquiry about the
patient’s background. There are the obvious things
such as the level of care and nutrition in children;
Loss of function dietary constraints which may cause specific deficien-
Functional disability is more than the sum of individ- cies; and, in certain cases, questions about smoking
ual symptoms and its expression depends upon the habits, alcohol consumption and drug abuse, all of
needs of that particular patient. The patient may say ‘I which call for a special degree of tact and non-judge-
can’t stand for long’ rather than ‘I have backache’; or mental enquiry. 5
1 Find out details about the patient’s work practices, neither an inspector nor a manipulator, and I am defi-
travel and recreation: could the disorder be due to a nitely not a palpator’.) Thus the traditional clinical
particular repetitive activity in the home, at work or routine, inspection, palpation, manipulation, was
on the sportsfield? Is the patient subject to any replaced by look, feel, move. With time his teaching has
unusual occupational strain? Has he or she travelled to been extended and we now add test, to include the
another country where tuberculosis is common? special manoeuvres we employ in assessing neurolog-
Finally, it is important to assess the patient’s home ical integrity and complex functional attributes.
circumstances and the level of support by family and
friends. This will help to answer the question: ‘What
Look
has the patient lost and what is he or she hoping to
regain?’ Abnormalities are not always obvious at first sight. A
systematic, step by step process helps to avoid mis-
takes.
EXAMINATION Shape and posture The first things to catch one’s
attention are the shape and posture of the limb or the
In A Case of Identity Sherlock Holmes has the follow- body or the entire person who is being examined. Is
ing conversation with Dr Watson. the patient unusually thin or obese? Does the overall
posture look normal? Is the spine straight or unusu-
Watson: You appeared to read a good deal upon
ally curved? Are the shoulders level? Are the limbs
[your client] which was quite invisible to me.
normally positioned? It is important to look for defor-
Holmes: Not invisible but unnoticed, Watson. mity in three planes, and always compare the affected
part with the normal side. In many joint disorders and
Some disorders can be diagnosed at a glance: who
in most nerve lesions the limb assumes a characteristic
would mistake the facial appearance of acromegaly or
posture. In spinal disorders the entire torso may be
the hand deformities of rheumatoid arthritis for any-
deformed. Now look more closely for swelling or
thing else? Nevertheless, even in these cases systematic
wasting – one often enhances the appearance of the
examination is rewarding: it provides information
other! Or is there a definite lump?
about the patient’s particular disability, as distinct
from the clinicopathological diagnosis; it keeps rein- Skin Careful attention is paid to the colour, quality
forcing good habits; and, never to be forgotten, it lets and markings of the skin. Look for bruising, wounds
the patient know that he or she has been thoroughly and ulceration. Scars are an informative record of the
attended to. past – surgical archaeology, so to speak. Colour
The examination actually begins from the moment reflects vascular status or pigmentation – for example
we set eyes on the patient. We observe his or her gen- the pallor of ischaemia, the blueness of cyanosis, the
eral appearance, posture and gait. Can you spot any redness of inflammation, or the dusky purple of an old
distinctive feature: Knock-knees? Spinal curvature? A bruise. Abnormal creases, unless due to fibrosis, sug-
short limb? A paralysed arm? Does he or she appear to gest underlying deformity which is not always obvi-
be in pain? Do their movements look natural? Do they ous; tight, shiny skin with no creases is typical of
walk with a limp, or use a stick? A tell-tale gait may oedema or trophic change.
suggest a painful hip, an unstable knee or a foot-drop.
The clues are endless and the game is played by every-
one (qualified or lay) at each new encounter through-
out life. In the clinical setting the assessment needs to
be more focussed.
When we proceed to the structured examination,
the patient must be suitably undressed; no mere
rolling up of a trouser leg is sufficient. If one limb is
affected, both must be exposed so that they can be
compared.
We examine the good limb (for comparison), then
the bad. There is a great temptation to rush in with
both hands – a temptation that must be resisted. Only
by proceeding in a purposeful, orderly way can we
avoid missing important signs. 1.3 Look Scars often give clues to the previous history.
The faded scar on this patient’s thigh is an old operation
Alan Apley, who developed and taught the system wound – internal fixation of a femoral fracture. The other
used here, shied away from using long words where scars are due to postoperative infection; one of the sinuses
6 short ones would do as well. (He also used to say ‘I’m is still draining.
General survey Attention is initially focussed on the degree of mobility and whether it is painful or 1
symptomatic or most obviously abnormal area, but we not. Active movement is also used to assess muscle
must also look further afield. The patient complains of power.
the joint that is hurting now, but we may see at a
glance that several other joints are affected as well. Passive movement Here it is the examiner who moves
the joint in each anatomical plane. Note whether
there is any difference between the range of active and
Feel passive movement.
Feeling is exploring, not groping aimlessly. Know Range of movement is recorded in degrees, starting
your anatomy and you will know where to feel for the from zero which, by convention, is the neutral or
landmarks; find the landmarks and you can construct anatomical position of the joint and finishing where
a virtual anatomical picture in your mind’s eye. movement stops, due either to pain or anatomical lim-
itation. Describing the range of movement is often
The skin Is it warm or cold; moist or dry; and is sen- made to seem difficult. Words such as ‘full’, ‘good’,
sation normal? ‘limited’ and ‘poor’ are misleading. Always cite the
The soft tissues Can you feel a lump; if so, what are its range or span, from start to finish, in degrees. For
characteristics? Are the pulses normal? example, ‘knee flexion 0–140°’ means that the range
of flexion is from zero (the knee absolutely straight)
The bones and joints Are the outlines normal? Is the through an arc of 140 degrees (the leg making an
synovium thickened? Is there excessive joint fluid? acute angle with the thigh). Similarly, ‘knee flexion
Tenderness Once you have a clear idea of the struc- 20–90°’ means that flexion begins at 20 degrees (i.e.
tural features in the affected area, feel gently for ten- the joint cannot extend fully) and continues only to
derness. Keep your eyes on the patient’s face; a 90 degrees.
grimace will tell you as much as a grunt. Try to local- For accuracy you can measure the range of move-
ize any tenderness to a particular structure; if you ment with a goniometer, but with practice you will
know precisely where the trouble is, you are halfway to learn to estimate the angles by eye. Normal ranges of
knowing what it is. movement are shown in chapters dealing with indi-
vidual joints. What is important is always to compare
the symptomatic with the asymptomatic or normal
Move side.
‘Movement’ covers several different activities: active While testing movement, feel for crepitus. Joint
movement, passive mobility, abnormal or unstable crepitus is usually coarse and fairly diffuse; tenosyn-
movement, and provocative movement. ovial crepitus is fine and precisely localized to the
affected tendon sheath.
Active movement Ask the patient to move without
your assistance. This will give you an idea of the Unstable movement This is movement which is inher-
ently unphysiological. You may be able to shift or
angulate a joint out of its normal plane of movement,
thus demonstrating that the joint is unstable. Such
abnormal movement may be obvious (e.g. a wobbly
knee); often, though, you have to use special manoeu-
vres to pick up minor degrees of instability.
Provocative movement One of the most telling clues

to diagnosis is reproducing the patient’s symptoms
by applying a specific, provocative movement. Shoul-
der pain due to impingement of the subacromial
structures may be ‘provoked’ by moving the joint in
a way that is calculated to produce such impinge-
ment; the patient recognizes the similarity between
this pain and his or her daily symptoms. Likewise, a
patient who has had a previous dislocation or sublux-
ation can be vividly reminded of that event by stress-
(a) (b) ing the joint in such a way that it again threatens to
dislocate; indeed, merely starting the movement may
1.4 Feeling for tenderness (a) The wrong way – there is
no need to look at your fingers, you should know where be so distressing that the patient goes rigid with anx-
they are. (b) It is much more informative to look at the iety at the anticipated result – this is aptly called the
patient’s face! apprehension test. 7
1
(a) (b) (c)
(d) (e) (f)
1.5 Testing for movement (a) Flexion, (b) extension, (c) rotation, (d) abduction, (e) adduction. The range of movement
can be estimated by eye or measured accurately using a goniometer (f).
(a) (b) (c) (d)
1.6 Move (a) Active movement – the patient moves the joint. The right shoulder is normal; the left has restricted active
movement. (b) Passive movement – the examiner moves the joint. (c) Unstable movement – the joint can be moved across
the normal planes of action, in this case demonstrating valgus instability of the right knee. (d) Provocative movement – the
examiner moves (or manipulates) the joint so as to provoke the symptoms of impending pain or dislocation. Here he is
reproducing the position in which an unstable shoulder is likely to dislocate.
Test Caveat
The apprehension test referred to in the previous para- We recognize that the sequence set out here may
graph is one of several clinical tests that are used to elicit sometimes have to be modified. We may need to
suspected abnormalities: some examples are Thomas’ test ‘move’ before we ‘look’: an early scoliotic deformity
for flexion deformity of the hip, Trendelenburg’s test for of the spine often becomes apparent only when the
instability of the hip, McMurray’s test for a torn meniscus patient bends forwards. The sequence may also have
of the knee, Lachman’s test for cruciate ligament insta- to be altered because a patient is in severe pain or dis-
bility and various tests for intra-articular fluid. These and abled: you would not try to move a limb at all in
others are described in the relevant chapters in Section 2. someone with a suspected fracture when an x-ray can
Tests for muscle tone, motor power, reflexes and provide the answer. When examining a child you may
various modes of sensibility are part and parcel of neu- have to take your chances with look or feel or move
8 rological examination, which is dealt with on page 10. whenever you can!
TERMINOLOGY midline of the body, and lateral away from the 1
median plane. These terms are usually applied to a
Colloquial terms such as front, back, upper, lower, limb, the clavicle or one half of the pelvis. Thus the
inner aspect, outer aspect, bow legs, knock knees have inner aspect of the thigh lies on the medial side of the
the advantage of familiarity but are not applicable to limb and the outer part of the thigh lies on the lateral
every situation. Universally acceptable anatomical def- side. We could also say that the little finger lies on the
initions are therefore necessary in describing physical medial or ulnar side of the hand and the thumb on
attributes. the lateral or radial side of the hand.
Bodily surfaces, planes and positions are always Proximal and distal are used mainly for parts of
described in relation to the anatomical position – as the limbs, meaning respectively the upper end and the
if the person were standing erect, facing the viewer, lower end as they appear in the anatomical position.
legs together with the knees pointing directly for- Thus the knee joint is formed by the distal end of the
wards, and arms held by the sides with the palms fac- femur and the proximal end of the tibia.
ing forwards. Axial alignment describes the longitudinal arrange-
The principal planes of the body are named sagit- ment of adjacent limb segments or parts of a single bone.
tal, coronal and transverse; they define the direction The knees and elbows, for example, are normally angu-
across which the body (or body part) is viewed in any lated slightly outwards (valgus) while the opposite –
description. Sagittal planes, parallel to each other, ‘bow legs’ – is more correctly described as varus (see on
pass vertically through the body from front to back; page 13, under Deformity). Angulation in the middle of
the midsagittal or median plane divides the body a long bone would always be regarded as abnormal.
into right and left halves. Coronal planes are also ori- Rotational alignment refers to the tortile arrange-
entated vertically, corresponding to a frontal view, at ment of segments of a long bone (or an entire limb)
right angles to the saggital planes; transverse planes around a single longitudinal axis. For example, in the
pass horizontally across the body. anatomical position the patellae face forwards while the
Anterior signifies the frontal aspect and posterior feet are turned slightly outwards; a marked difference
the rear aspect of the body or a body part. The terms in rotational alignment of the two legs is abnormal.
ventral and dorsal are also used for the front and the Flexion and extension are joint movements in the
back respectively. Note, though, that the use of these sagittal plane, most easily imagined in hinge joints like
terms is somewhat confusing when it comes to the the knee, elbow and the joints of the fingers and toes.
foot: here the upper surface is called the dorsum and In elbows, knees, wrists and fingers flexion means
the sole is called the plantar surface. bending the joint and extension means straightening
Medial means facing towards the median plane or it. In shoulders and hips flexion is movement in an
anterior direction and extension is movement posteri-
orwards. In the ankle flexion is also called plan-
tarflexion (pointing the foot downwards) and
extension is called dorsiflexion (drawing the foot
upwards). Thumb movements are the most compli-
Sagittal plane Coronal plane
cated and are described in Chapter 16.
Abduction and adduction are movements in the
coronal plane, away from or towards the median
Transverse plane
plane. Not quite for the fingers and toes, though: here
abduction and adduction mean away from and
towards the longitudinal midline of the hand or foot!
Lateral rotation and medial rotation are twisting
movements, outwards and inwards, around a longitu-
dinal axis.
Pronation and supination are also rotatory move-
ments, but the terms are applied only to movements
of the forearm and the foot.
Circumduction is a composite movement made up
of a rhythmic sequence of all the other movements. It
is possible only for ball-and-socket joints such as the
hip and shoulder.
Specialized movements such as opposition of the
thumb, lateral flexion and rotation of the spine, and
1.7 The principal planes of the body, as viewed in the inversion or eversion of the foot, will be described in
anatomical position: sagittal, coronal and transverse. the relevant chapters. 9
1 NEUROLOGICAL EXAMINATION important; if localized and asymmetrical, it may suggest
dysfunction of a specific motor nerve.
If the symptoms include weakness or incoordination
or a change in sensibility, or if they point to any dis- Muscle tone
order of the neck or back, a complete neurological Tone in individual muscle groups is tested by moving
examination of the related part is mandatory. the nearby joint to stretch the muscle. Increased tone
Once again we follow a systematic routine, first (spasticity) is characteristic of upper motor neuron
looking at the general appearance, then assessing disorders such as cerebral palsy and stroke. It must
motor function (muscle tone, power and reflexes) and not be confused with rigidity (the ‘lead-pipe’ or ‘cog-
finally testing for sensory function (both skin sensibil- wheel’ effect) which is seen in Parkinson’s disease.
ity and deep sensibility). Decreased tone (flaccidity) is found in lower motor
neuron lesions; for example, poliomyelitis. Muscle
Appearance power is diminished in all three states; it is important
to recognize that a ‘spastic’ muscle may still be weak.
Some neurological disorders result in postures that
are so characteristic as to be diagnostic at a glance: the
claw hand of an ulnar nerve lesion; drop wrist follow- Power
ing radial nerve palsy; or the ‘waiter’s tip’ deformity of Motor function is tested by having the patient per-
the arm in brachial plexus injury. Usually, however, it form movements that are normally activated by spe-
is when the patient moves that we can best appreciate cific nerves. We may learn even more about composite
the type and extent of motor disorder: the dangling movements by asking the patient to perform specific
arm following a brachial plexus injury; the flail lower tasks, such as holding a pen, gripping a rod, doing up
limb of poliomyelitis; the symmetrical paralysis of a button or picking up a pin.
spinal cord lesions; the characteristic drop-foot gait Testing for power is not as easy as it sounds; the dif-
following sciatic or peroneal nerve damage; and the ficulty is making ourselves understood. The simplest
jerky, ‘spastic’ movements of cerebral palsy. way is to place the limb in the ‘test’ position, then ask
Concentrating on the affected part, we look for troph- the patient to hold it there as firmly as possible and re-
ic changes that signify loss of sensibility: the smooth, hair- sist any attempt to change that position. The normal
less skin that seems to be stretched too tight; atrophy limb is examined first, then the affected limb, and the
of the fingertips and the nails; scars that tell of accidental two are compared. Finer muscle actions, such as those
burns; and ulcers that refuse to heal. Muscle wasting is of the thumb and fingers, may be reproduced by first
demonstrating the movement yourself, then testing it
in the unaffected limb, and then in the affected one.
Muscle power is usually graded on the Medical
Research Council scale:
Grade 0 No movement.
Grade 1 Only a flicker of movement.
Grade 2 Movement with gravity eliminated.
Grade 3 Movement against gravity.
Grade 4 Movement against resistance.
Grade 5 Normal power.
It is important to recognize that muscle weakness
may be due to muscle disease rather than nerve dis-
ease. In muscle disorders the weakness is usually more
widespread and symmetrical, and sensation is normal.
Tendon reflexes
A deep tendon reflex is elicited by rapidly stretching the
tendon near its insertion. A sharp tap with the tendon
hammer does this well; but all too often this is per-
formed with a flourish and with such force that the finer
1.8 Posture Posture is often diagnostic. This patient’s
‘drop wrist’ – typical of a radial nerve palsy – is due to gradations of response are missed. It is better to employ
carcinomatous infiltration of the supraclavicular lymph a series of taps, starting with the most forceful and re-
10 nodes on the right. ducing the force with each successive tap until there is
no response. Comparing the two sides in this way, we from the normal central inhibition and there is an ex- 1
can pick up fine differences showing that a reflex is aggerated response to tendon stimulation. This may
‘diminished’ rather than ‘absent’. In the upper limb we manifest as ankle clonus: a sharp upward jerk on the
test biceps, triceps and brachioradialis; and in the lower foot (dorsiflexion) causes a repetitive, ‘clonic’ move-
limb the patellar and Achilles tendons. ment of the foot; similarly, a sharp downward push on
The tendon reflexes are monosynaptic segmental re- the patella may elicit patellar clonus.
flexes; that is, the reflex pathway takes a ‘short cut’
through the spinal cord at the segmental level. De-
Superficial reflexes
pression or absence of the reflex signifies interruption
of the pathway at the posterior nerve root, the anterior The superficial reflexes are elicited by stroking the skin
horn cell, the motor nerve root or the peripheral nerve. at various sites to produce a specific muscle contrac-
It is a reliable pointer to the segmental level of dys- tion; the best known are the abdominal (T7–T12),
function: thus, a depressed biceps jerk suggests pressure cremasteric (L1, 2) and anal (S4, 5) reflexes. These
on the fifth or sixth cervical (C5 or 6) nerve roots while are corticospinal (upper motor neuron) reflexes.
a depressed ankle jerk signifies a similar abnormality at Absence of the reflex indicates an upper motor neuron
the first sacral level (S1). An unusually brisk reflex, on lesion (usually in the spinal cord) above that level.
the other hand, is characteristic of an upper motor
neuron disorder (e.g. cerebral palsy, a stroke or injury The plantar reflex
to the spinal cord); the lower motor neuron is released Forceful stroking of the sole normally produces flex-
ion of the toes (or no response at all). An extensor
Table 1.1 Nerve root supply and actions of main response (the big toe extends while the others remain
muscle groups in flexion) is characteristic of upper motor neuron dis-
orders. This is the Babinski sign – a type of withdrawal
Sternomastoids Spinal accessory C2, 3, 4
reflex which is present in young infants and normally
Trapezius Spinal accessory C3, 4 disappears after the age of 18 months.
Diaphragm C3, 4, 5
Deltoid C5, 6
Supra- and infraspinatus C5, 6
Serratus anterior C5, 6, 7
Pectoralis major C5, 6, 7, 8
Elbow flexion C5, 6
extension C7
Supination C5, 6
Pronation C6
Wrist extension C6, (7)
flexion C7, (8)
Finger extension C7
flexion C7, 8, T1
ab- and adduction C8, T1
Hip flexion L1, 2, 3
extension L5, S1
adduction L2, 3, 4
abduction L4, 5, S1
Knee extension L(2), 3, 4
flexion L5, S1
Ankle dorsiflexion L4, 5
plantarflexion S1, 2
inversion L4, 5
eversion L5, S1
Toe extension L5
flexion S1
abduction S1, 2 1.9 Examination Dermatomes supplied by the spinal
nerve roots. 11
1 Sensibility sign, a full examination of the central nervous system
Sensibility to touch and to pinprick may be increased will be necessary.
(hyperaesthesia) or unpleasant (dysaesthesia) in
certain irritative nerve lesions. More often, though, it
is diminished (hypoaesthesia) or absent (anaesthesia), EXAMINING INFANTS AND
signifying pressure on or interruption of a peripheral
CHILDREN
nerve, a nerve root or the sensory pathways in the
spinal cord. The area of sensory change can be
mapped out on the skin and compared with the Paediatric practice requires special skills. You may
known segmental or dermatomal pattern of innerva- have no first-hand account of the symptoms; a baby
tion. If the abnormality is well defined it is an easy screaming with pain will tell you very little, and over-
matter to establish the level of the lesion, even if the anxious parents will probably tell you too much.
precise cause remains unknown. When examining the child, be flexible. If he or she is
Brisk percussion along the course of an injured moving a particular joint, take your opportunity to
nerve may elicit a tingling sensation in the distal dis- examine movement then and there. You will learn
tribution of the nerve (Tinel’s sign). The point of much more by adopting methods of play than by
hypersensitivity marks the site of abnormal nerve applying a rigid system of examination. And leave any
sprouting: if it progresses distally at successive visits test for tenderness until last!
this signifies regeneration; if it remains unchanged this
suggests a local neuroma. INFANTS AND SMALL CHILDREN
Tests for temperature recognition and two-point The baby should be undressed, in a warm room, and
discrimination (the ability to recognize two touch- placed on the examining couch. Look carefully for
points a few millimetres apart) are also used in the birthmarks, deformities and abnormal movements –
assessment of peripheral nerve injuries. or absence of movement. If there is no urgency or dis-
Deep sensibility can be examined in several ways. In tress, take time to examine the head and neck, includ-
the vibration test a sounded tuning fork is placed over ing facial features which may be characteristic of
a peripheral bony point (e.g. the medial malleolus or specific dysplastic syndromes. The back and limbs are
the head of the ulna); the patient is asked if he or she then examined for abnormalities of position or shape.
can feel the vibrations and to say when they disappear. Examining for joint movement can be difficult. Active
By comparing the two sides, differences can be noted. movements can often be stimulated by gently stroking
Position sense is tested by asking the patient to find the limb. When testing for passive mobility, be careful
certain points on the body with the eyes closed – for to avoid frightening or hurting the child.
example, touching the tip of the nose with the fore- In the neonate, and throughout the first two years
finger. The sense of joint posture is tested by grasping of life, examination of the hips is mandatory, even if
the big toe and placing it in different positions of flex- the child appears to be normal. This is to avoid miss-
ion and extension. The patient (whose eyes are ing the subtle signs of developmental dysplasia of the
closed) is asked to say whether it is ‘up’ or ‘down’. hips (DDH) at the early stage when treatment is most
Stereognosis, the ability to recognize shape and tex- effective.
ture by feel alone, is tested by giving the patient It is also important to assess the child’s general
(again with eyes closed) a variety of familiar objects to development by testing for the normal milestones
hold and asking him or her to name each object. which are expected to appear during the first two
The pathways for deep sensibility run in the post- years of life.
erior columns of the spinal cord. Disturbances are,
therefore, found in peripheral neuropathies and in
spinal cord lesions such as posterior column injuries or
tabes dorsalis. The sense of balance is also carried in NORMAL DEVELOPMENTAL MILESTONES
the posterior columns. This can be tested by asking Newborn Grasp reflex present
the patient to stand upright with his or her eyes Morrow reflex present
closed; excessive body sway is abnormal (Romberg’s
sign). 3–6 months Holds head up unsupported
6–9 months Able to sit up
Cortical and cerebellar function 9–12 months Crawling and standing up
A staggering gait may imply an unstable knee – or a 9–18 months Walking

disorder of the spinal cord or cerebellum. If there is 18–24 months Running
12 no musculoskeletal abnormality to account for the
OLDER CHILDREN It is doubtful whether these individuals should be 1
Most children can be examined in the same way as considered ‘abnormal’. However, epidemiological
adults, though with different emphasis on particular studies have shown that they do have a greater than
physical features. Posture and gait are very important; usual tendency to recurrent dislocation (e.g. of the
subtle deviations from the norm may herald the shoulder or patella). Some experience recurrent
appearance of serious abnormalities such as scoliosis episodes of aching around the larger joints; however,
or neuromuscular disorders, while more obvious there is no convincing evidence that hypermobility by
‘deformities’ such as knock knees and bow legs may itself predisposes to osteoarthritis.
be no more than transient stages in normal develop- Generalized hypermobility is not usually associated
ment; similarly with mild degrees of ‘flat feet’ and with any obvious disease, but severe laxity is a feature
‘pigeon toes’. More complex variations in posture and of certain rare connective tissue disorders such as Mar-
gait patterns, when the child sits and walks with the fan’s syndrome, Ehlers–Danlos syndrome, Larsen’s
knees turned inwards (medially rotated) or outwards disease and osteogenesis imperfecta.
(laterally rotated) are usually due to anteversion or
retroversion of the femoral necks, sometimes associ-
Deformity
ated with compensatory rotational ‘deformities’ of the
femora and tibiae. Seldom need anything be done The boundary between variations of the normal and
about this; the condition usually improves as the child physical deformity is blurred. Indeed, in the develop-
approaches puberty and only if the gait is very awk- ment of species, what at one point of time might have
ward would one consider performing corrective been seen as a deformity could over the ages have
osteotomies of the femora. turned out to be so advantageous as to become essen-
tial for survival.
So too in humans. The word ‘deformity’ is derived
PHYSICAL VARIATIONS AND from the Latin for ‘misshapen’, but the range of ‘nor-
DEFORMITIES mal shape’ is so wide that variations should not auto-
matically be designated as deformities, and some
undoubted ‘deformities’ are not necessarily patholog-
JOINT LAXITY ical; for example, the generally accepted cut-off points
Children’s joints are much more mobile than those of for ‘abnormal’ shortness or tallness are arbitrary and
most adults, allowing them to adopt postures that people who in one population might be considered
would be impossible for their parents. An unusual abnormally short or abnormally tall could, in other
degree of joint mobility can also be attained by adults populations, be seen as quite ordinary. However, if
willing to submit to rigorous exercise and practice, as one leg is short and the other long, no-one would
witness the performances of professional dancers and quibble with the use of the word ‘deformity’!
athletes, but in most cases, when the exercises stop, Specific terms are used to describe the ‘position’
mobility gradually reverts to the normal range. and ‘shape’ of the bones and joints. Whether, in any
Persistent generalized joint hypermobility occurs in particular case, these amount to ‘deformity’ will be
about 5% of the population and is inherited as a sim- determined by additional factors such as the extent to
ple mendelian dominant. Those affected describe which they deviate from the norm, symptoms to
themselves as being ‘double-jointed’: they can hyper- which they give rise, the presence or absence of insta-
extend their metacarpophalangeal joints beyond a bility and the degree to which they interfere with
right angle, hyperextend their elbows and knees and function.
bend over with knees straight to place their hands flat Varus and valgus It seems pedantic to replace ‘bow
on the ground; some can even ‘do the splits’ or place legs’ and ‘knock knees’ with ‘genu varum’ and ‘genu
their feet behind their neck! valgum’, but comparable colloquialisms are not avail-
able for deformities of the elbow, hip or big toe; and,
besides, the formality is justified by the need for clar-
ity and consistency. Varus means that the part distal to
the joint in question is displaced towards the median
plane, valgus away from it.
Kyphosis and lordosis Seen from the side, the normal
spine has a series of curves: convex posteriorly in the
thoracic region (kyphosis), and convex anteriorly in
1.10 Tests for joint hypermobility Hyperextension of the cervical and lumbar regions (lordosis). Excessive
knees and elbows; metacarpophalangeal joints extending curvature constitutes kyphotic or lordotic deformity
to 90 degrees; thumb able to touch forearm. (also sometimes referred to as hyperkyphosis and 13
1
(a) (b) (c)
1.11 Varus and valgus (a) Valgus knees in a patient with rheumatoid arthritis. The toe joints are also valgus. (b) Varus
knees due to osteoarthritis. (c) Another varus knee? No – the deformity here is in the left tibia due to Paget’s disease.
hyperlordosis). Colloquially speaking, excessive tho- flexor aspect of a joint, e.g. due to a burn or fol-
racic kyphosis is referred to as ‘round-shouldered’. lowing surgery.
2. Contracture of the subcutaneous fascia The classi-
Scoliosis Seen from behind, the spine is straight. Any
cal example is Dupuytren’s contracture in the palm
curvature in the coronal plane is called scoliosis. The
of the hand.
position and direction of the curve are specified by
3. Muscle contracture Fibrosis and contracture of
terms such as thoracic scoliosis, lumbar scoliosis, con-
muscles that cross a joint will cause a fixed defor-
vex to the right, concave to the left, etc.
mity of the joint. This may be due to deep infec-
Postural deformity A postural deformity is one which tion or fibrosis following ischaemic necrosis
the patient can, if properly instructed, correct volun- (Volkmann’s ischaemic contracture).
tarily: e.g. thoracic ‘kyphosis’ due to slumped shoul- 4. Muscle imbalance Unbalanced muscle weakness or
ders. Postural deformity may also be caused by spasticity will result in joint deformity which, if not
temporary muscle spasm. corrected, will eventually become fixed. This is
seen most typically in poliomyelitis and cerebral
Structural deformity A deformity which results from a
palsy. Tendon rupture, likewise, may cause defor-
permanent change in anatomical structure cannot be
mity.
voluntarily corrected. It is important to distinguish
5. Joint instability Any unstable joint will assume a
postural scoliosis from structural (fixed) scoliosis. The
‘deformed’ position when subjected to force.
former is non-progressive and benign; the latter is
6. Joint destruction Trauma, infection or arthritis
usually progressive and may require treatment.
may destroy the joint and lead to severe deformity.
‘Fixed deformity’ This term is ambiguous. It seems to
mean that a joint is deformed and unable to move.
Not so – it means that one particular movement can-
CAUSES OF BONE DEFORMITY
not be completed. Thus the knee may be able to flex
Bone deformities in small children are usually due to
fully but not extend fully – at the limit of its extension
genetic or developmental disorders of cartilage and
it is still ‘fixed’ in a certain amount of flexion. This
bone growth; some can be diagnosed in utero by spe-
would be called a ‘fixed flexion deformity’.
cial imaging techniques (e.g. achondroplasia); some
become apparent when the child starts to walk, or
CAUSES OF JOINT DEFORMITY
later still during one of the growth spurts (e.g. hered-
There are six basic causes of joint deformity:
itary multiple exostosis); and some only in early adult-
1. Contracture of the overlying skin This is seen hood (e.g. multiple epiphyseal dysplasia). There are a
14 typically when there is severe scarring across the myriad genetic disorders affecting the skeleton, yet
any one of these conditions is rare. The least unusual JOINT STIFFNESS 1
of them are described in Chapter 8.
Acquired deformities in children may be due to The term ‘stiffness’ covers a variety of limitations. We
fractures involving the physis (growth plate); ask consider three types of stiffness in particular: (1) all
about previous injuries. Other causes include rickets, movements absent; (2) all movements limited; (3)
endocrine disorders, malunited diaphyseal fractures one or two movements limited.
and tumours.
All movements absent Surprisingly, although move-
Acquired deformities of bone in adults are usually
ment is completely blocked, the patient may retain
the result of previous malunited fractures. However,
such good function that the restriction goes unno-
causes such as osteomalacia, bone tumours and
ticed until the joint is examined. Surgical fusion is
Paget’s disease should always be considered.
called ‘arthrodesis’; pathological fusion is called
‘ankylosis’. Acute suppurative arthritis typically ends
in bony ankylosis; tuberculous arthritis heals by
BONY LUMPS fibrosis and causes fibrous ankylosis – not strictly a
‘fusion’ because there may still be a small jog of move-
A bony lump may be due to faulty development,
ment.
injury, inflammation or a tumour. Although x-ray
examination is essential, the clinical features can be All movements limited After severe injury, movement
highly informative. may be limited as a result of oedema and bruising.
Later, adhesions and loss of muscle extensibility may
Size A large lump attached to bone, or a lump that is
perpetuate the stiffness.
getting bigger, is nearly always a tumour.
With active inflammation all movements are
Site A lump near a joint is most likely to be a tumour restricted and painful and the joint is said to be ‘irri-
(benign or malignant); a lump in the shaft may be table’. In acute arthritis spasm may prevent all but a
fracture callus, inflammatory new bone or a tumour. few degrees of movement.
In osteoarthritis the capsule fibroses and move-
Margin A benign tumour has a well-defined margin;
ments become increasingly restricted, but pain occurs
malignant tumours, inflammatory lumps and callus
only at the extremes of motion.
have a vague edge.
Some movements limited When one particular move-
Consistency A benign tumour feels bony hard; malig-
ment suddenly becomes blocked, the cause is usually
nant tumours often give the impression that they can
mechanical. Thus a torn and displaced meniscus may
be indented.
prevent extension of the knee but not flexion.
Tenderness Lumps due to active inflammation, recent Bone deformity may alter the arc of movement,
callus or a rapidly growing sarcoma are tender. such that it is limited in one direction (loss of abduc-
tion in coxa vara is an example) but movement in the
Multiplicity Multiple bony lumps are uncommon:
opposite direction is full or even increased.
they occur in hereditary multiple exostosis and in
These are all examples of ‘fixed deformity’.
Ollier’s disease.
DIAGNOSTIC IMAGING
The map is not the territory

Alfred Korzybski
PLAIN FILM RADIOGRAPHY

Plain film x-ray examination is over 100 years old.
Notwithstanding the extraordinary technical advances
of the last few decades, it remains the most useful
method of diagnostic imaging. Whereas other meth-
ods may define an inaccessible anatomical structure
more accurately, or may reveal some localized tissue
1.12 Bony lumps The lump above the left knee is hard,
well-defined and not increasing in size. The clinical change, the plain film provides information simulta-
diagnosis of cartilage-capped exostosis (osteochondroma) neously on the size, shape, tissue ‘density’ and bone
is confirmed by the x-rays. architecture – characteristics which, taken together, 15
1 will usually suggest a diagnosis, or at least a range of The clinical details are important; it is surprising how
possible diagnoses. much more you can see on the x-ray when you know
the background. Similarly, when requesting an x-ray
The radiographic image examination, give the radiologist enough information
from the patient’s history and the clinical findings to
X-rays are produced by firing electrons at high speed help in guiding his or her thoughts towards the diag-
onto a rotating anode. The resulting beam of x-rays is nostic possibilities and options. For example, when
attenuated by the patient’s soft tissues and bones, considering a malignant bone lesion, simply knowing
casting what are effectively ‘shadows’ which are dis- the patient’s age may provide an important clue:
played as images on an appropriately sensitized plate under the age of 10 it is most likely to be a Ewing’s
or stored as digital information which is then available sarcoma; between 10 and 20 years it is more likely to
to be transferred throughout the local information be an osteosarcoma; and over the age of 50 years it is
technology (IT) network. likely to be a metastatic deposit.
The more dense and impenetrable the tissue, the
greater the x-ray attenuation and therefore the more THE SOFT TISSUES
blank, or white, the image that is captured. Thus, a Generalized change Muscle planes are often visible
metal implant appears intensely white, bone less so and may reveal wasting or swelling. Bulging outlines
and soft tissues in varying shades of grey depending around a hip, for example, may suggest a joint effu-
on their ‘density’. Cartilage, which causes little atten- sion; and soft-tissue swelling around interphalangeal
uation, appears as a dark area between adjacent bone joints may be the first radiographic sign of rheumatoid
ends; this ‘gap’ is usually called the joint space, arthritis. Tumours tend to displace fascial planes,
though of course it is not a space at all, merely a radi- whereas infection tends to obliterate them.
olucent zone filled with cartilage. Other ‘radiolucent’
areas are produced by fluid-filled cysts in bone. Localized change Is there a mass, soft tissue calcifica-
One bone overlying another (e.g. the femoral head tion, ossification, gas (from penetrating wound or
inside the acetabular socket) produces superimposed gas-forming organism) or the presence of a radio-
images; any abnormality seen in the resulting com- opaque foreign body?
bined image could be in either bone, so it is important
to obtain several images from different projections in THE BONES
order to separate the anatomical outlines. Similarly, Shape The bones are well enough defined to allow
the bright image of a metallic foreign body superim- one to check their general anatomy and individual
posed upon that of, say, the femoral condyles could shape. For example, for the spine, look at the overall
mean that the foreign body is in front of, inside or
behind the bone. A second projection, at right angles Articular
cartilage
to the first, will give the answer.
Epiphysis
Picture Archiving and Communication System (PACS) This Physis
(growth plate)
is the system whereby all digitally coded images are
filed, stored and retrieved to enable the images to be Metaphysis
sent to work stations throughout the hospital, to other Apophysis
hospitals or to the Consultant’s personal computer.
Diaphysis
Radiographic interpretation
Although radiograph is the correct word for the plain
image which we address, in the present book we have
chosen to retain the old-fashioned term ‘x-ray’, which
has become entrenched by long usage. Cortex
The process of interpreting this image should be as Medulla
methodical as clinical examination. It is seductively
easy to be led astray by some flagrant anomaly; sys-
tematic study is the only safeguard. A convenient
sequence for examination is: the patient – the soft tis-
sues – the bone – the joints. Physis
Epiphysis
THE PATIENT 1.13 The radiographic image X-ray of an anatomical

Make sure that the name on the film is that of your specimen to show the appearance of various parts of the
16 patient; mistaken identity is a potent source of error. bone in the x-ray image.
1
(a) (b) (c) (d) (e) (f)
1.14 X-rays – bent bones (a) Mal-united fracture. (b) Paget’s disease. (c) Dyschondroplasia. (d) Congenital
pseudarthrosis. (e) Syphilitic sabre tibia. (f) Osteogenesis imperfecta.
vertebral alignment, then at the disc spaces, and then lesions think of metastases (including myeloma and
at each vertebra separately, moving from the body to lymphoma) and also multifocal infection. By contrast,
the pedicles, the facet joints and finally the spinous most primary tumours are monostotic.
appendages. For the pelvis, see if the shape is sym-
Localized change Focal abnormalities should be
metrical with the bones in their normal positions,
approached in the same way as one would conduct a
then look at the sacrum, the two innominate bones,
clinical analysis of a soft tissue abnormality. Start
the pubic rami and the ischial tuberosities, then the
describing the abnormality from the centre and move
femoral heads and the upper ends of the femora,
outwards. Determine the lesion’s size, site, shape, den-
always comparing the two sides.
sity and margins, as well as adjacent periosteal changes
Generalized change Take note of changes in bone ‘den- and any surrounding soft tissue changes. Remember
sity’ (osteopaenia or osteosclerosis). Is there abnormal that benign lesions are usually well defined with scle-
trabeculation, as in Paget’s disease? Are there features rotic margins (Fig. 1.15b) and a smooth periosteal
suggestive of diffuse metastatic infiltration, either reaction. Ill-defined areas with permeative bone
sclerotic or lytic? Other polyostotic lesions include destruction (Fig. 1.15c) and irregular or spiculated
fibrous dysplasia, histiocyotis, multiple exostosis and periosteal reactions (Fig. 1.15d) suggest an aggressive
Paget’s disease. With aggressive looking polyostotic lesion such as infection or a malignant tumour.
(a) (b) (c) (d) (e)
1.15 X-rays – important features to look for (a) General shape and appearance, in this case the cortices are thickened
and the bone is bent (Paget’s disease). (b,c) Interior density, a vacant area may represent a true cyst (b), or radiolucent
material infiltrating the bone, like the metastatic tumour in (c). (d) Periosteal reaction, typically seen in healing fractures,
bone infection and malignant bone tumours – as in this example of Ewing’s sarcoma. Compare this with the smooth
periosteal new bone formation shown in (e). 17
1 1.16 Plain x-rays of the hip Stages in the
development of osteoarthritis (OA). (a) Normal
hip: anatomical shape and position, with joint
‘space’ (articular cartilage) fully preserved.
(b) Early OA, showing joint space slightly
decreased and a subarticular cyst in the femoral
head. (c) Advanced OA: joint space markedly
decreased; osteophytes at the joint margin.

(d) Hip replacement: the cup is radiolucent but
its position is shown by a circumferential wire
marker. Note the differing image ‘densities’:
(1) the metal femoral implant; (2) the
polyethylene cup (radiolucent); (3) acrylic
cement impacted into the adjacent bone.
(a) (b)
2
1
(c) (d)
THE JOINTS types of arthropathy. In rheumatoid arthritis and psori-

The radiographic ‘joint’ consists of the articulating asis the erosions are peri-articular (at the bare area where
bones and the ‘space’ between them. the hyaline cartilage covering the joint has ended and the
intracapsular bone is exposed to joint fluid). In gout the
The ‘joint space’ The joint space is, of course, illusory;
erosions are further away from the articular surfaces and
it is occupied by a film of synovial fluid plus radiolu-
are described as juxta-articular. Rheumatoid arthritis is
cent articular cartilage which varies in thickness from
classically symmetrical and predominantly involves the
1 mm or less (the carpal joints) to 6 mm (the knee). It
metacarpophalangeal and proximal interphalangeal joints
looks much wider in children than in adults because
in both hands. The erosions in psoriasis are usually more
much of the epiphysis is still cartilaginous and there-
feathery with ill-defined new bone at their margins. Ill-
fore radiolucent. Lines of increased density within the
defined erosions suggest active synovitis whereas corti-
radiographic articular ‘space’ may be due to calcifica-
cated erosions indicate healing and chronicity.
tion of the cartilage or menisci (chondrocalcinosis).
Loose bodies, if they are radio-opaque, appear as
rounded patches overlying the normal structures. Diagnostic associations
Shape Note the general orientation of the joint and the However carefully the individual x-ray features are
congruity of the bone ends (actually the subarticular observed, the diagnosis will not leap ready-made off
bone plates), if necessary comparing the abnormal with the x-ray plate. Even a fracture is not always obvious.
the normal opposite side. Then look for narrowing or It is the pattern of abnormalities that counts: if you
asymmetry of the joint ‘space’: narrowing signifies loss see one feature that is suggestive, look for others that
of hyaline cartilage and is typical of infection, inflam- are commonly associated.
matory arthropathies and osteoarthritis. Further stages
• Narrowing of the joint space + subchondral sclero-
of joint destruction are revealed by irregularity of the
sis and cysts + osteophytes = osteoarthritis.
radiographically visible bone ends and radiolucent cysts
• Narrowing of the joint space + osteoporosis + peri-
in the subchondral bone. Bony excrescences at the joint
articular erosions = inflammatory arthritis. Add to
margins (osteophytes) are typical of osteoarthritis.
this the typical distribution, more or less symmetri-
Erosions Look for associated bone erosions. The posi- cally in the proximal joints of both hands, and you
18 tion of erosions and symmetry help to define various must think of rheumatoid arthritis.
• Bone destruction + periosteal new bone formation in orthopaedics are mostly iodine-based liquids which 1
= infection or malignancy until proven otherwise. can be injected into sinuses, joint cavities or the spinal
• Remember: the next best investigation is either the theca. Air or gas also can be injected into joints to
previous radiograph or the subsequent follow-up produce a ‘negative image’ outlining the joint cavity.
radiograph. Sequential films demonstrate either Oily iodides are not absorbed and maintain maxi-
progression of changes in active pathology or status mum concentration after injection. However, because
quo in longstanding conditions. they are non-miscible, they do not penetrate well into
all the nooks and crannies. They are also tissue irri-
tants, especially if used intrathecally. Ionic, water-
Limitations of conventional radiography
soluble iodides permit much more detailed imaging
Conventional radiography involves exposure of the and, although also somewhat irritant and neurotoxic,
patient to ionizing radiation, which under certain cir- are rapidly absorbed and excreted.
cumstances can lead to radiation-induced cancer. The
Ionising Radiation Medical Exposure Regulations
Sinography
(IRMER) 2000 are embedded in European Law, requir-
ing all clinicians to justify any exposure of the patient to Sinography is the simplest form of contrast radio-
ionizing radiation. It is a criminal offence to breach these graphy. The medium (usually one of the ionic water-
regulations. Ionizing radiation can also damage a devel- soluble compounds) is injected into an open sinus; the
oping foetus, especially in the first trimester. film shows the track and whether or not it leads to the
As a diagnostic tool, conventional radiography pro- underlying bone or joint.
vides poor soft-tissue contrast: for example, it cannot
distinguish between muscles, tendons, ligaments and
hyaline cartilage. Ultrasound (US), computed tomog-
Arthrography
raphy (CT) and magnetic resonance imaging (MRI) Arthrography is a particularly useful form of contrast
are now employed to complement plain x-ray exami- radiography. Intra-articular loose bodies will produce
nation. However, in parts of the world where these filling defects in the opaque contrast medium. In the
techniques are not available, some modifications of knee, torn menisci, ligament tears and capsular rup-
plain radiography still have a useful role. tures can be shown. In children’s hips, arthrography is
a useful method of outlining the cartilaginous (and
therefore radiolucent) femoral head. In adults with
X-RAYS USING CONTRAST MEDIA avascular necrosis of the femoral head, arthrography
may show up torn flaps of cartilage. After hip replace-
Substances that alter x-ray attenuation characteristics ment, loosening of a prosthesis may be revealed by
can be used to produce images which contrast with seepage of the contrast medium into the
those of the normal tissues. The contrast media used cement/bone interface. In the hip, ankle, wrist and
(a) (b) (c)

1.17 Contrast radiography (a) Myelography shows the outline of the spinal theca. Where facilities are available,
myelography has been largely replaced by CT and MRI. (b) Discography is sometimes useful: note the difference between a
normal intervertebral disc (upper level) and a degenerate disc (lower level). (c) Contrast arthrography of the knee shows a
small popliteal herniation. 19
1 shoulder, the injected contrast medium may disclose PLAIN TOMOGRAPHY
labral tears or defects in the capsular structures. In the
spine, contrast radiography can be used to diagnose Tomography provides an image ‘focused’ on a
disc degeneration (discography) and abnormalities of selected plane. By moving the tube and the x-ray film
the small facet joints (facetography). in opposite directions around the patient during the
exposure, images on either side of the pivotal plane
are deliberately blurred out. When several ‘cuts’ are

Myelography
studied, lesions obscured in conventional x-rays may
Myelography was used extensively in the past for the be revealed. The method is useful for diagnosing seg-
diagnosis of disc prolapse and other spinal canal lesions. mental bone necrosis and depressed fractures in can-
It has been largely replaced by non-invasive methods cellous bone (e.g. of the vertebral body or the tibial
such as CT and MRI. However, it still has a place in the plateau); these defects are often obscured in the plain
investigation of nerve root lesions and as an adjunct to x-ray by the surrounding intact mass of bone. Small
other methods in patients with back pain. radiolucent lesions, such as osteoid osteomas and
The oily media are no longer used, and even with bone abscesses, can also be revealed.
the ionic water-soluble iodides there is a considerable A useful procedure in former years, conventional
incidence of complications, such as low-pressure tomography has been largely supplanted by CT and MRI.
headache (due to the lumbar puncture), muscular
spasms or convulsions (due to neurotoxicity, espe-
cially if the chemical is allowed to flow above the mid- COMPUTED TOMOGRAPHY (CT)
dorsal region) and arachnoiditis (which is attributed
to the hyperosmolality of these compounds in relation Like plain tomography, CT produces sectional images
to cerebrospinal fluid). Precautions, such as keeping through selected tissue planes – but with much
the patient sitting upright after myelography, must be greater resolution. A further advance over conven-
strictly observed. tional tomography is that the images are trans-axial
Metrizamide has low neurotoxicity and at working (like transverse anatomical sections), thus exposing
concentrations it is more or less isotonic with cere- anatomical planes that are never viewed in plain film
brospinal fluid. It can therefore be used throughout x-rays. A general (or ‘localization’) view is obtained,
the length of the spinal canal; the nerve roots are also the region of interest is selected and a series of cross-
well delineated (radiculography). A bulging disc, an sectional images is produced and digitally recorded.
intrathecal tumour or narrowing of the bony canal ‘Slices’ through the larger joints or tissue masses may
will produce characteristic distortions of the opaque be 5–10 mm apart; those through the small joints or
column in the myelogram. intervertebral discs have to be much thinner.
(a)
(c) (d)
1.18 Computed tomography (CT) The plain x-ray (a) shows a fracture of the vertebral
body but one cannot tell precisely how the bone fragments are displaced. The CT (b) shows
clearly that they are dangerously close to the cauda equina. (c) Congenital hip dislocation,
20 (b) defined more clearly by (d) three-dimensional CT reconstruction.
1
(a) (b) (c)
1.19 CT for complex fractures (a) A plain x-ray shows a fracture of the calcaneum but the details are obscure. CT
sagittal and axial views (b,c) give a much clearer idea of the seriousness of this fracture.
New multi-slice CT scanners provide images of MAGNETIC RESONANCE IMAGING (MRI)

high quality from which multi-planar reconstructions
in all three orthogonal planes can be produced. Magnetic resonance imaging produces cross-sectional
Three-dimensional surface rendered reconstructions images of any body part in any plane. It yields superb
and volume rendered reconstructions may help in soft-tissue contrast, allowing different soft tissues to
demonstrating anatomical contours, but fine detail is be clearly distinguished, e.g. ligaments, tendons, mus-
lost in this process. cle and hyaline cartilage. Another big advantage of
MRI is that it does not use ionizing radiation. It is,
however, contra-indicated in patients with pacemakers
Clinical applications
and possible metallic foreign bodies in the eye or
Because CT achieves excellent contrast resolution and brain, as these could potentially move when the
spatial localization, it is able to display the size, shape patient is introduced into the scanner’s strong mag-
and position of bone and soft-tissue masses in trans- netic field. Approximately 5% of patients cannot toler-
verse planes. Image acquisition is extremely fast. The ate the scan due to claustrophobia, but newer
technique is therefore ideal for evaluating acute scanners are being developed to be more ‘open’.
trauma to the head, spine, chest, abdomen and pelvis.
It is better than MRI for demonstrating fine bone
detail and soft-tissue calcification or ossification.
MRI physics
Computed tomography is also an invaluable tool The patient’s body is placed in a strong magnetic field (be-
for assisting with pre-operative planning in secondary tween 5 and 30 000 times the strength of the earth’s mag-
fracture management. It is routinely used for assessing netic field). The body’s protons have a positive charge and
injuries of the vertebrae, acetabulum, proximal tibial align themselves along this strong external magnetic field.
plateau, ankle and foot – indeed complex fractures The protons are spinning and can be further excited by ra-
and fracture-dislocations at any site. diofrequency pulses, rather like whipping a spinning top.
It is also useful in the assessment of bone tumour These spinning positive charges will not only induce a
size and spread, even if it is unable to characterize the small magnetic field of their own, but will produce a sig-
tumour type. It can be employed for guiding soft- nal as they relax (slow down) at different rates.
tissue and bone biopsies. A proton density map is recorded from these signals
and plotted in x, y and z coordinates. Different speeds
of tissue excitation with radiofrequency pulses (repeti-
Limitations
tion times, or TR) and different intervals between
An important limitation of CT is that it provides rela- recording these signals (time to echo, or TE) will yield
tively poor soft-tissue contrast when compared with anatomical pictures with varying ‘weighting’ and char-
MRI. acteristics. T1 weighted (T1W) images have a high spa-
A major disadvantage of this technique is the rela- tial resolution and provide good anatomical-looking
tively high radiation exposure to which the patient is pictures. T2 weighted (T2W) images give more infor-
subjected. It should, therefore, be used with discre- mation about the physiological characteristics of the tis-
tion. sue. Proton density (PD) images are also described as 21
1
(a) (b)
1.21 MRI A case of septic arthritis of the ankle, suspected

from the plain x-ray (a) and confirmed by MRI (b).
1.20 Magnetic resonance imaging MRI is ideal for
displaying soft-tissue injuries, particularly tears of the
menisci of the knee; this common injury is clearly shown in
the picture.
Magnetic resonance imaging is becoming cheaper and
more widely available. Its excellent anatomical detail,
soft-tissue contrast and multi-planar capability make it
‘balanced’ or ‘intermediate’ as they are essentially a ideal for non-invasive imaging of the musculoskeletal
combination of T1 and T2 weighting and yield excellent system. The multi-planar capability provides accurate
anatomical detail for orthopaedic imaging. Fat suppres- cross-sectional information and the axial images in
sion sequences allow highlighting of abnormal water, particular will reveal detailed limb compartmental
which is particularly useful in orthopaedics when assess- anatomy. The excellent soft-tissue contrast allows
ing both soft tissue and bone marrow oedema. identification of similar density soft tissues, for exam-
ple in distinguishing between tendons, cartilage and
ligaments. By using combinations of T1W, T2W and
Intravenous contrast
fat suppressed sequences, specific abnormalities can be
Just as in CT, enhancement by intravenous contrast further characterized with tissue specificity, so further
relies on an active blood supply and leaky cell mem- extending the diagnostic possibilities.
branes. Areas of inflammation and active tumour tis- In orthopaedic surgery, MRI of the hip, knee,
sue will be highlighted. Gadolinium compounds are ankle, shoulder and wrist is now fairly commonplace.
employed as they have seven unpaired electrons and It can detect the early changes of bone marrow
work by creating local magnetic field disturbances at oedema and osteonecrosis before any other imaging
their sites of accumulation. modality. In the knee, MRI is as accurate as
arthroscopy in diagnosing meniscal tears and cruciate
ligament injuries. Bone and soft-tissue tumours
Indirect arthrography
should be routinely examined by MRI as the intra-
Gadolinium compounds administered intravenously osseous and extra-osseous extent and spread of dis-
will be secreted through joint synovium into joint ease, as well as the compartmental anatomy, can be
effusions resulting in indirect arthrography. However, accurately assessed. Additional use of fat suppression
there is no additional distension of the joint, which sequences determines the extent of peri-lesional
limits its effect. oedema and intravenous contrast will demonstrate the
active part of the tumour.
Intravenous contrast is used to distinguish vascular-
Direct arthrography
ized from avascular tissue, e.g. following a scaphoid
Direct puncture of joints under image guidance with fracture, or in defining active necrotic areas of tumour,
a solution containing dilute gadolinium (1:200 con- or in demonstrating areas of active inflammation.
centration) is routinely performed. This provides a Direct MRI arthrography is used to distend the
positive contrast within the joint and distension of the joint capsule and outline labral tears in the shoulder
joint capsule, thereby separating many of the closely and the hip. In the ankle, it provides the way to
applied soft-tissue structures that can be demon- demonstrate anterolateral impingement and assess the
22 strated on the subsequent MRI scan. integrity of the capsular ligaments.
Limitations acetabulum (which are, of course, ‘invisible’ on x-ray) 1
can be clearly identified, and their relationship to each
Despite its undoubted value, MRI (like all singular other shows whether the hip is normal or abnormal.
methods of investigation) has its limitations and it Ultrasound imaging is quick, cheap, simple and
must be seen as one of a group of imaging techniques, readily available. However, the information obtained
none of which by itself is appropriate in every situa- is highly operator dependent, relying on the experi-
tion. Conventional radiographs and CT are more sen- ence and interpretation of the technician.
sitive to soft-tissue calcification and ossification,
changes which can easily be easily overlooked on
MRI. Conventional radiographs should, therefore, be Doppler ultrasound
used in combination with MRI to prevent such errors. Blood flow can be detected by using the principle of a
change in frequency of sound when material is mov-
ing towards or away from the ultrasound transducer.
DIAGNOSTIC ULTRASOUND This is the same principle as the change in frequency
of the noise from a passing fire engine when travelling
High-frequency sound waves, generated by a trans- towards and then away from an observer. Abnormal
ducer, can penetrate several centimetres into the soft increased blood flow can be observed in areas of
tissues; as they pass through the tissue interfaces some inflammation or in aggressive tumours. Different flow
of these waves are reflected back (like echoes) to the rates can be shown by different colour representations
transducer, where they are registered as electrical sig- (‘colour Doppler’).
nals and displayed as images on a screen. Unlike x-
rays, the image does not depend on tissue density but
rather on reflective surfaces and soft-tissue interfaces. RADIONUCLIDE IMAGING
This is the same principle as applies in sonar detection
for ships or submarines. Photon emission by radionuclides taken up in specific
Depending on their structure, different tissues are tissues can be recorded by a gamma camera to pro-
referred to as highly echogenic, mildly echogenic or duce an image which reflects physiological activity in
echo-free. Fluid-filled cysts are echo-free; fat is highly that tissue or organ. The radiopharmaceutical used for
echogenic; and semi-solid organs manifest varying radionuclide imaging has two components: a chemical
degrees of ‘echogenicity’, which makes it possible to compound that is chosen for its metabolic uptake in
differentiate between them. the target tissue or organ, and a radioisotope tracer
Real-time display on a monitor gives a dynamic im- that will emit photons for detection.
age, which is more useful than the usual static images.
A big advantage of this technique is that the equipment
is simple and portable and can be used almost any-
Isotope bone scans
where; another is that it is entirely harmless. For bone imaging the ideal isotope is technetium-
99m (99mTc): it has the appropriate energy characteris-
tics for gamma camera imaging, it has a relatively short
half-life (6 hours) and it is rapidly excreted in the urine.
Because of the marked echogenic contrast between A bone-seeking phosphate compound is used as the
cystic and solid masses, ultrasonography is particularly substrate as it is selectively taken up and concentrated
useful for identifying hidden ‘cystic’ lesions such as in bone. The low background radioactivity means that
haematomas, abscesses, popliteal cysts and arterial any site of increased uptake is readily visible.
aneurysms. It is also capable of detecting intra-articu- Technetium-labelled hydroxymethylene diphos-
lar fluid and may be used to diagnose a synovial effu- phonate (99mTc-HDP) is injected intravenously and its
sion or to monitor the progress of an ‘irritable hip’. activity is recorded at two stages: (1) the early perfu-
Ultrasound is commonly used for assessing tendons sion phase, shortly after injection, while the isotope is
and diagnosing conditions such as tendinitis and par- still in the blood stream or the perivascular space thus
tial or complete tears. The rotator cuff, patellar liga- reflecting local blood flow difference; and (2) the
ment, quadriceps tendon, Achilles tendon, flexor delayed bone phase, 3 hours later, when the isotope
tendons and peroneal tendons are typical examples. has been taken up in bone tissue. Normally, in the
The same technique is used extensively for guiding early perfusion phase the vascular soft tissues around
needle placement in diagnostic and therapeutic joint the joints produce the sharpest (most active) image; 3
and soft-tissue injections. hours later this activity has faded and the bone out-
Another important application is in the screening of lines are shown more clearly, the greatest activity
newborn babies for congenital dislocation (or dyspla- appearing in the cancellous tissue at the ends of the
sia) of the hip; the cartilaginous femoral head and long bones. 23
1 the site of abnormality and it should always be viewed
in conjunction with other modes of imaging.
Bone scintigraphy is relatively sensitive but non-
specific. One advantage is that the whole body can be
imaged to look for multiple sites of pathology (occult
metastases, multi-focal infection and multiple occult
fractures). It is also one of the only techniques to give

information about physiological activity in the tissues
being examined (essentially osteoblastic activity).
However, the technique carries a significant radiation
burden (equivalent to approximately 200 chest x-rays)
and the images yielded make anatomical localization
difficult (poor spatial resolution). For localized prob-
lems MRI has superseded bone scintigraphy as it
(a) (b) yields much greater specificity due to its superior
1.22 Radionuclide scanning (a) The plain x-ray showed anatomical depiction and tissue specificity.
a pathological fracture, probably through a metastatic
tumour. (b) The bone scan revealed generalized
secondaries, here involving the spine and ribs. Other radionuclide compounds
Gallium-67 ( 67Ga) Gallium-67 concentrates in inflam-

matory cells and has been used to identify sites of hid-
Changes in radioactivity are most significant when
den infection: for example, in the investigation of
they are localized or asymmetrical. Four types of
prosthetic loosening after joint replacement. How-
abnormality are seen:
ever, it is arguable whether it gives any more reliable
Increased activity in the perfusion phase This is due to information than the 99mTc bone scan.
increased soft-tissue blood flow, suggesting inflamma-
Indium-111-labelled leucocytes (111I) The patient’s own
tion (e.g. acute or chronic synovitis), a fracture, a
white blood cells are removed and labelled with
highly vascular tumour or regional sympathetic dys-
indium-111 before being re-injected into the patient’s
trophy.
blood stream. Preferential uptake in areas of infection
Decreased activity in the perfusion phase This is much is expected, thereby hoping to distinguish sites of active
less common and signifies local vascular insufficiency. infection from chronic inflammation. For example,
white cell uptake is more likely to be seen with an
Increased activity in the delayed bone phase This could be
infected total hip replacement as opposed to mechani-
due either to excessive isotope uptake in the osseous
cal loosening. However, as this technique is expensive
extracellular fluid or to more avid incorporation into
and still not completely specific, it is seldom performed.
newly forming bone tissue; either would be likely in a
fracture, implant loosening, infection, a local tumour
or healing after necrosis, and nothing in the bone scan
itself distinguishes between these conditions. SINGLE PHOTON EMISSION COMPUTED
Diminished activity in the bone phase This is due to an TOMOGRAPHY
absent blood supply (e.g. in the femoral head after a
fracture of the femoral neck) or to replacement of Single photon emission computed tomography
bone by pathological tissue. (SPECT) is essentially a bone scan in which images
are recorded and displayed in all three orthogonal
CLINICAL APPLICATIONS planes. Coronal, sagittal and axial images at multiple
Radionuclide imaging is useful in many situations: (1) levels make spatial localization of pathology possible:
the diagnosis of stress fractures or other undisplaced for example, activity in one side of a lumbar vertebra
fractures that are not detectable on the plain x-ray; (2) on the planar images can be further localized to the
the detection of a small bone abscess, or an osteoid body, pedicle or lamina of the vertebra on the SPECT
osteoma; (3) the investigation of loosening or infec- images.
tion around prostheses; (4) the diagnosis of femoral
head ischaemia in Perthes’ disease or avascular necro-
sis in adults; (5) the early detection of bone metas- POSITRON EMISSION TOMOGRAPHY
tases. The scintigraphic appearances in these
conditions are described in the relevant chapters. In Positron emission tomography (PET) is an advanced
24 most cases the isotope scan serves chiefly to pinpoint nuclear medicine technique that allows functional im-
aging of disease processes. Positron-emitting isotopes Various techniques have been developed, including 1
with short half-lives are produced on site at specialist radiographic absorptiometry (RA), quantitative com-
centres using a cyclotron. Various radiopharmaceuticals puted tomography (QCT) and quantitative ultra-
can be employed, but currently the most commonly sonometry (QUS). However, the most widely used
used is 18-fluoro-2-deoxy-D-glucose (18FDG). The technique is dual energy x-ray absorptiometry (DXA).
18
FDG is accumulated in different parts of the body RA uses conventional radiographic equipment and
where it can effectively measure the rate of consump- measures bone density in the phalanges. QCT meas-
tion of glucose. Malignant tumours metabolize glucose ures trabecular bone density in vertebral bodies, but is
at a faster rate than benign tumours and PET scanners not widely available and involves a higher dose of ion-
are extremely useful in looking for occult sites of dis- izing radiation than DXA. QUS assesses bone mineral
ease around the body on this basis. density in the peripheral skeleton (e.g. the wrist and
PET/CT is a hybrid examination performing both calcaneus) by measuring both the attenuation of ultra-
PET and CT on the patient in order to superimpose sound and the variation of speed of sound through
the two images produced. The combination of these the bone.
two techniques uses the sensitivity of PET for func- DXA employs columnated low-dose x-ray beams of
tional tissue changes and the cross-sectional anatomy two different energy levels in order to distinguish the
detail of CT to localize the position of this activity. density of bone from that of soft tissue. Although this
PET is useful in oncology to identify occult malig- involves the use of ionizing radiation, it is an
nant tumours and metastases and more accurately extremely low dose. A further advantage of DXA is
‘stage’ the disease. Furthermore, activity levels at the development of a huge international database that
known sites of disease can be used to assess treatment allows expression of bone mineral density values in
and distinguish ‘active’ residual tumour or tumour comparison to both an age and sex matched popula-
recurrence from ‘inactive’ post-surgical scarring and tion (Z score) and also to the peak adult bone mass (T
necrotic tumour. score). The T score in particular allows calculation of
relative fracture risk. Individual values for both the
lumbar spine and hips are obtained as there is often a
BONE MINERAL DENSITOMETRY discrepancy between these two sites and the fracture
risk is more directly related to the value at the target
Bone mineral density (BMD) measurement is now area. By World Health Organization (WHO) criteria,
widely used in identifying patients with osteoporosis T scores of <–1.0 indicate ‘osteopenia’ and T scores of
and an increased risk of osteoporotic fractures. <–2.5 indicate ‘osteoporosis’.
DXA Results Summary:
Region Area BMC BMD T - score PR(%) Z - score AM(%)

(cm2) (g) (g/cm2)
Neck 5.37 3.62 0.675 –1.6 79 –0.5 93
Troch 12.48 7.10 0.569 –1.3 81 –0.6 90
Inter 20.07 18.92 0.943 –1.0 86 –0.5 92
Total 37.92 29.64 0.782 –1.3 83 –0.6 92
Ward’s 1.03 0.54 0.527 –1.8 72 0.0 100
Total BMD CV 1.)%
WHO Classification: Osteopenia
Fracture Risk: Increased
(a) (b)
1.23 Measurement of bone mass (a) X-ray of the lumbar spine shows a compression fracture of L2. The general loss of
bone density accentuates the cortical outlines of the vertebral body end-plates. These features are characteristic of
diminished bone mass, which can be measured accurately by dual energy x-ray absorptiometry. (b) DXA scan from another
woman who attended for monitoring at the onset of the menopause. 25
1 BLOOD TESTS Biochemistry
Biochemical tests are essential in monitoring patients
Non-specific blood tests after any serious injury. They are also used routinely in
the investigation of rheumatic disorders and abnor-
Non-specific blood abnormalities are common in malities of bone metabolism. Their significance is dis-
bone and joint disorders; their interpretation hinges
cussed under the relevant conditions.

on the clinical and x-ray findings.
Hypochromic anaemia is usual in rheumatoid arthri-
tis, but it may also be a consequence of gastrointesti-
nal bleeding due to the anti-inflammatory drugs. SYNOVIAL FLUID ANALYSIS
Leucocytosis is generally associated with infection,
but a mild leucocytosis is not uncommon in rheuma-
Arthrocentesis and synovial fluid analysis is a much-
toid arthritis and during an attack of gout.
neglected diagnostic procedure; given the correct
The erythrocyte sedimentation rate (ESR) is usually
indications it can yield valuable information. It should
increased in acute and chronic inflammatory disorders
be considered in the following conditions.
and after tissue injury. However, patients with low-
grade infection may have a normal ESR and this Acute joint swelling after injury The distinction
should not be taken as a reassuring sign. The ESR is between synovitis and bleeding may not be obvious;
strongly affected by the presence of monoclonal aspiration will settle the question immediately.
immunoglobulins; a high ESR is almost mandatory in
Acute atraumatic synovitis in adults Synovial fluid analy-
the diagnosis of myelomatosis.
sis may be the only way to distinguish between infec-
C-reactive protein (and other acute phase proteins)
tion, gout and pseudogout. Characteristic crystals can
may be abnormally increased in chronic inflammatory
be identified on polarized light microscopy.
arthritis and (temporarily) after injury or operation.
The test is often used to monitor the progress and Suspected infection Careful examination and labora-
activity of rheumatoid arthritis and chronic infection. tory investigations may provide the answer, but they
Plasma gamma-globulins can be measured by pro- take time. Joint aspiration is essential for early
tein electrophoresis. Their precise characterization is diagnosis.
helpful in the assessment of certain rheumatic disor-
Chronic synovitis Here joint aspiration is less urgent,
ders, and more particularly in the diagnosis of myelo-
and is only one of many diagnostic procedures in the
matosis.
investigation of suspected tuberculosis or atypical
rheumatic disorders.
Rheumatoid factor tests
Rheumatoid factor, an IgM autoantibody, is present Technique
in about 75% of adults with rheumatoid arthritis.
However, it is not pathognomonic: some patients Joint aspiration should always be performed under
with undoubted rheumatoid arthritis remain strict aseptic conditions. After infiltrating the skin
‘seronegative’, while rheumatoid factor is found in with a local anaesthetic, a 20-gauge needle is intro-
some patients with other disorders such as systemic duced and a sample of joint fluid is aspirated; even a
lupus erythematosus and scleroderma. small quantity of fluid (less than 0.5 mL) is enough
Ankylosing spondylitis, Reiter’s disease and psori- for diagnostic analysis.
atic arthritis characteristically test negative for The volume of fluid and its appearance are immedi-
rheumatoid factor; they have been grouped together ately noted. Normal synovial fluid is clear and slightly
as the ‘seronegative spondarthritides’. yellow. A cloudy or turbid fluid is due to the presence
of cells, usually a sign of inflammation. Blood-stained
fluid may be found after injury, but is also seen in
Tissue typing
acute inflammatory disorders and in pigmented vil-
Human leucocyte antigens (HLA) can be detected in lonodular synovitis.
white blood cells and they are used to characterize A single drop of fresh synovial fluid is placed on a
individual tissue types. The seronegative spondarthri- glass slide and examined through the microscope. Blood
tides are closely associated with the presence of HLA- cells are easily identified; abundant leucocytes may sug-
B27 on chromosome 6; this is frequently used as a gest infection. Crystals may be seen, though this usually
confirmatory test in patients suspected of having requires a careful search; they are better characterized by
ankylosing spondylitis or Reiter’s disease, but it polarized light microscopy (see Chapter 4).
should not be regarded as a specific test because it is Dry smears are prepared with heparinized fluid;
26 positive in about 8% of normal western Europeans. more concentrated specimens can be obtained if the
Table 1.2 Examination of synovial fluid 1
Suspected condition Appearance Viscosity White cells Crystals Biochemistry Bacteriology
Normal Clear yellow High Few – As for plasma –
Septic arthritis Purulent Low + – Glucose low +
Tuberculous arthritis Turbid Low + – Glucose low +
Rheumatoid arthritis Cloudy Low ++ – – –
Gout Cloudy Normal ++ Urate – –
Pseudogout Cloudy Normal + Pyrophosphate – –
Osteoarthritis Clear yellow High Few Often+ – –
fluid is centrifuged. After suitable staining (Wright’s show: (a) the type of abnormality (osteoporosis,
and Gram’s), the smear is examined for pus cells and osteomalacia, hyperparathyroidism), and (b) the
organisms. Remember, though, that negative findings severity of the disorder.
do not exclude infection.
Open or closed?
Laboratory tests Open biopsy, with exposure of the lesion and excision
If enough fluid is available, it is sent for full laboratory of a sizeable portion of the bone, seems preferable, but
investigation (cells, biochemistry and bacteriological it has several drawbacks. (1) It requires an operation,
culture). A simultaneous blood specimen allows com- with the attendant risks of anaesthesia and infection.
parison of synovial and blood glucose concentration; a (2) New tissue planes are opened up, predisposing to
marked reduction of synovial glucose suggests infection. spread of infection or tumour. (3) The biopsy incision
A high white cell count (more than 10 000/mm3) may jeopardize subsequent wide excision of the lesion.
is usually indicative of infection, but a moderate leu- (4) The more inaccessible lesions (e.g. a tumour of the
cocytosis is also seen in gout and other types of acetabular floor) can be reached only by dissecting
inflammatory arthritis. widely through healthy tissue.
Bacteriological culture and tests for antibiotic sensi- A carefully performed ‘closed’ biopsy, using a nee-
tivity are essential in any case of suspected infection. dle or trephine of appropriate size to ensure the
removal of an adequate sample of tissue, is the proce-
dure of choice except when the lesion cannot be accu-
rately localized or when the tissue consistency is such
BONE BIOPSY that a sufficient sample cannot be obtained. Solid or
semi-solid tissue is removed intact by the cutting nee-
Bone biopsy is often the crucial means of making a dle or trephine; fluid material can be aspirated
diagnosis or distinguishing between local conditions through the biopsy needle.
that closely resemble one another. Confusion is most
likely to occur when the x-ray or MRI discloses an
area of bone destruction that could be due to a com-
Precautions
pression fracture, a bone tumour or infection (e.g. a • The biopsy site and approach should be carefully
collapsed vertebral body). In other cases it is obvious planned with the aid of x-rays or other imaging
that the lesion is a tumour – but what type of tumour? techniques.
Benign or malignant? Primary or metastatic? Radical • If there is any possibility of the lesion being malig-
surgery should never be undertaken for a suspected nant, the approach should be sited so that the
neoplasm without first confirming the diagnosis histo- wound and biopsy track can be excised if later rad-
logically, no matter how ‘typical’ or ‘obvious’ the x- ical surgery proves to be necessary.
ray appearances may be. • The procedure should be carried out in an operat-
In bone infection, the biopsy permits not only his- ing theatre, under anaesthesia (local or general) and
tological proof of acute inflammation but also bacte- with full aseptic technique.
riological typing of the organism and tests for • For deep-seated lesions, fluoroscopic control of the
antibiotic sensitivity. needle insertion is essential.
The investigation of metabolic bone disease some- • The appropriate size of biopsy needle or cutting
times calls for a tetracycline-labelled bone biopsy to trephine should be selected. 27
1 • A knowledge of the local anatomy and of the likely mits manipulation and opening of the joint compart-
consistency of the lesion is important. Large blood ments. The joint is distended with fluid and the
vessels and nerves must be avoided; potentially vas- arthroscope is introduced percutaneously. Various
cular tumours may bleed profusely and the means to instruments (probes, curettes and forceps) can be
control haemorrhage should be readily to hand. More inserted through other skin portals; they are used to
than one surgeon has set out to aspirate an ‘abscess’ help expose the less accessible parts of the joint, or to
only to plunge a wide-bore needle into an aneurysm! obtain biopsies for further examination. Guided by
• Clear instructions should be given to ensure that the image on the monitor, the arthroscopist explores
the tissue obtained at the biopsy is suitably the joint in a systematic fashion, manipulating the
processed. If infection is suspected, the material arthroscope with one hand and the probe or forceps
should go into a culture tube and be sent to the lab- with the other. At the end of the procedure the joint
oratory as soon as possible. A smear may also be is washed out and the small skin wounds are sutured.
useful. Whole tissue is transferred to a jar contain- The patient is usually able to return home later the
ing formalin, without damaging the specimen or same day.
losing any material. Aspirated blood should be
allowed to clot and can then be preserved in for-
malin for later paraffin embedding and sectioning.
Diagnosis
Tissue thought to contain crystals should not be The knee is the most accessible joint. The appearance
placed in formalin as this may destroy the crystals; of the synovium and the articular surfaces usually
it should either be kept unaltered for immediate allows differentiation between inflammatory and non-
examination or stored in saline. inflammatory, destructive and non-destructive lesions.
• No matter how careful the biopsy, there is always Meniscal tears can be diagnosed and treated immedi-
the risk that the tissue will be too scanty or too ately by repair or removal of partially detached seg-
unrepresentative for accurate diagnosis. Close con- ments. Cruciate ligament deficiency, osteocartilaginous
sultation with the radiologist and pathologist fractures, cartilaginous loose bodies and synovial
beforehand will minimize this possibility. In the ‘tumours’ are also readily visualized.
best hands, needle biopsy has an accuracy rate of Arthroscopy of the shoulder is more difficult, but
over 95%. the articular surfaces and glenoid labrum can be ade-
quately explored. Rotator cuff lesions can often be
diagnosed and treated at the same time.
Arthroscopy of the wrist is useful for diagnosing
DIAGNOSTIC ARTHROSCOPY torn triangular fibrocartilage and interosseous liga-
ment ruptures.
Arthroscopy is performed for both diagnostic and Arthroscopy of the hip is less widely used, but it is
therapeutic reasons. Almost any joint can be reached proving to be useful in the diagnosis of unexplained hip
but the procedure is most usefully employed in the pain. Labral tears, synovial lesions, loose bodies and
knee, shoulder, wrist, ankle and hip. If the suspect articular cartilage damage (all of which are difficult to
lesion is amenable to surgery, it can often be dealt detect by conventional imaging techniques) have been
with at the same sitting without the need for an open diagnosed with a reported accuracy rate of over 50%.
operation. However, arthroscopy is an invasive proce-
dure and its mastery requires skill and practice; it
should not be used simply as an alternative to clinical
Complications
examination and imaging. Diagnostic arthroscopy is safe but not entirely free of
complications, the commonest of which are
haemarthosis, thrombophlebitis, infection and joint
Technique stiffness. There is also a significant incidence of algo-
The instrument is basically a rigid telescope fitted with dystrophy following arthroscopy.
fibreoptic illumination. Tube diameter ranges from
about 2 mm (for small joints) to 4–5 mm (for the
knee). It carries a lens system that gives a magnified im-
age. The eyepiece allows direct viewing by the arthro- REFERENCES AND FURTHER READING
scopist, but it is far more convenient to fit a small, ster-
ilizable solid-state television camera which produces a Apley AG, Solomon L. Physical Examination in
picture of the joint interior on a television monitor. Orthopaedics. Oxford, Butterworth Heinemann, 1997.
The procedure is best carried out under general Resnick D. Diagnosis of Bone and Joint Disorders, Edn 4.
anaesthesia; this gives good muscle relaxation and per- Philadelphia, WB Saunders, 2002.
28
Infection
2
Louis Solomon, H. Srinivasan, Surendar Tuli, Shunmugam Govender
Micro-organisms may reach the musculoskeletal tis- Host susceptibility to infection is increased by (a)
sues by (a) direct introduction through the skin (a pin- local factors such as trauma, scar tissue, poor circula-
prick, an injection, a stab wound, a laceration, an tion, diminished sensibility, chronic bone or joint dis-
open fracture or an operation), (b) direct spread from ease and the presence of foreign bodies, as well as (b)
a contiguous focus of infection, or (c) indirect spread systemic factors such as malnutrition, general illness,
via the blood stream from a distant site such as the nose debility, diabetes, rheumatoid disease, corticosteroid
or mouth, the respiratory tract, the bowel or the gen- administration and all forms of immunosuppression,
itourinary tract. either acquired or induced. Resistance is also dimin-
Depending on the type of invader, the site of infec- ished in the very young and the very old.
tion and the host response, the result may be a pyo- Bacterial colonization and resistance to antibiotics is
genic osteomyelitis, a septic arthritis, a chronic enhanced by the ability of certain microbes (including
granulomatous reaction (classically seen in tuberculo- Staphylococcus) to adhere to avascular bone surfaces
sis of either bone or joint), or an indolent response to and foreign implants, protected from both host
an unusual organism (e.g. a fungal infection). Soft- defences and antibiotics by a protein-polysaccharide
tissue infections range from superficial wound sepsis slime (glycocalyx).
to widespread cellulitis and life-threatening necrotiz- Acute pyogenic bone infections are characterized by
ing cellulitis. Parasitic lesions such as hydatid disease the formation of pus – a concentrate of defunct leu-
also are considered in this chapter, although these are cocytes, dead and dying bacteria and tissue debris –
infestations rather than infections. which is often localized in an abscess. Pressure builds
up within the abscess and infection may then extend
into a contiguous joint or through the cortex and
along adjacent tissue planes. It may also spread further
afield via lymphatics (causing lymphangitis and lym-
phadenopathy) or via the blood stream (bacteraemia
GENERAL ASPECTS OF INFECTION and septicaemia). An accompanying systemic reaction
varies from a vague feeling of lassitude with mild
Infection – as distinct from mere residence of micro- pyrexia to severe illness, fever, toxaemia and shock.
organisms – is a condition in which pathogenic organ- The generalized effects are due to the release of bac-
isms multiply and spread within the body tissues. This terial enzymes and endotoxins as well as cellular
usually gives rise to an acute or chronic inflammatory breakdown products from the host tissues.
reaction, which is the body’s way of combating the Chronic pyogenic infection may follow on unre-
invaders and destroying them, or at least immobiliz- solved acute infection and is characterized by persist-
ing them and confining them to a single area. The ence of the infecting organism in pockets of necrotic
signs of inflammation are recounted in the classical tissue. Purulent material accumulates and may be dis-
mantra: redness, swelling, heat, pain and loss of func- charged through sinuses at the skin or a poorly healed
tion. In one important respect, bone infection differs wound. Factors which favour this outcome are the
from soft-tissue infection: since bone consists of a col- presence of damaged muscle, dead bone or a foreign
lection of rigid compartments, it is more susceptible implant, diminished local blood supply and a weak
than soft tissues to vascular damage and cell death host response. Resistance is likely to be depressed in
from the build-up of pressure in acute inflammation. the very young and the very old, in states of malnu-
Unless it is rapidly suppressed, bone infection will trition or immunosuppression, and in certain diseases
inevitably lead to necrosis. such as diabetes and leukaemia.
2 Table 2.1 Factors predisposing to bone infection 2001). However, it is almost certainly much higher
among less affluent populations.
Malnutrition and general debility
The causal organism in both adults and children is
Diabetes mellitus usually Staphylococcus aureus (found in over 70% of
Corticosteroid administration cases), and less often one of the other Gram-positive
Immune deficiency cocci, such as the Group A beta-haemolytic strepto-
coccus (Streptococcus pyogenes) which is found in

Immunosuppressive drugs
chronic skin infections, as well as Group B strepto-
Venous stasis in the limb coccus (especially in new-born babies) or the alpha-
Peripheral vascular disease haemolytic diplococcus S. pneumoniae.
Loss of sensibility In children between 1 and 4 years of age the Gram-
negative Haemophilus influenzae used to be a fairly
Iatrogenic invasive measures
common pathogen for osteomyelitis and septic arthri-
Trauma tis, but the introduction of H. influenzae type B vac-
cination about 20 years ago has been followed by a
much reduced incidence of this infection in many
Chronic non-pyogenic infection may result from countries. In recent years its place has been taken by
invasion by organisms that produce a cellular reaction
leading to the formation of granulomas consisting
largely of lymphocytes, modified macrophages and
multinucleated giant cells; this type of granulomatous
infection is seen most typically in tuberculosis. Sys-
temic effects are less acute but may ultimately be very
debilitating, with lymphadenopathy, splenomegaly
and tissue wasting.
The principles of treatment are: (1) to provide anal-
gesia and general supportive measures; (2) to rest the
affected part; (3) to identify the infecting organism
and administer effective antibiotic treatment or
chemotherapy; (4) to release pus as soon as it is
detected; (5) to stabilize the bone if it has fractured;
(6) to eradicate avascular and necrotic tissue; (7) to
restore continuity if there is a gap in the bone; and (8)
to maintain soft-tissue and skin cover. Acute infec-
tions, if treated early with effective antibiotics, can
usually be cured. Once there is pus and bone necrosis,
operative drainage will be needed.
ACUTE HAEMATOGENOUS
OSTEOMYELITIS
Aetiology and pathogenesis

Acute haematogenous osteomyelitis is mainly a dis-
ease of children. When adults are affected it is usually
because their resistance is lowered. Trauma may deter-
mine the site of infection, possibly by causing a small
haematoma or fluid collection in a bone, in patients
(a) (b)
with concurrent bacteraemia.
The incidence of acute haematogenous osteo- 2.1 Epiphyseal and metaphyseal blood supply (a) In
myelitis in western European children is thought to new-born infants some metaphyseal arterioles from the
nutrient artery penetrate the physis and may carry infection
have declined in recent years, probably a reflection of
directly from the metaphysis to the epiphysis. (b) In older
improving social conditions. A study from Glasgow, children the physis acts as a barrier and the developing
Scotland, covering the period 1990–99, suggests that epiphysis receives a separate blood supply from the
30 it is less than 3 cases per 100 000 per year (Blyth et al., epiphyseal and peri-articular blood vessels.
Kingella kingae, mainly following upper respiratory Acute osteomyelitis in children The ‘classical’ picture is 2
infection in young children. Other Gram-negative seen in children between 2 and 6 years. The earliest
organisms (e.g. Escherichia coli, Pseudomonas aerugi- change in the metaphysis is an acute inflammatory
nosa, Proteus mirabilis and the anaerobic Bacteroides reaction with vascular congestion, exudation of fluid
fragilis) occasionally cause acute bone infection. Curi- and infiltration by polymorphonuclear leucocytes.
ously, patients with sickle-cell disease are prone to The intraosseous pressure rises rapidly, causing intense
Infection
infection by Salmonella typhi. pain, obstruction to blood flow and intravascular
Anaerobic organisms (particularly Peptococcus mag- thrombosis. Even at an early stage the bone tissue is
nus) have been found in patients with osteomyelitis, threatened by impending ischaemia and resorption
usually as part of a mixed infection. Unusual organ- due to a combination of phagocytic activity and the
isms are more likely to be found in heroin addicts and local accumulation of cytokines, growth factors,
as opportunistic pathogens in patients with compro- prostaglandin and bacterial enzymes. By the second or
mised immune defence mechanisms. third day, pus forms within the bone and forces its
The blood stream is invaded, perhaps from a minor way along the Volkmann canals to the surface where
skin abrasion, treading on a sharp object, an injection it produces a subperiosteal abscess. This is much more
point, a boil, a septic tooth or – in the newborn – evident in children, because of the relatively loose
from an infected umbilical cord. In adults the source attachment of the periosteum, than in adults. From
of infection may be a urethral catheter, an indwelling the subperiosteal abscess pus can spread along the
arterial line or a dirty needle and syringe. shaft, to re-enter the bone at another level or burst
In children the infection usually starts in the vascu- into the surrounding soft tissues. The developing
lar metaphysis of a long bone, most often in the prox- physis acts as a barrier to direct spread towards the
imal tibia or in the distal or proximal ends of the epiphysis, but where the metaphysis is partly intracap-
femur. Predilection for this site has traditionally been sular (e.g. at the hip, shoulder or elbow) pus may dis-
attributed to the peculiar arrangement of the blood charge through the periosteum into the joint.
vessels in that area (Trueta, 1959): the non-anasto- The rising intraosseous pressure, vascular stasis,
mosing terminal branches of the nutrient artery twist small-vessel thrombosis and periosteal stripping
back in hairpin loops before entering the large net-
work of sinusoidal veins; the relative vascular stasis
and consequent lowered oxygen tension are believed
to favour bacterial colonization. It has also been sug-
gested that the structure of the fine vessels in the
hypertrophic zone of the physis allows bacteria more
easily to pass through and adhere to type 1 collagen in
that area (Song and Sloboda, 2001). In infants, in
whom there are still anastomoses between metaphy-
seal and epiphyseal blood vessels, infection can also
reach the epiphysis.
In adults, haematogenous infection accounts for
only about 20% of cases of osteomyelitis, mostly
affecting the vertebrae. Staphylococcus aureus is the (a) (b)
commonest organism but Pseudomonas aeruginosa
often appears in patients using intravenous drugs.
Adults with diabetes, who are prone to soft-tissue
infections of the foot, may develop contiguous bone
infection involving a variety of organisms.
Sequestrum
Pathology Involucrum
Acute haematogenous osteomyelitis shows a charac- Sinus

teristic progression marked by inflammation, suppu-
ration, bone necrosis, reactive new bone formation and, (c)
ultimately, resolution and healing or else intractable
2.2 Acute osteomyelitis (a) Infection in the metaphysis
chronicity. However, the pathological picture varies
may spread towards the surface, to form a subperiosteal
considerably, depending on the patient’s age, the site abscess (b). Some of the bone may die, and is encased in
of infection, the virulence of the organism and the periosteal new bone as a sequestrum (c). The encasing
host response. involucrum is sometimes perforated by sinuses. 31
2 increasingly compromise the blood supply; by the end comes about is still argued over. Following Trueta
of a week there is usually microscopic evidence of (1957) it has long been held that, during the first
bone death. Bacterial toxins and leucocytic enzymes 6–9 months of life, small metaphyseal vessels pene-
also may play their part in the advancing tissue trate the physeal cartilage and this permits the infec-
destruction. With the gradual ingrowth of granulation tion to spread into the cartilaginous epiphysis. Others
tissue the boundary between living and devitalized have disagreed with this hypothesis (Chung, 1976),
bone becomes defined. Pieces of dead bone may sep- but what is indisputable is that during infancy
arate as sequestra varying in size from mere spicules to osteomyelitis and septic arthritis often go together.
large necrotic segments of the cortex in neglected Another feature in infants is an unusually exuberant
cases. periosteal reaction resulting in sometimes bizarre new
Macrophages and lymphocytes arrive in increasing bone formation along the diaphysis; fortunately, with
numbers and the debris is slowly removed by a com- longitudinal growth and remodelling the diaphyseal
bination of phagocytosis and osteoclastic resorption. anatomy is gradually restored.
A small focus in cancellous bone may be completely
Acute osteomyelitis in adults Bone infection in the adult
resorbed, leaving a tiny cavity, but a large cortical or
usually follows an open injury, an operation or spread
cortico-cancellous sequestrum will remain entombed,
from a contiguous focus of infection (e.g. a neuropathic
inaccessible to either final destruction or repair.
ulcer or an infected diabetic foot). True haematogenous
Another feature of advancing acute osteomyelitis is
osteomyelitis is uncommon and when it does occur it
new bone formation. Initially the area around the
usually affects one of the vertebrae (e.g. following a
infected zone is porotic (probably due to hyperaemia
pelvic infection) or a small cuboidal bone. A vertebral
and osteoclastic activity) but if the pus is not released,
infection may spread through the end-plate and the in-
either spontaneously or by surgical decompression,
tervertebral disc into an adjacent vertebral body.
new bone starts forming on viable surfaces in the
If a long bone is infected, the abscess is likely to
bone and from the deep layers of the stripped perios-
spread within the medullary cavity, eroding the cortex
teum. This is typical of pyogenic infection and fine
and extending into the surrounding soft tissues.
streaks of subperiosteal new bone usually become
Periosteal new bone formation is less obvious than in
apparent on x-ray by the end of the second week.
childhood and the weakened cortex may fracture. If
With time this new bone thickens to form a casement,
the bone end becomes involved there is a risk of the
or involucrum, enclosing the sequestrum and infected
infection spreading into an adjacent joint. The out-
tissue. If the infection persists, pus and tiny seques-
come is often a gradual slide towards subacute and
trated spicules of bone may discharge through perfo-
chronic osteomyelitis.
rations (cloacae) in the involucrum and track by
sinuses to the skin surface.
If the infection is controlled and intraosseous pres-
sure released at an early stage, this dire progress can Clinical features
be halted. The bone around the zone of infection
Clinical features differ in the three groups described
becomes increasingly dense; this, together with the
above.
periosteal reaction, results in thickening of the bone.
In some cases the normal anatomy may eventually be Children The patient, usually a child over 4 years,
reconstituted; in others, though healing is sound, the presents with severe pain, malaise and a fever; in neg-
bone is left permanently deformed. lected cases, toxaemia may be marked. The parents
If healing does not occur, a nidus of infection may will have noticed that he or she refuses to use one
remain locked inside the bone, causing pus and some- limb or to allow it to be handled or even touched.
times bone debris to be discharged intermittently There may be a recent history of infection: a septic
through a persistent sinus (or several sinuses). The toe, a boil, a sore throat or a discharge from the ear.
infection has now lapsed into chronic osteomyelitis, Typically the child looks ill and feverish; the pulse
which may last for many years. rate is likely to be over 100 and the temperature is
raised. The limb is held still and there is acute tender-
Acute osteomyelitis in infants The early features of ness near one of the larger joints (e.g. above or below
acute osteomyelitis in infants are much the same as the knee, in the popliteal fossa or in the groin). Even
those in older children. However, a significant differ- the gentlest manipulation is painful and joint move-
ence, during the first year of life, is the frequency with ment is restricted (‘pseudoparalysis’). Local redness,
which the metaphyseal infection spreads to the epiph- swelling, warmth and oedema are later signs and sig-
ysis and from there into the adjacent joint. In the nify that pus has escaped from the interior of the bone.
process, the physeal anlage may be irreparably dam- Lymphadenopathy is common but non-specific. It is
aged, further growth at that site is severely retarded important to remember that all these features may be
32 and the joint will be permanently deformed. How this attenuated if antibiotics have been administered.
Infants In children under a year old, and especially in be a faint extra-cortical outline due to periosteal new 2
the newborn, the constitutional disturbance can be bone formation; this is the classic x-ray sign of early
misleadingly mild; the baby simply fails to thrive and pyogenic osteomyelitis, but treatment should not be
is drowsy but irritable. Suspicion should be aroused delayed while waiting for it to appear. Later the
by a history of birth difficulties, umbilical artery periosteal thickening becomes more obvious and
catheterization or a site of infection (however mild) there is patchy rarefaction of the metaphysis; later still
Infection
such as an inflamed intravenous infusion point or even the ragged features of bone destruction appear.
a heel puncture. Metaphyseal tenderness and resist- An important late sign is the combination of
ance to joint movement can signify either regional osteoporosis with a localized segment of
osteomyelitis or septic arthritis; indeed, both may be apparently increased density. Osteoporosis is a feature
present, so the distinction hardly matters. Look for of metabolically active, and thus living, bone; the seg-
other sites – multiple infection is not uncommon, ment that fails to become osteoporotic is metaboli-
especially in babies who acquire the infection in hos- cally inactive and possibly dead.
pital. Radionuclide bone scans may help to discover
additional sites. ULTRASONOGRAPHY
Ultrasonography may detect a subperiosteal collection
Adults The commonest site for haematogenous
of fluid in the early stages of osteomyelitis, but it can-
infection is the thoracolumbar spine. There may be a
not distinguish between a haematoma and pus.
history of some urological procedure followed by a
mild fever and backache. Local tenderness is not very
RADIONUCLIDE SCANNING
marked and it may take weeks before x-ray signs
Radioscintigraphy with 99mTc-HDP reveals increased
appear; when they do appear the diagnosis may still
activity in both the perfusion phase and the bone
need to be confirmed by fine-needle aspiration and
phase. This is a highly sensitive investigation, even in
bacteriological culture. Other bones are occasionally
the very early stages, but it has relatively low speci-
involved, especially if there is a background of dia-
ficity and other inflammatory lesions can show similar
betes, malnutrition, drug addiction, leukaemia,
changes. In doubtful cases, scanning with 67Ga-citrate
immunosuppressive therapy or debility.
or 111In-labelled leucocytes may be more revealing.
In the very elderly, and in those with immune defi-
ciency, systemic features are mild and the diagnosis is
MAGNETIC RESONANCE IMAGING
easily missed.
Magnetic resonance imaging can be helpful in cases of
doubtful diagnosis, and particularly in suspected
Diagnostic imaging infection of the axial skeleton. It is also the best
method of demonstrating bone marrow inflamma-
PLAIN X-RAY
tion. It is extremely sensitive, even in the early phase
During the first week after the onset of symptoms the
of bone infection, and can therefore assist in differen-
plain x-ray shows no abnormality of the bone. Dis-
tiating between soft-tissue infection and
placement of the fat planes signifies soft-tissue
osteomyelitis. However, specificity is too low to
swelling, but this could as well be due to a haematoma
exclude other local inflammatory lesions.
or soft-tissue infection. By the second week there may
Laboratory investigations
The most certain way to confirm the clinical diagnosis is
to aspirate pus or fluid from the metaphyseal sub-
periosteal abscess, the extraosseous soft tissues or an
adjacent joint. This is done using a 16- or 18-gauge
trocar needle. Even if no pus is found, a smear of the
aspirate is examined immediately for cells and organ-
isms; a simple Gram stain may help to identify the
type of infection and assist with the initial choice of
antibiotic. A sample is also sent for detailed microbio-
logical examination and tests for sensitivity to anti-
biotics. Tissue aspiration will give a positive result in
over 60% of cases; blood cultures are positive in less
2.3 Acute osteomyelitis The first x-ray, 2 days after than half the cases of proven infection.
symptoms began, is normal – it always is; metaphyseal
mottling and periosteal changes were not obvious until the The C-reactive protein (CRP) values are usually ele-
second film, taken 14 days later; eventually much of the vated within 12–24 hours and the erythrocyte sedimen-
shaft was involved. tation rate (ESR) within 24–48 hours after the onset 33
2 CARDINAL FEATURES OF ACUTE
trol towards muscle necrosis, septicaemia and death.
Intense pain and board-like swelling of the limb in a
OSTEOMYELITIS IN CHILDREN patient with fever and a general feeling of illness are
warning signs of a medical emergency. MRI will reveal
Pain muscle swelling and possibly signs of tissue break-
Fever down. Immediate treatment with intravenous antibi-
otics is essential. Surgical debridement of necrotic

Refusal to bear weight tissue – and sometimes even amputation – may be
Elevated white cell count needed to save a life.
Elevated ESR Acute rheumatism The pain is less severe and it tends
to flit from one joint to another. There may also be
Elevated CRP signs of carditis, rheumatic nodules or erythema mar-
ginatum.
of symptoms. The white blood cell (WBC) count rises Sickle-cell crisis The patient may present with features
and the haemoglobin concentration may be dimin- indistinguishable from those of acute osteomyelitis. In
ished. In the very young and the very old these tests are areas where Salmonella is endemic it would be wise to
less reliable and may show values within the range of treat such patients with suitable antibiotics until infec-
normal. tion is definitely excluded.
Antistaphylococcal antibody titres may be raised.
Gaucher’s disease ‘Pseudo-osteitis’ may occur with
This test is useful in atypical cases where the diagnosis
features closely resembling those of osteomyelitis. The
is in doubt.
diagnosis is made by finding other stigmata of the dis-
Osteomyelitis in an unusual site or with an unusual
ease, especially enlargement of the spleen and liver.
organism should alert one to the possibility of heroin
addiction, sickle-cell disease (Salmonella may be cul-
tured from the faeces) or deficient host defence mech- Treatment
anisms including HIV infection.
If osteomyelitis is suspected on clinical grounds, blood
and fluid samples should be taken for laboratory inves-
Differential diagnosis tigation and then treatment started immediately with-
out waiting for final confirmation of the diagnosis.
Cellulitis This is often mistaken for osteomyelitis.
There are four important aspects to the management
There is widespread superficial redness and lymphan-
of the patient:
gitis. The source of skin infection may not be obvious
and should be searched for (e.g. on the sole or • Supportive treatment for pain and dehydration.
between the toes). If doubt remains about the diag- • Splintage of the affected part.
nosis, MRI will help to distinguish between bone • Appropriate antimicrobial therapy.
infection and soft-tissue infection. The organism is • Surgical drainage.
usually staphylococcus or streptococcus. Mild cases
will respond to high dosage oral antibiotics; severe GENERAL SUPPORTIVE TREATMENT
cases need intravenous antibiotic treatment. The distressed child needs to be comforted and
treated for pain. Analgesics should be given at
Acute suppurative arthritis Tenderness is diffuse, and
repeated intervals without waiting for the patient to
movement at the joint is completely abolished by
ask for them. Septicaemia and fever can cause severe
muscle spasm. In infants the distinction between
dehydration and it may be necessary to give fluid
metaphyseal osteomyelitis and septic arthritis of the
intravenously.
adjacent joint is somewhat theoretical, as both often
coexist. A progressive rise in C-reactive protein values
SPLINTAGE
over 24–48 hours is said to be suggestive of concur-
Some type of splintage is desirable, partly for comfort
rent septic arthritis (Unkila-Kallis et al., 1994).
but also to prevent joint contractures. Simple skin
Streptococcal necrotizing myositis Group A beta- traction may suffice and, if the hip is involved, this also
haemolytic streptococci (the same organisms which helps to prevent dislocation. At other sites a plaster
are responsible for the common ‘sore throat’) occa- slab or half-cylinder may be used but it should not
sionally invade muscles and cause an acute myositis obscure the affected area.
which, in its early stages, may be mistaken for celluli-
tis or osteomyelitis. Although the condition is rare, it ANTIBIOTICS
should be kept well to the foreground in the differen- Blood and aspiration material are sent immediately for
34 tial diagnosis because it may rapidly spiral out of con- examination and culture, but the prompt intravenous
administration of antibiotics is so vital that treatment salmonella and/or other Gram-negative organisms. 2
should not await the result. Chloramphenicol, which is effective against Gram-
Initially the choice of antibiotics is based on the positive, Gram-negative and anaerobic organisms,
findings from direct examination of the pus smear and used to be the preferred antibiotic, though there
the clinician’s experience of local conditions – in other were always worries about the rare complication of
words, a ‘best guess’ at the most likely pathogen. aplastic anaemia. Nowadays the antibiotic of choice
Infection
Staphylococcus aureus is the most common at all ages, is a third-generation cephalosporin or a fluoro-
but treatment should provide cover also for other bac- quinolone like ciprofloxacin.
teria that are likely to be encountered in each age • Heroin addicts and immunocompromised patients
group; a more appropriate drug which is also capable Unusual infections (e.g. with Pseudomonas aerugi-
of good bone penetration can be substituted, if nec- nosa, Proteus mirabilis or anaerobic Bacteroides
essary, once the infecting organism is identified and its species) are likely in these patients. Infants with
antibiotic sensitivity is known. Factors such as the human immunodeficiency virus (HIV) infection
patient’s age, general state of resistance, renal func- may also have picked up other sexually transmitted
tion, degree of toxaemia and previous history of organisms during birth. All patients with this type
allergy must be taken into account. The following rec- of background are therefore best treated empirically
ommendations are offered as a guide. with a broad-spectrum antibiotic such as one of the
third-generation cephalosporins or a fluoro-
• Neonates and infants up to 6 months of age Initial quinolone preparation, depending on the results of
antibiotic treatment should be effective against sensitivity tests.
penicillin-resistant Staphylococcus aureus, Group B • Patients considered to be at risk of meticillin-resistant
streptococcus and Gram-negative organisms. Drugs Staphylococcus aureus (MRSA) infection Patients
of choice are flucloxacillin plus a third-generation admitted with acute haematogenous osteomyelitis
cephalosporin like cefotaxime. Alternatively, effec- and who have a previous history of MRSA infec-
tive empirical treatment can be provided by a com- tion, or any patient with a bone infection admitted
bination of flucloxacillin (for penicillin-resistant to a hospital or a ward where MRSA is endemic,
staphylococci), benzylpenicillin (for Group B strep- should be treated with intravenous vancomycin (or
tococci) and gentamicin (for Gram-negative organ- similar antibiotic) together with a third-generation
isms). cephalosporin.
• Children 6 months to 6 years of age Empirical treat-
The usual programme is to administer the drugs
ment in this age group should include cover against
intravenously (if necessary adjusting the choice of
Haemophilus influenzae, unless it is known for cer-
antibiotic once the results of antimicrobial sensitivity
tain that the child has had an anti-haemophilus vac-
become available) until the patient’s condition begins
cination. This is best provided by a combination of
to improve and the CRP values return to normal lev-
intravenous flucloxacillin and cefotaxime or
els – which usually takes 2–4 weeks depending on the
cefuroxime.
virulence of the infection and the patient’s general
• Older children and previously fit adults The vast
degree of fitness. By that time the most appropriate
majority in this group will have a staphylococcal
antibiotic would have been prescribed, on the basis of
infection and can be started on intravenous flu-
sensitivity tests; this can then be administered orally
cloxacillin and fusidic acid. Fusidic acid is preferred
for another 3–6 weeks, though if bone destruction is
to benzylpenicillin partly because of the high preva-
marked the period of treatment may have to be
lence of penicillin-resistant staphylococci and
longer. While patients are on oral antibiotics it is
because it is particularly well concentrated in bone.
important to track the serum antibiotic levels in order
However, for a known streptococcal infection ben-
to ensure that the minimal inhibitory concentration
zylpenicillin is better. Patients who are allergic to
(MIC) is maintained or exceeded. CRP, ESR and
penicillin should be treated with a second- or third-
WBC values are also checked at regular intervals and
generation cephalosporin.
treatment can be discontinued when these are seen to
• Elderly and previously unfit patients In this group
remain normal.
there is a greater than usual risk of Gram-negative
infections, due to respiratory, gastro-intestinal, or
urinary disorders and the likelihood of the patient DRAINAGE
needing invasive procedures. The antibiotic of If antibiotics are given early (within the first 48 hours
choice would be a combination of flucloxacillin and after the onset of symptoms) drainage is often unnec-
a second- or third-generation cephalosporin. essary. However, if the clinical features do not
• Patients with sickle-cell disease These patients are improve within 36 hours of starting treatment, or
prone to osteomyelitis, which may be caused by a even earlier if there are signs of deep pus (swelling,
staphylococcal infection but in many cases is due to oedema, fluctuation), and most certainly if pus is 35
2 When treating patients with bone or joint infection to miss secondary sites of infection when attention is
it is wise to maintain continuous collaboration with focussed on one particular area; it is important to be
a specialist in microbiology alert to this complication and to examine the child all
over and repeatedly.
aspirated, the abscess should be drained by open oper- Pathological fracture Fracture is uncommon, but it
ation under general anaesthesia. If pus is found – and may occur if treatment is delayed and the bone is
released – there is little to be gained by drilling into weakened either by erosion at the site of infection or
the medullary cavity. If there is no obvious abscess, it by overzealous debridement.
is reasonable to drill a few holes into the bone in var-
ious directions. There is no evidence that widespread Chronic osteomyelitis Despite improved methods of di-
drilling has any advantage and it may do more harm agnosis and treatment, acute osteomyelitis sometimes
than good; if there is an extensive intramedullary fails to resolve. Weeks or months after the onset of
abscess, drainage can be better achieved by cutting a acute infection a sequestrum appears in the follow-up
small window in the cortex. The wound is closed x-ray and the patient is left with a chronic infection and
without a drain and the splint (or traction) is reap- a draining sinus. This may be due to late or inadequate
plied. Once the signs of infection subside, movements treatment but is also seen in debilitated patients and in
are encouraged and the child is allowed to walk with those with compromised defence mechanisms.
the aid of crutches. Full weightbearing is usually pos-
sible after 3–4 weeks.
At present about one-third of patients with con-
firmed osteomyelitis are likely to need an operation; SUBACUTE HAEMATOGENOUS
adults with vertebral infection seldom do. OSTEOMYELITIS
This condition is no longer rare, and in some coun-

Complications tries the incidence is equal to that of acute
osteomyelitis. Its relative mildness is presumably due
A lethal outcome from septicaemia is nowadays ex- to the organism being less virulent or the patient
tremely rare; with antibiotics the child nearly always re- more resistant (or both). It is more variable in skele-
covers and the bone may return to normal. But mortal distribution than acute osteomyelitis, but the distal
bidity is common, especially if treatment is delayed or femur and the proximal and distal tibia are the
the organism is insensitive to the chosen antibiotic. favourite sites. The anatomical classification suggested
by Roberts et al. (1982) is useful in comparing fea-
Epiphyseal damage and altered bone growth In
tures in various reported series of cases.
neonates and infants whose epiphyses are still entirely
cartilaginous, metaphyseal vessels penetrate the physis
and may carry the infection into the epiphysis. If this Pathology
happens, the physeal growth plate can be irrevocably
Typically there is a well-defined cavity in cancellous
damaged and the cartilaginous epiphysis may be
bone – usually in the tibial metaphysis – containing
destroyed, leading to arrest of growth and shortening
glairy seropurulent fluid (rarely pus). The cavity is
of the bone. At the hip joint, the proximal end of the
lined by granulation tissue containing a mixture of
femur may be so badly damaged as to result in a
acute and chronic inflammatory cells. The surround-
pseudarthrosis.
ing bone trabeculae are often thickened. The lesion
Suppurative arthritis This may occur: (1) in very sometimes encroaches on and erodes the bony cortex.
young infants, in whom the growth disc is not an Occasionally it appears in the epiphysis and, in adults,
impenetrable barrier; (2) where the metaphysis is in one of the vertebral bodies.
intracapsular, as in the upper femur; or (3) from
metastatic infection. In infants it is so common as Clinical features
almost to be taken for granted, especially with
osteomyelitis of the femoral neck. Ultrasound will The patient is usually a child or adolescent who has
help to demonstrate an effusion, but the definitive had pain near one of the larger joints for several weeks
diagnosis is given by joint aspiration. or even months. He or she may have a limp and often
there is slight swelling, muscle wasting and local ten-
Metastatic infection This is sometimes seen – generally derness. The temperature is usually normal and there
in infants – and may involve other bones, joints, is little to suggest an infection.
serous cavities, the brain or lung. In some cases the The WBC count and blood cultures usually show
36 infection may be multifocal from the outset. It is easy no abnormality but the ESR is sometimes elevated.
2.4 Subacute osteomyelitis (a,b) 2
The classic Brodie’s abscess looks like a
small walled-off cavity in the bone with
little or no periosteal reaction;
(c) sometimes rarefaction is more
diffuse and there may be cortical
erosion and periosteal reaction.
Infection
(a) (b) (c)
Imaging months. If the diagnosis is in doubt, an open biopsy

is needed and the lesion may be curetted at the same
The typical radiographic lesion is a circumscribed, time. Curettage is also indicated if the x-ray shows
round or oval radiolucent ‘cavity’ 1–2 cm in diameter. that there is no healing after conservative treatment;
Most often it is seen in the tibial or femoral metaph- this is always followed by a further course of anti-
ysis, but it may occur in the epiphysis or in one of the biotics.
cuboidal bones (e.g. the calcaneum). Sometimes the
‘cavity’ is surrounded by a halo of sclerosis (the clas-
sic Brodie’s abscess); occasionally it is less well defined,
extending into the diaphysis. POST-TRAUMATIC OSTEOMYELITIS
Metaphyseal lesions cause little or no periosteal
reaction; diaphyseal lesions may be associated with Open fractures are always contaminated and are
periosteal new bone formation and marked cortical therefore prone to infection. The combination of tis-
thickening. If the cortex is eroded the lesion may be sue injury, vascular damage, oedema, haematoma,
mistaken for a malignant tumour. dead bone fragments and an open pathway to the
The radioisotope scan shows markedly increased atmosphere must invite bacterial invasion even if the
activity. wound is not contaminated with particulate dirt. This
is the most common cause of osteomyelitis in adults.
Diagnosis Staphylococcus aureus is the usual pathogen, but
other organisms such as E. coli, Proteus mirabilis and
The clinical and x-ray appearances may resemble those Pseudomonas aeruginosa are sometimes involved.
of cystic tuberculosis, eosinophilic granuloma or Occasionally, anaerobic organisms (clostridia, anaero-
osteoid osteoma; occasionally they mimic a malignant bic streptococci or Bacteroides) appear in contami-
bone tumour like Ewing’s sarcoma. Epiphyseal lesions nated wounds.
are easily mistaken for chondroblastoma. The diagno-
sis often remains in doubt until a biopsy is performed.
If fluid is encountered, it should be sent for bacte- Clinical features
riological culture; this is positive in about half the The patient becomes feverish and develops pain and
cases and the organism is almost always Staphylococcus swelling over the fracture site; the wound is inflamed
aureus. and there may be a seropurulent discharge. Blood
tests reveal increased CRP levels, leucocytosis and an
elevated ESR; it should be remembered, though, that
Treatment
these inflammatory markers are non-specific and may
Treatment may be conservative if the diagnosis is not be affected by tissue trauma.
in doubt. Immobilization and antibiotics (flu- X-ray appearances may be more difficult than usual
cloxacillin and fusidic acid) intravenously for 4 or 5 to interpret because of bone fragmentation. MRI can
days and then orally for another 6 weeks usually be helpful in differentiating between bone and soft-
result in healing, though this may take up to 12 tissue infection, but is less reliable in distinguishing 37
2 between longstanding infection and bone destruction The usual organisms (and with time there is always
due to trauma. a mixed infection) are Staphylococcus aureus,
Escherichia coli, Streptococcus pyogenes, Proteus
mirabilis and Pseudomonas aeruginosa; in the presence
Microbiological investigation
of foreign implants Staphylococcus epidermidis, which
A wound swab should be examined and cultured for is normally non-pathogenic, is the commonest of all.
organisms which can be tested for antibiotic sensitiv-

ity. Unfortunately, though, standard laboratory meth-
ods still yield negative results in about 20 per cent of
Predisposing factors
cases of overt infection. Acute haematogenous osteomyelitis, if left untreated
– and provided the patient does not succumb to sep-
ticaemia – will subside into a chronic bone infection
Treatment which lingers indefinitely, perhaps with alternating
The essence of treatment is prophylaxis: thorough ‘flare-ups’ and spells of apparent quiescence. The
cleansing and debridement of open fractures, the pro- host defences are inevitably compromised by the
vision of drainage by leaving the wound open, immo- presence of scar formation, dead and dying bone
bilization of the fracture and antibiotics. In most cases around the focus of infection, poor penetration of
a combination of flucloxacillin and benzylpenicillin new blood vessels and non-collapsing cavities in
(or sodium fusidate), given 6-hourly for 48 hours, will which microbes can thrive. Bacteria covered in a
suffice. If the wound is clearly contaminated, it is wise protein–polysaccharide slime (glycocalyx) that pro-
also to give metronidazole for 4 or 5 days to control tects them from both the host defences and antibi-
both aerobic and anaerobic organisms. otics have the ability to adhere to inert surfaces such
Pyogenic wound infection, once it has taken root, as bone sequestra and metal implants, where they
is difficult to eradicate. The presence of necrotic soft multiply and colonize the area. There is also evidence
tissue and dead bone, together with a mixed bacterial that bacteria can survive inside osteoblasts and osteo-
flora, conspire against effective antibiotic control. cytes and be released when the cells die (Ellington et
Treatment calls for regular wound dressing and al., 2003).
repeated excision of all dead and infected tissue. These processes are evident in patients who have
Traditionally it was recommended that stable been inadequately treated (perhaps ‘too little too
implants (fixation plates and medullary nails) should late’), but in any event certain patients are at greater
be left in place until the fracture had united, and this risk than others: those who are very old or debilitated,
advice is still respected in recognition of the adage those suffering from substance abuse and those with
that even worse than an infected fracture is an diabetes, peripheral vascular disease, skin infections,
infected unstable fracture. However, advances in malnutrition, lupus erythematosus or any type of
external fixation techniques have meant that almost immune deficiency. The commonest of all predispos-
all fractures can, if necessary, be securely fixed by that ing factors is local trauma, such as an open fracture or
method, with the added advantage that the wound a prolonged bone operation, especially if this involves
remains accessible for dressings and superficial the use of a foreign implant.
debridement.
If these measures fail, the management is essentially
that of chronic osteomyelitis.
Pathology
Bone is destroyed or devitalized, either in a discrete
area around the focus of infection or more diffusely
along the surface of a foreign implant. Cavities con-
POSTOPERATIVE taining pus and pieces of dead bone (sequestra) are
surrounded by vascular tissue, and beyond that by
OSTEOMYELITIS areas of sclerosis – the result of chronic reactive new
bone formation – which may take the form of a dis-
This subject is dealt with in Chapter 12. tinct bony sheath (involucrum). In the worst cases a
sizeable length of the diaphysis may be devitalized and
encased in a thick involucrum.
Sequestra act as substrates for bacterial adhesion in
CHRONIC OSTEOMYELITIS much the same way as foreign implants, ensuring the
persistence of infection until they are removed or
This used to be the dreaded sequel to acute discharged through perforations in the involucrum
haematogenous osteomyelitis; nowadays it more fre- and sinuses that drain to the skin. A sinus may seal off
38 quently follows an open fracture or operation. for weeks or even months, giving the appearance of
2.5 Chronic 2
osteomyelitis
Chronic osteomyelitis
may follow acute. The
young boy
(a) presented with
draining sinuses at the
Infection
site of a previous acute
infection. The x-ray
shows densely sclerotic
bone. (b) In adults,
chronic osteomyelitis is
usually a sequel to
open trauma or
operation.
(a) (b)
healing, only to reopen (or appear somewhere else) with 67Ga-citrate or 111In-labelled leucocytes is said to
when the tissue tension rises. Bone destruction, and be more specific for osteomyelitis; such scans are use-
the increasingly brittle sclerosis, sometimes results in a ful for showing up hidden foci of infection.
pathological fracture. CT and MRI are invaluable in planning operative
The histological picture is one of chronic inflamma- treatment: together they will show the extent of bone
tory cell infiltration around areas of acellular bone or destruction and reactive oedema, hidden abscesses
microscopic sequestra. and sequestra.
Investigations
Clinical features
During acute flares the CSR, ESR and WBC levels
The patient presents because pain, pyrexia, redness
may be increased; these non-specific signs are helpful
and tenderness have recurred (a ‘flare’), or with a dis-
in assessing the progress of bone infection but they
charging sinus. In longstanding cases the tissues are
are not diagnostic.
thickened and often puckered or folded inwards
Organisms cultured from discharging sinuses
where a scar or sinus adheres to the underlying bone.
should be tested repeatedly for antibiotic sensitivity;
There may be a seropurulent discharge and excoria-
with time, they often change their characteristics and
tion of the surrounding skin. In post-traumatic
become resistant to treatment. Note, however, that a
osteomyelitis the bone may be deformed or un-
superficial swab sample may not reflect the really per-
united.
sistent infection in the deeper tissues; sampling from
deeper tissues is important.
The most effective antibiotic treatment can be
Imaging applied only if the pathogenic organism is identified
X-ray examination will usually show bone resorption and tested for sensitivity. Unfortunately standard bac-
– either as a patchy loss of density or as frank excava- terial cultures still give negative results in about 20%
tion around an implant – with thickening and sclero- of cases of overt infection. In recent years more
sis of the surrounding bone. However, there are sophisticated molecular techniques have been devel-
marked variations: there may be no more than local- oped, based on the amplification of bacterial DNA or
ized loss of trabeculation, or an area of osteoporosis, RNA fragments (the polymerase chain reaction or
or periosteal thickening; sequestra show up as unnat- PCR) and their subsequent identification by gel elec-
urally dense fragments, in contrast to the surrounding trophoresis. However, although this has been shown
osteopaenic bone; sometimes the bone is crudely to reveal unusual and otherwise undetected organisms
thickened and misshapen, resembling a tumour. A in a significant percentage of cases, the technique is
sinogram may help to localize the site of infection. not widely available for routine testing.
Radioisotope scintigraphy is sensitive but not spe- A range of other investigations may also be needed
cific. 99mTc-HDP scans show increased activity in both to confirm or exclude suspected systemic disorders
the perfusion phase and the bone phase. Scanning (such as diabetes) that could influence the outcome. 39
2 Staging of chronic osteomyelitis in long kept at several times the minimal bactericidal concen-
bones tration. Continuous collaboration with a specialist in
microbiology is important. If surgical clearance fails, an-
‘Staging’ the condition helps in risk–benefit assess- tibiotics should be continued for another 4 weeks be-
ment and has some predictive value concerning the fore considering another attempt at full debridement.
outcome of treatment. The system popularized by
Cierny et al. (2003) is based on both the local patho- LOCAL TREATMENT
logical anatomy and the host background (Table 2.2). A sinus may be painless and need dressing simply to
The least serious, and most likely to benefit, are protect the clothing. Colostomy paste can be used to
patients classified as Stage 1 or 2, Type A, i.e. those stop excoriation of the skin. An acute abscess may
with localized infection and free of compromising dis- need urgent incision and drainage, but this is only a
orders. Type B patients are somewhat compromised temporary measure.
by a few local or systemic factors, but if the infection
is localized and the bone still in continuity and stable OPERATION
(Stage 1–3) they have a reasonable chance of recovery. A waiting policy, punctuated by spells of bed rest and
Type C patients are so severely compromised that the antibiotics to control flares, may have to be patiently
prognosis is considered to be poor. If the lesion is also endured until there is a clear indication for radical sur-
classified as Stage 4 (e.g. intractable diffuse infection gery: for chronic haematogenous infections this means
in an ununited fracture), operative treatment may be intrusive symptoms, failure of adequate antibiotic
contraindicated and the best option may be long-term treatment, and/or clear evidence of a sequestrum or
palliative treatment. Occasionally one may have to dead bone; for post-traumatic infections, an
advise amputation. intractable wound and/or an infected ununited
fracture; for postoperative infection, similar criteria and
Table 2.2 Staging for adult chronic osteomyelitis evidence of bone erosion.
The presence of a foreign implant is a further incen-
LESION TYPE tive to operate. Traditionally it was felt that internal
Stage 1 Medullary fixation devices (plates, screws and intramedullary
Stage 2 Superficial nails) should be retained, even though infected, in
order to maintain stability. Nowadays, however, a
Stage 3 Localized
range of ingenious external fixation systems are avail-
Stage 4 Diffuse able and it is possible to immobilize almost any frac-
HOST CATEGORY ture by this method, thus bypassing the fracture and
Type A Normal allowing earlier removal of infected material at that
site.
Type B Compromised by local or systemic conditions
When undertaking operative treatment, collabora-
Type C Severely compromised by local and systemic tion with a plastic surgeon is strongly recommended.
conditions
Debridement At operation all infected soft tissue and
dead or devitalized bone, as well as any infected
Treatment implant, must be excised. After three or four days the
ANTIBIOTICS wound is inspected and if there are renewed signs of
Chronic infection is seldom eradicated by antibiotics tissue death the debridement may have to be repeated
alone. Yet bactericidal drugs are important (a) to sup- – several times if necessary. Antibiotic cover is contin-
press the infection and prevent its spread to healthy ued for at least 4 weeks after the last debridement.
bone and (b) to control acute flares. The choice of Dealing with the ‘dead space’ There are several ways of
antibiotic depends on microbiological studies, but the dealing with the resulting ‘dead space’. Porous antibi-
drug must be capable of penetrating sclerotic bone otic-impregnated beads can be laid in the cavity and
and should be non-toxic with long-term use. Fusidic left for 2 or 3 weeks and then replaced with cancellous
acid, clindamycin and the cephalosporins are good bone grafts. Bone grafts have also been used on their
examples. Vancomycin and teicoplanin are effective in own; in the Papineau technique the entire cavity is
most cases of meticillin-resistant Staphylococcus aureus packed with small cancellous chips (preferably autoge-
infection (MRSA). nous) mixed with an antibiotic and a fibrin sealant.
Antibiotics are administered for 4–6 weeks (starting Where possible, the area is covered by adjacent mus-
from the beginning of treatment or the last debride- cle and the skin wound is sutured without tension. An
ment) before considering operative treatment. During alternative approach is to employ a muscle flap trans-
this time serum antibiotic concentrations should be fer: in suitable sites a large wad of muscle, with its
40 measured at regular intervals to ensure that they are blood supply intact, can be mobilized and laid into
the cavity; the surface is later covered with a split-skin X-rays show increased bone density and cortical 2
graft. In areas with too little adjacent muscle (e.g. the thickening; in some cases the marrow cavity is com-
distal part of the leg), the same objective can be pletely obliterated. There is no abscess cavity.
achieved by transferring a myocutaneous island flap Diagnosis can be difficult. If a small segment of
on a long vascular pedicle. A free vascularized bone bone is involved, it may be mistaken for an osteoid
graft is considered to be a better option, provided the osteoma. If there is marked periosteal layering of new
Infection
site is suitable and the appropriate facilities for bone, the lesion resembles a Ewing’s sarcoma. The
microvascular surgery are available. biopsy will disclose a low-grade inflammatory lesion
A different approach is the one developed and with reactive bone formation. Micro-organisms are
refined by Lautenbach in South Africa. This involves seldom cultured but the condition is usually ascribed
radical excision of all avascular and infected tissue fol- to a staphylococcal infection.
lowed by closed irrigation and suction drainage of the Treatment is by operation: the abnormal area is
bed using double-lumen tubes and an appropriate excised and the exposed surface thoroughly curetted.
antibiotic solution in high concentration (based on Bone grafts, bone transport or free bone transfer may
microbiological tests for bacterial sensitivity). The be needed.
‘dead space’ is gradually filled by vascular granulation
tissue. The tubes are removed when cultures remain
negative in three consecutive fluid samples and the
cavity is obliterated. The technique, which has been
MULTIFOCAL NON-SUPPURATIVE
used with considerable success, is described in detail OSTEOMYELITIS
by Hashmi et al. (2004).
In refractory cases it may be possible to excise the This obscure disorder – it is not even certain that it is
infected and/or devitalized segment of bone com- an infection – was first described in isolated cases in
pletely and then close the gap by the Ilizarov method the 1960s and 70s, and later in a more comprehensive
of ‘transporting’ a viable segment from the remaining report on 20 patients of mixed age and sex (Björkstén
diaphysis. This is especially useful if infection is associ- and Boquist, 1980). It is now recognized that: (1) it
ated with an ununited fracture (see Chapter 12). is not as rare as initially suggested; (2) it comprises
several different syndromes which have certain fea-
Soft-tissue cover Last but not least, the bone must be
tures in common; and (3) there is an association with
adequately covered with skin. For small defects split-
chronic skin infection, especially pustular lesions of
thickness skin grafts may suffice; for larger wounds
the palms and soles (palmo-plantar pustulosis) and
local musculocutaneous flaps, or free vascularized
pustular psoriasis.
flaps, are needed.
In children the condition usually takes the form of
Aftercare Success is difficult to measure; a minute multifocal (often symmetrical), recurrent lesions in
focus of infection might escape the therapeutic the long-bone metaphyses, clavicles and anterior rib-
onslaught, only to flare into full-blown osteomyelitis cage; in adults the changes appear predominantly in
many years later. Prognosis should always be guarded; the sterno-costo-clavicular complex and the vertebrae.
local trauma must be avoided and any recurrence of In recent years the various syndromes have been
symptoms, however slight, should be taken seriously drawn together under the convenient acronym
and investigated. The watchword is ‘cautious opti- SAPHO – standing for synovitis, acne, pustulosis,
mism’ – a ‘probable cure’ is better than no cure at all. hyperostosis and osteitis (Boutin and Resnick, 1998).
Early osteolytic lesions show histological features
suggesting a subacute inflammatory condition; in
longstanding cases there may be bone thickening and
GARRÉ’S SCLEROSING round cell infiltration. The aetiology is unknown.
OSTEOMYELITIS Despite the local and systemic signs of inflammation,
there is no purulent discharge and micro-organisms
Garré, in 1893, described a rare form of non- have seldom been isolated.
suppurative osteomyelitis which is characterized by The two most characteristic clinical syndromes will
marked sclerosis and cortical thickening. There is no be described.
abscess, only a diffuse enlargement of the bone at the
affected site – usually the diaphysis of one of the tubu- SUBACUTE RECURRENT MULTIFOCAL
lar bones or the mandible. The patient is typically an OSTEOMYELITIS
adolescent or young adult with a long history of This appears as an inflammatory bone disorder affect-
aching and slight swelling over the bone. Occasionally ing mainly children and adolescents. Patients develop
there are recurrent attacks of more acute pain accom- recurrent attacks of pain, swelling and tenderness
panied by malaise and slight fever. around one or other of the long-bone metaphyses 41
2 (usually the distal femur or the proximal or distal clavicles, the adjacent sternum and the anterior ends
tibia), the medial ends of the clavicles or a vertebral of the upper ribs, as well as ossification of the stern-
segment. Over the course of several years multiple oclavicular and costoclavicular ligaments. Vertebral
sites are affected, sometimes symmetrically and some- changes include sclerosis of individual vertebral bod-
times simultaneously; with each exacerbation the child ies, ossification of the anterior longitudinal ligament,
is slightly feverish and may have a raised ESR. anterior intervertebral bridging, end-plate erosions,
X-ray changes are characteristic. There are small disc space narrowing and vertebral collapse.
lytic lesions in the metaphysis, usually closely adjacent Radioscintigraphy shows increased activity around the
to the physis. Some of these ‘cavities’ are surrounded sternoclavicular joints and affected vertebrae.
by sclerosis; others show varying stages of healing. The condition usually runs a protracted course with
The clavicle may become markedly thickened. If the recurrent ‘flares’. There is no effective treatment but
spine is affected, it may lead to collapse of a vertebral in the long term symptoms tend to diminish or disap-
body. Radioscintigraphy shows increased activity pear; however, the patient may be left with ankylosis
around the lesions. of the affected joints.
Biopsy of the lytic focus is likely to show the typical
histological features of acute or subacute inflamma-
tion. In longstanding lesions there is a chronic inflam-
matory reaction with lymphocyte infiltration. INFANTILE CORTICAL HYPEROSTOSIS
Bacteriological cultures are almost invariably negative. (CAFFEY’S DISEASE)
Treatment is entirely palliative; antibiotics have no
effect on the disease. Although the condition may run Infantile cortical hyperostosis is a rare disease of
a protracted course, the prognosis is good and the infants and young children. It usually starts during the
lesions eventually heal without complications. first few months of life with painful swelling over the
tubular bones and/or the mandible. The child may be
STERNO-COSTO-CLAVICULAR HYPEROSTOSIS feverish and irritable, refusing to move the affected
Patients are usually in their forties or fifties, and men limb. Infection may be suspected but, apart from the
are affected more often than women. Clinical and swelling, there are no local signs of inflammation. The
radiological changes are usually confined to the ster- ESR, though, is usually elevated.
num and adjacent bones and the vertebral column. As X-rays characteristically show periosteal new-bone
with recurrent multifocal osteomyelitis, there is a curi- formation resulting in thickening of the affected
ous association with cutaneous pustulosis. The usual bone.
complaint is of pain, swelling and tenderness around After a few months the local features may resolve
the sternoclavicular joints; sometimes there is also a spontaneously, only to reappear somewhere else. Flat
slight fever and the ESR may be elevated. Patients bones, such as the scapula and cranial vault, may also
with vertebral column involvement may develop back be affected.
pain and stiffness. Other causes of hyperostosis (osteomyelitis, scurvy)
X-rays show hyperostosis of the medial ends of the must be excluded. The cause of Caffey’s disease is
2.6 Caffey’s
disease This infant
with Caffey’s
disease developed
marked thickening
of the mandible and
long bones. The
lesions gradually
cleared up, leaving
little or no trace of
their former
ominous
appearance.
42 (a) (b) (c) (d)

unknown but a virus infection has been suggested. severely damaged; in older children, vascular occlu- 2
Antibiotics are sometimes employed; it is doubtful sion may lead to necrosis of the epiphyseal bone. In
whether they have any effect. adults the effects are usually confined to the articular
cartilage, but in late cases there may be extensive ero-
sion due to synovial proliferation and ingrowth.
ACUTE SUPPURATIVE ARTHRITIS If the infection goes untreated, it will spread to the
Infection
underlying bone or burst out of the joint to form
A joint can become infected by: (1) direct invasion abscesses and sinuses.
through a penetrating wound, intra-articular injection With healing there may be: (1) complete resolution
or arthroscopy; (2) direct spread from an adjacent and a return to normal; (2) partial loss of articular car-
bone abscess; or (3) blood spread from a distant site. tilage and fibrosis of the joint; (3) loss of articular car-
In infants it is often difficult to tell whether the infec- tilage and bony ankylosis; or (4) bone destruction and
tion started in the metaphyseal bone and spread to the permanent deformity of the joint.
joint or vice versa. In practice it hardly matters and in
advanced cases it should be assumed that the entire Clinical features
joint and the adjacent bone ends are involved.
The causal organism is usually Staphylococcus The clinical features differ somewhat according to the
aureus; however, in children between 1 and 4 years age of the patient.
old, Haemophilus influenzae is an important pathogen In new-born infants the emphasis is on septicaemia
unless they have been vaccinated against this organ- rather than joint pain. The baby is irritable and refuses
ism. Occasionally other microbes, such as Streptococ- to feed; there is a rapid pulse and sometimes a fever.
cus, Escherichia coli and Proteus, are encountered. Infection is often suspected, but it could be anywhere!
Predisposing conditions are rheumatoid arthritis, The joints should be carefully felt and moved to elicit
chronic debilitating disorders, intravenous drug the local signs of warmth, tenderness and resistance to
abuse, immunosuppressive drug therapy and acquired movement. The umbilical cord should be examined
immune deficiency syndrome (AIDS). for a source of infection. An inflamed intravenous
infusion site should always excite suspicion. The
baby’s chest, spine and abdomen should be carefully
Pathology examined to exclude other sites of infection.
The usual trigger is a haematogenous infection which Special care should be taken not to miss a concomitant
settles in the synovial membrane; there is an acute osteomyelitis in an adjacent bone end.
inflammatory reaction with a serous or seropurulent In children the usual features are acute pain in a sin-
exudate and an increase in synovial fluid. As pus gle large joint (commonly the hip or the knee) and
appears in the joint, articular cartilage is eroded and reluctance to move the limb (‘pseudoparesis’). The
destroyed, partly by bacterial enzymes and partly by child is ill, with a rapid pulse and a swinging fever. The
proteolytic enzymes released from synovial cells, overlying skin looks red and in a superficial joint
inflammatory cells and pus. In infants the entire epi- swelling may be obvious. There is local warmth and
physis, which is still largely cartilaginous, may be marked tenderness. All movements are restricted, and
(a) (b) (c) (d)
2.7 Acute suppurative arthritis – pathology In the early stage (a), there is an acute synovitis with a purulent joint
effusion. (b) Soon the articular cartilage is attacked by bacterial and cellular enzymes. If the infection is not arrested, the
cartilage may be completely destroyed (c). Healing then leads to bony ankylosis (d). 43
2 Imaging
Ultrasonography is the most reliable method for
revealing a joint effusion in early cases. Both hips
should be examined for comparison. Widening of the
space between capsule and bone of more than 2 mm
is indicative of an effusion, which may be echo-free

(perhaps a transient synovitis) or positively echogenic
(more likely septic arthritis).
X-ray examination is usually normal early on but
signs to be watched for are soft-tissue swelling, loss of
tissue planes, widening of the radiographic ‘joint
(a) space’ and slight subluxation (because of fluid in the
joint). With E. coli infections there is sometimes gas in
the joint. Narrowing and irregularity of the joint
space are late features.
MRI and radionuclide imaging are helpful in diag-
nosing arthritis in obscure sites such as the sacroiliac
and sternoclavicular joints.
Investigations
The white cell count and ESR are raised and blood cul-
ture may be positive. However, special investigations
take time and it is much quicker (and usually more re-
liable) to aspirate the joint and examine the fluid. It may
be frankly purulent but beware! – in early cases the fluid
(b)
2.8 Suppurative may look clear. A white cell count and Gram stain
arthritis – x-ray should be carried out immediately: the normal synovial
(a) In this child the left fluid leucocyte count is under 300 per mL; it may be
hip is subluxated and over 10 000 per mL in non-infective inflammatory dis-
the soft tissues are orders, but counts of over 50 000 per mL are highly
swollen. (b) If the
infection persists suggestive of sepsis. Gram-positive cocci are probably
untreated, the S. aureus; Gram-negative cocci are either H. influenzae
cartilaginous epiphysis or Kingella kingae (in children) or Gonococcus (in
may be entirely adults). Samples of fluid are also sent for full micro-
destroyed, leaving a biological examination and tests for antibiotic
permanent
pseudarthrosis. sensitivity.
(c) Septic arthritis in
an adult knee joint.
(c) Differential diagnosis
Acute osteomyelitis In young children, osteomyelitis
may be indistinguishable from septic arthritis; often
often completely abolished, by pain and spasm. It is one must assume that both are present.
essential to look for a source of infection – a septic Other types of infection Psoas abscess and local infection
toe, a boil or a discharge from the ear. of the pelvis must be kept in mind. Systemic features
In adults it is often a superficial joint (knee, wrist, a will obviously be the same as those of septic arthritis.
finger, ankle or toe) that is painful, swollen and
Trauma Traumatic synovitis or haemarthrosis may be
inflamed. There is warmth and marked local tender-
associated with acute pain and swelling. A history of
ness, and movements are restricted. The patient
injury does not exclude infection. Diagnosis may
should be questioned and examined for evidence of
remain in doubt until the joint is aspirated.
gonococcal infection or drug abuse. Patients with
rheumatoid arthritis, and especially those on corticos- Irritable joint At the onset the joint is painful and lacks
teroid treatment, may develop a ‘silent’ joint infec- some movement, but the child is not really ill and
tion. Suspicion may be aroused by an unexplained there are no signs of infection. Ultrasonography may
deterioration in the patient’s general condition; every help to distinguish septic arthritis from transient
44 joint should be carefully examined. synovitis.
Haemophilic bleed An acute haemarthrosis closely nase-resistant penicillins (e.g. flucloxacillin) plus a 2
resembles septic arthritis. The history is usually con- third-generation cephalosporin.
clusive, but aspiration will resolve any doubt. Children from 6 months to puberty can be treated
Rheumatic fever Typically the pain flits from joint to
similarly. Unless they had been immunized there is a
joint, but at the onset one joint may be misleadingly risk of Haemophilus infection.
inflamed. However, there are no signs of septicaemia. Older teenagers and adults can be started on flu-
Infection
cloxacillin and fusidic acid. If the initial examination
Juvenile rheumatoid arthritis This may start with pain shows Gram-negative organisms a third-generation
and swelling of a single joint, but the onset is usually cephalosporin is added. More appropriate drugs can
more gradual and systemic symptoms less severe than be substituted after full microbiological investigation.
in septic arthritis. Antibiotics should be given intravenously for 4–7
Sickle-cell disease The clinical picture may closely days and then orally for another 3 weeks.
resemble that of septic arthritis – and indeed the bone
nearby may actually be infected! – so this condition DRAINAGE
should always be excluded in communities where the Under anaesthesia the joint is opened through a small
disease is common. incision, drained and washed out with physiological
saline. A small catheter is left in place and the wound
Gaucher’s disease In this rare condition acute joint is closed; suction–irrigation is continued for another 2
pain and fever can occur without any organism being or 3 days. This is the safest policy and is certainly
found (‘pseudo-osteitis’). Because of the predisposi- advisable (1) in very young infants, (2) when the hip
tion to true infection, antibiotics should be given. is involved and (3) if the aspirated pus is very thick.
Gout and pseudogout In adults, acute crystal-induced For the knee, arthroscopic debridement and copious
synovitis may closely resemble infection. On aspira- irrigation may be equally effective. Older children
tion the joint fluid is often turbid, with a high white with early septic arthritis (symptoms for less than 3
cell count; however, microscopic examination by days) involving any joint except the hip can often be
polarized light will show the characteristic crystals. treated successfully by repeated closed aspiration of
the joint; however, if there is no improvement within
48 hours, open drainage will be necessary.
Treatment AFTERCARE
The first priority is to aspirate the joint and examine the Once the patient’s general condition is satisfactory
fluid. Treatment is then started without further delay and the joint is no longer painful or warm, further
and follows the same lines as for acute osteomyelitis. damage is unlikely. If articular cartilage has been pre-
Once the blood and tissue samples have been served, gentle and gradually increasing active move-
obtained, there is no need to wait for detailed results ments are encouraged. If articular cartilage has been
before giving antibiotics. If the aspirate looks puru- destroyed the aim is to keep the joint immobile while
lent, the joint should be drained without waiting for ankylosis is awaited. Splintage in the optimum posi-
laboratory results (see below). tion is therefore continuously maintained, usually by
plaster, until ankylosis is sound.
GENERAL SUPPORTIVE CARE
Analgesics are given for pain and intravenous fluids for
dehydration.
Complications
Infants under 6 months of age have the highest inci-
SPLINTAGE dence of complications, most of which affect the hip.
The joint should be rested, and for neonates and The most obvious risk factors are a delay in diagnosis
infants this may mean light splintage; with hip infec- and treatment (more than 4 days) and concomitant
tion, the joint should be held abducted and 30 degrees osteomyelitis of the proximal femur.
flexed, on traction to prevent dislocation. Subluxation and dislocation of the hip, or instability
of the knee should be prevented by appropriate pos-
ANTIBIOTICS turing or splintage.
Antibiotic treatment follows the same guidelines as Damage to the cartilaginous physis or the epiphysis in
presented for acute haematogenous osteomyelitis (see the growing child is the most serious complication.
page 35). The initial choice of antibiotics is based on Sequelae include retarded growth, partial or complete
judgement of the most likely pathogens. destruction of the epiphysis, deformity of the joint, epi-
Neonates and infants up to the age of 6 months physeal osteonecrosis, acetabular dysplasia and
should be protected against staphylococcus and pseudarthrosis of the hip.
Gram-negative streptococci with one of the penicilli- Articular cartilage erosion (chondrolysis) is seen in 45
2 older patients and this may result in restricted move- haemophiliacs and other patients with AIDS. The
ment or complete ankylosis of the joint. usual organisms are Staphylococcus aureus and Strepto-
coccus; however, opportunistic infection by unusual
organisms is not uncommon.
GONOCOCCAL ARTHRITIS The patient may present with an acutely painful,
inflamed joint and marked systemic features of bacter-
Neisseria gonorrhoeae is the commonest cause of sep- aemia or septicaemia. In some cases the infection is
tic arthritis in sexually active adults, especially among confined to a single, unusual site such as the sacroiliac
poorer populations. Even in affluent communities the joint; in others several joints may be affected simulta-
incidence of sexually transmitted diseases has neously. Opportunistic infection by unusual organ-
increased (probably related to the increased use of isms may produce a more indolent clinical picture.
non-barrier contraception) and with it the risk of Treatment follows the general principles outlined
gonococcal and syphilitic bone and joint diseases and before. Patients with staphylococcal and streptococcal
their sequelae. The infection is acquired only by direct infections usually respond well to antibiotic treatment
mucosal contact with an infected person – carrying a and joint drainage; opportunistic infections may be
risk of greater than 50% after a single contact! more difficult to control.
Clinical features
Two types of clinical disorder are recognized: (a) dis- SPIROCHAETAL INFECTION
seminated gonococcal infection – a triad of polyarthritis,
tenosynovitis and dermatitis – and (b) septic arthritis of
Two conditions which are likely to be encountered by
a single joint (usually the knee, ankle, shoulder, wrist
the orthopaedic surgeon are dealt with here: syphilis
or hand). Both syndromes may occur in the same
and yaws. Lyme disease, which also originates with a
patient. There may be a slight pyrexia and the ESR and
spirochaetal infection, is better regarded as due to a
WBC count will be raised. If the condition is sus-
systemic autoimmune response and is dealt with in
pected, the patient should be questioned about poss-
Chapter 3.
ible contacts during the previous days or weeks and
they should be examined for other signs of genitouri-
nary infection (e.g. a urethral discharge or cervicitis).
Joint aspiration may reveal a high white cell count SYPHILIS
and typical Gram-negative organisms, but bacterio-
logical investigations are often disappointing. Samples Syphilis is caused by the spirochaete Treponema pal-
should also be taken from the various mucosal sur- lidum, generally acquired during sexual activity by
faces and tests should be performed for other sexually direct contact with infectious lesions of the skin or
transmitted infections. mucous membranes. The infection spreads to the
regional lymph nodes and thence to the blood stream.
The organism can also cross the placental barrier and
Treatment enter the foetal blood stream directly during the latter
Treatment is similar to that of other types of pyogenic half of pregnancy, giving rise to congenital syphilis.
arthritis. Patients will usually respond fairly quickly to In acquired syphilis a primary ulcerous lesion, or
a third-generation cephalosporin given intravenously chancre, appears at the site of inoculation about a
or intramuscularly. However, bear in mind that many month after initial infection. This usually heals with-
patients with gonococcal infection also have chlamy- out treatment but, a month or more after that, the
dial infection, which is resistant to cephalosporins; disease enters a secondary phase characterized by the
both are sensitive to quinolone antibiotics such as appearance of a maculopapular rash and bone and
ciprofloxacin and ofloxacin. If the organism is found joint changes due to periostitis, osteitis and osteo-
to be sensitive to penicillin (and the patient is not chondritis. After a variable length of time, this phase
allergic), treatment with ampicillin or amoxicillin and is followed by a latent period which may continue for
clavulanic acid is also effective. many years. The term is somewhat deceptive because
in about half the cases pathological lesions continue to
appear in various organs and 10–30 years later the
patient may present again with tertiary syphilis, which
SEPTIC ARTHRITIS AND HIV-1 takes various forms including the appearance of large
INFECTION granulomatous gummata in bones and joints and neu-
ropathic disorders in which the loss of sensibility gives
Septic arthritis has been encountered quite frequently rise to joint breakdown (Charcot joints).
46 in HIV-positive intravenous drug users, HIV-positive In congenital syphilis the primary infection may be
so severe that the foetus is either still-born or the (GPI). With modern treatment these late sequelae 2
infant dies shortly after birth. The ones who survive have become rare.
manifest pathological changes similar to those
described above, though with modified clinical
Clinical features of congenital syphilis
appearances and a contracted timescale.
Early congenital syphilis Although the infection is pres-
Infection
ent at birth, bone changes do not usually appear until
several weeks afterwards (Rasool and Govender,
Clinical features of acquired syphilis 1989). The baby is sick and irritable and examination
Early features The patient usually presents with pain, may show skin lesions, hepatosplenomegaly and
swelling and tenderness of the bones, especially those anaemia. Serological tests are usually positive in both
with little soft-tissue covering, such as the frontal mother and child.
bones of the skull, the anterior surface of the tibia, the The first signs of skeletal involvement may be joint
sternum and the ribs. X-rays may show typical features swelling and ‘pseudoparalysis’ – the child refuses to
of periostitis and thickening of the cortex in these move a painful limb. Several sites may be involved,
bones, as well as others that are not necessarily symp- often symmetrically, with slight swelling and tender-
tomatic. Osteitis and septic arthritis are less common. ness at the ends or along the shafts of the tubular
Occasionally these patients develop polyarthralgia or bones. The characteristic X-ray changes are of two
polyarthritis. Enquiry may reveal a history of sexually kinds: osteochondritis (‘metaphysitis’) – trabecular ero-
transmitted disease. sion in the juxta-epiphyseal regions of tubular bones
showing first as a lucent band near the physis and later
Late features The typical late feature, which may
as frank bone destruction which may result in epiphy-
appear only after many years, is the syphilitic gumma,
seal separation; and, less frequently, periostitis – diffuse
a dense granulomatous lesion associated with local
periosteal new bone formation along the diaphysis,
bone resorption and adjacent areas of sclerosis. Some-
usually of mild degree but sometimes producing an
times this results in a pathological fracture. X-rays
‘onion-peel’ effect. The condition must be distin-
may show thick periosteal new bone formation at
guished from scurvy (rare in the first 6 months of life),
other sites, especially the tibia.
multifocal osteomyelitis, the battered baby syndrome
The other well-recognized feature of tertiary
and Caffey’s disease (see page 42).
syphilis is a neuropathic arthropathy due to loss of
sensibility in the joint – most characteristically the Late congenital syphilis Bone lesions in older children
knee (see page 98). and adolescents resemble those of acquired syphilis
Other neurological disorders, the early signs of and some features occurring 10 or 15 years after birth
which may only be discovered on careful examination, may be manifestations of tertiary disease, the result of
are tabes dorsalis and ‘general paralysis of the insane’ gumma formation and endarteritis. Gummata appear
2.9 Syphilis (a–c) Congenital

syphilis, with diffuse periostitis of
many bones. (d) Acquired syphilitic
periostitis of the tibia.
(b)
(a) (c) (d) 47

2 either as discrete, punched-out radiolucent areas in Treatment
the medulla or as more extensive destructive lesions in
the cortex. The surrounding bone is thick and scle- Treatment with benzylpenicillin, preferably given by
rotic. Sometimes the predominant feature is dense intramuscular injection, is effective. For those who are
endosteal and periosteal new bone formation affecting hypersensitive to penicillin, erythromycin is a satisfac-
almost the entire bone (the classic ‘sabre tibia’). tory alternative.
Other abnormalities which have come to be

regarded as ‘classic’ features in older children are den-
tal malformations (‘Hutchinson’s teeth’), erosion of
the nasal bones, thickening and expansion of the fin-
TROPICAL ULCER
ger phalanges (dactylitis) and painless effusions in the
knees or elbows (‘Clutton’s joints’). Tropical ulcer, though the name sounds vague and
non-specific, is a distinct entity that is seen frequently
in tropical and subtropical regions, particularly in parts
Treatment of Africa, where people walk bare-legged through
Early lesions will usually respond to intramuscular rough terrain or long grass. It almost always occurs on
injections of benzylpenicillin given weekly for 3 or 4 the leg and men make up the majority of patients
doses. Late lesions will require high-dosage intra- (probably because they are out and about more often
venous penicillin for a week or 10 days, but some than women). The initial lesion is a small split in the
forms of tertiary syphilis will not respond at all. An skin (a cut, thorn-scratch, insect bite or other minor
alternative would be treatment with one of the third- abrasion), which is then contaminated with all kinds of
generation cephalosporins. dirt or stagnant water. The most likely infecting or-
ganisms are Fusiformis fusiformis and Borrelia vincen-
tii (both common in faeces). This results in an indolent
ulcer which defies most forms of topical treatment
YAWS (and certainly traditional remedies native to those parts
Yaws is a non-venereal spirochaetal infection caused of the world). The ulcer may eventually bore its way
by Treponema pertenue. It is seen mainly in the poorer into the soft tissues and the underlying bone; occa-
tropical parts of Africa, Asia and South America.
Though considered – at least in Europe – to be a
‘rare’ disease, several thousand cases a year are
reported in Indonesia.
The infection is contracted by skin-to-skin contact. A
knobbly ulcer covered by a scab (the primary or ‘mother’
yaw), usually develops on the face, hands or feet. Sec-
ondary skin lesions appear 1–4 months later and suc-
cessive lesions may go on to pustular ulceration; as each
one heals it leaves a pale tell-tale scar. This secondary
stage is followed by a long latent period, merging into a
tertiary stage during which skeletal changes similar to
those of syphilis develop – periosteal new bone forma-
tion, cortical destruction and osteochondritis.
Clinical features
Children under 10 years old are the usual victims. In
areas where the disease is endemic the typical skin
lesions and an associated lymphadenopathy are
quickly recognized. Elsewhere further investigations
may be called for – serological tests and dark-field
examination of scrapings from one of the skin lesions.
At a later stage deformities and bone tenderness
may become apparent. X-rays show features such as
cortical erosion, joint destruction and periosteal new (a) (b)
bone formation; occasionally thickening of a long 2.10 Tropical ulcer What started as a small ulcer has
bone may be so marked as to resemble the ‘sabre turned into a large spreading lesion. The x-ray shows the
48 tibia’ of late congenital syphilis. typical marked periosteal reaction in the underlying bone.
sionally, after many years, it gives rise to a locally inva- Late cases of ulceration will require painstaking 2
sive squamous-cell carcinoma. cleansing and de-sloughing together with broad-spec-
trum antibiotics effective against the causative anaer-
obic Gram-negative organisms as well as secondary
Clinical features
infecting microbes cultured from swab samples. Soft-
What starts as a small inflamed scratch or cut develops tissue and bone destruction may be severe enough to
Infection
over a few days into a large pustule. By the time the require extensive debridement and skin-grafting.
patient attends for medical treatment the pustule has Occasionally amputation is the best option.
usually ruptured, leaving a foul-smelling, discharging
ulcer with hard rolled edges on the leg, the ankle or
foot. In some cases the ulcer has already started to
spread and after 4–6 weeks it may be several centime- TUBERCULOSIS
tres in diameter! Two or three adjacent ulcers may
join up to form a large sloughing mass that erodes Once common throughout the world, tuberculosis
tendons, ligaments and the underlying bone. Even if showed a steady decline in its prevalence in developed
the bone is not directly involved, x-ray examination countries during the latter half of the twentieth cen-
may show a marked periosteal reaction to the overly- tury, due mainly to the effectiveness of public health
ing infection. With time that segment of the bone programmes, a general improvement in nutritional
may become thickened and sclerotic, or there may be status and advances in chemotherapy. In the last two
erosion of the cortex. With healing, soft-tissue scar- decades, however, the annual incidence (particularly
ring sometimes causes joint contractures at the knee, of extrapulmonary tuberculosis) has risen again, a
the ankle or the foot. phenomenon which has been attributed variously to a
Occasionally an invasive squamous cell carcinoma general increase in the proportion of elderly people,
develops in a chronic ulcer. changes in population movements, the spread of
intravenous drug abuse and the emergence of AIDS.
The skeletal manifestations of the disease are seen
Treatment chiefly in the spine and the large joints, but the infec-
‘Prevention is better than cure.’ For people living or tion may appear in any bone or any synovial or bursal
working in the tropics, the chance of infection can be sheath. Predisposing conditions include chronic debil-
reduced by wearing shoes and any type of covering for itating disorders, diabetes, drug abuse, prolonged cor-
the legs. Scratches and abrasions should be cleaned ticosteroid medication, AIDS and other disorders
and kept clean until they heal. resulting in reduced defence mechanisms.
Early cases of tropical ulcer may respond to ben-
zylpenicillin or erythromycin given daily for a week. If
this is not effective, a broad-spectrum antibiotic will
Pathology
be needed (e.g. a third-generation cephalosporin). Mycobacterium tuberculosis (usually human, some-
Ulcers should be cleansed every day and kept covered times bovine) enters the body via the lung (droplet
with moist or non-adherent dressings. Topical treat- infection) or the gut (swallowing infected milk
ment with metronidazole gel is advisable. products) or, rarely, through the skin. In contrast to
(a) (b) (c) (d)

2.11 Tuberculous arthritis – pathology The disease may begin as synovitis (a) or osteomyelitis (b). From either, it can
extend to become a true arthritis (c); not all the cartilage is destroyed, and healing is usually by fibrous ankylosis (d). 49
2 may occur, giving rise to miliary tuberculosis, menin-
gitis or multiple tuberculous lesions. More often,
blood spread occurs months or years later, perhaps
during a period of lowered immunity, and bacilli are
deposited in extrapulmonary tissues. Some of these
foci develop into destructive lesions to which the term
‘tertiary’ may be applied.

Tertiary lesion Bones or joints are affected in about 5
per cent of patients with tuberculosis. There is a
predilection for the vertebral bodies and the large syn-
ovial joints. Multiple lesions occur in about one-third
of patients. In established cases it is difficult to tell
whether the infection started in the joint and then
2.12 Tuberculosis – histology A typical tuberculous spread to the adjacent bone or vice versa; synovial
granuloma, with central necrosis and scattered giant cells membrane and subchondral bone have a common
surrounded by lymphocytes and histiocytes. blood supply and they may, of course, be infected
simultaneously.
Once the bacilli have gained a foothold, they elicit
pyogenic infection, it causes a granulomatous reaction a chronic inflammatory reaction. The characteristic
which is associated with tissue necrosis and caseation. microscopic lesion is the tuberculous granuloma (or
‘tubercle’) – a collection of epithelioid and multinu-
Primary complex The initial lesion in lung, pharynx or cleated giant cells surrounding an area of necrosis,
gut is a small one with lymphatic spread to regional with round cells (mainly lymphocytes) around the
lymph nodes; this combination is the primary complex. periphery.
Usually the bacilli are fixed in the nodes and no clinical Within the affected area, small patches of caseous
illness results, but occasionally the response is excessive, necrosis appear. These may coalesce into a larger yel-
with enlargement of glands in the neck or abdomen. lowish mass, or the centre may break down to form an
Even though there is often no clinical illness, the abscess containing pus and fragments of necrotic bone.
initial infection has two important sequels: (1) within Bone lesions tend to spread quite rapidly. Epiphy-
nodes which are apparently healed or even calcified, seal cartilage is no barrier to invasion and soon the
bacilli may survive for many years, so that a reservoir infection reaches the joint. Only in the vertebral bod-
exists; (2) the body has been sensitized to the toxin (a ies, and more rarely in the greater trochanter of the
positive Heaf test being an index of sensitization) and, femur or the metatarsals and metacarpals, does the
should reinfection occur, the response is quite differ- infection persist as a pure chronic osteomyelitis.
ent, the lesion being a destructive one which spreads If the synovium is involved, it becomes thick and
by contiguity. oedematous, giving rise to a marked effusion. A pan-
Secondary spread If resistance to the original infection nus of granulation tissue may extend from the syn-
is low, widespread dissemination via the blood stream ovial reflections across the joint; articular cartilage is
(a) (b) (c)
2.13 Tuberculosis – clinical and x-ray features (a) Generalized wasting used to be a common feature of all forms of
tuberculosis. Nowadays, skeletal tuberculosis occurs in deceptively healthy-looking individuals. An early feature is
peri-articular osteoporosis due to synovitis – the left knee in (b). This often resolves with treatment, but if cartilage and
50 bone are destroyed (c), healing occurs by fibrosis and the joint retains a ‘jog’ of painful movement.
slowly destroyed, though the rapid and complete erosion of the subarticular bone; characteristically this 2
destruction elicited by pyogenic organisms does not is seen on both sides of the joint, indicating an inflam-
occur in the absence of secondary infection. At the matory process starting in the synovium. Cystic
edges of the joint, along the synovial reflections, there lesions may appear in the adjacent bone ends but
may be active bone erosion. In addition, the increased there is little or no periosteal reaction. In the spine the
vascularity causes local osteoporosis. characteristic appearance is one of bone erosion and
Infection
If unchecked, caseation and infection extend into collapse around a diminished intervertebral disc space;
the surrounding soft tissues to produce a ‘cold’ the soft-tissue shadows may define a paravertebral
abscess (‘cold’ only in comparison to a pyogenic abscess.
abscess). This may burst through the skin, forming a
sinus or tuberculous ulcer, or it may track along the
tissue planes to point at some distant site. Secondary Investigations
infection by pyogenic organisms is common. If the
The ESR is usually increased and there may be a rela-
disease is arrested at an early stage, healing may be by
tive lymphocytosis. The Mantoux or Heaf test will be
resolution to apparent normality. If articular cartilage
positive: these are sensitive but not specific tests; i.e. a
has been severely damaged, healing is by fibrosis and
negative Mantoux virtually excludes the diagnosis,
incomplete ankylosis, with progressive joint defor-
but a positive test merely indicates tuberculous infec-
mity. Within the fibrocaseous mass, mycobacteria may
tion, now or at some time in the past.
remain imprisoned, retaining the potential to flare up
If synovial fluid is aspirated, it may be cloudy, the
into active disease many years later.
protein concentration is increased and the white cell
count is elevated.
Clinical features Acid-fast bacilli are identified in synovial fluid in
10–20 per cent of cases, and cultures are positive in
There may be a history of previous infection or recent
over half. A synovial biopsy is more reliable: sections
contact with tuberculosis. The patient, usually a child
will show the characteristic histological features and
or young adult, complains of pain and (in a superficial
acid-fast bacilli may be identified; cultures are positive
joint) swelling. In advanced cases there may be attacks
in about 80 per cent of patients who have not received
of fever, night sweats, lassitude and loss of weight.
antimicrobial treatment.
Relatives tell of ‘night cries’: the joint, splinted by
muscle spasm during the waking hours, relaxes with
sleep and the inflamed or damaged tissues are
stretched or compressed, causing sudden episodes of
Diagnosis
intense pain. Muscle wasting is characteristic and syn- Except in areas where tuberculosis is common, diag-
ovial thickening is often striking. Regional lymph nosis is often delayed simply because the disease is not
nodes may be enlarged and tender. Movements are suspected. Features that should trigger more active
limited in all directions. As articular erosion pro- investigation are:
gresses the joint becomes stiff and deformed.
• a long history of pain or swelling
In tuberculosis of the spine, pain may be decep-
• involvement of only one joint
tively slight – often no more than an ache when the
• marked synovial thickening
spine is jarred. Consequently the patient may not
• severe muscle wasting
present until there is a visible abscess (usually in the
• enlarged and matted regional lymph nodes
groin or the lumbar region to one side of the midline)
• periarticular osteoporosis on x-ray
or until collapse causes a localized kyphosis. Occa-
• a positive Mantoux test.
sionally the presenting feature is weakness or instabil-
ity in the lower limbs. Synovial biopsy for histological examination and cul-
Multiple foci of infection are sometimes found, ture is often necessary. Joint tuberculosis must be dif-
with bone and joint lesions at different stages of ferentiated from the following.
development. This is more likely in people with low-
Transient synovitis This is fairly common in children.
ered resistance.
At first it seems no different from any other low-grade
inflammatory arthritis; however, it always settles down
X-ray after a few weeks’ rest in bed. If the synovitis recurs,
further investigation (even a biopsy) may be necessary.
Soft-tissue swelling and peri-articular osteoporosis are
characteristic. The bone ends take on a ‘washed-out’ Monarticular rheumatoid arthritis Occasionally rheuma-
appearance and the articular space is narrowed. In toid arthritis starts in a single large joint. This is clinically
children the epiphyses may be enlarged, probably the indistinguishable from tuberculosis and the diagnosis
result of long-continued hyperaemia. Later on there is may have to await the results of synovial biopsy. 51
2 Subacute arthritis Diseases such as amoebic dysentery dosage may have to be adjusted and modified,
or brucellosis are sometimes complicated by arthritis. depending on the individual patient’s age, size, gen-
The history, clinical features and pathological investi- eral health and drug reactions.
gations usually enable a diagnosis to be made.
OPERATION
Haemorrhagic arthritis The physical signs of blood in a
Operative drainage or clearance of a tuberculous focus
joint may resemble those of tuberculous arthritis. If

is seldom necessary nowadays. However, a cold
the bleeding has followed a single recent injury, the
abscess may need immediate aspiration or draining.
history and absence of marked wasting are diagnostic.
Once the condition is controlled and arthritis has
Following repeated bleeding, as in haemophilia, the
completely subsided, normal activity can be resumed,
clinical resemblance to tuberculosis is closer, but there
though the patient must report any renewed symp-
is also a history of bleeding elsewhere.
toms. If, however, the joint is painful and the articu-
Pyogenic arthritis In longstanding cases it may be dif- lar surface is destroyed, arthrodesis or replacement
ficult to exclude an old septic arthritis. arthroplasty may be considered. The longer the
period of quiescence, the less the risk of reactivation
of the disease; there is always some risk and it is essen-
Treatment
tial to give chemotherapy for 3 months before and
REST after the operation.
Hugh Owen Thomas long ago urged that tuberculo-
sis should be treated by rest – which had to be ‘pro-
longed, uninterrupted, rigid and enforced’. This often
involved splintage of the joint and traction to over- BRUCELLOSIS
come muscle spasm and prevent collapse of the artic-
ular surfaces. With modern chemotherapy this is no Brucellosis is an unusual but nonetheless important
longer mandatory; rest and splintage are varied cause of subacute or chronic granulomatous infection
according to the needs of the individual patient. in bones and joints. Three species of organism are
Those who are diagnosed and treated early are kept in seen in humans: Brucella melitensis, B. abortus (from
bed only until pain and systemic symptoms subside, cattle) and B. suis (from pigs). Infection usually occurs
and thereafter are allowed restricted activity until the from drinking unpasteurized milk or from coming
joint changes resolve (usually 6 months to a year). into contact with infected meat (e.g. among farmers
Those with progressive joint destruction may need a and meat packers). In the past it has been more com-
longer period of rest and splintage to prevent ankylo- mon in countries around the Mediterranean and in
sis in a bad position; however, as soon as symptoms certain parts of Africa and India. About 50 per cent of
permit, movements are again encouraged. patients with chronic brucellosis develop arthritis.
CHEMOTHERAPY
The most effective treatment is a combination of anti-
Pathology
tuberculous drugs, which should always include The organism enters the body with infected milk pro-
rifampicin and isoniazid. During the last decade the ducts or, occasionally, directly through the skin or
incidence of drug resistance has increased and this has mucosal surfaces. It is taken up by the lymphatics and
led to the addition of various ‘potentiating’ drugs to then carried by the blood stream to distant sites. Foci
the list. The following is one of several recommended of infection may occur in bones (usually the vertebral
regimens. bodies) or in the synovium of the larger joints. The
Initial, ‘intensive phase treatment’, consists of isoni- characteristic lesion is a chronic inflammatory granu-
azid 300–400 mg, rifampicin 450–600 mg and fluoro- loma with round-cell infiltration and giant cells. There
quinolones 400–600 mg daily for 5 or 6 months. All may be central necrosis and caseation leading to abscess
replicating sensitive bacteria are likely to be killed by formation and invasion of the surrounding tissues.
this bactericidal attack. This is followed by a ‘continu-
ation phase treatment’ lasting 9 months, the purpose of
which is to eliminate the ‘persisters’, slow-growing,
Clinical features
intermittently-growing, dormant or intracellular The patient usually presents with fever, headache and
mycobacteria. This involves the use of isoniazid and generalized weakness, followed by joint pains and back-
pyrazinamide 1500 mg per day for 4½ months and iso- ache. The initial illness may be acute and alarming;
niazid and rifampicin for another 4½ months. Then a more often it begins insidiously and progresses until the
‘prophylactic phase’, consisting of isoniazid and etham- symptoms localize in a single large joint (usually the hip
butol 1200 mg per day for a further 3 or 4 months. or knee) or in the spine. The joint becomes painful,
52 During the entire treatment period drugs and swollen and tender; movements are restricted in all
directions. If the spine is affected, there is usually local peripheral nerves, the skin and the mucosa of the 2
tenderness and back movements are restricted. upper respiratory tract.
The systemic illness follows a fluctuating course, Leprosy was once common throughout the world.
with alternating periods of fever and apparent Today it is rarely seen outside parts of South Asia,
improvement (hence the older term ‘undulant fever’). Africa, Latin America and some of the Pacific Islands.
Diagnosis is often long delayed and may not be While the disease is easily cured with drugs, its crip-
Infection
resolved until destructive changes are advanced. pling effects persist in a cumulative number of people.
The infection is acquired mainly by respiratory
transmission; unbroken skin to skin contact is thought
X-rays
not to be dangerous. Several years may elapse before
The picture is that of a subacute arthritis, with loss of clinical features appear.
articular space, slowly progressive bone erosion and
peri-articular osteoporosis. In the spine there may be
Pathology
destruction and collapse of adjacent vertebral bodies
with obliteration of the disc. Most people infected with M. leprae develop protec-
tive immunity and get rid of the infection. Some
develop a few skin lesions, appearing as vague
Investigations hypopigmented macules (indeterminate leprosy), that
A positive agglutination test (titre above 1/80) is recover spontaneously. If the condition progresses, it
diagnostic. Joint aspiration or biopsy may allow the takes one of several forms, depending on the host’s
organism to be cultured and identified. immune response.
Tuberculoid leprosy occurs where there is delayed
type hypersensitivity (DTH) to M. leprae antigens,
Diagnosis combined with some decrease in cell-mediated immu-
Diagnosis is usually delayed while other types of sub- nity (CMI). The granuloma in tuberculoid leprosy is
acute arthritis are excluded. focal and circumscribed and is made up of epithelioid
Tuberculosis and brucellosis have similar clinical and cells, with a few scattered giant cells and a cuff of lym-
radiological features. The distinction is often difficult phocytes, very similar to tuberculosis.
and may have to await the results of agglutination Lepromatous leprosy is seen in patients who are
tests, synovial biopsy and bacteriological investiga- unable to mount effective CMI against M. leprae.
tion. Here the granuloma is diffuse and extensive and it
Reiter’s disease and other forms of reactive arthritis consists of macrophages, many loaded with acid-fast
often follow an initial systemic illness. However, fever bacilli. There may be a sprinkling of round cells in the
is not so marked and joint erosion is usually late and lepromatous granuloma. The entire body skin may
mild. thus be affected.
Borderline types are intermediate forms that show
some features of both of the above conditions. With-
Treatment out treatment, they tend to progress increasingly
Antibiotics The infection usually responds to a com- towards the lepromatous form.
bined onslaught with tetracycline and streptomycin Peripheral nerves are always affected in leprosy.
for 3–4 weeks. Alternative drugs, which are equally Dermal nerve twigs, cutaneous nerves as well as major
effective and which may be used as ‘combination ther- nerve trunks may thus be involved. The affected
apy’, are rifampicin and the newer cephalosporins. nerves become thickened. Besides the granuloma
there is hypertrophy of the epineurium and per-
Operation An abscess will need drainage, and necrotic
ineurium, demyelination, axonal degeneration and
bone and cartilage should be meticulously excised. If
endoneurial fibrosis. A thickened nerve trunk may be
the joint is destroyed, arthrodesis or arthroplasty may
strangulated by its own sheath or by the rigid walls of
be necessary once the infection is completely
a fibro-osseous tunnel through which it passes (e.g.
controlled.
the ulnar nerve at the elbow). Sometimes, a tubercu-
loid granuloma in a nerve undergoes caseation. An
important factor contributing to nerve damage is that
medication is less likely to reach the segment of the
LEPROSY nerve thus rendered ischaemic.
The chronic course of leprosy is often punctuated
Leprosy is a mildly infectious chronic inflammatory by acute inflammatory episodes – so-called ‘reactions’
disease caused by acid-fast Mycobacterium leprae. It – which are due to the deposition of immune com-
is characterized by granulomatous lesions in the plexes (erythema nodosum leprosum or ENL or Type 53
2
(a) (b)
2.14 Leprosy – late features (a) Patient showing typical ulnar claw-hand deformity. (b) This patient was even worse off,
having lost all the fingers of both hands.
II reaction) or due to an increase in CMI and DTH Nerve lesions in tuberculoid leprosy may undergo
levels (reversal reaction or RR or Type I reaction). caseation and liquefaction resulting in an intraneural
Reactions occurring in the nerves (acute neuritis) ‘cold abscess’ mimicking an intraneural tumour, or
greatly increase the risk of nerve damage. the pus may break through the epineurium to present
as a chronic collar-stud abscess.
Clinical features
Diagnosis
Hypopigmented skin patches with impaired sensibility
develop in all types of leprosy. Thickened cutaneous In countries where the disease is common the clinical
nerves may be seen and thickened nerve trunks may diagnosis is seldom in doubt. Suggestive signs are the
be felt where they are superficial, especially where they appearance of skin lesions with loss of sensibility, pal-
cross a bone (typically behind the medial condyle of pably or even visibly thickened nerves which may also
the humerus at the elbow). Irrecoverable nerve dam- be tender, areas of anaesthesia, chronic ulcers of the
age with characteristic patterns of muscle weakness feet and typical deformities of hands and feet due to
and deformities of the hands and feet may also be muscle weakness and imbalance. In countries where
seen. Trophic ulcers, causing progressive destruction the disease is not endemic, diagnosis may have to
of the affected part, appear in the hands and feet. await the results of skin smear examination, serologi-
Skin lesions in tuberculoid leprosy are sparse, well-de- cal tests and skin or nerve biopsy.
marcated, hypopigmented and anaesthetic. In contrast,
in lepromatous leprosy, the skin is affected diffusely and
extensively and the lesions present as multiple, sym-
Patterns of nerve involvement
metrically distributed macular patches with some sen- Nerve trunks of the upper limbs are involved more
sory impairment. Plaques and nodules develop in ad- often than those of the lower limbs. There is a pattern
vanced stages. Coarsening of the facial skin and loss of in the selection, site of involvement, risk of damage
eyebrows may produce typical leonine features. Lep- and chances of recovery (see Table 2.3). In the upper
romatous ulceration of the nasal mucosa leads to de- limb ulnar nerve paralysis is the most common and
struction of the nasal septum and nasal deformity. combined ulnar and median nerve paralysis is seen less
Peripheral nerves are affected extensively in lepro- frequently. Occasionally, triple nerve paralysis (paraly-
matous leprosy whereas in tuberculoid leprosy the neural sis of ulnar, median and radial nerves) may occur. Any
lesions are few and focal in distribution. Cutaneous other pattern is extremely rare.
nerves as well as major nerve trunks of the upper and
lower limbs are usually involved. Except for the Vth
and VIIth nerves, the cranial nerves are not affected.
Treatment
Clinical defects in nerve function appear early in tuber- For purposes of treatment, patients are categorized as
54 culoid leprosy but much later in lepromatous leprosy. having paucibacillary (cases of indeterminate and
Table 2.3 Features of nerve trunk involvement in leprosy 2
Nerve affected Preferred site Involvementa Motor paralysis Recovery
Ulnarc Above elbow/wrist ++++ ++++ +
Median Above wrist ++ ++ ++
Infection
Common peroneal Back of knee +++ + ++
Tibial Behind ankle +++ +++ b
Radial Cutaneous division +++ NA NA
Radial groove ++ (forearm muscles only) +++
a Thickening; b tenderness/pain; c most commonly involved nerve trunk; + uncommon; ++ common; +++ quite common; ++++ very
common; NA, not applicable.
tuberculoid leprosy) or multibacillary (cases of lepro- MANAGEMENT OF RESIDUAL PARALYSIS AND

matous and borderline leprosy) leprosy. TROPHIC LESIONS
The long-term neuropathic complications of leprosy
MULTIDRUG THERAPY are dealt with in Chapter 11. The notorious deformi-
Combined chemotherapy with rifampicin as one of the ties and disablement result from: (a) local leprous
drugs is the mainstay of treatment; however, the choice granulomas (as in the face); (b) damage to nerves of
of drugs and duration of treatment depend on the the hands and feet and consequent muscle paralysis;
type of disease. Following the recommendations of the and (c) so-called ‘trophic lesions’ (ulcers, shortening of
World Health Organization, patients with paucibacil- digits and mutilations) arising from injuries to insen-
lary disease are treated with rifampicin 600 mg once sitive hands and feet. These conditions are prevented
monthly and dapsone 100 mg once daily, for 6 months; by early treatment of the disease, adequate treatment
and patients with multibacillary disease are given ri- of neuritis and protection of anaesthetic hands and
fampicin 600 mg and clofazimine 300 mg once feet.
monthly and dapsone 100 mg and clofazimine 50 mg Paresis and established deformities can usually be cor-
once daily, for 12 months. Reactions, especially acute rected or at least improved by surgery (see Chapter 11).
neuritis, are treated with anti-inflammatory medica- Although this is done mainly to improve function,
tion, of which prednisolone is the most important, and restoration of normal appearance is also important for
other supportive therapy. leprosy patients. Deformities such as claw-fingers and
drop-foot stigmatize affected individuals as ‘leprosy
NERVE DECOMPRESSION patients’, with dire social consequences.
Surgical decompression of a nerve trunk is sometimes Those requiring surgery should have had anti-
required in order to improve perfusion of the nerve leprosy treatment and should not have had acute neu-
and allow the anti-leprosy and anti-inflammatory ritis of any nerve trunk for at least 6 months prior to
drugs to reach the affected segment and thus prevent surgery. They must be well motivated and there
or abort nerve damage. Surgical decompression is should be proper pre-operative preparation with
indicated: (a) in acute neuritis when, even while under appropriate physiotherapy. Absence of facilities for
treatment with corticosteroids, there is increasing pre- and post-operative therapy is an absolute contra-
neurological deficit; and (b) in cases of severe, unre- indication for corrective surgery.
sponsive nerve pain, for relief of pain. Decompression
involves tunnel release (often with excision of the
medial epicondyle for the ulnar nerve) combined with
incision of the epineurium over the entire sclerosed MYCOTIC INFECTIONS
segment of the nerve. Stripping the epineurium should
not be done. Mycotic or fungal infection causes an indolent granu-
lomatous reaction, often leading to abscess formation,
TREATMENT OF NERVE ABSCESS tissue destruction and ulceration. When the muscu-
Cold abscesses associated with deteriorating neuro- loskeletal system is involved, it is usually by direct
logical function and those that are likely to burst spread from the adjacent soft tissues. Occasionally,
through the skin need to be excised or surgically evac- however, a bone or joint may be infected by
uated. If there is no associated neural deficit it is not haematogenous spread from a distant site.
necessary to intervene immediately, provided the These disorders are conveniently divided into
patient can be reviewed periodically. ‘superficial’ and ‘deep’ infections. 55
2 Superficial mycoses These are primarily infections of sinuses and ulcers. X-rays may show multiple bone
the skin or mucous surfaces which spread into the cavities or progressive bone destruction. The organ-
adjacent soft tissues and bone. The more common ism can be identified in the sinus discharge or in tissue
examples are the maduromycoses (a group consisting biopsies.
of several species), Sporothrix and various species of Treatment is unsatisfactory as there is no really
Candida. effective chemotherapy. Intravenous amphotericin B
The actinomycoses are usually included with the is advocated, but it is fairly toxic and causes side
superficial fungal infections. The causal organisms, of effects such as headaches, vomiting and fever.
which Actinomyces israelii is the commonest in Necrotic tissue should be widely excised. Even then it
humans, are not really fungi but anaerobic bacilli with is sometimes difficult to stop further invasion, and
fungus-like appearance and behaviour. amputation is sometimes necessary.
Deep mycoses This group comprises infections by
Blastomyces, Histoplasma, Coccidioides, Cryptococcus,
Aspergillus and other rare fungi. The organisms, CANDIDIASIS
which occur in rotting vegetation and bird droppings,
Candida albicans is a normal commensal in humans
gain entry through the lungs and, in humans, may
and it often causes superficial infection of the skin or
cause an influenza-like illness. Bone or joint infection
mucous membranes. Deep and systemic infections are
is uncommon except in patients with compromised
rare except under conditions of immunosuppression.
host defences.
Candida osteomyelitis and arthritis may follow
direct contamination during surgery or other invasive
procedures such as joint aspiration or arthroscopy.
MADUROMYCOSIS The diagnosis is usually made only after tissue sam-
pling and culture.
This chronic fungal infection is seen mainly in
Treatment consists of thorough joint irrigation and
northern Africa and the Indian subcontinent. The
curettage of discrete bone lesions, together with intra-
organisms usually enter through a cut in the foot;
venous amphotericin B.
from there they spread through the subcutaneous
tissues and along the tendon sheaths. The bones and
joints are infected by direct invasion; local abscesses
form and break through the skin as multiple sinuses. ACTINOMYCOSIS
The patient may present at an early stage with a tender
Infection is usually by Actinomyces israelii, an anaero-
subcutaneous nodule (when the diagnosis is seldom
bic Gram-positive bacillus. Although rare, it is impor-
entertained); more often he or she is seen when the
tant that it should be diagnosed because the organism
foot is swollen and indurated, with discharging
is sensitive to antibiotics.
The most common site of infection is the mandible
(from the mouth and pharynx), but bone lesions are also
seen in the vertebrae (spreading from the lung or gut)
and the pelvis (spreading from the caecum or colon).
Peripheral lesions may occur by direct infection of the
soft tissues and later extension to the bones. There may
be a firm, tender swelling in the soft tissues, going on to
form an abscess and one or more chronic discharging
sinuses. X-rays may show cyst-like areas of bone
destruction. The organism can be readily identified in the
sinus discharge, but only on anaerobic culture.
Treatment, by large doses of benzylpenicillin G,
tetracycline or erythromycin, has to be continued for
several months.
THE DEEP MYCOSES

2.15 Maduromycosis This Mediterranean market-worker Histoplasmosis, blastomycosis and coccidioidomycosis
was perpetually troubled by tiny abscesses and weeping
sinuses in her foot. X-rays showed that bone destruction are rare causes of bone and joint infection, but they
had already spread to the tarsal bones, and after 2 years of should always be considered in patients on immuno-
56 futile treatment the foot had to be amputated. suppressive therapy who develop arthritis of one of the
large joints or osteomyelitis in an unusual site. Diagno- 2
sis is usually delayed and often involves specialized
microbiological investigations to identify the organism.
Treatment with intravenous amphotericin B is
moderately effective. Operation may be necessary to
drain an abscess or to remove necrotic tissue.
Infection
HYDATID DISEASE
Hydatid disease is caused by the tapeworm Echinococ-

cus. Parasitic infestation is common among sheep
farmers, but bone lesions are rare.
The organism, a cestode worm, has a complicated
life-cycle. The definitive host is the dog or some other
carnivore that carries the tapeworm in its bowel. Seg- (a) (b)
ments of worm and ova pass out in the faeces and are
2.17 Hydatid disease of bone Two examples of hydatid
later ingested by one of the intermediate hosts – usu-
involvement of bone: there is no expansion of the cortex in
ally sheep or cattle or man. Here the larvae are carried (a) and very little in (b).
via the portal circulation to the liver, and occasionally
beyond to other organs, where they produce cysts
containing numerous scolices. Infested meat is then ture or compression of the spinal cord. Infestation
eaten by dogs (or humans), giving rise to a new gen- sometimes starts in childhood but the cysts take so
eration of tapeworm. long to enlarge that clinical symptoms and signs may
Scolices carried in the blood stream occasionally not become apparent for many years. The diagnosis is
settle in bone and produce hydatid cysts that slowly more likely if the patient comes from a sheep-farming
enlarge with little respect for cortical or epiphyseal district.
boundaries. The bones most commonly affected are
the vertebrae, pelvis, femur, scapula and ribs.
Imaging
X-rays show solitary or multiloculated bone cysts, but
Clinical features only moderate expansion of the cortices. However,
The patient may complain of pain and swelling, or cortical thinning may lead to a pathological fracture.
may present for the first time with a pathological frac- In the spine, hydatid disease may involve adjacent ver-
tebrae, with large cysts extending into the paraverte-
bral soft tissues. These features are best seen on CT
and MRI, which should always be performed if oper-
ative excision of the lesion is contemplated.
Investigations
Casoni’s (complement fixation) test may be positive,
especially in longstanding cases.
Diagnosis
Hydatid disease must be included in the differential di-
agnosis of benign and malignant bone cysts and cyst-
like tumours. If the clinical and radiological features are
not conclusive, needle biopsy should be considered,
though there is a risk of spreading the disease.
Treatment
2.16 Hydatid disease The life-cycle of the tapeworm The anthelminthic drug albendazole is moderately
which causes hydatid disease. effective in destroying the parasite. It has to be given 57
2 in repeated courses: a recommended programme is end of the human femur. J Bone Joint Surg 1976; 58A:
oral administration of 10 mg per kg per day for 3 961–70,
weeks, repeated at least 4 times with a one-week ‘rest’ Cierny G 3rd, Mader JT, Penninck JJ. A clinical staging
between courses. Liver, renal and bone marrow func- system for adult osteomyelitis. Clin Orthop Relat Res
tion should be monitored during treatment. 2003; 414: 7–24.
However, the bone cysts do not heal and recur- Ebong WW. Acute osteomyelitis in Nigerians with sickle-
rence is common. The indications for surgery are con- cell disease. Ann Rheum Dis 1986; 45: 911–5.
tinuing enlargement or spread of the lesion, a risk of Ellington JK, Harris M, Webb L, et al. Intracellular
fracture, invasion of soft tissues and pressure on Staphylococcus aureus. A mechanism for the indolence of
important structures. Curettage and bone grafting osteomyelitis. J Bone Joint Surg 2003; 85B: 918–21.
will lessen the risk of pathological fracture; at opera- Gristina AG. Biomaterial-centred infection: microbial
tion the cavity can be ‘sterilized’ with copious adhesion versus tissue integration. Science 1988; 237:
amounts of hypertonic saline, alcohol or formalin to 437–51
lessen the risk of recurrence. Hashmi MA, Norman P, Saleh M. The management of
Radical resection, with the margin at least 2 cm chronic osteomyelitis using the Lautenbach method.
beyond the cyst, is more certain, but also much more J Bone Joint Surg 2004; 86B: 269–75.
challenging. In a long bone the space can sometimes Lidwell OM. Clean air at operation and subsequent sepsis
be filled with a tumour-prosthesis, to include an in the joint. Clin Orthop Relat Res 1986; 211: 91–102.
arthroplasty if necessary. Large cysts of the vertebral Perez-Stable EJ & Hopewell PC. Current tuberculosis
column, or the pelvis and hip joint, are particularly treatment regimens: choosing the right one for your
difficult to manage in this way and in some cases sur- patient. Clin Chest Med, 1989; 10: 323–39.
gical excision is simply impractical or impossible. Rasool MN & Govender S. The skeletal manifestations of
congenital syphilis. J Bone Joint Surg 1989; 71B: 752–5.
Roberts JM, Drummond DS, Breed AL, et al. Subacute
haematogenous osteomyelitis in children: a retrospective
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Song Kit M, Sloboda John F. Acute hematogenous
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changing epidemiology of acute and subacute 9: 166–75.
haematogenous osteomyelitis in children. J Bone Joint Trueta J. The normal vascular anatomy of the human femoral
Surg 2001; 83B: 99–102. head during growth. J Bone Joint Surg 1957; 39B: 358–94.
Björkstén B & Boquist L. Histopathological aspects of Trueta J. Three types of acute haematogenous
chronic recurrent multifocal osteomyelitis. J Bone Joint osteomyelitis. J Bone Joint Surg 1959; 41B: 671–80.
Surg 1980; 62B: 276–380. Unkila-Kallis L, Kallis MJT, Peltola H. The usefulness of C-
Boutin RD & Resnick D. The SAPHO syndrome: an evolv- reactive protein levels in the identification of concurrent
ing concept for unifying several idiopathic disorders of septic arthritis in children who have acute haematogenous
bone and skin. Am J Roentgenol 1998; 170: 585–91. osteomyelitis. J Bone Joint Surg, 1994; 76A: 848–53.
Carr AJ, Cole WG, Robertson DM, Chow CW. Chronic Whalen JL, Fitzgeral RH Jr, Morrissy RT. A histological
multifocal osteomyelitis. J Bone Joint Surg 1993; 75B: study of acute haematogenous osteomyelitis following
582–91. physeal injuries in rabbits. J Bone Joint Surg 1988; 70A:
Chung SMK. The articular supply of the developing proximal 1383–92.
58
Inflammatory
rheumatic disorders 3
Christopher Edwards, Louis Solomon
The term ‘inflammatory rheumatic disorders’ covers a circumstances in which RA develops, and hypotheses
number of diseases that cause chronic pain, stiffness about its aetiology and pathogenesis have been sug-
and swelling around joints and tendons. In addition, gested. Important factors in the evolution of RA are:
they are commonly associated with extra-articular fea- (1) genetic susceptibility; (2) an immunological reac-
tures including skin rashes and inflammatory eye dis- tion, possibly involving a foreign antigen, preferen-
ease. Individuals with these diseases tend to die tially focussed on synovial tissue; (3) an inflammatory
younger than their peers as a result of the effects of reaction in joints and tendon sheaths; (4) the appear-
chronic inflammation. Many – perhaps all – are due to ance of rheumatoid factors (RF) in the blood and syn-
a faulty immune reaction resulting from a combina- ovium; (5) perpetuation of the inflammatory process;
tion of environmental exposures against a background and (6) articular cartilage destruction.
of genetic predisposition.
Genetic susceptibility A genetic association is sug-
gested by the fact that RA is more common in first-
degree relatives of patients than in the population at
RHEUMATOID ARTHRITIS large; furthermore twin studies have revealed a con-
cordance rate of around 30 per cent if one of the pair
is affected. The human leucocyte antigen (HLA) DR4
Rheumatoid arthritis (RA) is the most common cause
occurs in about 70 per cent of people with RA,
of chronic inflammatory joint disease. The most typi-
compared to a frequency of less than 30 per cent in
cal features are a symmetrical polyarthritis and
normal controls. HLA-DR4 is encoded in the major
tenosynovitis, morning stiffness, elevation of the ery-
histocompatibility complex (MHC) region on chro-
throcyte sedimentation rate (ESR) and the appearance
mosome 6. There are strong associations between
of autoantibodies that target immunoglobulins
HLA-DR4 and RA. In particular a key structural
(rheumatoid factors) in the serum. Rheumatoid arthri-
conformation within the HLA-DR4 binding groove
tis is a systemic disease and changes can be widespread
called the ‘shared epitope’ seems important. This may
in a number of tissues of the body. Individuals with
suggest that a particular antigen that fits into this may
RA tend to die younger than their peers as a result of
be playing a part.
the effects of chronic inflammation on a number of
HLA Class II molecules appear as surface antigens
organ systems. Chief among these is early ischaemic
on cells of the immune system (B lymphocytes,
heart disease secondary to the effects of inflammation
macrophages, dendritic cells), which can act as anti-
on the cardiovascular system.
gen-presenting cells (APCs). In some T-cell immune
The reported prevalence of RA in most populations
reactions, the process is initiated only when the anti-
is 1–3 per cent, with a peak incidence in the fourth or
genic peptide is presented in association with a specific
fifth decades. Women are affected 3 or 4 times more
HLA allele. It has been suggested that this is the case
commonly than men. Both the prevalence and the clin-
in people who develop RA; the idea is even more
ical expression vary between populations; the disease is
attractive if one proposes that the putative antigen has
more common (and generally more severe) in Cau-
a special affinity for synovial tissue. So far no such
casians living in the urban communities of Europe and
antigen has been discovered.
North America than in the rural populations of Africa.
The inflammatory reaction Once the APC/T-cell inter-
action is initiated, various local factors come into play
Cause and lead to a progressive enhancement of the immune
The cause of RA is still incompletely worked out. response. There is a marked proliferation of cells in
However, a great deal is now known about the the synovium, with the appearance of new blood
3 vessel formation. Immune cells coordinate their
action by the use of ‘short-range hormones’ (cyto-
kines), which can activate inflammatory cells such as
macrophages and B cells. Some cytokines called
chemokines attract other inflammatory cells to the
area.
Over recent years it has become clear that certain

(a) (b) (c) (d)
cytokines are important in RA. These include tumour
necrosis factor (TNF), interleukin-1 (IL-1) and inter- 3.1 Rheumatoid arthritis – pathology (a) Stage 1 –
leukin-6 (IL-6). The resulting synovitis, both in joints pre-clinical. (b) Stage 2 – synovitis and joint swelling.
(c) Stage 3 – early joint destruction with peri-articular
and in tendon sheath linings, is the hallmark of early erosions. (d) Stage 4 – advanced joint destruction and
RA. deformity.
Rheumatoid factor B-cell activation in RA leads to
tinue to seriously damage systemic health by acceler-
the production of anti-IgG autoantibodies, which
ating other disease processes such as ischaemic heart
are detected in the blood as ‘rheumatoid factor’
disease.
(RF). Low levels of RF can be found in many ‘nor-
mal’ individuals but when the levels are high an Stage 1 – pre-clinical Well before RA becomes clini-
inflammatory disease is likely. Other autoimmune cally apparent the immune pathology is already begin-
conditions such as systemic lupus erythematosus ning. Raised ESR, C-reactive protein (CRP) and RF
(SLE) and Sjögren’s syndrome are also associated may be detectable years before the first diagnosis.
with the presence of RF.
Stage 2 – synovitis Early changes are vascular conges-
In recent years other autoantibodies associated with
tion with new blood vessel formation, proliferation of
RA have been identified. The most important are anti-
synoviocytes and infiltration of the subsynovial layers
cyclic citrullinated peptide antibodies (anti-CCP).
by polymorphs, lymphocytes and plasma cells. There
The presence of anti-CCP is very specific for RA.
is thickening of the capsular structures, villous forma-
Patients with a positive RF test tend to be more
tion of the synovium and a cell-rich effusion into the
severely affected than those with a negative test.
joints and tendon sheaths. Although painful, swollen
Chronic synovitis and joint destructionChronic rheuma- and tender, these structures are still intact and mobile,
toid synovitis is associated with the production of pro- and the disorder is potentially reversible.
teolytic enzymes, prostaglandins and the cytokines
Stage 3 – destruction Persistent inflammation causes
TNF and IL-1. Immune complexes are deposited in
joint and tendon destruction. Articular cartilage is
the synovium and on the articular cartilage, where
eroded, partly by proteolytic enzymes, partly by vascu-
they appear to augment the inflammatory process.
lar tissue in the folds of the synovial reflections, and
This combination of factors leads to depletion of the
partly due to direct invasion of the cartilage by a pan-
cartilage matrix and, eventually, damage to cartilage
nus of granulation tissue creeping over the articular
and underlying bone. Vascular proliferation and
surface. At the margins of the joint, bone is eroded by
osteoclastic activity, most marked at the edges of the
granulation tissue invasion and osteoclastic resorption.
articular surface, may contribute further to cartilage
Similar changes occur in tendon sheaths, causing
destruction and peri-articular bone erosion.
tenosynovitis, invasion of the collagen bundles and,
eventually, partial or complete rupture of tendons.
Pathology A synovial effusion, often containing copious
amounts of fibrinoid material, produces swelling of
Rheumatoid arthritis is a systemic disease but the
the joints, tendons and bursae.
most characteristic lesions are seen in the synovium or
within rheumatoid nodules. The synovium is Stage 4 – deformity The combination of articular
engorged with new blood vessels and packed full of destruction, capsular stretching and tendon rupture
inflammatory cells. leads to progressive instability and deformity of the
joints. The inflammatory process usually continues
JOINTS AND TENDONS but the mechanical and functional effects of joint and
The pathological changes, if unchecked, proceed in tendon disruption now become vital.
four stages. Previously it was felt that having gone
through these stages the disease activity could be EXTRA-ARTICULAR TISSUES
‘burnt out’. This does not appear to be the case. In Rheumatoid nodules The rheumatoid nodule is a small
any one joint features of different stages can be occur- granulomatous lesion consisting of a central necrotic
ring simultaneously and even when joints are very zone surrounded by a radially disposed palisade of
60 badly destroyed the ongoing inflammation can con- local histiocytes, and beyond that by inflammatory
3
Inflammatory rheumatic disorders

(a) (b)
3.2 Rheumatoid synovitis (a) The macroscopic appearance of rheumatoid synovitis with fibrinoid material oozing
through a rent in the capsule. (b) Histology shows proliferating synovium with round-cell infiltration and fibrinoid particles
in the joint cavity (x120).
granulation tissue. Nodules occur under the skin Typically, a woman of 30–40 years complains of pain,
(especially over bony prominences), in the synovium, swelling and loss of mobility in the proximal joints of
on tendons, in the sclera and in many of the viscera. the fingers. There may be a previous history of ‘mus-
cle pain’, tiredness, loss of weight and a general lack
Lymphadenopathy Not only the nodes draining
of well-being. As time passes, the symptoms ‘spread’
inflamed joints, but also those at a distance such as the
to other joints – the wrists, feet, knees and shoulders
mediastinal nodes, can be affected. This, as well as a
in order of frequency. Another classic feature is gener-
mild splenomegaly, is due to hyperactivity of the reti-
alized stiffness after periods of inactivity, and espe-
culoendothelial system. More severe splenomegaly
cially after rising from bed in the early morning. This
can also be associated with neutropaenia as part of
early morning stiffness typically lasts longer than 30
Felty’s syndrome.
minutes.
Vasculitis This can be a serious and life-threatening Physical signs may be minimal, but usually there is
complication of RA. Involvement of the skin, includ- symmetrically distributed swelling and tenderness of
ing nailfold infarcts, is common but organ infarction the metacarpophalangeal joints, the proximal inter-
can occur. phalangeal joints and the wrists. Tenosynovitis is com-
mon in the extensor compartments of the wrist and
Muscle weakness Muscle weakness is common. It may
the flexor sheaths of the fingers; it is diagnosed by
be due to a generalized myopathy or neuropathy, but it
feeling thickening, tenderness and crepitation over
is important to exclude spinal cord disease or cord
the back of the wrist or the palm while passively mov-
compression due to vertebral displacement (atlanto-
ing the fingers. If the larger joints are involved, local
axial subluxation). Sensory changes may be part of a
warmth, synovial hypertrophy and intra-articular effu-
neuropathy, but localized sensory and motor symp-
sion may be more obvious. Movements are often lim-
toms can also result from nerve compression by thick-
ited but the joints are still stable and deformity is
ened synovium (e.g. carpal tunnel syndrome).
unusual.
Visceral disease The lungs, heart, kidneys, gastroin- In the later stages joint deformity becomes increas-
testinal tract and brain are sometimes affected. ingly apparent and the acute pain of synovitis is
Ischaemic heart disease and osteoporosis are common replaced by the more constant ache of progressive
complications. joint destruction. The combination of joint instability
and tendon rupture produces the typical ‘rheumatoid’
deformities: ulnar deviation of the fingers, radial and
Clinical features volar displacement of the wrists, valgus knees, valgus
The onset of RA is usually insidious, with symptoms feet and clawed toes. Joint movements are restricted
emerging over a period of months. Occasionally the and often very painful. About a third of all patients
disease starts quite suddenly. develop pain and stiffness in the cervical spine.
In the early stages the picture is mainly that of a Function is increasingly disturbed and patients may
polysynovitis, with soft-tissue swelling and stiffness. need help with grooming, dressing and eating. 61
3
(a) (b) (c)
(d) (e) (f)
3.3 Rheumatoid arthritis – clinical features (a) Early features of swelling and stiffness of the proximal finger joints and
the wrists. (b) The late hand deformities are so characteristic as to be almost pathognomonic. (c) Occasionally rheumatoid
disease starts with synovitis of a single large joint (in this case the right knee). Extra-articular features include subcutaneous
nodules (d,e) and tendon ruptures (f).
Extra-articular features These often appear in patients Ultrasound scanning and MRI The use of other imaging
with severe disease. The most characteristic is the techniques to look at soft-tissue changes and early
appearance of nodules. They are usually found as small erosions within joints has become more common.
subcutaneous lumps, rubbery in consistency, at the Ultrasound can be particularly useful in defining the
back of the elbows, but they also develop in tendons presence of synovitis and early erosions. Additional
(where they may cause ‘triggering’ or rupture), in the information on vascularity can be obtained if Doppler
viscera and the eye. They are pathognomonic of RA, techniques are used.
but occur in only 25% of patients.
Less specific features include muscle wasting, lym- Blood investigations
phadenopathy, scleritis, nerve entrapment syndromes,
skin atrophy or ulceration, vasculitis and peripheral sen- Normocytic, hypochromic anaemia is common and is
sory neuropathy. Marked visceral disease, such as pul- a reflection of abnormal erythropoiesis due to disease
monary fibrosis, is rare. activity. It may be aggravated by chronic gastrointesti-
nal blood loss caused by non-steroidal anti-inflamma-
tory drugs. In active phases the ESR and CRP
concentration are usually raised.
Imaging Serological tests for rheumatoid factor are positive
in about 80 per cent of patients and antinuclear fac-
X-rays Early on, x-rays show only the features of syn- tors are present in 30 per cent. Neither of these tests
ovitis: soft-tissue swelling and peri-articular osteo- is specific and neither is required for a diagnosis of
porosis. The later stages are marked by the appearance rheumatoid arthritis. Newer tests such as those for
of marginal bony erosions and narrowing of the artic- anti-CCP antibodies have added much greater speci-
ular space, especially in the proximal joints of the ficity but at the expense of sensitivity.
hands and feet. However, most individuals have evi-
dence of erosions within 2 years. In advanced disease,
articular destruction and joint deformity are obvious. Synovial biopsy
Flexion and extension views of the cervical spine often Synovial tissue may be obtained by needle biopsy, via
show subluxation at the atlanto-axial or mid-cervical the arthroscope, or by open operation. Unfortunately,
levels; surprisingly, this causes few symptoms in the most of the histological features of rheumatoid arthri-
62 majority of cases. tis are non-specific.
3

(a) (b) (c)
3.4 Rheumatoid arthritis – x-ray changes The progress of disease is well shown in this patient’s x-rays. First, there was
only soft-tissue swelling and peri-articular osteoporosis; later juxta-articular erosions appeared (arrow); ultimately, the joints
became unstable and deformed.
Diagnosis In the differential diagnosis of polyarthritis several

disorders must be considered.
The usual criteria for diagnosing rheumatoid arthritis
are the presence of a bilateral, symmetrical polyarthri- Seronegative inflammatory polyarthritis Polyarthritis is a
tis involving the proximal joints of the hands or feet, feature of a number of conditions including psoriatic
and persisting for at least 6 weeks. If there are subcu- arthritis, adult Still’s disease, systemic lupus erythe-
taneous nodules or x-ray signs of peri-articular ero- matosus and other connective-tissue diseases. These
sions, the diagnosis is certain. A positive test for are considered in later sections.
rheumatoid factor in the absence of the above features is
not sufficient evidence of rheumatoid arthritis, nor does Ankylosing spondylitis This is primarily an inflamma-
a negative test exclude the diagnosis if the other features tory disease of the sacroiliac and intervertebral joints,
are all present. The chief value of the rheumatoid fac- causing back pain and progressive stiffness; however,
tor tests is in the assessment of prognosis: persistently it may also involve the peripheral joints.
high titres herald more serious disease including
Reiter’s disease The larger joints and the lumbosacral
extra-articular features.
spine are the main targets. There is usually a history of
Atypical forms of presentation are not uncommon.
urethritis or colitis and often also conjunctivitis.
The early stages may be punctuated by spells of qui-
escence, during which the diagnosis is doubted, but Polyarticular gout Tophaceous gout affecting multiple
sooner or later the more characteristic features appear. joints can, at first sight, be mistaken for rheumatoid
Occasionally, in older people, the onset is explosive, arthritis. On x-ray the erosions are quite different
with the rapid appearance of severe joint pain and from those of rheumatoid arthritis; the diagnosis is
stiffness; paradoxically these patients have a relatively clinched by identifying typical birefringent urate crys-
good prognosis. Now and then (more so in young tals in the joint fluid or a nodular tophus.
women) the disease starts with chronic pain and It is a curious fact that, although both gout and RA
swelling of a single large joint and it may take months are fairly common, the two conditions are rarely seen
or years before other joints are involved. in the same patient. The reason for this is unknown.
The presence of tenderness on squeezing across all
metacarpophalangeal or metatarsophalangeal joints, Calcium pyrophosphate deposition disease This condi-
early morning stiffness of at least 30 minutes and a tion is usually seen in older people. Typically it affects
raised ESR are highly suggestive of a diagnosis of large joints, but it may occur in the wrist and metacar-
rheumatoid arthritis. A rapid diagnosis is vital so that pophalangeal joints as well. X-ray signs are fairly char-
early treatment can be started with disease-modifying acteristic and crystals may be identified in synovial
antirheumatic drugs. fluid or synovium. 63
3
(a) (b) (c)
3.5 Rheumatoid arthritis – differential diagnosis All three patients presented with painful swollen fingers. In
(a) mainly the proximal joints were affected (rheumatoid arthritis); in (b) the distal joints were the worst (Heberden’s
osteoarthritis); in (c) there were asymmetrical nodular swellings around the joints (gouty tophi).
Sarcoidosis Sarcoid disease sometimes presents with a Polymyalgia rheumatica This condition, which is seen
symmetrical small-joint polyarthritis and no bone mainly in the middle-aged or elderly, is characterized
involvement; in other cases a large joint such as the by aching discomfort around the pectoral and pelvic
knee or ankle may be involved. Erythema nodosum girdles, post-inactivity stiffness and muscular weakness.
and hilar lymphadenopathy on chest x-ray are clues to The joints are not tender but the muscles may be. The
the diagnosis. ESR and CRP are almost always elevated.
Acute sarcoidosis usually subsides spontaneously Corticosteroids (as little as 10 mg a day) provide rapid
within 6 months. Chronic sarcoidosis produces granu- and dramatic relief of all symptoms, and this response
lomatous infiltration of lungs, bone, synovium and is often used as a diagnostic test. The condition may
other organs and is more common in Afro-Caribbean be associated with, and certainly carries the risk of,
than Caucasian peoples. In addition to polyarthritis temporal arteritis resulting in blindness.
and tenosynovitis, there are usually x-ray features of
Osteoarthritis Polyarticular osteoarthritis (OA), which
punched-out ‘cysts’ and cortical erosions in the bones
typically involves the finger joints, is often mistaken
of the hands and feet. The ESR and serum
for RA. A moment’s reflection will usually dispel any
angiotensin converting enzyme (SACE) may be
doubt: OA always involves the distal interphalangeal
raised. Biopsy of affected tissue shows typical non-
joints and causes a nodular arthritis with radiologically
caseating granulomas. Treatment with non-steroidal
obvious osteophytes, whereas RA affects the proximal
anti-inflammatory drugs (NSAIDs) may be adequate
joints of the hand and causes predominantly erosive
but in more intractable cases corticosteroids or other
features.
immunosuppressive preparations are necessary.
Some confusion may arise from the fact that RA, in
Lyme disease This tick-borne spirochaetal infection its later stages, is associated with loss of articular car-
usually starts with a skin lesion and flu-like symptoms tilage and secondary OA. Enquiry into the early his-
and then spreads to multiple organs. If the initial tory will usually untangle the diagnosis. Sometimes,
lesions are missed or left untreated, patients may pres- however, RA atypically affects only a few of the larger
ent with an asymmetrical inflammatory polyarthritis joints and it is then very difficult to distinguish from
affecting mainly the larger joints. It is most likely to OA; x-ray features such as loss of articular cartilage
be encountered in known endemic areas in North throughout the entire joint and lack of hypertrophic
America, Europe and Asia. In late cases serological bone changes (sclerosis and osteophytes) should sug-
tests may be positive. Treatment with doxycycline or gest an inflammatory arthritis.
one of the newer cephalosporins is usually effective for
the arthritic features. Treatment
Viral arthritis Viral infections are often associated with There is no cure for rheumatoid arthritis. However,
a transient polyarthralgia; flu-like illness and a rash advances in therapy have revolutionized the treatment
will suggest the diagnosis. However, some infections approach with associated major improvements in out-
– most typically parvovirus B19 – occasionally cause a come (Kennedy et al., 2005). Medical treatment is
symmetrical polysynovitis (including the finger joints) guided by the principle that inflammation should be
and early morning stiffness, symptoms which may last reduced rapidly and aggressively. A multidisciplinary
for several months or may recur over a few years. The approach is needed from the beginning: ideally the
absence of ‘rheumatoid’ x-ray features and subcuta- therapeutic team should include a rheumatologist,
neous nodules will raise suspicions about the diagno- orthopaedic surgeon, physiotherapist, occupational
64 sis. therapist, orthotist and social worker. Their deploy-
Control of pain and stiffness with non-steroidal 3
anti-inflammatory drugs (NSAIDs) may be needed,
maintaining muscle tone and joint mobility by a bal-
anced programme of exercise, and general advice on
coping with the activities of daily living.
If there is no satisfactory response to DMARDs, it

is wise to progress rapidly to biological therapies such
as the TNF inhibitors infliximab, etanercept and adal-
imumab (Scott & Kingsley, 2006; Deighton et al.,
2006).
(a) Additional measures include the injection of long-
acting corticosteroid preparations into inflamed joints
and tendon sheaths. It is sometimes feared that such
injections may themselves cause damage to articular
cartilage or tendons. However, there is little evidence
that they are harmful, provided they are used spar-
ingly and with full precautions against infection.
Prolonged rest and immobility is likely to weaken
muscles and lead to a worse prognosis. However, some
splinting can be helpful at any stage of the disease.
KEY ELEMENTS IN MEDICAL TREATMENT

Identify patients with RA as early as possible
Start disease-modifying antirheumatic drugs

(b) (DMARDs) immediately
3.6 Rheumatoid arthritis – aftermath After the acute Consider combination therapy with multiple
inflammatory phase has passed, the patient may be left DMARDs
with features of secondary osteoarthritis, especially in the
hips (a) and the knees (b). If DMARDs fail, progress rapidly to biological
therapies such as the TNF inhibitors infliximab,
etanercept and adalimumab
ment and priorities will vary according to the individ-
ual and stage of the disease.
At the onset of the disease both the patient and the PHYSIOTHERAPY AND OCCUPATIONAL THERAPY
doctor will be uncertain about the likely rate of Preventative splinting and orthotic devices may delay
progress. An attempt should be made to determine the march of events; however, it is important to
the likely prognosis. Poor prognosis is associated with encourage activity. If these fail to restore and maintain
female sex, multiple joint involvement, high ESR and function, operative treatment is indicated.
CRP, positive RF and anti-CCP, younger age and the
presence of erosions at diagnosis. SURGICAL MANAGEMENT
At first this consists mainly of soft-tissue procedures
PRINCIPLES OF MEDICAL MANAGEMENT (synovectomy, tendon repair or replacement and joint
Treatment should be aimed at controlling inflamma- stabilization); in some cases osteotomy may be more
tion as rapidly as possible. This is likely to require the appropriate.
use of corticosteroids for their rapid onset (initially In late rheumatoid disease, severe joint destruction,
oral doses of 30 mg of prednisolone or 120 mg i.m. fixed deformity and loss of function are clear indica-
methylprednisolone may be used). Steroids should be tions for reconstructive surgery. Arthrodesis, osteo-
rapidly tapered to prevent significant side effects. tomy and arthroplasty all have their place and are
In addition, disease-modifying antirheumatic drugs considered in the appropriate chapters. However, it
(DMARDs) should be started at this time. The first should be recognized that patients who are no longer
choice is now methotrexate at doses of 10–25 mg/week. suffering the pain of active synovitis and who are con-
This may be used initially alone or in combination with tented with a limited pattern of life may not want or
sulfasalazine and hydroxychloroquine. Leflunomide can need heroic surgery merely to improve their anatomy.
also be considered if methotrexate is not tolerated. Gold Careful assessment for occupational therapy, the pro-
and penicillamine are now used rarely. vision of mechanical aids and adjustments to their 65
3 home environment may be much more useful. It ular erosions, rheumatoid nodules, severe muscle
appears safe to continue methotrexate during elective wasting, joint contractures and evidence of vasculitis
orthopaedic surgery. However, doses of corticos- are bad prognostic signs. Women, on the whole, fare
teroids should be as low as possible and biological somewhat worse than men. Without effective treat-
therapies such as the TNF inhibitors should be ment about 10 per cent of patients improve steadily
stopped prior to surgery where possible. after the first attack of active synovitis; 60 per cent
have intermittent phases of disease activity and remis-

sion, but with a slow downhill course over many years;
Complications
20 per cent have severe joint erosion, which is usually
Fixed deformities The perils of rheumatoid arthritis evident within the first 5 years; and 10 per cent end
are often the commonplace ones resulting from igno- up completely disabled. In addition, a reduction in life
rance and neglect. Early assessment and planning expectancy by 5–10 years is common and is often due
should prevent postural deformities, which will result to premature ischaemic heart disease. However, early
in joint contractures. aggressive medical treatment appears to reduce the
morbidity and mortality.
Muscle weakness Even mild degrees of myopathy or
neuropathy, when combined with prolonged inactiv-
ity, may lead to profound muscle wasting and weak-
ness. This should be prevented by control of SERONEGATIVE
inflammation, physiotherapy and pain control, if pos- SPONDYLOARTHROPATHIES
sible; if not, the surgeon must be forewarned of the
difficulty of postoperative rehabilitation.
ANKYLOSING SPONDYLITIS
Joint rupture Occasionally the joint lining ruptures
and synovial contents spill into the soft tissues. Treat- Like rheumatoid arthritis, this is a generalized chronic
ment is directed at the underlying synovitis, i.e. splin- inflammatory disease, but its effects are seen mainly in
tage and injection of the joint, with synovectomy as a the spine and sacroiliac joints. It is characterized by
second resort. pain and stiffness of the back, with variable involve-
ment of the hips and shoulders and (more rarely) the
Infection Patients with rheumatoid arthritis – and
peripheral joints. Its reported prevalence is 0.1 to 0.2
even more so those on corticosteroid therapy – are
per cent in western Europe and North America, but is
susceptible to infection. Sudden clinical deterioration,
much lower in Japanese and African peoples. Males
or increased pain in a single joint, should alert one to
are affected more frequently than females (estimates
the possibility of septic arthritis and the need for joint
vary from 2:1 to 10:1) and the usual age at onset is
aspiration.
between 15 and 25 years. There is a strong tendency
Spinal cord compression This is a rare complication of to familial aggregation and association with the
cervical spine (atlanto-axial) instability. The onset of genetic marker HLA-B27.
weakness and upper motor neuron signs in the lower
limbs is suspicious. If they occur, immobilization of
the neck is essential and spinal fusion should be car-
Cause
ried out as soon as possible. There is considerable evidence for regarding ankylos-
ing spondylitis (AS) as a genetically determined
Systemic vasculitis Vasculitis is a rare but potentially
immunopathological disorder. The disease is much
serious complication. Corticosteroids and immuno-
more common in family members of patients than in
suppressives such as intravenous cyclophosphamide
the general population – HLA-B27 is present in over
may be required.
95 per cent of Caucasian patients and in half of their
Amyloidosis This is another rare but potentially lethal first-degree relatives (as compared with 8 per cent of
complication of longstanding rheumatoid arthritis. The the general population); and racial groups with an
patient presents with proteinuria and progressive renal unusually low prevalence of AS also show a very low
failure. Finding amyloid in a rectal or renal biopsy prevalence of HLA-B27 (e.g. less than 1 per cent in
makes the diagnosis. Aggressive control of inflamma- Japanese people).
tion has reduced this complication significantly. There are various theories about the ‘triggering fac-
tor’ that initiates the abnormal immune response. It
may be a bacterial antigen, which closely resembles
Prognosis HLA-B27 that induces an antibody response, which
Rheumatoid arthritis runs a variable course. When the also targets the HLA-B27 positive cells; or, as in the
patient is first seen it is difficult to predict the out- case of RA, the HLA-B27 molecule may be involved
66 come, but high titres of rheumatoid factor, peri-artic- in the presentation of a specific antigen to the T cells,
which then react with the antigen-presenting cells. 3.7 Ankylosing spondylitis 3
Since classic ankylosing spondylitis is sometimes asso- – early The cardinal clinical
ciated with genitourinary or bowel infection, and dis- feature is marked stiffness of
the spine. (a) This patient
orders such as Reiter’s disease and ulcerative colitis manages to stand upright by
cause vertebral and sacroiliac changes indistinguish- keeping his knees slightly
able from those of ankylosing spondylitis, it has been flexed. (b) It looks as if he can

suggested that the putative organism may be carried bend down to touch his toes,
to the spine by local lymphatic drainage. but his back is rigid and all the
movement takes place at his
hips.
Pathology
There are two basic lesions: synovitis of diarthrodial
joints and inflammation at the fibro-osseous junctions
of syndesmotic joints and tendons. The preferential
involvement of the insertion of tendons and ligaments
(the entheses) has resulted in the unwieldy term
enthesopathy.
Synovitis of the sacroiliac and vertebral facet joints
causes destruction of articular cartilage and peri-artic-
ular bone. The costovertebral joints also are fre-
quently involved, leading to diminished respiratory (a) (b)
excursion. When peripheral joints are affected the
same changes occur.
Inflammation of the fibro-osseous junctions affects
the intervertebral discs, sacroiliac ligaments, symph- Early on there is little to see apart from slight flat-
ysis pubis, manubrium sterni and the bony insertions tening of the lower back and limitation of extension in
of large tendons. Pathological changes proceed in the lumbar spine. There may be diffuse tenderness
three stages: (1) an inflammatory reaction with cell over the spine and sacroiliac joints, or (occasionally)
infiltration, granulation tissue formation and erosion swelling and tenderness of a single large joint.
of adjacent bone; (2) replacement of the granulation In established cases the posture is typical: loss of the
tissue by fibrous tissue; and (3) ossification of the normal lumbar lordosis, increased thoracic kyphosis
fibrous tissue, leading to ankylosis of the joint. and a forward thrust of the neck; upright posture and
Ossification across the surface of the disc gives rise balance are maintained by standing with the hips and
to small bony bridges or syndesmophytes linking adja- knees slightly flexed, and in late cases these may
cent vertebral bodies. If many vertebrae are involved become fixed deformities. Spinal movements are
the spine may become absolutely rigid. diminished in all directions, but loss of extension is
always the earliest and the most severe disability. It is
revealed dramatically by the ‘wall test’: the patient is
Clinical features
asked to stand with his back to the wall; heels, but-
The disease starts insidiously: a teenager or young adult tocks, scapulae and occiput should all be able to touch
complains of backache and stiffness recurring at inter- the wall simultaneously. If extension is seriously
vals over a number of years. This is often diagnosed as diminished the patient will find this impossible. In the
‘simple mechanical back pain’, but the symptoms are most advanced stage the spine may be completely
worse in the early morning and after inactivity. Referred ankylosed from occiput to sacrum – sometimes in
pain in the buttocks and thighs may appear as ‘sciatica’ positions of grotesque deformity. Marked loss of cer-
and some patients are mistakenly treated for interverte- vical extension may restrict the line of vision to a few
bral disc prolapse. Gradually pain and stiffness become paces.
continuous and other symptoms begin to appear: gen- Chest expansion, which should be at least 7 cm in
eral fatigue, pain and swelling of joints, tenderness at young men, is often markedly decreased. In old peo-
the insertion of the Achilles tendon, ‘foot strain’, or ple, who may have pulmonary disease, this test is
intercostal pain and tenderness. unreliable.
Occasionally the disease starts with pain and slight Peripheral joints (usually shoulders, hips and knees)
swelling in a peripheral joint such as the ankle, or pain are involved in over a third of the patients; they show
and stiffness of the hip. Sooner or later, though, back- the features of inflammatory arthritis – swelling, ten-
ache will come to the fore. In women the axial skele- derness, effusion and loss of mobility. There may also
tal disease may remain restricted to the sacroiliac be tenderness of the ligament and tendon insertions
joints making diagnosis challenging. close to a large joint or under the heel. 67
3
(c)
(b)
3.8 Ankylosing spondylitis – x-rays (a) An early sign is ‘squaring’ of the lumbar
vertebrae. (b,c) Bony bridges (syndesmophytes) between the vertebral bodies convert
the spine into a rigid column.
(a)
Peripheral joints may show erosive arthritis or pro-
gressive bony ankylosis.
MRI MRI allows detailed investigation of sacroiliac
Extraskeletal manifestationsGeneral fatigue and loss of joints and may show typical erosions and features of
weight are common. Acute anterior uveitis occurs in inflammation such as bone oedema. Various tech-
about 25 per cent of patients; it usually responds well niques including gadolinium contrast can be used to
to treatment but, if neglected, may lead to permanent demonstrate inflammatory lesions in other areas of
damage including glaucoma. Other extraskeletal dis- the spine.
orders, such as aortic valve disease, carditis and pul-
monary fibrosis (apical), are rare and occur very late in
the disease. Special investigations
The ESR and CRP are usually elevated during active
Imaging phases of the disease. HLA-B27 is present in 95 per
cent of cases. Serological tests for rheumatoid factor
X-rays The cardinal sign – and often the earliest – is are usually negative.
erosion and fuzziness of the sacroiliac joints. Later
there may be peri-articular sclerosis, especially on the
iliac side of the joint and finally bony ankylosis. Diagnosis
The earliest vertebral change is flattening of the Diagnosis is easy in patients with spinal rigidity and
normal anterior concavity of the vertebral body typical deformities, but it is often missed in those with
(‘squaring’). Later, ossification of the ligaments early disease or unusual forms of presentation. In over
around the intervertebral discs produces delicate 10 per cent of cases the disease starts with an asym-
bridges (syndesmophytes) between adjacent verte- metrical inflammatory arthritis – usually of the hip,
brae. Bridging at several levels gives the appearance of knee or ankle – and it may be several years before back
a ‘bamboo spine’. pain appears. Atypical onset is more common in
Osteoporosis is common in longstanding cases and women, who may show less obvious changes in the
there may be hyperkyphosis of the thoracic spine due sacroiliac joints. A history of AS in a close relative is
68 to wedging of the vertebral bodies. strongly suggestive.
Mechanical disorders Low back pain in young adults is Non-steroidal anti-inflammatory drugs It is doubtful 3
usually attributed to one of the more common disor- whether these drugs prevent or retard the progress to
ders such as muscular strain, facet joint dysfunction or ankylosis, but they do control pain and counteract
spondylolisthesis. These conditions differ from AS in soft-tissue stiffness, thus making it possible to benefit
several ways: the onset of pain is related to specific from exercise and activity. They may have to be con-
physical activities, stiffness is less pronounced and tinued for many years.

symptoms are eased rather than aggravated by inactiv-
TNF inhibitors With the introduction of the TNF
ity. Tenderness is also more localized and the periph-
inhibitors it has become possible to treat the underly-
eral joints are normal.
ing inflammatory processes active in AS. This can
This
Diffuse idiopathic hyperostosis (Forestier’s disease) result in significant improvement in disease activity
is a fairly common disorder, predominantly of older including remission. These therapies are generally
men, characterized by widespread ossification of liga- reserved for individuals who have failed to be con-
ments and tendon insertions. X-rays show pro- trolled with non-steroidal anti-inflammatory drugs.
nounced but asymmetrical intervertebral spur
Operation Significantly damaged hips can be treated
formation and bridging throughout the dorsolumbar
by joint replacement, though this seldom provides
spine (see Fig. 5.13b). Although it bears a superficial
more than moderate mobility. Moreover, the inci-
resemblance to AS, it is not an inflammatory disease,
dence of infection is higher than usual and patients
spinal pain and stiffness are seldom severe, the sacro-
may need prolonged rehabilitation.
iliac joints are not eroded and the ESR is normal.
Deformity of the spine may be severe enough to
Other seronegative spondyloarthropathies A number of warrant lumbar or cervical osteotomy. These are diffi-
disorders are associated with vertebral and sacroiliac cult and potentially hazardous procedures; fortu-
lesions indistinguishable from those of ankylosing nately, with improved activity and exercise
spondylitis. They are Reiter’s disease, psoriatic arthri- programmes, they are seldom needed. If spinal defor-
tis, ulcerative colitis, Crohn’s disease, Whipple’s disease mity is combined with hip stiffness, hip replacements
and Behçet’s syndrome. In each there are certain char- (permitting full extension) often suffice.
acteristic features: the rash or nail changes of psoriasis,
intestinal ulceration in inflammatory bowel disease,
genitourinary and ocular inflammation in Reiter’s dis-
Complications
ease, buccal and genital ulceration in Behçet’s syn- Spinal fractures The spine is often both rigid and
drome. Yet there is considerable overlap between osteoporotic; fractures may be caused by compara-
them; all show some familial aggregation and all are tively mild injuries. The commonest site is C5–7, but
associated with the histocompatibility antigen, HLA- it is prudent to x-ray the entire spine in accident
B27. Patients with one of these disorders (including
AS) often have close relatives with another, or with a
positive HLA-B27.
Treatment
The disease is not usually as damaging as rheumatoid
arthritis and many patients continue to lead an active
life. Treatment consists of: (1) general measures to
maintain satisfactory posture and preserve movement;
(2) anti-inflammatory drugs to counteract pain and
stiffness; (3) the use of TNF inhibitors for severe dis-
ease; and (4) operations to correct deformity or
restore mobility (Manadan et al., 2007; Siridopoulos
et al., 2008).
General measures Patients are encouraged to remain
active and follow their normal pursuits as far as possi- (a) (b)
ble. They should be taught how to maintain satisfac-
tory posture and urged to perform spinal extension 3.9 Ankylosing spondylitis – operative treatment
Spinal osteotomy is occasionally performed to correct a
exercises every day. Swimming, dancing and gymnas-
severe, rigid deformity. (a) Before operation this man could
tics are ideal forms of recreation. Rest and immobi- see only a few paces ahead; (b) after osteotomy his back is
lization are contraindicated because they tend to still rigid but his posture, function and outlook are
increase the general feeling of stiffness. improved. 69
3 victims who have AS. Treatment in these cases is
directed at preventing further deformity.
Hyperkyphosis In longstanding cases the spine may
become severely kyphotic, so much so that the patient
has difficulty lifting his head to see in front of his feet.
Spinal cord compression This is uncommon, but it

should be thought of in patients who develop long- (a)
tract symptoms and signs. It may be caused by (b)
atlanto-axial subluxation or by ossification of the pos-
terior longitudinal ligament. 3.10 Reiter’s
syndrome – the
Lumbosacral nerve root compression Patients may occa- classic ‘Reiter’s triad’
sionally develop root symptoms, including lower limb consists of conjunctivitis
weakness and paraesthesia, in addition to their ‘usual’ (a), urethritis
pelvic girdle symptoms. (b) (sometimes colitis)
and arthritis (c).
Tenderness of the tendo
Achilles and the plantar
REITER’S SYNDROME AND REACTIVE fascia is also common.
(c)
ARTHRITIS
The syndrome described by Hans Reiter in 1916 (and
100 years before that by Benjamin Brodie) is a clinical
Pathology
triad of urethritis, arthritis and conjunctivitis occur- The pathological changes are essentially the same as
ring some weeks after either dysentery or genitouri- those in ankylosing spondylitis, with the emphasis first
nary infection. It is now recognized that this is one of on subacute large-joint synovitis and in some individ-
the classic forms of reactive arthritis, i.e. an aseptic uals with a chronic disease course tending towards
inflammatory arthritis associated with non-specific sacroiliitis and spondylitis.
infection (often urogenital or bowel).
Its prevalence is difficult to assess, but it is probably
the commonest type of large-joint polyarthritis in
Clinical features
young men. It is thought to occur in 1–3 per cent of The acute phase of the disease is marked by an asym-
all people who develop either non-specific urogenital metrical inflammatory arthritis of the lower limb
infection or Shigella dysentery, but its incidence may joints – usually the knee and ankle but often the tarsal
be as high as 25 per cent in those who are HLA-B27 and toe joints as well. The joint may be acutely
positive. Men are affected more often than women painful, hot and swollen with a tense effusion, sug-
(the ratio is about 10:1), but this may simply reflect gesting gout or infection. Tendo Achilles tenderness
the difficulty of diagnosing the genitourinary infec- and plantar fasciitis (evidence of enthesopathy) are
tion in women. The usual age at onset is between 20 common, and the patient may complain of backache
and 40 years, but children are affected too – perhaps even in the early stage. Conjunctivitis, urethritis and
after an episode of diarrhoea. bowel infections are often mild and easily missed; the
patient should be carefully questioned about symp-
toms during the previous few weeks. Cystitis and cer-
Cause
vicitis may occur in women.
Familial aggregation, overlap with other forms of Less frequent, but equally characteristic, features
seronegative spondyloarthropathy in first-degree rela- are a vesicular or pustular dermatitis of the feet (kera-
tives and a close association with HLA-B27 point to a toderma blennorrhagica), balanitis and mild buccal
genetic predisposition, the bowel or genitourinary ulceration.
infection acting as a trigger. Gut pathogens include The acute disorder usually lasts for a few weeks or
Shigella flexneri, Salmonella, Campylobacter species months and then subsides, but most patients have
and Yersinia enterocolitica. Lymphogranuloma either recurrent attacks of arthritis or other features of
venereum and Chlamydia trachomatis have been chronic disease.
implicated as sexually transmitted infections. All these The chronic phase is more characteristic of a spondy-
bacteria can survive in human cells; assuming that loarthropathy. Over half of the patients with Reiter’s
either the bacterium or a peptide bacterial fragment disease complain of mild, recurrent episodes of poly-
acts as the antigen, the pathogenesis could be the arthritis (including upper limb joints). About half of
70 same as that suggested for ankylosing spondylitis. those again develop sacroiliitis and spondylitis with
from Reiter’s disease, and diagnosis may depend on 3
identifying the organism or gonococcal antibodies.
Enteropathic arthritis Ulcerative colitis and Crohn’s
disease may be associated with subacute synovitis,
causing pain and swelling of one or more of the
peripheral joints. These subside when the intestinal

disease is controlled.
Treatment
Initial treatment for Reiter’s disease should be aimed
at ensuring the infectious organism responsible has
been cleared. This is particularly important for sexu-
3.11 Reiter’s disease – other features The characteristic ally transmitted infections such as Chlamydia tra-
pustular dermatitis of the feet – keratoderma chomatis.
blennorrhagicum. Even if the triggering infection is identified, treating
it will have no effect on the reactive arthritis. However,
there is some evidence that treatment of Chlamydia in-
features resembling those of ankylosing spondylitis. fection with tetracycline for periods of up to 3 months
Uveitis is also fairly common and may give rise to pos- can reduce the risk of recurrent joint disease.
terior synechiae and glaucoma. Symptomatic treatment could include the use of
analgesia and non-steroidal anti-inflammatory drugs.
If the inflammatory response is aggressive then local
X-rays injection of corticosteroids or even intramuscular
Sacroiliac and vertebral changes are similar to those of methylprednisolone may be useful. If symptoms and
ankylosing spondylitis. If peripheral joints are signs do not resolve then DMARDs used in the treat-
involved, they may show features of erosive arthritis. ment of RA may be needed. Topical steroids may be
used for uveitis.
Special investigations
Tests for HLA-B27 are positive in 75 per cent of PSORIATIC ARTHRITIS
patients with sacroiliitis. The ESR may be high in the
active phase of the disease. The causative organism Polyarthritis and psoriasis are often seen together.
can sometimes be isolated from urethral fluids or fae- Usually this is simply a chance concurrence of two
ces, and tests for antibodies may be positive. fairly common disorders. In some cases, however, the
patient has a true psoriatic arthritis – a distinct entity
characterized by seronegative polysynovitis, erosive
Diagnosis (sometimes very destructive) arthritis, and a signifi-
The diagnosis should be considered in any young cant incidence of sacroiliitis and spondylitis.
adult who presents with an acute or subacute arthritis The prevalence of psoriasis is 1–2 per cent, but only
in the lower limbs. It is more likely to be missed in about 5 per cent of those affected will develop psori-
women, in children and in those with very mild (and atic arthritis. The usual age at onset is 30–50 years
often forgotten) episodes of genitourinary or bowel (often later than the skin lesions).
infection. Some patients never develop the full syn-
drome and one should be alert to the formes fruste
with large-joint arthritis alone.
Cause
As with the other seronegative spondyloarthropathies,
Reiter’s disease, gout and
Gout and infective arthritis
there is a strong genetic component: patients often
infection should all be considered in the differential
give a family history of psoriasis; there is a significantly
diagnosis of inflammation in a large peripheral joint.
increased incidence of other spondyloarthropathies in
Examination of synovial fluid for organisms and crys-
close relatives; and 60 per cent of those with psoriatic
tals may provide important clues.
spondylitis or sacroiliitis have HLA-B27.
Gonococcal arthritis Gonococcal arthritis takes two Psoriatic skin lesions may well be a reactive phe-
forms: (1) bacterial infection of the joint; and (2) a re- nomenon, and the joint lesions a form of ‘reactive
active arthritis with sterile joint fluid. A history of gen- arthritis’. However, no specific trigger agent has thus
itourinary infection further complicates the distinction far been identified. 71
3 Pathology
The joint changes are similar to those in rheumatoid
arthritis – chronic synovitis with cell infiltration and
exudate, going on to fibrosis. Cartilage and bone
destruction may be unusually severe (‘arthritis muti-
lans’). However, rheumatoid nodules are not seen.

Sacroiliac and spine changes, which occur in about
30 per cent of patients, are similar to those in anky-
losing spondylitis.
(a)
Clinical features
The patient may present with one of several patterns
of joint involvement. These include: arthritis of distal
interphalangeal joints, ‘arthritis mutilans’, asymmetri-
cal large joint oligoarthritis and patterns mimicking
rheumatoid arthritis or ankylosing spondylitis. Psoria-
sis of the skin or nails usually precedes the arthritis,
but hidden lesions (in the natal cleft or umbilicus) are
easily overlooked. (b)
The condition can progress slowly or very rapidly
and may become quiescent. Sometimes (particularly
in women) joint involvement is more symmetrical,
and in these cases the condition may be indistinguish-
able from seronegative rheumatoid arthritis. Asym-
metrical swelling of two or three fingers may be due
to a combination of interphalangeal arthritis and
tenosynovitis.
Sacroiliitis and spondylitis are seen in about one-third
of patients, and occasionally this is the predominant
(c)
change with a clinical picture resembling ankylosing
spondylitis. As in the other spondyloarthropathies, heel 3.12 Psoriatic arthritis (1) (a) Psoriasis of the elbows
pain (enthesitis) is not uncommon. and forearms; (b) typical finger deformities, and (c) x-rays
In the worst cases both the spine and the peripheral show distal joint involvement – clearly the disease is not
simply rheumatoid arthritis in a patient with psoriasis.
joints may be involved. Fingers and toes are severely
deformed due to erosion and instability of the inter-
phalangeal joints (arthritis mutilans).
Ocular inflammation occurs in about 30 per cent of Diagnosis
patients. The main difficulty is to distinguish ‘psoriatic arthritis’
from ‘psoriasis with seronegative RA’. The important
Imaging distinguishing features of psoriatic arthritis are: (1)
asymmetrical joint distribution; (2) involvement of dis-
X-ray examination may show severe destruction of tal finger joints; (3) the presence of sacroiliitis or
the interphalangeal joints of the hands and feet; spondylitis; and (4) the absence of rheumatoid nodules.
changes in the large joints are similar to those of
rheumatoid disease. Sacroiliac erosion is fairly com-
mon; if the spine is involved the appearances are iden- Treatment
tical to those of ankylosing spondylitis. In mild disease no more than topical preparations to
Ultrasound scanning and MRI may show greater control the skin disease and NSAIDs for the arthritis
definition of the extent and activity of synovitis. are needed. In resistant forms of arthritis, immuno-
suppressive agents (methotrexate) and TNF inhibitors
(infliximab, etanercept and adalimumab) have proved
effective.
Tests for rheumatoid factor are almost always nega- Surgery may be needed for unstable joints.
tive. HLA-B27 occurs in 50–60 per cent, especially in Arthrodesis of the distal interphalangeal joints may
72 those with overt sacroiliitis. greatly improve function.
Complications 3
In addition to spondyloarthritis, there are several
unusual but important complications of inflammatory
bowel disease that may confuse the clinical picture.
Septic arthritis of the hip Infection may spread directly

from the bowel. The patient presents with a fever and
pain in the groin. Hip movements are limited and
there may be swelling due to an abscess. Treatment is
by antibiotics and operative drainage.
(a) (b)
Psoas abscess In Crohn’s disease a posterior fistula
3.13 Psoriatic arthritis (2) The feet and toes are often may track into the psoas sheath. The patient com-
involved. In this case the patient developed a severely plains of back pain and may develop a typical psoas
destructive form of the disease (arthritis mutilans). abscess with pain in the hip, limitation of movement
and a tender mass in the groin. Treatment is by oper-
ative drainage of the abscess.
ENTEROPATHIC ARTHRITIS Osteopaenia Patients with chronic bowel disease
often develop osteoporosis and osteomalacia – partly
Both Crohn’s disease and ulcerative colitis may be due to malabsorption and partly as a consequence of
associated with either peripheral arthritis or sacroiliitis treatment with corticosteroids. Compression fractures
and spondylitis. of the spine may cause severe back pain.
Peripheral arthritis
Peripheral arthritis is fairly common, occurring in
about 15 per cent of patients with inflammatory JUVENILE IDIOPATHIC ARTHRITIS
bowel disease. Typically one or perhaps a few of the
larger joints are involved. Pain and swelling may Juvenile idiopathic arthritis (JIA) is the preferred term
appear quite suddenly and last for 2–3 months before for non-infective inflammatory joint disease of more
subsiding. Synovitis is usually the only feature but than 3 months’ duration in children under 16 years of
joint erosion can occur. Men and women are affected age. It embraces a group of disorders in all of which
with equal frequency and there is no particular associ- pain, swelling and stiffness of the joints are common
ation with HLA-B27. features. The prevalence is about 1 per 1000 children,
Treatment is directed at the underlying disorder: and boys and girls are affected with equal frequency.
attacks of arthritis are often triggered by a flare-up of The cause is similar to that of rheumatoid arthritis:
bowel disease and when the latter is brought under an abnormal immune response to some antigen in
control the arthritis can disappear. Anti-inflammatory children with a particular genetic predisposition.
drugs should not generally be used as they may have However, rheumatoid factor is usually absent.
a deleterious effect on the bowel disease. Other treat- The pathology, too, may be like that of rheumatoid
ment options are local corticosteroid injection and arthritis: primarily a synovial inflammation leading to
disease-modifying treatments such as methotrexate. fibrosis and ankylosis. Stiffening tends to occur in
This may also improve the bowel disease. In severe whatever position the joint is allowed to assume; thus
cases TNF inhibitors may be needed. flexion deformities are a common and characteristic
feature. Chronic inflammation and alterations in the
local blood supply may affect the epiphyseal growth
Sacroiliitis and spondylitis
plates, leading to both local bone deformities and an
This pattern is seen in about 10 per cent of patients overall retardation of growth. However, cartilage ero-
with inflammatory bowel disease, and in half of these sion is less marked than in rheumatoid arthritis and
patients the clinical picture closely resembles that of severe joint instability is uncommon.
ankylosing spondylitis. HLA-B27 is positive in 60 per
cent and there is an increased incidence of ankylosing
spondylitis in close relatives. Unlike the peripheral
Clinical features
arthritis, sacroiliitis shows no temporal relationship to Children with JIA present in several characteristic
gastrointestinal inflammation and its course is unaf- ways. About 15 per cent have a systemic illness, and
fected by treatment of the bowel disease. Manage- arthritis only develops somewhat later; the majority
ment is the same as that of ankylosing spondylitis. (60–70 per cent) have a pauciarticular arthritis 73
3
(a) (b) (c) (d) (e)
3.14 Juvenile idiopathic arthritis (a–d) This young girl developed JIA when she was 5 years old. Here we see her at 6, 9
and 14 years of age. The arthritis has become inactive, leaving her with a knee deformity which was treated by osteotomy.
Her eyes, too, were affected by iridocyclitis. (Courtesy of Mr Malcolm Swann and Dr Barbara Ansell). (e) X-ray of another
young girl who required hip replacements at the age of 14 years and, later, surgical correction of her scoliosis.
affecting a few of the larger joints; about 10 per cent complication is chronic iridocyclitis, which occurs in
present with polyarticular arthritis, sometimes closely about 50 per cent of patients. The arthritis often goes
resembling RA; the remaining 5–10 per cent develop into remission after a few years but by then the child
a seronegative spondyloarthritis. is left with asymmetrical deformities and growth
defects that may be permanent.
SYSTEMIC JIA
This, the classic Still’s disease, is usually seen below the POLYARTICULAR JIA
age of 3 years and affects boys and girls equally. It Polyarticular arthritis, typically with involvement of
starts with intermittent fever, rashes and malaise; the temporomandibular joints and the cervical spine,
during these episodes, which occur almost daily, the is usually seen in older children, mainly girls. The
child appears to be quite ill but after a few hours the hands and wrists are often affected, but the classic
clinical condition improves again. Less constant deformities of rheumatoid arthritis are uncommon
features are lymphadenopathy, splenomegaly and and rheumatoid factor is usually absent. In some
hepatomegaly. Joint swelling occurs some weeks or cases, however, the condition is indistinguishable
months after the onset; fortunately, it usually resolves from adult rheumatoid arthritis, with a positive
when the systemic illness subsides but it may go on to rheumatoid factor test; these probably warrant the
progressive seronegative polyarthritis, leading to per- designation ‘juvenile rheumatoid arthritis’.
manent deformity of the larger joints and fusion of
the cervical apophyseal joints. By puberty there may
be stunting of growth, often abetted by the earlier use SERONEGATIVE SPONDYLOARTHROPATHY
of corticosteroids. In older children – usually boys – the condition may
take the form of sacroiliitis and spondylitis; hips and
knees are sometimes involved as well. Tests for HLA-
PAUCIARTICULAR JIA
B27 are often positive and this should probably be
This is by far the commonest form of JIA. It usually
regarded as ‘juvenile ankylosing spondylitis’.
occurs below the age of 6 years and is much more
common in girls; occasionally older children are
affected. Only a few joints are involved and there is no
systemic illness. The child presents with pain and
X-rays
swelling of medium-sized joints (knees, ankles, elbows In early disease non-specific changes such as soft-
and wrists); sometimes only one joint is affected. tissue swelling may be seen, but x-ray is mainly useful
Rheumatoid factor tests are negative but antinuclear to exclude other painful disorders. Later there may be
74 antibodies (ANA) may be positive. A serious signs of progressive joint erosion and deformity.
Investigations elbows may be unable to extend fully, and in the 3
spondylitic form of JIA the spine, hips and knees may
The white cell count and ESR are markedly raised in be almost rigid. Temporomandibular ankylosis and
systemic JIA, less so in the other forms. Rheumatoid stiffness of the cervical spine can make general anaes-
factor tests are positive only in juvenile RA. Joint aspi- thesia difficult and dangerous.
ration and synovial fluid examination may be essential
Growth defects There is a general retardation of

to exclude infection or haemarthrosis.
growth, aggravated by prolonged corticosteroid ther-
apy. In addition, epiphyseal disturbances lead to char-
Diagnosis acteristic deformities: external torsion of the tibia,
In the early stages, before chronic arthritis is fully dysplasia of the distal ulna, underdevelopment of the
established, diagnosis may be difficult. Systemic JIA mandible, shortness of the neck and scoliosis.
may start with an illness resembling a viral infection.
Fractures Children with chronic joint disease may suf-
Pauciarticular JIA, especially if only one joint is
fer osteoporosis and they are prone to fractures.
involved, is indistinguishable from Reiter’s disease or
septic arthritis (if the signs are acute) or tuberculous Iridocyclitis This is most common in ANA-positive
synovitis (if they are more subdued). pauciarticular disease; untreated it may lead to blind-
Other conditions that need to be excluded are rheu- ness.
matic fever, one of the bleeding disorders and leukaemia.
Amyloidosis In children with longstanding active dis-
In most cases the problem is resolved once the full
ease there is a serious risk of amyloidosis, which may
pattern of joint involvement is established, but blood
be fatal.
investigations, joint aspiration and synovial biopsy
may be required to clinch the diagnosis.
Prognosis
Treatment Fortunately, most children with JIA recover from the
arthritis and are left with only moderate deformity
General treatment Systemic treatment is similar to
and limitation of function. However, 5–10 per cent
that of rheumatoid arthritis, including the use of
(and especially those with juvenile rheumatoid arthri-
second-line drugs such as hydroxychloroquine, sul-
tis) are severely crippled and require treatment
fasalazine or low-dose methotrexate for those with
throughout life.
seropositive juvenile RA. Corticosteroids should be
A significant number of children with JIA (about
used only for severe systemic disease and for chronic
3 per cent) still die – usually as a result of renal failure
iridocyclitis unresponsive to topical therapy. Severe
due to amyloidosis, or following overwhelming
inflammatory disease may need to be treated with
infection.
cytokine inhibitors such as anti-TNF therapies.
Children and parents alike need sympathetic coun-
selling to help them cope with the difficulties of social
adjustments, education and training. CONNECTIVE TISSUE DISEASES
Local treatment The priorities are to prevent stiffness and
deformity. Night splints may be useful for the wrists, This term is applied to a group of closely related con-
hands, knees and ankles; prone lying for some period of ditions that have features which overlap with those of
each day may prevent flexion contracture of the hips. Be- rheumatoid arthritis. Like RA, these are ‘autoimmune
tween periods of splinting, active exercises are encour- disorders’, probably triggered by environmental expo-
aged; these are started by the physiotherapist but the par- sures, such as viral infections, in genetically predis-
ents must be taught how to continue the programme. posed individuals. They include systemic lupus
Fixed deformities may need correction by serial erythematosus, scleroderma, Sjögren’s syndrome,
plasters or by a spell in hospital on a continuous pas- polymyositis, dermatomyositis and a number of over-
sive motion (CPM) machine; when progress is no lap syndromes with features of more than one disease.
longer being made, joint capsulotomy may help. For
painful eroded joints, useful procedures include cus-
tom-designed arthroplasties of the hip and knee (even SYSTEMIC LUPUS ERYTHEMATOSUS (SLE)
in children), and arthrodesis of the wrist or ankle.
Systemic lupus occurs mainly in young females and
may be difficult to differentiate from RA. Although
Complications
joint pain is usual, it is often overshadowed by sys-
Ankylosis While most patients recover good function, temic symptoms such as malaise, anorexia, weight loss
some loss of movement is common. Hips, knees and and fever. Characteristic clinical features are skin 75
3 rashes (especially the ‘butterfly rash’ of the face), Ray- REFERENCES AND FURTHER READING
naud’s phenomenon, peripheral vasculitis,
splenomegaly, and disorders of the kidney, heart, Deighton CM, George E, Kiely PDW, Ledingham, J et al.
lung, eye and central nervous system. Anaemia, leu- Updating the British Society for Rheumatology guide-
copaenia and elevation of the ESR are common. Tests lines for anti-tumour necrosis factor therapy in adult
for ANA are usually positive. rheumatoid arthritis (again). Rheumatology 2006; 45:
Treatment Corticosteroids are indicated for severe 649–52.

systemic disease and may have to be continued for life. Kennedy T, McCabe C, Struthers G, et al. BSR guidelines
Progressive joint deformity is unusual and the arthri- on standards of care for persons with rheumatoid arthri-
tis can almost always be controlled. tis. Rheumatology 2005; 44: 553–6
Manadan AM, James N, Block JA. New therapeutic
Complications A curious complication of SLE is avas-
approaches for spondyloarthritis. Curr Opin Rheumatol
cular necrosis (usually of the femoral head). This may 2007; 19: 259–64.
be due in part to the corticosteroid treatment, but the Scott DL, Kingsley GH. Tumor necrosis factor inhibitors
disease itself seems to predispose to bone ischaemia, for rheumatoid arthritis. N Engl J Med 2006; 355: 704–
possibly as a manifestation of the antiphospholipid 12.
(Hughes) syndrome which sometimes accompanies Sidiropoulos PI, Hatemi G, Song M, et al. Evidence-
SLE. based recommendations for the management of ankylos-
ing spondylitis: systematic literature search of the 3E
Initiative in Rheumatology involving a broad panel of
experts and practising rheumatologists. Rheumatology
2008; 47: 355–61.
76
Crystal deposition
disorders 4
Louis Solomon
The crystal deposition disorders are a group of condi- Pathology

tions characterized by the presence of crystals in and
around joints, bursae and tendons. Although many Hyperuricaemia Nucleic acid and purine metabolism
different crystals are found, three clinical conditions normally proceeds, through complex pathways, to the
in particular are associated with this phenomenon: production of hypoxanthine and xanthine; the final
breakdown to uric acid is catalysed by the enzyme
• gout xanthine oxidase. Monosodium urate appears in ionic
• calcium pyrophosphate dihydrate (CPPD) deposi- form in all the body fluids; about 70 per cent is
tion disease derived from endogenous purine metabolism and 30
• calcium hydroxyapatite (HA) deposition disorders. per cent from purine-rich foods in the diet. It is
Characteristically, in each of the three conditions, excreted (as uric acid) mainly by the kidneys and
crystal deposition has three distinct consequences: (1) partly in the gut.
it may be totally inert and asymptomatic; (2) it may Urate is poorly soluble, with a plasma saturation
induce an acute inflammatory reaction; or (3) it may value of only 7 mg/dL (0.42 mmol/L). This concen-
result in slow destruction of the affected tissues. tration is commonly exceeded in normal individuals
and epidemiological studies have identified entire
populations (for example the Maoris of New Zealand)
who have unusually high levels of serum uric acid.
GOUT The term ‘hyperuricaemia’ is therefore generally
reserved for individuals with a serum urate concentra-
tion which is significantly higher than that of the pop-
Gout is a disorder of purine metabolism characterized
ulation to which they belong (more than two
by hyperuricaemia, deposition of monosodium urate
standard deviations above the mean); this is about
monohydrate crystals in joints and peri-articular tis-
0.42 mmol/L for men and 0.35 mmol/L for women
sues and recurrent attacks of acute synovitis. Late
in western Caucasian peoples. By this definition,
changes include cartilage degeneration, renal dysfunc-
about 5 per cent of men and less than 1 per cent of
tion and uric acid urolithiasis.
women have hyperuricaemia; the majority suffer no
The clinical disorder was known to Hippocrates
pathological consequences and they remain asympto-
and its association with hyperuricaemia was recog-
matic throughout life.
nized well over 100 years ago. The prevalence of
symptomatic gout varies from 1 to over 10 per 1000, Gout Urate crystals are deposited in minute clumps
depending on the race, sex and age of the population in connective tissue, including articular cartilage; the
studied: it is much commoner in Caucasian than in commonest sites are the small joints of the hands and
Negroid peoples; it is more widespread in men than in feet. For months, perhaps years, they remain inert.
women (the ratio may be as high as 20:1); and it is Then, possibly as a result of local trauma, the needle-
rarely seen before the menopause in females. like crystals are dispersed into the joint and the sur-
Although the risk of developing clinical features of rounding tissues where they excite an acute
gout increases with increasing levels of serum uric inflammatory reaction. Individual crystals may be
acid, only a fraction of those with hyperuricaemia phagocytosed by synovial cells and polymorphs or
develop symptoms. However, ‘hyperuricaemia’ and may float free in the synovial fluid.
‘gout’ are generally regarded as part and parcel of the With the passage of time, urate deposits may build
same disorder. up in joints, peri-articular tissues, tendons and bursae;
4 Clinical features
Patients are usually men over the age of 30 years;
women are seldom affected until after the menopause.
Often there is a family history of gout.
The gouty stereotype is obese, rubicund, hyperten-
sive and fond of alcohol. However, many patients

have none of these attributes and some are nudged
into an attack by the uncontrolled administration of
diuretics or aspirin.
THE ACUTE ATTACK

The sudden onset of severe joint pain which lasts for a
4.1 Gout - pathology Histological section through a week or two before resolving completely is typical of
gouty MTP joint, showing the urate tophus occupying a acute gout. The attack usually comes out of the blue
cavity in the articular surface. but may be precipitated by minor local trauma, opera-
tion, intercurrent illness, unaccustomed exercise or
alcohol consumption. The commonest sites are the
Table 4.1 Some factors predisposing to
hyperuricaemia metatarsophalangeal joint of the big toe, the ankle and
finger joints, and the olecranon bursa. Occasionally,
Older age, male gender more than one site is involved. The skin looks red and
Genetic enzyme defects, hyperparathyroidism
shiny and there is considerable swelling. The joint feels
hot and extremely tender, suggesting a cellulitis or sep-
Haemolytic disorders, myeloproliferative disorders tic arthritis. Sometimes the only feature is acute pain
Obesity, diabetes, hypertension and tenderness in the heel or the sole. Hyperuricaemia
High consumption of red meat, hyperlipidaemia is present at some stage, though not necessarily during
Chronic inflammatory diseases
an acute attack. However, while a low serum uric acid
makes gout unlikely, hyperuricaemia is not ‘diagnostic’
Long-term use of aspirin or diuretics and is often seen in normal middle-aged men.
Alcohol abuse
common sites are around the metatarsophalangeal

joints of the big toes, the Achilles tendons, the ole-
cranon bursae and the pinnae of the ears. These
clumps of chalky material, or tophi (L. tophus =
porous stone), vary in size from less than 1 mm to
several centimetres in diameter. They may ulcerate
through the skin or destroy cartilage and peri-articu-
lar bone. (a)
Classification
Gout is often classified into ‘primary’ and ‘secondary’
forms. Primary gout (95 per cent) occurs in the
absence of any obvious cause and may be due to con-
stitutional under-excretion (the vast majority) or over-
production of urate. Secondary gout (5 per cent)
results from prolonged hyperuricaemia due to
acquired disorders such as myeloproliferative diseases,
administration of diuretics or renal failure.
This division is somewhat artificial; people with an (b) (c)
initial tendency to ‘primary’ hyperuricaemia may
develop gout only when secondary factors are intro- 4.2 Gout (a) This is the typical ‘gouty type’, with his
rubicund face, large olecranon bursae and small
duced – for example obesity, alcohol abuse, or treat- subcutaneous tophi over the elbows. (b,c) Tophaceous
ment with diuretics or salicylates which increase gout affecting the hands and feet; the swollen big toe joint
78 tubular reabsorption of uric acid. is particularly characteristic.
The true diagnosis can be established beyond doubt This may present with acute pain and
Reiter’s disease 4
by finding the characteristic negatively birefringent urate swelling of a knee or ankle, but the history is more
crystals in the synovial fluid. A drop of fluid on a glass protracted and the response to anti-inflammatory
slide is examined by polarizing microscopy. Crystals may drugs less dramatic.
be sparse but if the fluid specimen is centrifuged a con-
Pseudogout Pyrophosphate crystal deposition may
centrated pellet may be obtained for examination.
cause an acute arthritis indistinguishable from gout –
Crystal deposition disorders

except that it tends to affect large rather than small
CHRONIC GOUT
joints and is somewhat more common in women than
Recurrent acute attacks may eventually merge into
in men. Articular calcification may show on x-ray.
polyarticular gout. Joint erosion causes chronic pain,
Demonstrating the crystals in synovial fluid establishes
stiffness and deformity; if the finger joints are
the diagnosis.
affected, this may be mistaken for rheumatoid arthri-
tis. Tophi may appear around joints over the olecra- Rheumatoid arthritis (RA) Polyarticular gout affecting
non, in the pinna of the ear and – less frequently – in the fingers may be mistaken for rheumatoid arthritis,
almost any other tissue. A large tophus can ulcerate and elbow tophi for rheumatoid nodules. In difficult
through the skin and discharge its chalky material. cases biopsy will establish the diagnosis. RA and gout
Renal lesions include calculi, due to uric acid precipi- seldom occur together.
tation in the urine, and parenchymal disease due to
deposition of monosodium urate from the blood.
X-rays
During the acute attack x-rays show only soft-tissue
swelling. Chronic gout may result in joint space nar-
rowing and secondary osteoarthritis. Tophi appear as
characteristic punched-out ‘cysts’ or deep erosions in
the para-articular bone ends; these excavations are
larger and slightly further from the joint margin than
the typical rheumatoid erosions. Occasionally, bone
destruction is more marked and may resemble neo-
plastic disease (see Fig. 9.1).
(a)
Differential diagnosis
Infection Cellulitis, septic bursitis, an infected bunion
or septic arthritis must all be excluded, if necessary by
immediate joint aspiration. Remember that crystals
and sepsis may coexist, so always send fluid for both
culture and crystal analysis.
(b)
4.4 Crystals In polarized light, crystals appear bright on a
dark background. If a compensator is added to the optical
system, the background appears in shades of mauve and
birefringent crystals as yellow or blue, depending on their
spatial orientation. In these two specimens (obtained from
crystal deposits in cartilage) there are differences in shape,
size and type of birefringence of the crystals. (a) Urate
crystals are needle-like, 5–20 μm long and exhibit strong
negative birefringence. (b) Pyrophosphate crystals are
4.3 Gout – x-rays The typical picture is of large rhomboid-shaped, slightly smaller than urate crystals and
periarticular excavations – tophi consisting of uric acid show weak positive birefringence. (Courtesy of Professor P.
deposits. A. Dieppe). 79
4 Treatment calcific material in articular cartilage and menisci;
(2) pseudogout – a crystal-induced synovitis; and
The acute attack The acute attack should be treated (3) chronic pyrophosphate arthropathy – a type of
by resting the joint, applying ice packs if pain is severe, degenerative joint disease. Any one of these condi-
and giving full doses of a non-steroidal anti-inflam- tions may occur on its own or in any combination
matory drug (NSAID). Colchicine, one of the oldest with the others (Dieppe et al., 1982). In contrast to
of medications, is less effective and may cause diar- classic gout, serum biochemistry shows no consistent
rhoea, nausea and vomiting. A tense joint effusion abnormality.
may require aspiration and intra-articular injection of CPPD crystal deposition is known to occur in
corticosteroids. Oral corticosteroids are sometimes certain metabolic disorders (e.g. hyperparathyroidism
used for patients who cannot tolerate NSAIDs or in and haemochromatosis) that cause a critical change in
whom NSAIDs are contraindicated. The sooner treat- ionic calcium and pyrophosphate equilibrium in
ment is started the sooner is the attack likely to end. cartilage. The rare familial forms of chondrocalcinosis
Interval therapy Between attacks, attention should be are probably due to a similar biochemical defect.
given to simple measures such as losing weight, cut- However, in the vast majority of cases chondrocalci-
ting out alcohol and eliminating diuretics. Urate-low- nosis follows some local change in the cartilage due
ering drug therapy is indicated if acute attacks recur at to ageing, degeneration, enzymatic degradation or
frequent intervals, if there are tophi or if renal function trauma.
is impaired. It should also be considered for asympto-
matic hyperuricaemia if the plasma urate concentration Pathology
is persistently above 6 mg/dL (0.36 mmol/L). How-
The incidence of CPPD arthropathy rises with
ever, one must remember that this starts a life-long
increasing age; men and women are equally affected
commitment and many clinicians feel that people who
and in some cases the disease runs in families
have never had an attack of gout and are free of tophi
Pyrophosphate is probably generated in abnormal
or urinary calculi do not need treatment.
cartilage by enzyme activity at chondrocyte surfaces; it
Uricosuric drugs (probenecid or sulfinpyrazone)
combines with calcium ions in the matrix where crys-
can be used if renal function is normal. However,
tal nucleation occurs on collagen fibres. The crystals
allopurinol, a xanthine oxidase inhibitor, is usually
grow into microscopic ‘tophi’, which appear as nests
preferred, and for patients with renal complications or
of amorphous material in the cartilage matrix.
chronic tophaceous gout allopurinol is definitely the
Chondrocalcinosis is most pronounced in fibrocarti-
drug of choice.
laginous structures (e.g. the menisci of the knee, tri-
Urate-lowering drugs should never be started before
angular ligament of the wrist, pubic symphysis and
the acute attack has completely subsided, and they should
intervertebral discs) but may also occur in hyaline
always be covered by an anti-inflammatory preparation
articular cartilage, tendons and peri-articular soft tis-
or colchicine, otherwise they may actually prolong or pre-
sues. From time to time CPPD crystals are extruded
cipitate an acute attack. Patients who suffer an acute
into the joint where they excite an inflammatory reac-
attack of gout while already on a constant dose of
tion similar to gout. The longstanding presence of
urate-lowering treatment should be advised to con-
CPPD crystals also appears to influence the develop-
tinue taking the drug at the usual dosage while the
ment of osteoarthritis in joints not usually prone to
acute episode is being treated.
this condition (e.g. shoulders, elbows and ankles).
Surgery With prolonged urate-lowering therapy, Characteristically, there is a hypertrophic reaction
adjusted to maintain a normal serum uric acid level with marked osteophyte formation. Synovitis is more
(less than 0.36 mmol/L), tophi may gradually dis- obvious than in ‘ordinary’ osteoarthritis.
solve. However, ulcerating tophi that fail to heal with
conservative treatment can be evacuated by curettage; Clinical features
the wound is left open and dressings are applied until
it heals. The clinical disorder takes several forms, all of them
appearing with increasing frequency in relation to age.
Asymptomatic chondrocalcinosis Calcification of the
CALCIUM PYROPHOSPHATE menisci is common in elderly people and is usually
DIHYDRATE ARTHROPATHY asymptomatic. When it is seen in association with
osteoarthritis, this does not necessarily imply cause
(PSEUDOGOUT) and effect. Both are common in elderly people and
they are bound to be seen together in some patients;
‘CPPD deposition’ encompasses three overlapping x-rays may reveal chondrocalcinosis in other, asymp-
80 conditions: (1) chondrocalcinosis – the appearance of tomatic, joints. Chondrocalcinosis in patients under
4

(a)
(b) (c) (d)
4.5 Chondrocalcinosis and pyrophosphate arthropathy Calcium pyrophosphate crystals may be deposited in cartilage,
causing (a) calcification of menisci and (b) a thin, dense line within the articular cartilage. (c,d) Chronic calcium
pyrophosphate arthropathy, on the other hand, is much more serious, as seen in this man who presented with osteoarthritis
in several of the larger joints, including unusual sites such as the elbow and ankle. X-ray of the right knee showed the
characteristic features of articular calcification, loose bodies in the joint and large trailing osteophytes around the
patellofemoral joint.
50 years of age should suggest the possibility of an

underlying metabolic disease or a familial disorder. GOUT AND PSEUDOGOUT
Acute synovitis (pseudogout) The patient, typically a GOUT PSEUDOGOUT

middle-aged woman, complains of acute pain and Smaller joints Large joints
swelling in one of the larger joints – usually the knee.
Sometimes the attack is precipitated by a minor illness Pain intense Pain moderate
or operation. The joint is tense and inflamed, though Joint inflamed Joint swollen
usually not as acutely as in gout. Untreated the con-
dition lasts for a few weeks and then subsides sponta- Hyperuricaemia Chondrocalcinosis
neously. X-rays may show signs of chondrocalcinosis, Uric acid crystals Ca pyrophosphate crystals
and the diagnosis can be confirmed by finding posi-
tively birefringent crystals in the synovial fluid.
Chronic pyrophosphate arthropathy The patient, usually
an elderly woman, presents with polyarticular
‘osteoarthritis’ affecting the larger joints (hips, knees)
and – more helpfully – unusual joints, such as the Calcification is usually seen in and around the
ankles, shoulders, elbows and wrists where osteoarthri- knees, wrists, shoulders, hips, pubic symphysis and
tis is seldom seen. There are the usual features of pain, intervertebral discs; it is often bilateral and symmetri-
stiffness, swelling, joint crepitus and loss of movement. cal. In articular cartilage it appears as a thin line paral-
It is often diagnosed, simply, as ‘generalized lel to the joint. In the fibrocartilaginous menisci and
osteoarthritis’, but the x-ray features are distinctive. discs it produces cloudy, irregular opacities. Less com-
Sometimes alternating bouts of acute synovitis and mon sites are the joint synovium, capsule, ligaments,
chronic arthritis may mimic rheumatoid disease. tendons and bursae.
Degenerative changes are similar to those of
straightforward osteoarthritis but notably involving
X-rays
unusual sites such as the non-weightbearing joints,
The characteristic x-ray features arise from a combina- the isolated patellofemoral compartment in the knee
tion of (1) intra-articular and peri-articular calcifica- and the talonavicular joint in the foot. In advanced
tion, and (2) degenerative arthritis in distinctive sites cases joint destruction may be marked, with the for-
(Resnick and Resnick, 1983). mation of loose bodies. 81
4 Diagnosis
THE ACUTE ATTACK
‘Pseudogout’ must be distinguished from other acute
inflammatory disorders.
Acute gout usually occurs in men, and typically in
smaller joints or in the olecranon bursa. The final

word often lies with joint aspiration and identification
of the characteristic crystals.
Post-traumatic haemarthrosis can be misleading;
pseudogout is often precipitated by trauma. A clear
history and aspiration of blood-stained fluid will solve
the problem.
Septic arthritis must not be missed; a delay of 24
hours can mean the difference between successful and (a) (b)
unsuccessful treatment. Systemic features are more
4.6 Haemochromatosis and alkaptonuria
evident, but blood tests and joint aspiration are essen- (a) Haemochromatosis: the degenerative arthritis of the
tial to clinch the diagnosis; joint fluid should be sub- proximal finger joints is typical. (b) Alkaptonuria: the
mitted with a request for both crystal analysis and intervertebral discs are calcified – this man has backache.
bacteriological culture.
Reiter’s disease can start in a single large joint;
always enquire about (and look for) signs of conjunc-
tivitis, urethritis and colitis.
ized by the appearance of homogentisic acid in the
CHRONIC CPPD ARTHROPATHY urine, dark pigmentation of the connective tissues
Chronic pyrophosphate arthropathy usually affects (ochronosis) and calcification of hyaline and fibrocarti-
multiple joints and it has to be distinguished from lage. The inborn error is an absence of homogentisic
other types of polyarticular arthritis. acid oxidase in the liver and kidney. Those affected
Osteoarthritis and joint calcification are both com- usually remain asymptomatic until the third or fourth
mon in older people; the two together do not neces- decade when they present with pain and stiffness of
sarily make it a CPPD arthropathy. The distinctive the spine and (later) larger joints. There may also be
x-ray features, and especially the involvement of dark pigmentation of the ear cartilage and the sclerae,
unusual joints (the elbow, wrist and ankle), point to a and clothes may become stained by homogentisic
CPPD disorder rather than a simple concurrence of acid in the sweat. X-rays reveal narrowing and calcifi-
two common conditions. cation of the intervertebral discs at multiple levels,
Inflammatory polyarthritis usually involves the and spinal osteoporosis. At a later stage the large
smaller joints as well, and systemic features of inflam- peripheral joints may show chondrocalcinosis and
mation are more marked. severe osteoarthritis. The feature which gives the
Metabolic disorders such as hyperparathyroidism, condition its name is that the urine turns dark brown
haemochromatosis and alkaptonuria may be associ- when it is alkalinized or if it is left to stand for some
ated with calcification of articular cartilage and hours.
fibrocartilage as well as joint symptoms. It is impor- Hyperparathyroidism is described on page 140.
tant to exclude such generalized disorders before
labelling a patient as ‘just another case of chondro-
Treatment
calcinosis’.
Haemochromatosis is an uncommon disorder of The treatment of pseudogout is the same as that of
middle-aged people (usually men), resulting from acute gout: rest and high-dosage anti-inflammatory
chronic iron overload. The clinical features are those therapy. In elderly patients, joint aspiration and
of cirrhosis and diabetes, with a typical bronze pig- intra-articular corticosteroid injection is the treat-
mentation of the skin. About half of the patients ment of choice as these patients are more vulnerable
develop joint symptoms (particularly in the hands to the side effects of non-steroidal anti-inflammatory
and fingers); some also have chronic backache. X- drugs.
rays reveal chondrocalcinosis and a destructive Chronic chondrocalcinosis appears to be irreversible.
arthropathy, typically in the metacarpophalangeal Fortunately it usually causes few symptoms and little
joints. The plasma iron and iron-binding capacity are disability. When it is associated with progressive joint
raised. degeneration the treatment is essentially that of
82 Alkaptonuria is a rare, heritable disorder character- advanced osteoarthritis.
BASIC CALCIUM PHOSPHATE 4
CRYSTAL DEPOSITION DISEASE
Basic calcium phosphate (BCP) is a normal compo-

nent of bone mineral, in the form of calcium hydrox-

yapatite crystals. It also occurs abnormally in dead or
damaged tissue. Minute deposits in joints and peri-
articular tissues can give rise to either an acute reac-
tion (synovitis or tendinitis) or a chronic, destructive
arthropathy.
(a) (b)
Prolonged hypercalcaemia or hyperphosphataemia,
of whatever cause, may result in widespread metasta- 4.7 Acute calcification of supraspinatus (a) Dense
tic calcification. However, by far the most common mass in the tendon. (b) Following the ‘reaction’ some
cause of BCP crystal deposition in and around joints calcium has escaped into the subdeltoid bursa.
is local tissue damage – strained or torn ligaments,
tendon attrition and cartilage damage or degenera- At other times the onset is more gradual and it is eas-
tion. ier to localize the area of tenderness to one of the peri-
articular structures. Both forms of the condition are
Pathology seen most commonly in rotator cuff lesions of the
shoulder. Symptoms usually subside after a few weeks
The minute (less than 1 mm) BCP crystals are
or months; sometimes they are aborted only when the
deposited around chondrocytes in articular cartilage
calcific deposit is removed or the surrounding tissues
and in relatively avascular or damaged parts of ten-
are decompressed. In acute cases, operation may
dons and ligaments – most notably around the shoul-
der and knee. The deposits grow by crystal accretion
and eventually may be detectable by x-ray in the peri-
articular tendons or ligaments. Calcification of the
posterior longitudinal ligament of the cervical spine
may also be associated with BCP crystal deposition.
Sometimes the calcific deposit has a creamy consis-
tency but in longstanding cases it is more like chalk.
The mini-tophus may be completely inert, but in
symptomatic cases it is surrounded by an acute vascu-
lar reaction and inflammation. Crystal shedding into
joints may give rise to synovitis. More rarely this is (a) (b)
complicated by the development of a rapidly destruc- 4.8 BCP destructive arthropathy (a) Knee joint exposed
tive, erosive arthritis. Bits of articular cartilage and at operation. The articular surface is severely eroded.
bone or fragments of a meniscus may be found in the (b) Fragments of meniscus. Note the white crystalline
synovial cavity. material on the large meniscal fragment.
Clinical features
Two clinical syndromes are associated with BCP crys-
tal deposition: (1) an acute or subacute peri-arthritis;
and (2) a chronic rapidly destructive arthritis.
ACUTE OR SUBACUTE PERI-ARTHRITIS

This is by far the commonest form of BCP crystal dep-
osition disorder affecting joints. The patient, usually an
adult between 30 and 50 years, complains of pain close
to one of the larger joints – most commonly the shoul- (a) (b)
der or the knee. Symptoms may start suddenly, perhaps
after minor trauma, and rise to a crescendo during 4.9 Rapidly destructive OA X-rays of two patients with
rapidly destructive OA of a large joint, (a) the hip in one
which the tissues around the joint are swollen, warm and (b) the shoulder in the other. Common features are
and exquisitely tender – but tender near the joint in re- rapid progression to joint disruption, crumbling of the
lation to a tendon or ligament, rather than in the joint. sub-articular bone and peri-articular ossification. 83
4 disclose a tense globule of creamy material oozing polymorphonuclear leucocytes, but this hardly serves
from between the frayed fibres of tendon or ligament. to distinguish the condition from other types of sub-
acute synovitis. BCP crystals are too small to be seen
CHRONIC DESTRUCTIVE ARTHRITIS by light microscopy but can be identified by electron
BCP crystals are sometimes found in association with probe or transmission electron microscopy.
a chronic erosive arthritis; whether they cause the
arthritis or modify a pre-existing disorder remains Treatment

uncertain.
A more dramatic type of rapidly destructive arthri- Acute peri-arthritis should be treated by rest and non-
tis of the shoulder is occasionally seen in elderly steroidal anti-inflammatory drugs. Resistant cases may
patients with rotator cuff lesions. This was described respond to local injection of corticosteroids; this
in 1981 by McCarty and his colleagues from Milwau- treatment should be used only to weather the acute
kee and acquired the sobriquet ‘Milwaukee shoulder’. storm – repeated injections for lesser pain may
Similar conditions affect the hip and knee. They have dampen the repair process in damaged tendons or lig-
been attributed to BCP crystal (or mixed BCP and aments and thus predispose to recurrent attacks. Per-
CPPD crystal) shedding into the joint. sistent pain and tenderness may call for operative
removal of the calcific deposit or ‘decompression’ of
the affected tendon or ligament.
X-rays Erosive arthritis is treated like osteoarthritis. How-
With peri-arthritis, calcification may be seen in ten- ever, rapidly progressive bone destruction calls for
dons or ligaments close to the joint, most commonly early operation: in the case of the shoulder, synovec-
in the rotator cuff around the shoulder. tomy and soft-tissue repair; for the hip, usually total
Articular cartilage and fibrocartilaginous menisci and joint replacement.
discs never show the type of calcification seen in CPPD
deposition disease, but ‘loose bodies’ may be seen in
synovial joints. Erosive arthritis causes loss of the artic-
ular space, with little or no sclerosis or osteophyte for- REFERENCES AND FURTHER READING
mation. The typical picture of rapidly destructive
arthritis is one of severe erosion and destruction of sub- Dieppe PA, Alexander GJM., Jones HE, et al. Pyrophos-
chondral bone. In advanced cases the joint may phate arthropathy: a clinical and radiological study of 105
become unstable and, eventually, dislocated. cases. Ann Rheum Dis 1982; 41: 371–6.
McCarty DJ, Halverson PB, Carrera GF, et al. ‘Milwaukee
shoulder’ – association of microspheroids containing
Investigations hydroxyapatite crystals, active collagenase and neutral
There is little help from special investigations. Serum protease with rotator cuff defects. Arth Rheum 1981; 24:
biochemistry is usually normal, except in those 464–73.
patients with hypercalcaemia or hyperphosphataemia. Resnick CS, Resnick D. Crystal deposition disease. Semin
Synovial fluid examination may reveal high counts of Arthritis Rheum 1983; 12: 390–403.
84
Osteoarthritis
5
Louis Solomon
THE PHYSIOLOGY OF SYNOVIAL

Muscle
JOINTS
ARTICULAR CARTILAGE
Hyaline cartilage, the pearly gristle which covers the
bone ends in every diarthrodeal joint, is supremely Tendon
adapted to transmit load and movement from one
skeletal segment to another. It increases the area of Joint capsule
the articular surfaces and helps to improve their
adaptability and stability; it changes its shape under Synovium
load and distributes compressive forces widely to the
subarticular bone; and, covered by a film of synovial
fluid, it is more slippery than any man-made material, Articular cartilage
offering very little frictional resistance to movement
and surface gliding. Subchondral bone
This specialized connective tissue has a gel-like
matrix consisting of a proteoglycan ground substance Cancellous bone
in which are embedded an architecturally structured
collagen network and a relatively sparse scattering of
specialized cells, the chondrocytes, which are respon-
sible for producing all the structural components of 5.1 Diagram showing the components of a synovial
the tissue. It has a high water content (60–80 per joint
cent), most of which is exchangeable with the synovial
fluid.
Chondrocytes of adult hyaline cartilage have little
capacity for cell division in vivo and direct damage to Hundreds of aggrecan molecules are linked, in turn,
the articular surface is poorly repaired, or repaired to a long unbranched hyalurinate chain (hyaluronan),
only with fibrocartilage. The fact that the normal wear to form an even larger molecule with a molecular
of daily joint activity does not result in degradation of weight of over 100 million daltons. These negatively
the articular surface is due to the highly effective charged macromolecules are responsible for the stiff-
lubricating mechanisms bestowed by synovial fluid. In ness and springiness of articular cartilage.
another sense, though, chondrocytes do undertake The fibrillar component of articular cartilage is
repair: in the early stages of cartilage degradation, mainly type II collagen. The collagen bundles are
matrix molecular constituents will be replenished by arranged in structured patterns, parallel to the articu-
increased chondrocyte activity. lar surface in the superficial zones and perpendicular
The proteoglycans exist mainly in the form of to the surface in the deeper layers where they anchor
aggrecan, a large aggregating molecule with a protein the articular cartilage to the subchondral bone.
core along which are arranged up to 100 chondroitin The main functions of aggrecan are to absorb
sulphate and keratan sulphate glycosaminoglycans changes in load and mitigate deformation, while the
(GAGs), rather like the bristles on a bottlebrush. collagen network copes with tensile forces. There is
5 considerable interaction between the molecules of each muscles, help to provide stability. The ligaments run-
component and between the molecules of the different ning from one bone to another are inelastic and have
components of cartilage: if these links are degraded or a fixed length. Not surprisingly, therefore, they are
broken, the cartilage will tend to unravel. This happens under different degrees of tension in different posi-
to some degree with ageing, but much more so in tions of the joint. When the joint assumes a position
pathological states leading to osteoarthritis. where the ligaments are fully taut, they provide maxi-
Proteoglycan has a strong affinity for water, result- mum stability and may keep the joint ‘locked’ even
ing in the collagen network being subjected to con- without the assistance of muscles; when less taut they
siderable tensile stresses. With loading, the cartilage permit a certain degree of laxity in the joint; and when
deforms and water is slowly squeezed onto the surface they are overstretched or torn the joint becomes
where it helps to form a lubricating film. When load- unstable.
ing ceases, the surface fluid seeps back into the carti- Non-pathological ligamentous laxity is a fairly com-
lage up to the point where the swelling pressure in the mon heritable trait which is employed to astonishing
cartilage is balanced by the tensile force of the colla- (and sometimes bizarre) effect by acrobatic perform-
gen network. As long as the network holds and the ers; stability is maintained by highly developed muscle
proteoglycans remain intact, cartilage retains its com- power and the articular cartilage is not necessarily
pressibility and elasticity. If the collagen network is damaged.
degraded or disrupted, the matrix becomes water- Inflamed or injured joints that need splinting
logged and soft; this, in turn, is followed by loss of should always be held in the position where the liga-
proteoglycans, cellular damage and splitting (‘fibrilla- ments are fully taut; if the ligaments are allowed to
tion’) of the articular cartilage. Trouble mounts up fibrose and shorten in the ‘relaxed’ position it may
further as the damaged chondrocytes begin to release take months (or be impossible) to regain full passive
matrix-degrading enzymes. movement afterwards.
THREATS TO CARTILAGE INTEGRITY

Synovium and synovial fluid
The interior surface of the capsule is lined by a thin
Loss of joint stability membrane, the synovium, which is richly supplied with
Localized increase in loading stress blood vessels, lymphatics and nerves. It provides a non-
adherent covering for the articular surfaces and it pro-
Increased stiffness of the cartilage duces synovial fluid, a viscous plasma dialysate laced
Inflammatory (enzymatic) degradation with hyaluronan. This fluid nourishes the avascular
articular cartilage, plays an important part in reducing
Restriction of free joint movement friction during movement and has slight adhesive prop-
Sclerosis in the subchondral bone erties which assist in maintaining joint stability.
In normal life the volume of synovial fluid in any
particular joint remains fairly constant, regardless of
movement. When a joint is injured fluid increases (as
in any bruised or oedematous connective tissue) and
this appears as a joint effusion. Synovium is also the
target tissue in joint infections and autoimmune dis-
orders such as rheumatoid arthritis.
MECHANISMS FOR MAINTAINING

JOINT STABILITY
5.2 Normal articular cartilage Normal articular cartilage,
smooth and glistening, is well preserved into old age. Alignment of joint components
These specimens were taken from elderly patients with
fractures of the femoral neck. Shape and fit of articular surfaces
Adhesive property of synovial fluid
CAPSULE AND LIGAMENTS Integrity of capsule and ligaments
Muscle tone and power

The soft tissues enclosing the joint consist of a fibrous
capsule with tough condensations on its surface – the Neurological control of balance
86 ligaments – which, together with the overlying
Joint lubrication 5
The coefficient of friction in the normal joint is
extremely low – one reason why, barring trauma or
disease, there is little difference in the amount of wear
on articular surfaces between young adults and old
Osteoarthritis
people. This extraordinary slipperiness of cartilage
surfaces is produced by a highly efficient combination
of lubricating systems.
Boundary layer lubrication at the bearing surfaces is
mediated by a large, water soluble glycoprotein frac-
(a) (b)
tion, lubricin, in the viscous synovial fluid. A single
layer of molecules attaches to each articular surface 5.3 Osteoarthritis: non-progressive and progressive
and these glide upon each other in a manner that has (a) Non-progressive OA changes are common in older
people; here we see them along the inferomedial edge of
been likened to surfaces rolling on miniscule ball-
the femoral head, while the articular cartilage over the rest
bearings. This is most effective at points of direct con- of the head looks perfect. (b) Progressive OA changes are
tact. seen characteristically in the maximal load-bearing area; in
Fluid film lubrication is provided by the hydrody- the hip this is the superior part of the joint. Articular
namic mechanism described earlier (see under Articu- cartilage has been destroyed, leaving a bald patch on the
dome of the femoral head.
lar cartilage). During movement and loading fluid is
squeezed out of the proteoglycan-rich cartilage and
forms a thin ‘cushion’ where contact is uneven, then areas of osteonecrosis in the subchondral bone; the
seeps back into the cartilage when loading ceases. appearance of joint instability; and the effects of pro-
Lubrication between synovial folds is provided by longed anti-inflammatory medication.
hyalurinate molecules in the synovial fluid.
Aetiology
The most obvious thing about OA is that it increases
OSTEOARTHRITIS in frequency with age. This does not mean that OA is
simply an expression of senescence. Cartilage does
Osteoarthritis (OA) is a chronic disorder of synovial ‘age’, showing diminished cellularity, reduced proteo-
joints in which there is progressive softening and dis- glycan concentration, loss of elasticity and a decrease
integration of articular cartilage accompanied by new in breaking strength with advancing years. These fac-
growth of cartilage and bone at the joint margins tors may well predispose to OA, but it is significant
(osteophytes), cyst formation and sclerosis in the sub- that the progressive changes which are associated with
chondral bone, mild synovitis and capsular fibrosis. It clinical and radiological deterioration are restricted to
differs from simple wear and tear in that it is asym- certain joints, and to specific areas of those joints,
metrically distributed, often localized to only one part while other areas show little or no progression with
of a joint and often associated with abnormal loading age (Byers et al., 1970).
rather than frictional wear. Primary changes in cartilage matrix might (theoret-
In its most common form, it is unaccompanied by ically) weaken its structure and thus predispose to car-
any systemic illness and, although there are sometimes tilage breakdown; crystal deposition disease and
local signs of inflammation, it is not primarily an ochronosis are well-known examples.
inflammatory disorder. ‘Inheritance’ has for many years been thought to
It is also not a purely degenerative disorder, and the play a role in the development of OA. A number of
term ‘degenerative arthritis’ – which is often used as a studies have demonstrated a significant increase in the
synonym for OA – is a misnomer. Osteoarthritis is a prevalence of generalized OA in first-degree relatives
dynamic phenomenon; it shows features of both of patients with OA as compared with controls (Kell-
destruction and repair. Cartilage softening and disin- gren, 1963) and others have published similar obser-
tegration are accompanied from the very outset by vations for OA of the hip (Lanyon et al., 2000).
hyperactive new bone formation, osteophytosis and However, one should bear in mind that OA of large
remodelling. The final picture is determined by the joints is often attributable to anatomical variations,
relative vigour of these opposing processes. In addi- e.g. acetabular dysplasia and other forms of epiphyseal
tion, there are various secondary factors which influ- dysplasia, and it is these that are inherited rather than
ence the progress of the disorder: the appearance of any tendency to develop OA as a primary abnormal-
calcium-containing crystals in the joint; ischaemic ity. At the molecular level, genetic defects in type II
changes (especially in elderly people) which result in collagen have been demonstrated in some cases 87
5 (Palotie et al., 1989; Knowlton et al., 1990), but it is Collagen
unlikely that this is a major aetiological factor in the failure
majority of cases.
Articular cartilage may be damaged by trauma or
previous inflammatory disorders. Enzymes released by Release of Proteoglycan Cartilage
synovial cells and leucocytes can cause leaching of proteolytic enzymes matrix depletion deformation
proteoglycans from the matrix, and synovial-derived

interleukin-1 (IL-1) may suppress proteoglycan syn-
Chondrocyte
thesis. This could explain the appearance of ‘sec- damage
ondary’ OA in patients with rheumatoid diseases;
whether similar processes operate in ‘primary’ (‘idio- 5.4 The cycle of articular cartilage deformation and
pathic’) OA is unknown. collagen failure
In most cases the precipitating cause of OA is
increased mechanical stress in some part of the articu-
lar surface. This may be due to increased load (e.g. in defects appear in the cartilage. As the cartilage
deformities that affect the lever system around a joint) becomes less stiff, secondary damage to chondrocytes
or to a reduction of the articular contact area (e.g. may cause release of cell enzymes and further matrix
with joint incongruity or instability). Both factors breakdown. Cartilage deformation may also add to
operate in varus deformity of the knee and in acetab- the stress on the collagen network, thus amplifying
ular dysplasia – common precursors of OA. Changes the changes in a cycle that leads to tissue breakdown.
in the subchondral bone may also increase stress con- Articular cartilage has an important role in distrib-
centration in the overlying cartilage, either by altering uting and dissipating the forces associated with joint
the shape of the articular surface or by an increase in loading. When it loses its integrity these forces are
bone density (e.g. following fracture healing) which increasingly concentrated in the subchondral bone.
reduces the shock-absorbing effect of the supporting The result: focal trabecular degeneration and cyst for-
cancellous bone. mation, as well as increased vascularity and reactive
From the foregoing outline it should be apparent sclerosis in the zone of maximal loading.
that the division of osteoarthritis into ‘primary’ (when What cartilage remains is still capable of regenera-
there is no obvious antecedent factor) and ‘secondary’ tion, repair and remodelling. As the articular surfaces
(when it follows a demonstrable abnormality) is become increasingly malapposed and the joint unsta-
somewhat artificial. This is borne out in clinical prac- ble, cartilage at the edges of the joint reverts to the
tice: patients with ‘secondary’ OA of the knee follow- more youthful activities of growth and endochondral
ing meniscectomy have been found also to have a ossification, giving rise to the bony excrescences, or
higher than usual incidence of ‘primary’ OA in other osteophytes, that so clearly distinguish osteoarthritis
joints (Doherty et al., 1983). Perhaps primary, gener- (once called ‘hypertrophic arthritis’) from ‘atrophic’
alized factors (genetic, metabolic or endocrine) alter disorders such as rheumatoid disease.
the physical properties of cartilage and thereby deter-
mine who is likely to develop OA, while secondary
factors such as anatomical defects or trauma specify
Pathology
when and where it will occur. OA is, ultimately, more The cardinal features are: (1) progressive cartilage
process than disease, occurring in any condition destruction; (2) subarticular cyst formation, with (3)
which causes a disparity between the mechanical stress sclerosis of the surrounding bone; (4) osteophyte for-
to which articular cartilage is exposed and the ability mation; and (5) capsular fibrosis.
of the cartilage to withstand that stress. Initially the cartilaginous and bony changes are
confined to one part of the joint – the most heavily
loaded part. There is softening and fraying, or fibrilla-
Pathogenesis tion, of the normally smooth and glistening cartilage.
The initial stages of OA have been studied in animal The term chondromalacia (Gr = cartilage softening)
models with induced joint instability and may not be seems apt for this stage of the disease, but it is used
representative of all types of OA. only of the patellar articular surfaces where it features
The earliest changes, while the cartilage is still mor- as one of the causes of anterior knee pain in young
phologically intact, are an increase in water content of people.
the cartilage and easier extractability of the matrix With progressive disintegration of cartilage, the
proteoglycans; similar findings in human cartilage underlying bone becomes exposed and some areas
have been ascribed to failure of the internal collagen may be polished, or burnished, to ivory-like smooth-
network that normally restrains the matrix gel. At a ness (eburnation). Sometimes small tufts of fibrocarti-
88 slightly later stage there is loss of proteoglycans and lage may be seen growing out of the bony surface. At
5
Osteoarthritis
(a) (b) (c) (d)
5.5 Osteoarthritis – pathology (a) The x-ray shows loss of articular cartilage at the superior pole and cysts in the
underlying bone; the specimen (b) shows that the top of the femoral head was completely denuded of cartilage and
there are large osteophytes around the periphery. In the coronal section (c) the subarticular cysts are clearly revealed.
(d) A fine-detail x-ray shows the extent of the subarticular bone destruction.
a distance from the damaged area the articular carti- more to a batch. In later stages, the clefts become
lage looks relatively normal, but at the edges of the more extensive and in some areas cartilage is lost to
joint there is remodelling and growth of osteophytes the point where the underlying bone is completely
covered by thin, bluish cartilage. denuded. The biochemical abnormalities correspon-
Beneath the damaged cartilage the bone is dense ding to these changes were described by Mankin et al.
and sclerotic. Often within this area of subchondral (1971).
sclerosis, and immediately subjacent to the surface, The subchondral bone shows marked osteoblastic
are one or more cysts containing thick, gelatinous activity, especially on the deep aspect of any cyst. The
material. cyst itself contains amorphous material; its origin is
The joint capsule usually shows thickening and mysterious – it could arise from stress disintegration
fibrosis, sometimes of extraordinary degree. The syn- of small trabeculae, from local areas of osteonecrosis
ovial lining, as a rule, looks only mildly inflamed; or from the forceful pumping of synovial fluid
sometimes, however, it is thick and red and covered through cracks in the subchondral bone plate. As in
by villi. all types of arthritis, small areas of osteonecrosis are
The histological appearances vary considerably, quite common. The osteophytes appear to arise from
according to the degree of destruction. Early on, the cartilage hyperplasia and ossification at the edge of the
cartilage shows small irregularities or splits in the sur- articular surface.
face, while in the deeper layers there is patchy loss of The capsule and synovium are often thickened but
metachromasia (obviously corresponding to the cellular activity is slight; however, sometimes there is
depletion of matrix proteoglycans). Most striking, marked inflammation or fibrosis of the capsular tissues.
however, is the increased cellularity, and the appear- A feature of OA that is difficult to appreciate from
ance of clusters, or clones, of chondrocytes – 20 or the morbid anatomy is the marked vascularity and
(a) (b) (c)
5.6 Osteoarthritis – histology (a) Destructive changes (loss of articular cartilage and cyst formation) are most marked
where stress is greatest; reparative changes are represented by sclerosis around the cysts and new bone formation
(osteophytes) in less stressed areas. (b) In this high-power view, the articular cartilage shows loss of metachromasia and
deep clefts in the surface (fibrillation). Attempts at repair result in (c) subarticular sclerosis and buds of fibrocartilage
mushrooming where the articular surface is destroyed. 89
5 venous congestion of the subchondral bone. This can injuries which result in joint instability. What is less
be shown by angiographic studies and the demonstra- certain is whether malunion of a long-bone fracture
tion of increased intraosseous pressure. It is also predisposes to OA by causing segmental overload in a
apparent from the intense activity around joint above or below the healed fracture (for example,
osteoarthritic joints on radionuclide scanning. in the knee or ankle after a tibial fracture). Contrary
to popular belief, research has shown that moderate
Prevalence angular deformities of the tibia (up to 15 degrees) are

not associated with an increased risk of OA (Merchant
Osteoarthritis is the commonest of all joint diseases. It and Dietz, 1989). This applies to mid-shaft fractures;
is a truly universal disorder, affecting both sexes and malunion close to a joint may well predispose to
all races; everyone who lives long enough will have it secondary OA.
somewhere, in some degree. However, there are sig-
nificant differences in its rate of occurrence in differ- Occupation There is good evidence of an association
ent ethnic groups, in the different sexes within any between OA and certain occupations which cause
group, and in the different joints. repetitive stress, for example OA of the knees in work-
Reports of prevalence rates vary, depending on the ers engaged in knee-bending activities (Felson, 1991),
method of evaluation. Autopsy studies show OA OA in the upper limbs in people working with heavy
changes in everyone over the age of 65 years. Radi- vibrating tools (Schumacher et al., 1972) and OA of
ographic surveys suggest that the prevalence rises the hands in cotton mill workers (Lawrence, 1961).
from 1 per cent below the age of 30 years to over 50 More controversial is the relationship of OA to sport-
per cent in people above the age of 60. Osteoarthritis ing activity. Boxers are certainly prone to developing
of the finger joints is particularly common in elderly OA of the hands but this may be due to trauma. The
women, affecting more than 70 per cent of those over same applies to footballers with OA of the knees and
70 years. baseball pitchers with OA of the shoulder. More con-
Men and women are equally likely to develop OA, vincing evidence of a causative relationship comes
but more joints are affected in women than in men. from recent studies which have shown a significant
Osteoarthritis is much more common in some increase in the risk of hip and knee OA in athletes
joints (the fingers, hip, knee and spine) than in others (Harris et al., 1994; Kulkala et al., 1994).
(the elbow, wrist and ankle). This may simply reflect Bone density It has long been known that women
the fact that some joints are more prone to predispos- with femoral neck fractures seldom have OA of the
ing abnormalities than others. hip. This negative association between OA and osteo-
A similar explanation may account for certain geo- porosis is reflected in more recent studies which have
graphical and ethnic differences in prevalence. For demonstrated a significant increase in bone mineral
example, the female-to-male ratio for OA of the hip is density in people with OA compared to those without
about 1:1 in northern Europe but is nearer 2:1 in (Hannan et al., 1992; Hart et al., 1994). However,
southern Europe where there is a high incidence of this may not be simple cause and effect: bone density
acetabular dysplasia in girls. Even more striking is the is determined by a variety of genetic, hormonal and
virtual absence of hip OA in southern Chinese and metabolic factors which may also influence cartilage
African blacks (Hoagland et al., 1973; Solomon, metabolism independently of any effect due to bone
1976); this may simply be because predisposing disor- density.
ders such as developmental displacement of the hip,
Perthes’ disease and slipped femoral epiphysis are Obesity The simple idea that obesity causes increased
uncommon in these populations. That they have no joint loading and therefore predisposes to OA may be
inherent resistance to OA is shown by the fact that correct – at least in part. The association is closer in
they often develop the condition in other joints, for women than in men and therefore (as with bone
example the knee. density) it may reflect other endocrine or metabolic
factors in the pathogenesis of OA.
Risk factors Family history Women whose mothers had general-
ized OA are more likely to develop the same condi-
Joint dysplasia Disorders such as congenital acetabular
tion. The particular trait responsible for this is not
dysplasia and Perthes’ disease presage a greater than
known (see above under Aetiology).
normal risk of OA in later life. It is not always easy to
spot minor degrees of dysplasia and careful studies
may have to be undertaken if these are not to be Symptoms
missed.
Patients usually present after middle age. Joint involve-
Trauma Fractures involving the articular surface are ment follows several different patterns: symptoms
90 obvious precursors of secondary OA, so too lesser centre either on one or two of the weightbearing joints
(hip or knee), on the interphalangeal joints (especially Signs 5
in women) or on any joint that has suffered a previous
affliction (e.g. congenital dysplasia, osteonecrosis or Joint swelling may be the first thing one notices in
intra-articular fracture). A family history is common in peripheral joints (especially the fingers, wrists, knees
patients with polyarticular OA. and toes). This may be due to an effusion, but hard
Pain is the usual presenting symptom. It is often (‘knobbly’) ridges around the margins of the distal
Osteoarthritis
quite widespread, or it may be referred to a distant site interphalangeal, the first metatarsophalangeal or knee
– for example, pain in the knee from OA of the hip. It joints can be just as obvious.
starts insidiously and increases slowly over months or Tell-tale scars denote previous abnormalities, and
years. It is aggravated by exertion and relieved by rest, muscle wasting suggests longstanding dysfunction.
although with time relief is less and less complete. In Deformity is easily spotted in exposed joints (the
the late stage the patient may have pain in bed at knee or the large-toe metatarsophalangeal joint), but
night. There are several possible causes of pain: mild deformity of the hip can be masked by postural adjust-
synovial inflammation, capsular fibrosis with pain on ments of the pelvis and spine.
stretching the shrunken tissue; muscular fatigue; and, Local tenderness is common, and in superficial joints
perhaps most important of all, bone pressure due to fluid, synovial thickening or osteophytes may be felt.
vascular congestion and intraosseous hypertension. Limited movement in some directions but not oth-
Stiffness is common; characteristically it occurs after ers is usually a feature, and is sometimes associated
periods of inactivity, but with time it becomes con- with pain at the extremes of motion.
stant and progressive. Crepitus may be felt over the joint (most obvious in
Swelling may be intermittent (suggesting an effu- the knee) during passive movements.
sion) or continuous (with capsular thickening or large Instability is common in the late stages of articular
osteophytes). destruction, but it may be detected much earlier by
Deformity may result from capsular contracture or special testing. Instability can be due to loss of carti-
joint instability, but be aware that the deformity may lage and bone, asymmetrical capsular contracture
actually have preceded and contributed to the onset and/or muscle weakness.
of OA. Other joints should always be examined; they may
Loss of function, though not the most dramatic, is show signs of a more generalized disorder. It is also
often the most distressing symptom. A limp, difficulty helpful to know whether problems in other joints add
in climbing stairs, restriction of walking distance, or to the difficulties in the one complained of (e.g. a stiff
progressive inability to perform everyday tasks or lumbar spine or an unstable knee making it more dif-
enjoy recreation may eventually drive the patient to ficult to cope with restricted movement in an
seek help. osteoarthritic hip).
Typically, the symptoms of OA follow an intermit- Function in everyday activities must be assessed.
tent course, with periods of remission sometimes last- X-ray appearances do not always correlate with either
ing for months. the degree of pain or the patient’s actual functional
capacity. Can the patient with an arthritic knee walk
up and down stairs, or rise easily from a chair? Does
he or she limp? Or use a walking stick?
Detailed examination of specific joints is dealt with
in Section 2 of the book.
Imaging
X-rays X-ray appearances are so characteristic that
other forms of imaging are seldom necessary for ordi-
nary clinical assessment. The cardinal signs are asym-
metrical loss of cartilage (narrowing of the ‘joint
space’), sclerosis of the subchondral bone under the
area of cartilage loss, cysts close to the articular sur-
face, osteophytes at the margins of the joint and
(a) (b) remodelling of the bone ends on either side of the
joint. Late features may include joint displacement
5.7 Osteoarthritis – clinical and x-ray (a) Varus and bone destruction.
deformity of the right knee due to osteoarthritis. (b) The
x-ray shows the classic features: disappearance of the joint Look carefully for signs of previous disorders (e.g.
‘space’, subarticular sclerosis and osteophyte formation at congenital defects, old fractures, Perthes’ disease or
the margins of the joint. rheumatoid arthritis). Such cases are usually designated 91
5
(a) (b) (c)
5.8 Osteoarthritis – x-rays The cardinal features of osteoarthritis are remarkably constant whether in (a) the hip, (b) the
knee or (c) the ankle: loss of articular cartilage seen as narrowing of the ‘joint space’, subarticular cyst formation and
sclerosis, osteophyte formation and bone remodelling.
Radionuclide scanning Scanning with 99mTc-HDP

THE CARDINAL SIGNS OF OSTEOARTHRITIS shows increased activity during the bone phase in the
Narrowing of the ‘joint space’
subchondral regions of affected joints. This is due to
increased vascularity and new bone formation.
Subchondral sclerosis
CT and MRI Advanced imaging is sometimes needed
Marginal osteophytes to elucidate a specific problem, e.g. early detection of
Subchondral cysts
an osteocartilaginous fracture, bone oedema or avas-
cular necrosis. These methods are also used for sever-
Bone remodelling ity grading in clinical trials.
Arthroscopy
Arthroscopy may show cartilage damage before x-ray
changes appear. The problem is that it reveals too
much, and the patient’s symptoms may be ascribed to
chondromalacia or OA when they are, in fact, due to
some other disorder.
Natural history
Osteoarthritis usually evolves as a slowly progressive
disorder. However, symptoms characteristically wax
and wane in intensity, sometimes disappearing for sev-
eral months.
The x-rays show no such fluctuation. However,
there is considerable variation between patients in the
5.9 Secondary osteoarthritis The flattened femoral degrees of destruction and repair. Most of the men
heads and shortened femoral necks are tell-tale signs of and half of the women have a hypertrophic reaction,
multiple epiphyseal dysplasia in this patient with secondary
OA. Her mother had an almost identical x-ray picture. with marked sclerosis and large osteophytes. In about
20 per cent of cases – most of them women – reactive
changes are more subdued, inviting descriptions such
as ‘secondary osteoarthritis’, though in a certain sense as atrophic or osteopaenic OA. Occasionally OA takes
OA is always secondary to some previous abnormality the form of a rapidly progressive disorder (Solomon,
92 if only we could discover what it was! 1976; Solomon, 1984).
5
Osteoarthritis
(a) (b)
(c) (d) (e)
5.10 Polyarticular (generalized) osteoarthritis (a,b) An almost invariable feature of polyarticular OA is involvement of
the terminal finger joints – Heberden’s nodes. There is a strong association with OA of the knees (c,d) and the lumbar facet
joints (e).
Complications MONARTICULAR AND PAUCIARTICULAR

OSTEOARTHRITIS
Capsular herniation Osteoarthritis of the knee is some-
In its ‘classic’ form, OA presents with pain and dys-
times associated with a marked effusion and hernia- function in one or two of the large weightbearing
tion of the posterior capsule (Baker’s cyst). joints. There may be an obvious underlying abnor-
Loose bodies Cartilage and bone fragments may give mality: multiple epiphyseal dysplasia, localized acetab-
rise to loose bodies, resulting in episodes of locking. ular dysplasia, old Perthes’ disease, previous slipped
epiphysis, inflammatory joint disease, avascular necro-
Rotator cuff dysfunction Osteoarthritis of the acromio- sis, a previous fracture or damage to ligaments or
clavicular joint may cause rotator cuff impingement, menisci. In the majority, however, the abnormality is
tendinitis or cuff tears. more subtle and may come to light only with special
Spinal stenosis Longstanding hypertrophic OA of the imaging techniques.
lumbar apophyseal joints may give rise to acquired
spinal stenosis. The abnormality is best demonstrated POLYARTICULAR (GENERALIZED) OSTEOARTHRITIS
by CT and MRI. This is by far the most common form of OA, though
most of the patients never consult an orthopaedic sur-
Spondylolisthesis In patients over 60 years of age,
geon. The patient is usually a middle-aged woman
destructive OA of the apophyseal joints may result in who presents with pain, swelling and stiffness of the
severe segmental instability and spondylolisthesis (so- finger joints. The first carpometacarpal and the big toe
called ‘degenerative’ spondylolisthesis, which almost metatarsophalangeal joints, the knees and the lumbar
always occurs at L4/5). facet joints may be affected at more or less the same
time.
The changes are most obvious in the hands. The
Clinical variants of osteoarthritis interphalangeal joints become swollen and tender,
Although the features of OA in any particular joint are and in the early stages they often appear to be
fairly consistent, the overall clinical picture shows vari- inflamed. Over a period of years osteophytes and soft-
ations which define a number of subgroups. tissue swelling produce a characteristic knobbly 93
5
(a) (b)
5.11 Rapidly destructive osteoarthritis (a) X-ray obtained when the patient was first seen, complaining of pain in the
left hip. This shows the typical features of an atrophic form of osteoarthritis on the painful side. (b) Eleven months later
there is marked destruction of the left hip, with crumbling of both the femoral head and the acetabular floor, and similar
features are beginning to appear on the right side.
appearance of the distal interphalangeal joints (Her- Avascular necrosis ‘Idiopathic’ osteonecrosis causes
berden’s nodes) and, less often, the proximal inter- joint pain and local effusion. Early on the diagnosis is
phalangeal joints (Bouchard’s nodes); pain may later made by MRI. Later x-ray appearances are usually
disappear but stiffness and deformity persist. Some pathognomonic; however, once bone destruction
patients present with painful knees or backache and occurs the x-ray changes can be mistaken for those of
the knobbly fingers are noticed only in passing. There OA. The cardinal distinguishing feature is that in
is a strong association with carpal tunnel syndrome osteonecrosis the ‘joint space’ (articular cartilage) is
and isolated tenovaginitis. preserved in the face of progressive bone collapse and
X-rays show the characteristic features of OA, usu- deformity, whereas in OA articular cartilage loss pre-
ally maximal in the distal interphalangeal joints of the cedes bone destruction.
fingers.
Inflammatory arthropathies Rheumatoid arthritis,
ankylosing spondylitis and Reiter’s disease may start in
OSTEOARTHRITIS IN UNUSUAL SITES
one or two large joints. The history is short and there
Osteoarthritis is uncommon in the shoulder, elbow,
are local signs of inflammation. X-rays show a pre-
wrist and ankle. If any of these joints is affected one
dominantly atrophic or erosive arthritis. Sooner or
should suspect a previous abnormality – congenital or
later other joints are affected and systemic features
traumatic – or an associated generalized disease such
appear.
as a crystal arthropathy.
RAPIDLY DESTRUCTIVE OSTEOARTHRITIS (see also

page 84)
Every so often a patient with apparently straightfor-
ward OA shows rapid and startling progression of
bone destruction. The condition was at one time
thought to be due to the dampening of pain impulses
by powerful anti-inflammatory drugs – a notional type
of ‘analgesic arthropathy’. It is now recognized that it
occurs mainly in elderly women and that it is associ-
ated with the deposition of calcium pyrophosphate
dihydrate crystals, though whether this is the cause of
the condition or a consequence thereof is still unde- (a) (b)
cided. 5.12 Differential diagnosis – osteoarthritis and
osteonecrosis (a) Osteoarthritis with marked subarticular
bone collapse is sometimes mistaken for osteonecrosis. The
Differential diagnosis of osteoarthritis clue to the diagnosis is that in OA the articular ‘space’
(cartilage) is progressively reduced before bone collapse
A number of conditions may mimic OA, some pre- occurs, whereas in primary osteonecrosis (b) articular
senting as a monarthritis and some as a polyarthritis cartilage is preserved even while the underlying bone
94 affecting the finger joint. crumbles.
of the symptoms, the age of the patient and his or her 5
functional needs. Three observations should be borne
in mind: (1) symptoms characteristically wax and
wane, and pain may subside spontaneously for long
periods; (2) some forms of OA actually become less
painful with the passage of time and the patient may
Osteoarthritis
need no more than reassurance and a prescription for
pain killers; (3) at the other extreme, the recognition
(from serial x-rays) that the patient has a rapidly pro-
gressive type of OA may warrant an early move to
reconstructive surgery before bone loss compromises
the outcome of any operation.
EARLY TREATMENT
There is, as yet, no drug that can modify the effects of
(a) (b) OA. Treatment is, therefore, symptomatic. The prin-
ciples are: (1) maintain movement and muscle
5.13 Diffuse idiopathic skeletal hyperostosis – DISH
(a) The large bony outgrowths around the knee suggest
strength; (2) protect the joint from ‘overload’; (3)
something more than the usual OA. X-rays of the spine (b) relieve pain; and (4) modify daily activities.
show the typical features of DISH. The spinal condition is
Physical therapy The mainstay of treatment in the
also known as Forestier’s disease.
early case is physical therapy, which should be directed
at maintaining joint mobility and improving muscle
strength. The programme can include aerobic exer-
Polyarthritis of the fingers Polyarticular OA may be cise, but care should be taken to avoid activities which
confused with other disorders which affect the finger increase impact loading. Other measures, such as mas-
joints (see Fig. 5.10). Close observation shows several sage and the application of warmth, may reduce pain
distinguishing features. Nodal OA affects predomi- but improvement is short-lived and the treatment has
nantly the distal joints, rheumatoid arthritis the proxi- to be repeated.
mal joints. Psoriatic arthritis is a purely destructive
arthropathy and there are no interphalangeal ‘nodes’. Load reduction Protecting the joint from excessive
Tophaceous gout may cause knobbly fingers, but the load may slow down the rate of cartilage loss. It is also
knobs are tophi, not osteophytes. X-rays will show the effective in relieving pain. Common sense measures
difference. such as weight reduction for obese patients, wearing
shock-absorbing shoes, avoiding activities like climb-
Diffuse idiopathic skeletal hyperostosis (DISH) This is a ing stairs and using a walking stick are worthwhile.
fairly common disorder of middle-aged people, char-
acterized by bone proliferation at the ligament and Analgesic medication Pain relief is important, but not
tendon insertions around peripheral joints and the all patients require drug therapy and those who do
intervertebral discs (Resnick et al., 1975). On x-ray may not need it all the time. If other measures do not
examination the large bony spurs are easily mistaken provide symptomatic improvement, patients may
for osteophytes. DISH and OA often appear together, respond to a simple analgesic such as paracetamol. If
but DISH is not OA: the bone spurs are symmetri- this fails to control pain, a non-steroidal anti-inflam-
cally distributed, especially along the pelvic apophyses matory preparation may be better.
and throughout the vertebral column. When DISH
INTERMEDIATE TREATMENT
occurs by itself it is usually asymptomatic.
Joint debridement (removal of loose bodies, cartilage
Multiple diagnosis Osteoarthritis is so common after tags, interfering osteophytes or a torn or impinging
middle age that it is often found in patients with other acetabular or glenoid labrum) may give some
conditions that cause pain in or around a joint. Before improvement. This may be done either by
jumping to the conclusion that the symptoms are due arthroscopy or by open operation.
to the OA features seen on x-ray, be sure to exclude If appropriate radiographic images suggest that
peri-articular disorders as well as more distant abnor- symptoms are due to localized articular overload aris-
malities giving rise to referred pain. ing from joint malalignment (e.g. varus deformity of
the knee) or incongruity (e.g. acetabular and femoral
head dysplasia), a corrective osteotomy may prevent
Management or delay progression of the cartilage damage. These
The management of OA depends on the joint (or techniques are discussed in the relevant chapters in
joints) involved, the stage of the disorder, the severity Section 2. 95
5 5.14 Operative treatment The three basic
operations: (a) osteotomy, (b) arthroplasty,
(c) athrodesis – at the hip.
(a) (b) (c)
LATE TREATMENT longer. Similar operations for the shoulder, elbow and
Progressive joint destruction, with increasing pain, ankle are less successful but techniques are improving
instability and deformity (particularly of one of the year by year. However, joint replacement operations
weightbearing joints), usually requires reconstructive are highly dependent on technical skills, implant
surgery. Three types of operation have, at different design, appropriate instrumentation and postopera-
times, held the field: realignment osteotomy, arthro- tive care – requirements that cannot always be met, or
plasty and arthrodesis. may not be cost-effective, in all parts of the world.
Realignment osteotomy Until the development of Arthrodesis Arthrodesis is still a reasonable choice if
joint replacement surgery in the 1970s, realignment the stiffness is acceptable and neighbouring joints are
osteotomy was widely employed. Refinements in tech- not likely to be prejudiced. This is most likely to apply
niques, fixation devices and instrumentation led to to small joints that are prone to OA, e.g. the carpal
acceptable results from operations on the hip and and tarsal joints and the large toe metatarsophalangeal
knee, ensuring that this approach has not been com- joint.
pletely abandoned. High tibial osteotomy is still con-
sidered to be a viable alternative to partial joint
replacement for unicompartmental OA of the knee, ENDEMIC OSTEOARTHRITIS
and intertrochanteric femoral osteotomy is sometimes
preferred for young patients with localized destructive
Osteoarthritis occasionally occurs as an endemic dis-
OA of the hip. These operations should be done while
order affecting entire communities. This phenome-
the joint is still stable and mobile and x-rays show that
non may be due either to an underlying generalized
a major part of the articular surface (the radiographic
dysplasia in a genetically isolated community or some
‘joint space’) is preserved. Pain relief is often dramatic
environmental factor peculiar to that region.
and is ascribed to (1) vascular decompression of the
subchondral bone, and (2) redistribution of loading
forces towards less damaged parts of the joint. After
load redistribution, fibrocartilage may grow to cover KASHIN–BECK DISEASE
exposed bone.
In 1859 Kashin, a Russian physician, reported the
Joint replacement Joint replacement, in one form or occurrence of an unusual form of polyarticular
another, is nowadays the procedure of choice for OA osteoarthritis associated with stunted growth in a
in patients with intolerable symptoms, marked loss of Siberian population. It is now known that the condi-
function and severe restriction of daily activities. For tion affects large numbers of children and adults (esti-
OA of the hip and knee in middle-aged and older mates vary from 1 to 6 million!) in the area stretching
patients, total joint replacement by modern tech- from Northern China across Eastern Siberia to North
96 niques promises improvement lasting for 15 years or Korea (Allander, 1994).
Clinical features Treatment 5
The condition starts in childhood with joint pain and There is no specific treatment for this condition. Pre-
progressive signs of polyarticular swelling, deformity ventive measures consist mainly of selenium supple-
and shortness of stature. Adults with this condition mentation in children’s diet or added to agricultural
may be severely crippled. fertilizer. In iodine-deficient areas, iodine is given as
Osteoarthritis
X-rays show distortion of the epiphyses in tubular well. Dosage should be monitored since selenium
bones during growth, and increasing signs of excess can cause unpleasant side effects and, in some
osteoarthritis in affected joints during adult life. cases, severe illness.
Patients with established arthritis will need treat-
ment as for other forms of OA.
Pathogenesis
There is, as yet, no agreement about the aetiology and
pathogenesis of this condition. Hypothetical causes
that have received the most attention are (a) defi- MSELENI JOINT DISEASE
ciency of trace elements such as selenium and iodine For many years visitors travelling along the eastern
in the soil and (b) contamination of the staple grain seaboard of South Africa have known about a crip-
product by mycotoxins during storage. This combina- pling type of polyarticular OA that was common
tion could lead to an accumulation of free radicals and among the Tsonga people living around the Mseleni
subsequent damage to growing chondrocytes in the Mission Station in Northern Zululand (now
exposed community. There are, however, some argu- Kwazulu). The first report in the medical literature
ments against a purely environmental causation: first, appeared in 1970 (Wittman and Fellingham, 1970).
there is no consistent correlation between local sele- Further studies suggested an overall prevalence rate of
nium and iodine levels and the prevalence of Kashin– at least 5 per cent, with women affected more often
Beck disease; second, the condition may be common than men and relatives of affected individuals much
in one village and completely absent in another only more commonly than relatives of unaffected people
30–50 miles away (Allender, 1994). The early radi- (Fellingham et al., 1973; Yach and Botha, 1985). A
ographic changes appear only in the epiphyses and the later radiographic survey showed that the
adjacent growth plates and not in other parts of the polyarthropathy actually comprises two distinct dis-
tubular bones which must, at an earlier stage, have orders: one with features of multiple epiphyseal dys-
consisted largely of cartilage. This, as well as the clin- plasia affecting males and females in equal proportions
ical appearances and the tendency for the condition to and another with typical features of protrusio acetab-
appear in familial clusters, are reminiscent of a genetic uli occurring almost exclusively in females (Solomon
disorder such as spondylo-epiphyseal dysplasia, a rec- et al., 1986).
ognized cause of stunted growth, bone deformities
and ‘secondary’ polyarticular OA. The most likely
explanation for this endemic disorder is that it is either
Clinical features
an expression of a straightforward genetic defect caus- In the first group, symptoms such as joint discomfort,
ing a type of chondrodysplasia or that the genetic slight deformity and stunting of growth start to
defect causes an increased susceptibility to the toxic appear in both boys and girls during childhood. When
effects of certain trace element deficiencies. x-ray changes appear they are those of symmetrically
(a) (b)
5.15 Endemic osteoarthritis – Mseleni disease X-rays showing the two forms of osteoarthritis endemic in the African
population of eastern Kwazulu: (a) generalized epiphyseal dysplasia and (b) bilateral protrusio acetabuli. 97
5 distributed epiphyseal dysplasia affecting particularly in Handigodu, South-Western India. It evidently
the hips, knees and ankles; sometimes the vertebral starts in childhood and by early adulthood patients
bodies also develop abnormally. During adulthood appear with painful, swollen joints (mainly the hips
the affected joints develop secondary OA: they and knees), deformities and stunting of growth. In
become painful and swollen, unstable and increasingly the most severe cases they have great difficulty walk-
deformed. ing and are reduced to crawling. As with Mseleni joint
The second group consists mainly of girls at disease in the past, this community is isolated from
puberty or a year or two later. Their main complaint the general population and patients appear in family
is of pain in the hip joints and even at that age x-ray clusters. It is, in all probability, a heritable form of
features of early protrusio acetabuli can be discerned. multiple epiphyseal dysplasia.
During adulthood those with the most marked
changes develop typical features of secondary OA.
NEUROPATHIC JOINT DISEASE

Causation
Various studies on the aetiology of Mseleni joint dis- Charcot, in 1868, described a type of destructive
ease have failed to identify a convincing nutritional or arthropathy associated with disease of the central
other environmental cause for this condition. There nervous system. Almost all his patients had tabes dor-
seems little doubt that the group with typical features salis, but the name ‘Charcot’s joint disease’ came to be
of epiphyseal changes represent a heritable form of applied to any destructive arthropathy arising from
multiple epiphyseal dysplasia or spondylo-epiphyseal loss of pain sensibility and position sense.
dysplasia. Nowadays the most common cause is diabetic neu-
It is uncertain whether those with protrusio acetab- ropathy, which occurs in 0.2–0.5 per cent of patients
uli have been fully investigated as a separate entity. with diabetes mellitus; other causes are tabes dorsalis,
However, what is well recognized is that features of leprosy (affecting mainly the lower limb joints),
calcium deficiency rickets are found in African chil- syringomyelia (upper and lower limbs), multiple scle-
dren from this area (Pettifor, 2008) and that (at least rosis, myelomeningocele, spinal cord compression
in the past) young girls in Kwazulu were the ones who and congenital indifference to pain. The term is also
traditionally were given the work of carrying the loads applied (less accurately) to rapidly destructive forms of
of water and other foodstuffs needed by their families osteoarthritis where there is no neurological lesion.
– often over long distances. Young boys were not
expected to do this work. Could this combination of
factors affecting girls have caused some distortion of
Pathogenesis and pathology
the acetabular socket before closure of the triradiate Neuropathic joints lack the normal reflex safeguards
cartilage? Whether a changing cultural milieu will against abnormal stress or injury and the subchondral
improve the situation remains to be seen. bone disintegrates with alarming speed. Unlike the
usual forms of osteoarthritis, this is a mainly destruc-
tive condition and there are few signs of repair. Some
Treatment cases show increased vascularity and osteoclastic activ-
In the past the people of Mseleni lived as a fairly iso- ity in the subchondral bone; in others, capsular and
lated group without intermarrying among neighbour- ligamentous laxity and joint instability go hand in
ing peoples and thereby changing the gene pool. As hand with articular disintegration.
with other endemic disorders, Mseleni disease is in The early changes are similar to those of
part a social problem and one can expect its preva- osteoarthritis. However, it soon becomes apparent
lence to fall with increasing social mobility and that this is a rapidly destructive process; the articular
improved living conditions. Meanwhile, patients are surface breaks up, fragments of bone and cartilage
treated as are those with other types of OA, i.e. by appear in the joint or embedded in the synovium, and
employing a mixture of analgesic medication, physical there is thickening of the synovial membrane and
therapy and reconstructive surgery where necessary marked joint effusion. In the late stages, there is com-
and feasible. plete loss of articular cartilage, fragmentation of the
subchondral bone and joint subluxation.
HANDIGODU JOINT DISEASE Clinical features

This is yet another endemic polyosteoarthopathy, sim- The patient complains of weakness, instability,
ilar to that of Mseleni joint disease, which was swelling, laxity and progressive deformity of the joint:
98 encountered some 30 years ago in a Dalit community usually the tarsal or ankle joints in diabetics, the large
5.16 Charcot’s disease The
vertebrae are distorted and
5
dense, the buttocks show the
radio-opaque remains of
former injections; the knee,
elbow and hip joints look
grotesque. Moral: ‘If it’s bizarre,
Osteoarthritis
do a “WR”’. Note also the
happy smile (though not all
Charcot joints are tabetic nor
are they always painless).
lower limb joints in leprosy and tabes dorsalis and the

upper limb joints (especially the shoulder) in
HAEMOPHILIC ARTHROPATHY
syringomyelia. The joint is neither warm nor particu-
larly tender, but swelling is marked, fluid is greatly Recurrent intra-articular bleeding may lead to chronic
increased and in the late stages bits of bone may be synovitis and progressive articular destruction. Clini-
felt everywhere. There is always some instability and cally this is seen only in classic haemophilia, in which
in the worst cases the joint is flail. The appearances there is a deficiency of clotting factor VIII, and
suggest that movement would be agonizing and yet it Christmas disease, due to deficiency of factor IX. Both
is often painless. The paradox is diagnostic. General are X-linked recessive disorders manifesting in males
examination may reveal features of the underlying but carried by females. Their incidence is about 1 per
neurological disorder. 10 000 male births. Plasma clotting factor levels
A fracture or dislocation may initiate the destructive above 40 per cent of the normal are compatible with
process and in those cases clinical deterioration is normal control of haemorrhage. Patients with clotting
more rapid and more painful than usual. factor levels above 5 per cent (‘mild haemophilia’)
may have prolonged bleeding after injury or opera-
tion; those with levels below 1 per cent (‘severe
X-rays haemophilia’) have frequent spontaneous joint and
The radiogaphic changes may at first be mistaken for muscle haemorrhages.
those of osteoarthritis. However, thinning of the
articular space is unusually rapid and there is little in
Pathology
the way of osteophyte formation. Joint swelling and
the appearance of intra-articular ‘calcification’ are fur- Haemorrhage into the joint causes synovial irritation,
ther clues. Ultimately there is gross erosion of the inflammation and subsynovial fibrosis. Haemosiderin
articular surfaces and displacement of the joint. appears in the synovial cells and macrophages and
after repeated bleeds the synovium becomes thick and
heavily pigmented. A vascular pannus creeps over the
Treatment articular surface and the cartilage is gradually eroded.
There is no way of halting or slowing the destructive The subchondral bone may be exposed and pene-
process. Treatment is usually conservative and consists trated, and occasionally large cysts develop at the
of splintage of the unstable joint. Despite the bizarre bone ends. These changes are attributed to cartilage-
appearances, patients often seem to manage well. degrading enzymes released by the proliferative syn-
Some patients complain of pain and may need anal- ovitis and by cells that have accumulated iron, but an
gesic medication. additional factor may be the interference with normal
Weightbearing joints are sometimes so unstable cartilage nutrition due to prolonged or repeated joint
that splintage is useless. Arthrodesis may be immobilization.
attempted, but the patient should be warned that Bleeding into muscles is less common but equally
there is only a small chance of success. harmful. Increased tension may lead to muscle necro- 99
5 (e.g. with groin pain due to iliopsoas haemorrhage);
usually only those movements that stretch the affected
muscles are painful, whereas in haemarthrosis all
movements are painful.
Bleeding into a peripheral nerve causes intense pain
followed by a variable degree of sensory change and
muscle weakness. Nerve function usually recovers

after several months.
Neurological symptoms and signs may also be
caused by a large soft-tissue haematoma. Following
effective treatment, the haematoma is usually
resorbed within 10–14 days but full movement may
take longer to return.
Bleeding into the forearm or leg may give rise to a
classical compartment syndrome. The tell-tale signs of
(a) (b) acute pain and tissue tension should be heeded before
5.17 Haemophilic arthropathy – clinical features sensory and motor impairment are obvious.
(a) Recurrent haemarthrosis and chronic synovitis led to
contractures of the elbow joints and deformities of the JOINT DEGENERATION
knees and ankles. (b) This man had difficulty staying This, the sequel to repeated bleeding, usually begins
upright, let alone walking, without support.
before the age of 15 years. Chronic synovitis is fol-
lowed by cartilage degeneration. An affected joint
shows wasting, limitation of movement and fixed
sis, reactive fibrosis and joint contractures. Sometimes deformity not unlike a tuberculous or rheumatoid
nerves are compressed, causing a neurapraxia; tempo- joint. In longstanding cases, articular destruction may
rary weakness may contribute further to the develop- lead to instability.
ment of joint deformity. X-ray changes vary according to the stage of the
Cysts and pseudotumours are rare phenomena. A disorder. A useful classification is that of Arnold and
large soft-tissue haematoma may become encapsu- Hilgartner (1977): Stage I – soft-tissue swelling; Stage
lated before it is absorbed, and may then draw in II – osteoporosis and epiphyseal overgrowth; Stage III
more fluid by osmosis to produce a slowly expanding – slight narrowing of the articular space and squaring
‘cyst’. A subperiosteal haematoma occasionally stimu- of the bone ends; Stage IV – marked narrowing of the
lates cystic resorption of bone resembling a tumour. articular space; and Stage V – joint disintegration.
Cysts and pseudotumours are rare complications.
Clinical features
Only males are affected and in severe haemophilia Treatment
joint bleeds usually begin when the child starts to
The most important aspect of treatment is to coun-
walk. The clinical picture depends on the severity of
teract bleeding as soon as it occurs, or better still to
the disorder, the site of bleeding and the efficacy of
prevent recurrent bleeds. Patients are taught to rec-
long-term treatment. The commonest features are
ognize the early symptoms of bleeding and to admin-
acute bleeding into joints or muscles, chronic arthritis
ister the appropriate clotting factor concentrate
and joint contractures. The sites most frequently
themselves. In some centres factor concentrate is
involved are the knees, ankles, elbow, shoulders and
administered prophylactically two or three times a
hips.
week; this is given intravenously, if necessary by
indwelling catheter. It is essential to establish the pre-
ACUTE BLEEDING INTO A JOINT, MUSCLE OR NERVE
cise diagnosis: factor VIII or IX is effective only for
With trivial injury a joint (usually the knee, elbow or
the specific disorder.
ankle) may rapidly fill with blood. Pain, warmth,
In former years (and probably still in some parts of
boggy swelling, tenderness and limited movement are
the world) fresh-frozen plasma or concentrates that
the outstanding features. The resemblance to a low-
had not undergone viral inactivation were used for
grade inflammatory joint is striking, but the history is
factor replacement, but this carried the risk of HIV
diagnostic.
contamination. These products are no longer recom-
Acute bleeding into muscles (especially the fore-
mended and have been replaced by virally inactivated
arm, calf or thigh) is less common. A painful swelling
factor concentrates.
appears and movement of the related joint is resisted.
100 The distinction from a haemarthosis may be difficult The acute bleed Bleeding into the tissues is treated by
5
Osteoarthritis
(a) (b) (c) (d) (e) (f)
5.18 Haemophilic arthritis (a) At first, there is blood in the joint but the surfaces are intact; (b) later the cartilage is
attacked and the joint ‘space’ narrows; (c) bony erosions appear and eventually the joint becomes deformed and unstable;
in (d) early subluxation is obvious. (e,f) This large pseudotumour was extirpated and, at the same time, massive bone grafts
were inserted – no light undertaking in a haemophilic.
immediate factor replacement. Analgesics are given haemostasis is needed. Not surprisingly, the complica-
for pain and the limb is immobilized in a splint – but tion rate is higher than for hip replacement in non-
not for more than a day or two. Once the acute bleeders (Nelson et al., 1992).
episode has passed, movement is encouraged, under
continuing cover with factor concentrate. Aspiration
is avoided unless distension is severe or there is a
strong suspicion of infection. Nerve palsy may require REFERENCES AND FURTHER READING
intermittent splintage and physiotherapy until the
neurapraxia recovers, and during this time the skin
Allander E. Kashin–Beck disease. An analysis of research
must be protected from injury.
and public health activities based on a bibliography 1849–
Chronic arthropathy The aim is to prevent the devel- 1992. Scand J Rheumat 1994; 23(suppl 99): 1–36.
opment of joint contractures, stiffness and progressive Arnold WD, Hilgartner MW. Hemophilic arthropathy.
muscle weakness. Under cover of factor infusions the J Bone Joint Surg, 1977; 59A: 287–305.
patient is given physiotherapy, and impending con- Byers PD, Contepomi CA, Farkas TA. A post mortem
tractures are managed by intermittent splintage and, if study of the hip joint including the prevalence of features
necessary, traction or passive correction by an inflat- on the right side. Ann Rheum Dis 1970; 29: 15–31.
able splint. Doherty M, Holt M, MacMillan P et al. A reappraisal of
Operative treatment has become safer since the ‘analgesic hip’. Ann Rheum Dis 1986; 45: 272–6.
introduction of clotting factor concentrates. How- Doherty M, Watt I, Dieppe P. Influence of primary gener-
ever, patients who develop anti-factor antibodies are alised osteoarthritis on development of secondary
unsuitable for any form of surgery. It is also important osteoarthritis. Lancet 1983; 2: 8–11.
to screen patients for hepatitis B virus and HIV anti- Fellingham SA, Elphinstone RD, Wittman W. Mseleni
bodies, as their presence demands special precautions joint disease: background and prevalence. S Afr Med J
during the operation. 1973; 47: 2173–80.
The clotting factor concentration should be raised Felson DT, Anderson JJ, Namack A et al. Obesity and
to above 25 per cent for factor VIII and above 15 per symptomatic knee osteoarthritis. Arthr Rheum 1987; 30:
cent for factor IX, and it should be kept at those lev- S130.
els throughout the postoperative period. It goes with- Felson DT, Hannan MT, Naimark A, et al. Occupational
out saying that operative treatment should be carried physical demands, knee bending, and knee osteoarthritis:
out in a hospital with the appropriate multidiscipli- results from the Framingham Study. J Rheumatol, 1991;
nary expertise on site. 18: 1587–92.
Useful procedures are tendon lengthening (to cor- Hannan MT, Zhang Y, Anderson JJ, et al. Bone mineral
rect contractures), osteotomy (for established defor- density and knee osteoarthritis in elderly men and
mity) and arthrodesis of the knee or ankle (for painful women: The Framingham Study. Arthr Rheum 1992; 35:
joint destruction). Synovectomy is sometimes per- S1 (S40).
formed but the benefits are dubious. Total hip Harris PA, Hart DJ, Jawad S et al. Risk of osteoarthritis
replacement is technically feasible, but tissue dissec- (OA) associated with running: A radiological survey.
tion should be kept to a minimum and meticulous Arthr Rheum 1994; 37: S369. 101
5 Hart DJ, Mootoosamy I, Doyle DV, et al. The relationship Nelson IW, Sivamerugan S, Latham PD et al. Total hip
between osteoarthritis and osteoporosis in the general arthroplasties for haemophilic arthropathies. Clin Orthop
population: The Chingford Study. Ann Rheum Dis 1994; Relat Res 1992; 276: 210–13.
53: 158–62. Palotie A, Vaisanen P, Ott J et al. Predisposition to fami-
Hoaglund FT, Yau ACMC, Wong WL. Osteoarthritis of the lial osteoarthrosis linked to type II collagen gene. Lancet
hip and other joints in Southern Chinese in Hong Kong. 1989; 2: 924.
J Bone Joint Surg 1973; 55A: 545–7. Pettifor JM. Vitamin D and/or calcium deficiency rickets
Kellgren JH. Genetic factors in generalized osteoarthrosis. in infants and children: a global perspective. Indian J Med
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102
Osteonecrosis and
related disorders 6
Louis Solomon
Avascular necrosis has long been recognized as a com- once established, may come to dominate the clinical
plication of femoral head fractures, the usual explana- picture, demanding attention in its own right.
tion being traumatic severance of the blood supply to
the femoral head. Segmental osteonecrosis also
appears as a distinctive feature in a number of non-
traumatic disorders: joint infection, Perthes’ disease, Sites which are peculiarly vulnerable to ischaemic
caisson disease, Gaucher’s disease, systemic lupus ery- necrosis are the femoral head, the femoral condyles,
thematosus (SLE), high-dosage corticosteroid admin- the head of the humerus, the capitulum and the prox-
istration and alcohol abuse, to mention only the more imal parts of the scaphoid and talus. These subarticu-
common ones. Whatever the cause, the condition, lar regions lie at the most distant parts of the bone’s
vascular territory, and they are largely enclosed by car-
tilage, giving restricted access to local blood vessels.
The subchondral trabeculae are further compromised
MAIN CONDITIONS ASSOCIATED WITH
in that they are sustained largely by a system of endar-
NON-TRAUMATIC OSTEONECROSIS
terioles with limited collateral connections.
Infections Another factor which needs to be taken into
• Osteomyelitis account is that the vascular sinusoids which nourish
• Septic arthritis the marrow and bone cells, unlike arterial capillaries,
have no adventitial layer and their patency is deter-
Haemoglobinopathy mined by the volume and pressure of the surrounding
• Sickle cell disease marrow tissue, which itself is encased in unyielding
Storage disorders bone. The system functions essentially as a closed
• Gaucher’s disease compartment within which one element can expand
Caisson disease
• Dysbaric osteonecrosis
Coagulation disorders
• Familial thrombophilia
• Hypofibrinolysis
• Hypolipoproteinaemia
• Thrombocytopenic purpura
Other
• Perthes’ disease
• Cortisone administration
• Alcohol abuse
• SLE (? increase in antiphospholipid antibodies)
• Pregnancy (? decreased fibrinolysis; ? fatty liver)
• Anaphylactic shock 6.1 Avascular necrosis – pathogenesis The medullary
• Ionizing radiation cavity of bone is virtually a closed compartment containing
myeloid tissue, marrow fat and capillary blood vessels. Any
SLE, systemic lupus erythematosus. increase in fat cell volume will reduce capillary circulation
and may result in bone ischaemia.
6 only at the expense of the others. Local changes such in both adult osteonecrosis and Perthes’ disease.
as decreased blood flow, haemorrhage or marrow Other coagulopathies have been implicated, e.g.
swelling can, therefore, rapidly spiral to a vicious cycle antiphospholipid deficiency in SLE (Asherson et al.,
of ischaemia, reactive oedema or inflammation, mar- 1993) and enhanced coagulability in sickle-cell disease
row swelling, increased intraosseous pressure and fur- (Francis, 1991), and it now seems likely that coagula-
ther ischaemia. tion abnormalities of one sort or another play at least
The process described above can be initiated in at a contributory role in some of the disorders associated
least four different ways: (1) severance of the local with non-traumatic osteonecrosis.
blood supply; (2) venous stasis and retrograde arteri-
Extravascular marrow swelling High-dosage cortico-
olar stoppage; (3) intravascular thrombosis; and (4)
steroid administration and alcohol overuse cause fat
compression of capillaries and sinusoids by marrow
cell swelling in the marrow, a feature which is very
swelling. Ischaemia, in the majority of cases, is due to a
obvious in bone specimens obtained during joint
combination of several of these factors.
replacement. There is a demonstrable rise in
TRAUMATIC OSTEONECROSIS intraosseous pressure and contrast venography shows
In traumatic osteonecrosis the vascular anatomy is slowing of venous blood flow from the bone. Ficat
particularly important. In fractures and dislocations of and Arlet (1980) posited that the increase in marrow
the hip the retinacular vessels supplying the femoral fat volume in the femoral head caused sinusoidal com-
head are easily torn. If, in addition, there is damage to pression, venous stasis and retrograde ischaemia lead-
or thrombosis of the ligamentum teres, osteonecrosis ing to trabecular bone death; in other words, the
is inevitable. Little wonder that displaced fractures of establishment of a compartment syndrome.
the femoral neck are complicated by osteonecrosis in Whichever of these mechanisms offers the primary
over 20 per cent of cases. Undisplaced fractures, or pathway to non-traumatic bone ischaemia, it is almost
lesser injuries, also sometimes result in subchondral certain that both intravascular and extravascular fac-
necrosis; this may be due to thrombosis of tors come into play at a fairly early stage and each
intraosseous capillaries or sinusoidal occlusion due to enhances the effect of the other.
marrow oedema.
Other injuries which are prone to osteonecrosis are
Sickle-cell disease
fractures of the scaphoid and talus. Significantly, in Dysbaric ischaemia
these cases it is always the proximal fragment which Thrombocytopenia
suffers. This is because the principal vessels enter the Fat embolism
bones near their distal ends and take an intraosseous
course from distal to proximal.
Impact injuries and osteoarticular fractures at any of
the convex articular surfaces behave in the same way Arteriolar
occlusion
and often develop localized ischaemic changes. These
small lesions are usually referred to as ‘osteochon-
droses’ and many of them have acquired eponyms
which are firmly embedded in orthopaedic history.
NON-TRAUMATIC OSTEONECROSIS Vascular

Marrow
The mechanisms here are more complex and may stasis
oedema
involve several pathways to intravascular stasis or
thrombosis, as well as extravascular swelling and cap-
illary compression.
Intravascular thrombosis Various mechanisms leading
to capillary thrombosis have been demonstrated in Sinusoidal
patients with non-traumatic osteonecrosis. Over 80 compression
per cent of cases are associated with high-dosage cor-
ticosteroid medication or alcohol abuse (or both, act-
Gaucher’s disease
ing cumulatively). These conditions give rise to
Tuberculosis
hyperlipidaemia and fatty degeneration of the liver. Cortisone/alcohol
Jones (1994) has favoured the idea that fat embolism Dysbaric ischaemia
plays a part, giving rise to capillary endothelial dam-
age, platelet aggregation and thrombosis. Glueck et 6.2 Avascular necrosis Algorithm showing how various
al. (1996, 1997a) have suggested that thrombophilia disorders may enter the vicious cycle of capillary stasis and
104 and hypofibrinolysis are important aetiological factors marrow engorgement.
6
Osteonecrosis and related disorders

(a) (b) (c) (d)
6.3 Osteonecrosis – pathology (a,b) Normal femoral
head and cut section. The articular cartilage is obviously
intact and the subchondral bone is well vascularized.
(c,d) In this femoral head with osteonecrosis the articular
cartilage is lifted off the bone; the coronal section in
(d) shows that this is due to a subarticular fracture through
the necrotic segment in the dome of the femoral head.
(e) Histological section across the junction between
articular cartilage and bone showing living cartilage cells
but necrotic subchondral marrow and bone. (f) High
power view showing islands of dead bone with empty
osteocytic lacunae enfolded by new, living bone.
(e) (f)
Pathology and natural history its thickness and viability. In the final stages, fragmen-
tation of the necrotic bone leads to progressive defor-
Bone cells die after 12–48 hours of anoxia, yet for mity and destruction of the joint surface.
days or even weeks the gross appearance of the In the past, when diagnosis was based entirely on
affected segment remains unaltered. During this time x-ray changes, it was thought that osteonecrosis
the most striking histological changes are seen in the always progressed to bone collapse. Now that it is
marrow: loss of fat cell outlines, inflammatory cell possible to detect the earliest signs by MRI, it has
infiltration, marrow oedema, the appearance of tissue become apparent that this is not the case.
histiocytes, and eventual replacement of necrotic mar- The size of the necrotic segment, as defined by the
row by undifferentiated mesenchymal tissue. hypo-intense band in the T1 weighted MRI, is usually
A characteristic feature of ischaemic segmental established at the time of the initiating ischaemic
necrosis is the tendency to bone repair, and within a event, and from then on it rarely increases; indeed,
few weeks one may see new blood vessels and there is evidence that non-traumatic lesions some-
osteoblastic proliferation at the interface between times diminish in size and occasionally even disappear
ischaemic and live bone. As the necrotic sector (Sakamoto et al., 1997). In persistent lesions, the rate
becomes demarcated, vascular granulation tissue of bone collapse depends largely on the site and
advances from the surviving trabeculae and new bone extent of the necrotic segment: lesions which lie out-
is laid down upon the dead; it is this increase in min- side the normal stress trajectories may remain struc-
eral mass that later produces the radiographic appear- turally intact while those that involve large segments
ance of increased density or ‘sclerosis’. of the load-bearing surface usually collapse within
Reparative new bone formation proceeds slowly 3 years (see under Staging).
and probably does not advance for more than 8–
10 mm into the necrotic zone. With time, structural
failure begins to occur in the most heavily stressed Clinical features
part of the necrotic segment. Usually this takes the The earliest stage of bone death is asymptomatic; by
form of a linear tangential fracture close to the articu- the time the patient presents, the lesion is usually well
lar surface, possibly due to shearing stress. The crack advanced. Pain is a common complaint. It is felt in or
may break through the articular cartilage and at oper- near a joint, and perhaps only with certain move-
ation it may be possible to lift the ‘lid’ off the necrotic ments. Some patients complain of a ‘click’ in the joint,
segment like the cracked shell of a hard-boiled egg. probably due to snapping or catching of a loose artic-
However, until very late the articular cartilage retains ular fragment. In the later stages the joint becomes 105
6
(a) (b) (c) (d)
6.4 Avascular necrosis of bone – pathology (a) This is a diagramatic guide to the fine-detail x-rays of necrotic femoral
heads (b–d) which show the progress of osteonecrosis. The articular cartilage (A) remains intact for a long time. The
necrotic segment (B) has a texture similar to that of normal bone, but it may develop fine cracks. New bone surrounds the
dead trabeculae and causes marked sclerosis (C). Beyond this the bone remains unchanged (D). In the later stages the
necrotic bone breaks up and finally the joint surface is destroyed.
stiff and deformed. Local tenderness may be present of the articular surface and more intense ‘sclerosis’,
and, if a superficial bone is affected, there may be now partly due to bone compression in a collapsed
some swelling. Movements – or perhaps one particu- segment.
lar movement – may be restricted; in advanced cases Occasionally the necrotic portion separates from
there may be fixed deformities. the parent bone as a discrete fragment. However, it is
now recognized that in the case of the femoral head
Imaging and the medial femoral condyle such necrotic frag-
ments may have resulted from small osteo-articular
X-ray The early signs of ischaemia are confined to the fractures which only later failed to unite and lost their
bone marrow and cannot be detected by plain x-ray blood supply.
examination. X-ray changes, when they appear (sel- With all the changes described here (and this is the
dom before 3 months after the onset of ischaemia), cardinal feature distinguishing primary avascular
are due to (a) reactive new bone formation at the necrosis from the sclerotic and destructive forms of
boundary of the ischaemic area and (b) trabecular fail- osteoarthritis) the ‘joint space’ retains its normal
ure in the necrotic segment. An area of increased radi- width because the articular cartilage is not destroyed
ographic density appears in the subchondral bone; until very late.
soon afterwards, suitable views may show a thin tan-
99m
gential fracture line just below the articular surface – Radioscintigraphy Radionuclide scanning with Tc-
the ‘crescent sign’. In the late stages there is distortion sulphur colloid, which is taken up in myeloid tissue,
(a) (b) (c)
6.5 Avascular necrosis – x-ray (a) The earliest x-ray sign is a thin radiolucent crescent just below the convex articular
surface where load bearing is at its greatest. This represents an undisplaced subarticular fracture in the early necrotic
segment. (b) At a later stage the avascular segment is defined by a band of increased density due to vital new bone
formation. At this stage the femoral head may still be spherical and (unlike osteoarthritis) the articular space is still
106 well-defined. (c) In late cases there is obvious collapse and distortion of the articular surface.
image), corresponding to the interface between 6
ischaemic and normal bone. The site and size of the
demarcated necrotic zone have been used to predict
the progress of the lesions (see Chapter 19).
Computed tomography CT involves considerable radi-
ation exposure and it is not very useful for diagnosing

osteonecrosis. However, it does show the area of bone
destruction very clearly and it may be useful in plan-
ning surgery.
(a) (b)
6.6 Osteonecrosis – MRI (a) Before any change is

discernible on the plain x-ray, MRI will show a typical hypo- Tests for haemodynamic function
intense band in the T1 weighted image, outlining the
ischaemic segment beneath the articular surface. (b) In this
During the early stage of ischaemic necrosis the
case the size of the ischaemic segment is much larger – intramedullary pressure is often markedly raised. This
and the likelihood of bone crumbling much greater. phenomenon is most easily demonstrated in the
femoral head. A cannula introduced into the metaph-
ysis enables measurements to be taken (1) at rest and
(2) after rapid injection of saline. The normal resting
may reveal an avascular segment. This is most likely in
pressure is 10–20 mmHg, rising by about 15 mm
traumatic avascular necrosis, where a large segment of
after saline injection; in early osteonecrosis both the
bone is involved, or in sickle-cell disease where a
intramedullary pressure and the response to saline
‘cold’ area contrasts significantly with the generally
injection may be increased three- or four-fold. Venous
high nuclide uptake due to increased erythroblastic
stasis can also be demonstrated by venography after
activity. 99mTc-HDP scans (in the bone phase) may
injection of radio-opaque medium into the bone.
also show a ‘cold’ area, particularly if a large segment
Similar findings have been recorded in osteoarthri-
of bone is avascular (e.g. after fracture of the femoral
tis, but the change is not nearly as marked as in
neck). More often, however, the picture is dominated
osteonecrosis.
by increased activity, reflecting hyperaemia and new
bone formation in the area around the infarct.
Staging the lesion
Magnetic resonance imaging MRI is the most reliable
way of diagnosing marrow changes and bone Ficat and Arlet (1980) introduced the concept of radi-
ischaemia at a comparatively early stage. The first sign ographic staging for osteonecrosis of the hip to distin-
is a band-like low-intensity signal on the T1 weighted guish between early (pre-symptomatic) signs and later
spin echo (SE) image (and a similar but high-intensity features of progressive demarcation and collapse of the
signal on the short-tau inversion recovery (STIR) necrotic segment in the femoral head. Stage 1 showed no
(a) (b) (c)
6.7 Osteonecrosis – distribution The most common sites for osteonecrosis are the head of the femur, the head of the
humerus and, as shown here, the medial condyle of the femur, the talus and the capitulum. All these areas are located
beneath convex articular surfaces; osteonecrosis is seldom seen beneath a concave articular surface. 107
6 x-ray change and the diagnosis was based on measure- The most widely used system, which permits com-
ment of intraosseous pressure and histological features of parison between series from different participating
bone biopsy (or nowadays on MRI). In Stage 2 the centres, is the one promoted by the International
femoral head contour was still normal but there were Association of Bone Circulation and Bone Necrosis
early signs of reactive change in the subchondral area. (Association Research Circulation Osseous – ARCO)
Stage 3 was defined by clearcut x-ray signs of osteonecro- which applies mainly to femoral head necrosis (Table
sis with evidence of structural damage and distortion of 6.1).

the bone outline. In Stage 4 there were collapse of the
articular surface and signs of secondary OA.
Diagnosis of the underlying disorder
Later modifications involving assessment of both
the extent and the location of the early changes on plain In many cases of osteonecrosis an underlying disorder
x-ray and MRI have proved to be more reliable as pre- will be obvious from the history: a known episode of
dictors of outcome, at least in relation to femoral head trauma, an occupation such as deep-sea diving or
necrosis (Shimizu et al., 1994; Steinberg et al., 1995). working under compressed air, a family background
The location and size of the necrotic segment in of Gaucher’s disease or sickle-cell disease. There may
Ficat stages 1–3 are defined by the hypo-intense band be a record of high-dosage corticosteroid administra-
on the T1 weighted MRI. Two general observations tion; for example, after renal transplantation where
can be made: (1) the size of the ischaemic segment is the drug is used for immunosuppression. However,
determined at a very early stage and it rarely increases smaller doses (e.g. as short-term treatment for asthma
after that; (2) small lesions which do not involve the or as an adjunct in neurosurgical emergencies) and
maximally loaded zone of the articular surface tend even topical steroid preparations can also be danger-
not to collapse, whereas large lesions extending under ous in patients with other risk factors (Solomon and
the maximally loaded articular surface break down in Pearse, 1994). Combinations of drugs (e.g. cortico-
over 60 per cent of cases. Shimizu’s classification is steroids and azathioprine, or corticosteroids after a
particularly useful in planning treatment; this is dis- period of alcohol abuse) also can be potent causes of
cussed in Chapter 19. osteonecrosis; occasionally corticosteroids have been
given without the patient’s knowledge.
Table 6.1 ARCO staging of osteonecrosis Alcohol abuse is often difficult to determine
because patients tend to hide the information. There
Stage 0 Patient asymptomatic and all clinical investigations is no biochemical marker that is specific for high
‘normal’
alcohol intake but elevation of three or four of the fol-
Biopsy shows osteonecrosis
lowing is suggestive: aspartate transaminase, γ-glu-
Stage 1 X-rays normal. MRI or radionuclide scan shows tamyl transpeptidase, serum urate, serum triglyceride
osteonecrosis
and mean red cell volume (Whitehead et al., 1978).
Stage 2 X-rays and/or MRI show early signs of osteonecrosis Ideally patients with very early non-traumatic
but no distortion of bone shape or subchondral ‘cres- osteonecrosis, and children with early Perthes’ dis-
cent sign’
ease, should undergo laboratory tests for coagu-
Subclassification by area of articular surface involved:
lopathies; this is justified by reports of cases in which
A = less than 15 per cent
the condition has been halted or reversed by treat-
B = 15–30 per cent ment with antithrombotic preparations such as war-
C = more than 30 per cent farin and stanozolol (Glueck et al., 1997b).
Stage 3 X-ray shows ‘crescent sign’ but femoral head still Unfortunately the tests are very expensive and there is
spherical understandable resistance to adopting this approach
Subclassification by length of ‘crescent’/articular sur- in routine management.
face: In cases of suspected SLE, antiphospholipid anti-
A = less than 15 per cent bodies may be measured.
B = 15–30 per cent
C = more than 30 per cent
Prevention
Stage 4 Signs of flattening or collapse of femoral head
A = less than 15 per cent of articular surface Where risk factors for osteonecrosis are recognized,
B = 15–30 per cent of articular surface preventive steps can be taken especially in the man-
C = more than 30 per cent of articular surface agement of corticosteroid medication and alcohol
abuse. Corticosteroids should be used only when
Stage 5 Changes as above plus loss of ‘joint space’ (secondary
OA) essential and in minimal effective dosage. It is impor-
tant also to be aware of the cumulative effect of even
Stage 6 Changes as above plus marked destruction of articular
surfaces
moderate doses of corticosteroids in patients with a his-
108 tory of alcohol abuse. Anoxia must be prevented in
6.8 Osteonecrosis – treatment 6
(a) Alcohol abuse has led to bilateral
femoral head necrosis, advanced on the
left but detectable only by MRI on the
right. (b) The left hip had to be
replaced; at the same time the right side
was treated by drilling of the femoral

neck (medullary decompression). This
x-ray was taken 8 years later.
(a)
(b)
patients with haemoglobinopathies. Decompression head collapse than untreated controls (Lai et al.,
procedures for divers and compressed-air workers 2005). Other studies have shown similar results
should be rigorously applied. (Nishii et al., 2006). However, it is still too early to
comment on the long-term success of this treatment.
Lesions in heavily loaded joints have a poor prog-
Treatment
nosis and will probably end in structural failure if left
In planning treatment, all the factors that influence untreated. Simple measures to reduce loading of
the natural course of the condition must be taken into weight-bearing joints may help, though their value
account: the general medical background, the type of has not been proven. If the bone contour is still intact,
ischaemic necrosis, the site and extent of the necrotic an ‘unloading’ osteotomy will help to preserve the
segment, its stage of development, the patient’s age anatomy while remodelling proceeds. This approach
and capacity for bone repair, the persistence or other- is applicable especially to the hip and knee.
wise of the aetiological agent and its effect on bone Medullary decompression and bone grafting may
turnover. have a place in ARCO stage 1 and 2 osteonecrosis of
Only general principles will be discussed here; the the femoral head (Chapter 19).
treatment of osteonecrosis in specific sites is dealt with
in the appropriate chapters on regional orthopaedics. INTERMEDIATE STAGE OSTEONECROSIS
Once there is structural damage and distortion of the
EARLY OSTEONECROSIS articular surface, conservative operations are inappro-
While the bone contour is intact there is always the priate. However, the joint may still be salvageable and
hope that structural failure can be prevented. Some in this situation realignment osteotomy – either alone
lesions heal spontaneously and with minimal defor- or combined with curettage and bone grafting of the
mity; this is seen especially in areas which are not necrotic segment – has a useful role.
severely stressed: the non-weightbearing joints, the If mobility can be sacrificed without severe loss of
superomedial part of the femoral head and the non- function (e.g. in the ankle or wrist), arthrodesis will
weightbearing surfaces of the femoral condyles and relieve pain and restore stability.
talus. Here one can afford to pursue a waiting policy.
In the past, various types of medication failed to LATE STAGE OSTEONECROSIS
show convincing evidence of preventing collapse of Destruction of the articular surface may be give rise to
the subchondral bone in cases of early osteonecrosis. pain and severe loss of function. Three options are
Recently, however, there have been promising reports available: (1) non-operative management, concentrat-
of the effect of bisphosphonates in these cases. In a ing on pain control, modification of daily activities
controlled study of the patients (54 femoral heads) and, where appropriate, splintage of the joint; (2)
with ARCO stage 2 or 3 osteonecrosis, those treated arthrodesis of the joint, e.g. the ankle or wrist; or (3)
with oral alendronate for 25 weeks were found after 2 partial or total joint replacement, the preferred option
years to show a significantly lower rate of femoral for the shoulder, hip and knee. 109
6 SYSTEMIC DISORDERS ASSOCIATED
tries along the Mediterranean, the Persian Gulf, parts
of India and across the Atlantic where it appears in
WITH OSTEONECROSIS people of Afro-American descent. In recent years it
has spread more widely in Europe but it is rarely
encountered south of the equator.
DRUG-INDUCED NECROSIS Sickle-cell disease is most likely in homozygous off-
Alcohol, corticosteroids, immunosuppressives and spring (those with HbS genes from both mother and
cytotoxic drugs, either singly or in combination, are father), but it may also occur in heterozygous children
the commonest causes of non-traumatic osteonecro- with HbS/C haemoglobinopathy and HbS/thalas-
sis. ‘At risk’ doses for these drugs have not been estab- saemia. Inheritance of one HbS gene and one normal
lished; the threshold depends not only on the total b-globin gene confers the (heterozygous) sickle-cell
intake but also on the time over which the intake is trait; HbS concentration is low and sickling occurs
spread and the presence or absence of associated dis- only under conditions of hypoxia (e.g. under ineffi-
orders which themselves may predispose to cient anaesthesia, in extreme cold, at very high alti-
osteonecrosis. A cumulative dose of 2000 mg of pred- tudes and when flying in unpressurized aircraft).
nisone equivalent administered over several years (for In the established disorder, the main clinical fea-
example in the treatment of rheumatoid arthritis) is tures are due to a combination of chronic haemolytic
less likely to cause osteonecrosis than the same dose anaemia and a tendency to clumping of the sickle-
given over a period of a few months (e.g. after organ shaped cells which results in diminished capillary flow
transplantation). It is important to bear in mind that and recurrent episodes of intracapillary thrombosis.
multiple causative agents have an additive effect; thus, Secondary changes such as trabecular coarsening,
osteonecrosis has been encountered after compara- infarctions of the marrow, periostitis and osteonecro-
tively short courses and low doses of corticosteroids sis are common. Complications include hyperuri-
(totals of 800 mg or less), but in these cases an addi- caemia (due to increased red cell turnover) and an
tive factor can almost always be identified (Solomon increased susceptibility to bacterial infection.
and Pearse, 1994).
The threshold dose for alcohol is equally vague. Clinical features
However, based on the known dose relationship of
alcohol-induced fatty degeneration of the liver, we Children during the first two years of life may present
would set it at around 150 mg of ethanol per day with swelling of the hands and feet. X-rays at first
(for men) – the equivalent of 300 mL of spirits, 1.2 seem normal, but later there may be suggestive fea-
litres of table wine or 3 litres of beer – continuing for tures such as marrow densities and periosteal new
over 2 years. The dose for women is considerably bone formation (‘dactylitis’). These changes are usu-
less. Asking patients ‘How much do you drink?’ is ally transient, but treatment is required for pain.
unlikely to elicit an accurate response. However, the In older children a typical feature is recurrent
presence of raised serum triglyceride and g-GT lev- episodes of severe pain, sometimes associated with
els, together with an increased mean corpuscular vol- fever. These ‘crises’, which may affect almost any part
ume (MCV), is suggestive of excessive alcohol of the body, are thought to be due to infarcts.
intake. Osteonecrosis of the femoral head is common, both
in children (when it is sometimes mistaken for Perthes’
disease) and in young adults, in whom other causes of
non-traumatic osteonecrosis have to be excluded
SICKLE-CELL DISEASE (Iwegbu and Fleming, 1985). Males and females are
affected with almost equal frequency. The child devel-
Sickle-cell disease is a genetic disorder in which the ops a painful limp and movements are restricted.
red cells contain abnormal haemoglobin (HbS). In X-rays may show no more than a diffuse increase in
deoxygenated blood there is increased aggregation of density of the epiphysis; however, in most cases the
the haemoglobin molecules and distortion of the red changes are very similar to those of Perthes’ disease,
cells, which become somewhat sickle-shaped. At first usually going on to flattening of the epiphysis. In
this is reversible and the cells reacquire their normal young adults there are both destructive lesions and
shape when the blood is oxygenated. Eventually, how- diffuse sclerosis of the femoral head. The head of the
ever, the red cell membrane becomes damaged and humerus and the femoral condyles may be similarly
the cells are permanently deformed. affected.
The sickle-cell trait, which originated in West and Other bone changes are due to a combination of
Central Africa centuries ago, is an example of natural marrow hyperplasia and medullary infarctions. Tra-
selection for survival in areas where malaria was becular coarsening and thickening of the cortices may
110 endemic. From there the gene was carried to coun- be mistaken for signs of infection.
vatism. Anaesthesia carries definite risks; failure to 6
maintain adequate oxygenation may precipitate vascu-
lar occlusion in the central nervous system, lungs or
kidneys. Prophylactic antibiotics are advisable as the
risk of postoperative infection is high.

CAISSON DISEASE AND DYSBARIC
OSTEONECROSIS
(a)
Decompression sickness (caisson disease) and
osteonecrosis are important causes of disability in
deep-sea divers and compressed-air workers building
tunnels or underwater structures. Under increased air
pressure the blood and other tissues (especially fat)
become supersaturated with nitrogen; if decompres-
sion is too rapid the gas is released as bubbles, which
cause local tissue damage, generalized embolic phe-
nomena and intracapillary coagulation. Prolonged
compression may also cause swelling of marrow fat
cells and decreased intramedullary blood flow, possi-
(b) (c) bly due to oxygen toxicity (Pooley and Walder, 1984).
The symptoms of decompression sickness, which
6.9 Sickle-cell disease (a) Typical features of may develop within minutes, are pain near the joints
osteonecrosis are seen in the femoral head, often
accompanied by patchy areas of bone destruction and (‘the bends’), breathing difficulty and vertigo (‘the
endosteal sclerosis in the femoral shaft. (b) The spine also staggers’). In the most acute cases there can be circu-
may be involved, producing appearances similar to those of latory and respiratory collapse, severe neurological
bone infection. (c) In severe cases infarctions of tubular changes, coma and death. Only 10 per cent of patients
bones may resemble osteomyelitis, with sequestra and a with bone necrosis give a history of decompression
marked periosteal reaction.
sickness.
Radiological bone lesions have been found in 17 per
cent of compressed-air workers in the UK; almost half
Bacterial osteomyelitis and septic arthritis, some- the lesions are juxta-articular – mainly in the humeral
times involving multiple sites, are serious complica- head and femoral head – but microscopic bone death is
tions, particularly in children. In over 50 per cent of much more widespread than x-rays suggest.
cases the organism is Salmonella. Clinical and x-ray features The necrosis may cause pain
and loss of joint movement, but many lesions remain
Treatment ‘silent’ and are found only on routine x-ray examina-
tion. Medullary infarcts cause mottled calcification or
A follow-up study of untreated children with femoral areas of dense sclerosis. Juxta-articular changes are
head necrosis due to sickle-cell disease showed that 80 similar to those in other forms of osteonecrosis.
per cent of them had permanently damaged hips with
severe loss of function (Hernigou et al., 1991). This Management The aim is prevention; the incidence of
may be due to recurrent infarction and inflammatory osteonecrosis is proportional to the working pressure,
changes in the joint. the length of exposure, the rate of decompression and
Hypoxic conditions favouring the occurrence of the number of exposures. Strict enforcement of suit-
crises should be avoided. If episodes of bone pain are able working schedules has reduced the risks consid-
frequent, transfusions may be necessary to reduce the erably. The treatment of established lesions follows
concentration of HbS. During a crisis the patient the principles already outlined.
should be given adequate analgesia and should be kept
fully oxygenated. Infections should be guarded against,
or treated promptly with the appropriate antibiotics. GAUCHER’S DISEASE (see also page 177)
Femoral head necrosis in children should be treated
in the same way as Perthes’ disease (see page 511). In this familial disorder lack of a specific enzyme
Adults are treated along the lines described on page results in the abnormal storage of glucocerebroside in
531. The emphasis in all cases should be on conser- the macrophages of the reticuloendothelial system. 111
6 The effects are seen chiefly in the liver, spleen and number of bones. A special feature (due to replacement
bone marrow, where the large polyhedral ‘Gaucher of myeloid tissue by Gaucher cells) is expansion of the
cells’ accumulate. Bone complications are common tubular bones, especially the distal femur, producing
and osteonecrosis is among the worst of them. The the Erlenmeyer flask appearance. Cortical thinning and
hip is most frequently affected, but lesions also appear osteoporosis may lead to pathological fracture.
in the distal femur, the talus and the head of the
humerus. Bone ischaemia is usually attributed to the

Treatment
increase in medullary cell volume and sinusoidal com-
pression, but it is likely that other effects (abnormal The condition can now be treated by replacement of
cell emboli and increased blood viscosity) are equally the missing enzyme and there is evidence that this will
important. reduce the incidence of bone complications.
The management of established osteonecrosis fol-
lows the principles outlined earlier. However, there is
Clinical features a greater risk of infection following operation and
Bone necrosis may occur at any age and causes pain suitable precautions should be taken. For adults, total
around one of the larger joints (usually the hip). In joint replacement is probably preferable to other pro-
longstanding cases movements are restricted. There is cedures.
a tendency for the Gaucher deposits to become
infected and the patient may present with septicaemia.
Blood tests reveal anaemia, leucopenia and thrombo- RADIATION NECROSIS
cytopaenia. A diagnostic, though inconstant, finding
is a raised serum acid phosphatase level. Ionizing radiation, if sufficiently intense or prolonged,
may cause bone death. This is due to the combined
effects of damage to small blood vessels, marrow cells
X-ray and bone cells. Such changes, which are dose-related,
The appearances resemble those in other types of often occurred in the past when low-energy radiation
osteonecrosis, and ‘silent’ lesions may be found in a was in use. Nowadays, with megavoltage apparatus
and more sophisticated planning techniques, long-
term bone damage is much less likely; patients who
present with osteonecrosis are usually those who were
treated some years ago. Areas affected are mainly the
shoulder and ribs (after external irradiation for breast
cancer), the sacrum, pelvis and hip (after irradiation of
pelvic lesions) and the jaws (after treatment of
tumours around the head and neck).
(a)
(c) (d)
(b)
6.10 Gaucher’s disease (a) Gaucher deposits are seen (a) (b)
throughout the femur. The cortices are thin and there is
osteonecrosis of the femoral head. (b) Bone infarction is 6.11 Radiation necrosis – x-rays This patient received
seen in the distal end of the tibia and the talus. (c) The radiation therapy for carcinoma of the bladder. One year
typical Erlenmeyer flask appearance is seen in the x-ray of later he developed pain in the left hip and x-ray showed
this teenager. (d) Ten years later the bone changes are (a) a fracture of the acetabulum. Diagnosis of radiation
much more marked, the cortices are extremely thin and the necrosis was confirmed when (b) the fracture failed to heal
112 patient has obviously suffered a pathological fracture. and the joint crumbled.
Pathology OSTEOCHONDROSIS (OSTEOCHONDRITIS) 6
Unlike the common forms of ischaemic necrosis, The terms ‘osteochondrosis’ or ‘osteochondritis’ have
which always involve subchondral bone, radiation for many years been applied to a group of conditions
necrosis is more diffuse and the effects more variable. in which there is demarcation, and sometimes separa-
Marrow and bone cells die, but for months or even tion and necrosis, of a small segment of articular car-

years there may be no structural change in the bone. tilage and bone. The affected area shows many of the
Gradually, however, stress fractures appear and may features of ischaemic necrosis, including death of
result in widespread bone destruction. A striking fea- bone cells in the osteoarticular fragment and reactive
ture is the absence of repair and remodelling. The sur- vascularity and osteogenesis in the surrounding bone.
rounding bone is usually osteoporotic; in the jaw, The disorder occurs mainly in adolescents and young
infection may follow tooth extraction. adults, often during phases of increased physical activ-
ity, and may be initiated by trauma or repetitive stress.
Clinical features The pathogenesis of these lesions is still not com-
pletely understood. Impact injuries can cause oedema
The patient usually presents with pain around the or bleeding in the subarticular bone, resulting in cap-
shoulder, the hip, the sacrum or the pubic symphysis. illary compression or thrombosis and localized
There will always be a history of previous treatment ischaemia. The critical event may well be a small
by ionizing radiation, though this may not come to osteochondral fracture, too faint to show up on plain
light unless appropriate questions are asked. x-ray examination but often visible on MRI. If the
There may be local signs of irradiation, such as skin crack fails to unite, the isolated fragment may lose its
pigmentation, and the area is usually tender. Move- blood supply and become necrotic. Traction injuries
ments in the nearby joint are restricted. General may similarly damage the blood supply to an apoph-
examination may reveal scars or other evidence of the ysis. However, it is thought that there must be other
original lesion. predisposing factors, for the condition is sometimes
X-rays show areas of bone destruction and patchy multifocal and sometimes runs in families.
sclerosis; in the hip there may be an unsuspected frac-
ture of the acetabulum or femoral neck, or collapse of
the femoral head. Clinical presentation
The classic example of this disorder is the condition
known as osteochondritis dissecans. This occurs typi-
Treatment
cally in young adults, usually men, and affects partic-
Treatment depends on the site of osteonecrosis, the ular sites: the inner (medial) surface of the medial
quality of the surrounding bone and the life femoral condyle in the knee, the anteromedial corner
expectancy of the patient. If a large joint is involved of the talus in the ankle, the superomedial part of the
(e.g. the hip), replacement arthroplasty may be con- femoral head, the humeral capitulum and the head of
sidered; however, bone quality is often poor and there the second metatarsal bone. (Note that these are all
is a high risk of early implant loosening. Nevertheless, slightly bulbous areas with convex articular surfaces).
if pain cannot be adequately controlled, and if the The patient usually complains of intermittent pain;
patient has a reasonable life expectancy, joint replace- sometimes there is swelling and a small effusion in the
ment is justified. joint. If the necrotic fragment becomes completely
(a) (b) (c)

6.12 Osteochondritis dissecans The osteochondral fragment usually remains in place at the articular surface. The most
common sites are (a) the medial femoral condyle, (b) the talus and (c) the capitulum. 113
6 detached it may cause locking of the joint, or unex- osteoporosis of one or both hips affecting women in the
pected episodes of ‘giving way’ in the knee or ankle. last trimester of pregnancy. It is now recognized that
the condition can occur in patients of either sex and at
all ages from late adolescence onwards. Although
Imaging
quite distressing at its onset, the condition typically
X-rays must be taken with the joint in the appropriate lasts for only 6–12 months, after which the symptoms
position to show the affected part of the articular sur- subside and radiographic bone density is restored.
face in tangential projection. The dissecting fragment Sometimes successive joints are affected (‘regional
is defined by a radiolucent line of demarcation. When migratory osteoporosis’), with similar symptoms
it separates, the resulting ‘crater’ may be obvious. occurring at each site.
The early changes (i.e. before demarcation of the The aetiology of this condition is obscure. The
dissecting fragment) are better shown by MRI: there intense activity shown on radionuclide scanning sug-
is decreased signal intensity in the area around the gests a neurovascular abnormality akin to that of
affected osteochondral segment. reflex sympathetic dystrophy (RSD). However, there
Radionuclide scanning with 99mTc-HDP shows are no trophic changes in the soft tissues and no long-
markedly increased activity in the same area. term effects, such as one sees in RSD. The demon-
stration of diffuse changes on MRI – low signal
intensity on T1 weighted images and matching high
Treatment signal intensity on T2 weighted images – correspon-
Treatment in the early stage consists of load reduction ding to the areas of increased scintigraphic activity are
and restriction of activity. In young people complete characteristic of bone marrow oedema (Wilson et al.,
healing may occur, though it can take up to two years. 1988), and this is now thought to be an important
For a large joint like the knee, it is generally recom- aspect of transient osteoporosis. What causes it is still
mended that partially detached fragments be pinned unknown.
back in position after roughening of the base, while Similar ‘marrow oedema changes’ are sometimes
completely detached fragments should be pinned back seen in areas around typical lesions of osteonecrosis
only if they are fairly large and completely preserved. and it has been suggested that transient osteoporosis
These procedures may be carried out by arthroscopy. is due to a sub-lethal, reversible episode of ischaemia
If the fragment becomes detached and causes symp- associated with reactive hyperaemia in the surround-
toms, it should be fixed back in position or else coming bone (Hofmann et al., 1993). Many would dis-
pletely removed. agree with this hypothesis; the most significant
Treatment of osteochondrosis at the elbow, wrist differences between the two conditions are listed in
and metatarsal head is discussed in the relevant chap- Table 6.2. The issue is important because transient
ters. osteoporosis has until now been regarded as a
reversible disorder which requires only symptomatic
treatment while osteonecrosis often calls for operative
‘Spontaneous’ osteonecrosis of the knee intervention.
(‘SONK’)
This condition is similar to osteochondritis dissecans
of the medial femoral condyle, but is distinguished by
three important features: it appears in elderly people
(usually women) who are osteoporotic and the lesion
invariably appears on the highest part of the medial
femoral condyle. A detailed description appears in
Chapter 20.
BONE MARROW OEDEMA

SYNDROME
6.13 Bone marrow oedema MRI showing the typical
In 1959 Curtiss and Kincaid described an uncommon diffuse area of low signal intensity in the right femoral
clinical syndrome characterized by pain and transient head in the T1 weighted image.
114
Table 6.2 Differences between transient bone mar- Hernigou P, Galacteros F, Bachir D, et al. Deformities of 6
row oedema and osteonecrosis the hip in adults who have sickle-cell disease and had avas-
cular necrosis in childhood. J Bone Joint Surg 1991; 73A:
Bone marrow Osteonecrosis
81–92.
oedema
Hofmann S, Engel A, Neuhold A, et al. Bone-marrow
Sex distribution (M:F) 1:3 1:1 oedema syndrome and transient osteoporosis of the hip.

Predisposing factors Pregnancy Systemic disorders J Bone Joint Surg 1993; 75B: 210–16.
Corticosteroids Iwegbu CF, Fleming AF. Avascular necrosis of the femoral
head in sickle-cell disease. J Bone Joint Surg 1985; 67B:
Onset Acute Gradual
29–32.
Clinical progress Self-limiting Progressive Jones JP Jr. Concepts of etiology and early pathogenesis of
X-ray Osteopaenia Sclerosis osteonecrosis. In Schafer IM (ed.). Instructional Course
Scintigraphy Increased activity Reduced activity Lectures, Am Acad Orthop Surg 1994; 43: 499–512.
Lai KA, Shen WJ, Yang CY et al. The use of alendronate
MRI Diffuse changes Focal changes
to prevent early collapse of the femoral head in patients
Histology Marrow oedema Marrow necrosis with nontraumatic osteonecrosis. J Bone Joint Surg 2005;
Minimal bone death Bone necrosis 87A: 2155–59.
Nishii T, Sugano N, Miki H et al. Does alendronate pre-
vent collapse in osteonecrosis of the femoral head? Clin
Orthop Relat Res 2006; 443: 273–9.
REFERENCES AND FURTHER READING Pooley J, Walder DN. The effect of compressed air on
bone marrow blood flow and its relationship to caisson
Asherson RA, Lioté F, Page B et al. Avascular necrosis of disease of bone. In Bone Circulation eds Arlet J, Ficat RP,
bone and antiphospholipid antibodies in systemic lupus Hungerford DS. Baltimore, Williams & Wilkins, pp 63–
erythematosus. J Rheum 1993; 20: 284–8. 67, 1984.
Curtiss PH, Kincaid WE. Transitory demineralization of the Sakamoto M, Shimizu K, Iida S, et al. Osteonecrosis of
hip in pregnancy: A report of three cases. J Bone Joint the femoral head. A prospective study with MRI. J Bone
Surg 1959; 41A: 1327–33. Joint Surg 1997; 79B: 213–19.
Ficat RP. Idiopathic bone necrosis of the femoral head: Shimizu K, Moriya H, Akita T. Prediction of collapse with
Early diagnosis and treatment. J Bone Joint Surg 1985; magnetic resonance imaging of avascular necrosis of the
67B: 3–9. femoral head. J Bone Joint Surg 1994; 76A: 215–23.
Ficat RP, Arlet J. Ischemia and Necroses of Bone (edited and Solomon L, Pearse MF. Osteonecrosis following low-dose
adapted by DS Hungerford), Williams & Wilkins, Balti- short-course corticosteroids. J Orthop Rheumatol 1994;
more, 1980. 7: 203–5.
Francis RB Jr. Platelets, coagulation and fibrinolysis in Steinberg ME, Hayken GD, Steinberg DR. A quantitative
sickle-cell disease: Their possible role in vascular occlu- system for staging avascular necrosis. J Bone Joint Surg
sion. Blood Coagul Fibrinolysis 1991; 2: 341–53. 1995; 77B: 34–41.
Glueck CJ, Crawford A, Roy D et al. Association of Whitehead TP, Clarke CA, Whitfield AGW. Biochemical
antithrombotic factor deficiencies and hypofibrinolysis and haematological markers of alcohol intake. Lancet
with Legg-Perthes’ disease. J Bone Joint Surg 1996; 78A: 1978; 1: 978–81.
3–13. Wilson AJ, Murphy WA, Hardy DC, et al. Transient
Glueck CJ, Freiberg R, Tracy T et al. Thrombophilia and osteoporosis: transient bone marrow oedema? Radiology
hypofibrinolysis. Pathophysiologies of osteonecrosis. Clin 1988; 167: 757–60.
Orthop 1997a; 334: 43–56. Yamamoto T, Bullough PG. Spontaneous osteonecrosis of
Glueck CJ, Crawford A, Roy D et al. Correspondence. the knee: the result of spontaneous insufficiency fracture.
J Bone Joint Surg 1997b; 79A: 1114–15. J Bone Joint Surg 2000; 82A: 858–66.
Guerra JJ, Steinberg ME. Distinguishing transient osteo- Yamamoto T, Kubo T, Hirasawa Y, et al. A clinicopatho-
porosis from avascular necrosis of the hip. J Bone Joint logic study of transient osteoporosis of the hip. Skeletal
Surg 1995; 77A: 616–24. Radiol 1999; 28: 621–7.
115
Metabolic and
endocrine disorders 7
Louis Solomon
Metabolic bone disorders are associated with critical From then onwards further development goes hand
alterations in the regulation of bone formation, bone in hand with growth. Bone formation in the cartilagi-
resorption and distribution of minerals in bone. Clin- nous model progresses along the diaphysis but the
ical features arise from both systemic responses to epiphyseal ends remain unossified until after birth.
changes in mineral exchange and local effects of The entire sequence has been aptly summarized as
abnormal bone structure and composition. condensation → chondrification → ossification.
Orthopaedic surgeons deal mainly with the bone Soon after birth secondary ossification centres
abnormalities (e.g. rickety deformities in growing begin to appear in the still cartilaginous ends of the
bones or insufficiency fractures in the elderly) but it is tubular bones, a process that will occur during child-
important also to be aware of the systemic disorders hood in all the endochondrial bones (bones formed in
that may lie behind apparently straightforward cartilage). By then each bone end is defined as an epi-
‘orthopaedic’ defects and to understand the unseen physis, the still-growing cartilage beyond that as the
metabolic changes that influence the outcome of physis and the shaft as the diaphysis.
many of our surgical interventions. Longitudinal growth continues through adoles-
cence until the epiphysis is fully ossified and fused to
the diaphysis. At the same time an increase in bone
girth occurs by a different process – appositional bone
BONE AND BONES formation by generative cells in the deepest layer of
the periosteum. The small cuboidal bones also grow
by interstitial cartilage proliferation and appositional
Understanding of disorders of the musculoskeletal
(periosteal) bone formation.
system begins with a basic knowledge of the anatom-
After the end of bone growth (which varies for dif-
ical structure and physiology of the bones and joints –
ferent bones) no further increase in size occurs, but
the framework that supports the body, protects the
bone and joint remodelling continues throughout life.
soft tissues, transmits load and power from one part of
Where bones connect with each other, i.e. at the
the body to another and mediates movement and
joints, the contact surfaces remain cartilaginous. In
locomotion.
diarthrodial joints (freely movable, synovial joints)
Embryonic development of the limbs begins with
this is hyaline cartilage, which is ideally suited to per-
the appearance of the arm buds at about 4 weeks from
mit low-friction movement and to accommodate both
ovulation and the leg buds shortly afterwards. These
compressive and tensile forces. In synarthroses, where
at first have the appearance of miniature paddles but
greater resistance to shearing forces is needed, the
by around 5 weeks the finger and toe rays become dif-
interface usually consists of tough fibrocartilage (e.g.
ferentiated. By then primitive skeletal elements and
the pubic symphysis).
pre-muscle masses have begun to differentiate in the
limbs. From about 6 weeks after ovulation the primi-
tive cartilaginous bone-models start to become vascu-
larized and primary ossification centres appear in the
chondroid anlage. By now spinal nerves would be
BONE STRUCTURE AND
growing into the limbs. At 7 or 8 weeks cavitation PHYSIOLOGY
occurs where the joints will appear and during the
next few weeks the cartilaginous epiphyseal precursors Bones as structural organs have three main functions:
become vascularized. Between 8 and 12 weeks the support, protection and leverage. They support every
primitive joints and synovium become defined. part of the body in a wide variety of positions and
7
Articular cartilage
Epiphysis
Physis
Metaphysis
Ossification
centre
Physis
Diaphysis
Cartilage
Growth zone
Fetus Child Adolescent Adult
7.1 Stages in bone development Schematic representation of the stages in the development of a tubular bone showing
the progress from diaphyseal ossification, through endochondral growth at the physis and increase in width of the diaphysis
by sub-periosteal appositional bone formation.
load-bearing; they protect important soft tissues such BONE COMPOSITION

as the brain, the spinal cord, the heart and the lungs;
and they act as jointed levers that facilitate a range of Bone consists of a largely collagenous matrix which is
movements from straightforward locomotion to the impregnated with mineral salts and populated by cells
breathtaking feats of musical virtuosos, ballet dancers (osteoblasts and osteoclasts).
and Olympic athletes.
Bone as tissue has an equally important role: it is a
mineral reservoir which helps to regulate the compo-
The matrix
sition – and in particular the calcium ion concentra- Type I collagen fibres, derived from tropocollagen
tion – of the extracellular fluid. For all its solidity, it is molecules produced by osteoblasts, make up over 80
in a continuous state of flux, its internal shape and per cent of the unmineralized matrix. They form a
structure changing from moment to moment in con- network which embodies a mucopolysaccharide (pro-
cert with the normal variations in mechanical function teoglycans) ground substance and also acts as a scaf-
and mineral exchange. fold on which the mineral component – crystalline
All modulations in bone structure and composition hydroxyapatite – is deposited.
are brought about by cellular activity, which is regu- Other non-collagenous proteins exist in small
lated by hormones and local factors; these agents, in amounts in the mineralized matrix – mainly sialopro-
turn, are controlled by alterations in mineral ion con- teins (osteopontin), osteonectin, osteocalcin (bone Gla
centrations. Disruption of this complex interactive protein) and alkaline phosphatases. Their functions
system results in systemic changes in mineral metabo- have not been fully elucidated but they appear to be
118 lism and generalized skeletal abnormalities. involved in the regulation of bone cells and matrix
mineralization. Osteocalcin is produced only by teins and for the mineralization of bone matrix (Peck 7
osteoblasts and its concentration in the blood is, to and Woods, 1988). Stimulated by parathyroid hor-
some extent, a measure of osteoblastic activity. mone (PTH), they play a critical role in the initiation
A number of growth factors have now been identified; and control of osteoclastic activity (page 122).
they are produced by the osteoblasts and some of them, At the end of each bone remodelling cycle the
acting in combination, have a regulatory effect on bone osteoblasts either remain on the newly formed bone
Metabolic and endocrine disorders

cell development, differentiation and metabolism. surface as quiescent lining cells or they become
Bone morphogenetic protein (BMP) – a collection of embedded in the matrix as ‘resting’ osteocytes. Dur-
growth factor proteins – has attracted a great deal of ing advanced ageing their numbers decrease.
attention. It was originally found by Marshall Urist in
Osteocytes These cells can be regarded as spent
1964 (Urist, 1965) and is now produced in purified
osteoblasts; however, they are by no means inactive.
form from bone matrix. It has been shown to have the
Lying in their bony lacunae, they communicate with
important property of inducing the differentiation of
each other and with the surface lining cells by slender
progenitor cells into cartilage and thereafter into
cytoplasmic processes. Their function is obscure: they
bone. It is now produced commercially and is being
may, under the influence of PTH, participate in bone
used to enhance osteogenesis in bone fusion opera-
resorption (‘osteocytic osteolysis’) and calcium ion
tions (Rihn et al., 2008).
transport (Peck and Woods, 1988). It has also been
suggested that they are sensitive to mechanical stimuli
Bone mineral and communicate information and changes in stress and
Almost half the bone volume is mineral matter – strain to the active osteoblasts (Skerry et al., 1989) which
mainly calcium and phosphate in the form of crystalline can then modify their osteogenic activity accordingly.
hydroxyapatite which is laid down in osteoid at the cal- Ultimately the ageing osteocytes are phagocytosed
cification front. The interface between bone and during osteoclastic bone resorption and remodelling.
osteoid can be labelled by administering tetracycline, Osteoclasts These large multinucleated cells are the
which is taken up avidly in newly mineralized bone principal mediators of bone resorption. They develop
and shows as a fluorescent band on ultraviolet light from mononuclear precursors in the haemopoietic
microscopy. In mature bone the proportions of cal- marrow (the same lineage as macrophages) under the
cium and phosphate are constant and the molecule is influence of local osteoblastic stromal cells that
firmly bound to collagen. It is important to appreciate generate an essential osteoclast differentiating factor –
that in life ‘demineralization’ of bone occurs only by
resorption of the entire matrix.
While the collagenous component lends tensile
strength to bone, the crystalline mineral enhances its
ability to resist compression.
Unmineralized matrix is known as osteoid; in nor-
mal life it is seen only as a thin layer on surfaces where
active new bone formation is taking place, but the
proportion of osteoid to mineralized bone increases
significantly in rickets and osteomalacia.
Bone cells
There are three types of bone cell: osteoblasts, osteo-
cytes and osteoclasts.
Osteoblasts Osteoblasts are concerned with bone
formation and osteoclast activation. They are derived
from mesenchymal precursors in the bone marrow and
the deep layer of the periosteum. Differentiation is
controlled by a number of interacting growth factors, (a) (b)
including bone morphogenetic proteins. 7.2 Bone cells (a) Histological section showing a
Mature osteoblasts form rows of small (20 μm) trabecula lined on one surface by excavating osteoclasts
mononuclear cells along the free surfaces of trabecu- and on the other surface by a string of much smaller
lae and haversian systems where osteoid is laid down osteoblasts. These two types of cell, working in concert,
continuously remodel the internal bone structure. (b) In
prior to calcification. They are rich in alkaline phos- compact bone the osteoclasts burrow deeply into the
phatase and are responsible for the production of type existing bone, with the osteoblasts following close behind
I collagen as well as the non-collagenous bone pro- to re-line the cavity with new bone. 119
7 receptor activator of nuclear factor-κβ ligand Cancellous bone
(RANKL) – which binds with a specific receptor site Cancellous (trabecular) bone has a honeycomb
(RANK) on the osteoclast precursors. appearance; it makes up the interior meshwork of all
Mature osteoclasts have a foamy appearance, due to bones and is particularly well developed in the ends of
the presence of numerous vesicles in the cytoplasm. In the tubular bones and the vertebral bodies. The struc-
response to appropriate stimuli the osteoclast forms a
tural units of trabecular bone are flattened sheets or

sealed attachment to a bone surface, where the cell spars that can be thought of as unfolded osteons.
membrane develops a ruffled border within which Three-dimensionally the trabecular sheets are inter-
bone resorption takes place. This process, and the connected (like a honeycomb) and arranged accord-
important interactions between RANKL and RANK, ing to the mechanical needs of the structure, the
are discussed further on page 122. thickest and strongest along trajectories of compres-
Following resorption of the bone matrix, the osteo- sive stress and the thinnest in the planes of tensile
clasts are left in shallow excavations – Howship’s lacu- stress. The interconnectedness of this meshwork lends
nae – along free bone surfaces. By identifying these added strength to cancellous bone beyond the simple
excavations one can distinguish ‘resorption surfaces’ effect of tissue mass. The spaces between trabeculae –
from the smooth ‘formation surfaces’ or ‘resting sur- the ‘opened out’ vascular spaces – contain the marrow
faces’ in histological sections. and fine sinusoidal vessels that course through the tis-
sue, nourishing both marrow and bone.
Trabecular bone is obviously more porous than cor-
BONE STRUCTURE tical bone. Although it makes up only one-quarter of
the total skeletal mass, it provides two-thirds of the
Bone in its immature state is called woven bone; the col- total bone surface. Add to this the fact that it is cov-
lagen fibres are arranged haphazardly and the cells ered with marrow and it is easy to understand why the
have no specific orientation. Typically it is found in the effects of metabolic disorders are usually seen first in
early stages of fracture healing, where it acts as a tem- trabecular bone.
porary weld before being replaced by mature bone.
The mature tissue is lamellar bone, in which the col-
lagen fibres are arranged parallel to each other to form Haversian system
multiple layers (or laminae) with the osteocytes lying Bones vary greatly in size and shape. At the most basic
between the lamellae. Unlike woven bone, which is level, however, they are similar: compact on the out-
laid down in fibrous tissue, lamellar bone forms only side and spongy on the inside. Their outer surfaces
on existing bone surfaces. (except at the articular ends) are covered by a tough
Lamellar bone exists in two structurally different periosteal membrane, the deepest layer of which con-
forms, compact (cortical) bone and cancellous (trabec- sists of potentially bone-forming cells. The inner,
ular) bone. endosteal, surfaces are irregular and lined by a fine
endosteal membrane in close contact with the marrow
spaces.
Compact bone
The osteonal pattern in the cortex is usually
Compact (cortical) bone is dense to the naked eye. It is depicted from two-dimensional histological sections.
found where support matters most: the outer walls of A three-dimensional reconstruction would show that
all bones but especially the shafts of tubular bones, and the haversian canals are long branching channels run-
the subchondral plates supporting articular cartilage. It ning in the longitudinal axis of the bone and con-
is made up of compact units – haversian systems or necting extensively with each other and with the
osteons – each of which consists of a central canal (the endosteal and periosteal surfaces by smaller channels
haversian canal) containing blood vessels, lymphatics (Volkmann canals). In this way the vessels in the
and nerves and enclosed by closely packed, more or less haversian canals form a rich anastomotic network
concentric lamellae of bone. Between the lamellae lie between the medullary and periosteal blood supply.
osteocytes, bedded in lacunae which appear to be dis- Blood flow in this capillary network is normally cen-
crete but which are in fact connected by a network of trifugal – from the medullary cavity outwards – and it
fine canaliculi. The haversian canal offers a free surface has long been held that the cortex is supplied entirely
lined by bone cells; its size varies, depending on from this source. However, it seems likely that at least
whether the osteon is in a phase of resorption or for- the outermost layers of the cortex are normally also
mation. During resorption osteoclasts eat into the sur- supplied by periosteal vessels, and if the medullary
rounding lamellae and the canal widens out; during vessels are blocked or destroyed the periosteal circula-
formation osteoblasts lay down new lamellae on the tion can take over entirely and the direction of blood
120 inner surface and the canal closes down again. flow is reversed.
7

(b)
(a) (c)
7.3 The haversian systems (a) A schematic diagram representing a wedge taken from the cortex of a long bone. It
shows the basic elements of compact bone: densely packed osteons, each made up of concentric layers of bone and
osteocytes around a central haversian canal which contains the blood vessels; outer laminae of sub-periosteal bone; and
similar laminae on the interior surface (endosteum) merging into a lattice of cancellous bone. (b,c) Low- and high-power
views showing the osteons in various stages of formation and resorption.
proliferative zone in which the chondrocytes are lined

BONE DEVELOPMENT AND up longitudinally; being capable of interstitial growth,
GROWTH they add progressively to the overall length of the
bone. The older cells in this zone (those ‘left behind’
Bones develop in two different ways: by ossification of nearest the advancing new bone of the diaphysis)
a prior cartilage model or framework (endochondral gradually enlarge and constitute a hypertrophic zone.
ossification) and by direct intramembranous ossification. Close to the interface between cartilage and bone the
cartilage becomes calcified (probably with the
involvement of alkaline phosphatase produced by the
hypertrophic cells); this zone of calcified cartilage
ENDOCHONDRAL OSSIFICATION finally undergoes osteoclastic resorption and, with the
This is the usual manner in which tubular bones ingrowth of new blood vessels from the metaphysis,
develop. At birth the cartilage model is complete and ossification. Woven bone is laid down on the calcified
ossification has already begun at the centre of the dia- scaffolding and this in turn is replaced by lamellar
physis. After secondary ossification of the epiphyseal bone which forms the newest part of the bone shaft,
ends has begun, further growth in length takes place now called the metaphysis.
in the still cartilaginous zone between the extending It should be noted that a similar process takes place
area of diaphyseal bone and the epiphysis. In this way in the late stage of fracture repair.
the still-cartilaginous zone between the ossifying dia-
physis and the epiphysis gradually narrows down but
does not disappear until late adolescence. This actively INTRAMEMBRANOUS OSSIFICATION
growing cartilage disc is called the physis, seated as it
is between the epiphysis and the diaphysis. With the growth in length, the bone also has to increase
The physis (or what used to be known as the in girth and, since a tubular bone is an open cylinder,
‘growth plate’) consists of four distinct zones. Co- this inevitably demands that the medullary cavity in-
extensive with the epiphysis is a zone of resting chon- crease in size proportionately. New bone is added to the
drocytes in haphazard array. This merges into a outside by direct ossification at the deepest layer of the 121
7 long been known that PTH promotes bone resorp-
tion, osteoclasts have no receptor for PTH but the
hormone acts indirectly through its effect on the
vitamin D metabolite 1,25-dihydroxycholecalciferol
[1,25(OH)2D3] and osteoblasts.
Proliferation of osteoclastic progenitor cells
requires the presence of an osteoclast differentiating

factor produced by the stromal osteoblasts after stim-
ulation by (for example) PTH, glucocorticoids or
pro-inflammatory cytokines. It is now known that this
‘osteoclast differentiating factor’ is the receptor acti-
vator of nuclear factor-κβ ligand (RANKL for short),
and that it has to bind with a RANK receptor on the
osteoclast precursor in the presence of a macrophage
colony-stimulating factor (M-CSF) before full matu-
ration and osteoclastic resorption can begin.
It is thought that osteoblasts first ‘prepare’ the
resorption site by removing osteoid from the bone
surface while other matrix constituents act as osteo-
clast attractors. During resorption each osteoclast
forms a sealed attachment to the bone surface where
the cell membrane folds into a characteristic ruffled
border within which hydrochloric acid and proteolytic
enzymes are secreted. At this low pH minerals in the
matrix are dissolved and the organic components are
destroyed by lysosomal enzymes. Calcium and phos-
phate ions are absorbed into the osteoclast vesicles
from where they pass into the extracellular fluid and,
ultimately, the blood stream.
In cancellous bone this process results in thinning
(and sometimes actual perforation) of existing trabec-
ulae. In cortical bone the cells either enlarge an exist-
ing haversian canal or else burrow into the compact
bone to create a cutting cone – like miners sinking a
new shaft in the ground. During hyperactive bone
7.4 Blood supply to bone Schematic presentation of resorption these processes are reflected in the appear-
blood supply in tubular bones. (Reproduced from Bullough ance of hydroxyproline in the urine and a rise in
PG. Atlas of Orthopaedic Pathology: With Clinical and serum calcium and phosphate levels.
Radiological Correlations (2nd edition). Baltimore:
University Park Press, 1985. By kind permission of Dr Peter
G Bullough and Elsevier.)
BONE MODELLING AND REMODELLING
periosteum where mesenchymal cells differentiate into The sequential process of bone resorption and forma-
osteoblasts (intramembranous, or ‘appositional’, bone tion has been likened to sculpting and is, in fact,
formation) and old bone is removed from the inside of known as bone modelling and remodelling. During
the cylinder by osteoclastic endosteal resorption. growth each bone has continuously to be ‘sculpted’
Intramembranous periosteal new bone formation into the normal shape of that particular part of the
also occurs as a response to periosteal stripping due to skeleton. How else can a long bone retain its basic
trauma, infection or tumour growth, and its appear- shape as the flared ends are constantly re-formed fur-
ance is a useful radiographic pointer. ther and further from the midshaft during growth?
The internal architecture of the bone is also subject
to remodelling, not only during growth but through-
BONE RESORPTION out life. This serves several crucial purposes: ‘old
bone’ is continually replaced by ‘new bone’ and in this
Bone resorption is carried out by the osteoclasts under way the skeleton is protected from exposure to cumu-
the influence of stromal cells (including osteoblasts) lative loading frequencies and the risk of stress failure;
122 and both local and systemic activators. Although it has bone turnover is sensitive to the demands of function
Epiphyseal 7
artery
Reserve cells
Proliferative cells

Hypertrophic cells
Degenerate cells
Calcified zone
Vascular invasion
Ossification
7.5 Endochondral ossification Histological section of a growing endochondral bone with a schematic figure showing
the layers of the growth disc (physis). (Reproduced from Bullough PG. Atlas of Orthopaedic Pathology: With Clinical and
Radiological Correlations (2nd edition). Baltimore: University Park Press, 1985. Second figure by kind permission of
Dr Peter G Bullough and Elsevier.)
and trabeculae are fashioned (or refashioned) in lying in the planes of tensile stress; besides, the main-
accordance with the stresses imposed upon the bone, tenance of calcium homeostasis requires a constant
the thicker and stronger trabeculae following the tra- turnover of the mineral deposits which would other-
jectories of compressive stress and the finer trabeculae wise stay locked in bone.
At each remodelling site work proceeds in an orderly
sequence. Prompted by the osteoblasts, osteoclasts
gather on a free bone surface and proceed to excavate
a cavity. After 2–4 weeks resorption ceases; the osteo-
clasts undergo apoptosis and are phagocytosed. There
is a short quiescent period, then the excavated surface
is covered with osteoblasts and for the next 3 months
osteoid is laid down and mineralized to leave a new
‘packet’ of bone (or osteon). The entire remodelling
cycle takes from 4 to 6 months and at the end the
boundary between ‘old’ and ‘new’ bone is marked by
a histologically identifiable ‘cement line’.
The osteoblasts and osteoclasts participating in
each cycle of bone turnover work in concert, together
acting as a bone remodelling unit (of which there are
more than a million at work in the adult skeleton at
any time). Resorption and formation are coupled, the
one ineluctably following the other. Systemic hor-
mones and local growth factors are involved in coor-
dinating this process; indeed it is likely that PTH and
1,25-(OH)2D are involved in initiating both forma-
tion and resorption. This ensures that (at least over
the short term) a balance is maintained though at any
moment and at any particular site one or other phase
may predominate.
In the long term, change does occur. The annual
rate of bone turnover in healthy adults has been esti-
mated as 4 per cent for cortical bone and 25 per cent
7.6 Wolff’s Law Wolff’s Law is beautifully demonstrated
in the trabecular pattern at the upper end of the femur. for trabecular bone (Parfitt, 1988). The rate may be
The thickest trabeculae are arranged along the trajectories increased or decreased either by alterations in the
of greatest stress. number of remodelling units at work or by changes in 123
7 the remodelling time. During the first half of life for- rapidly compensated for by changes in renal tubular
mation slightly exceeds resorption and bone mass absorption. A more persistent fall in extracellular cal-
increases; in later years resorption exceeds formation cium concentration can be accommodated by increas-
and bone mass steadily diminishes. Connecting spars ing bone resorption.
may be perforated or lost, further diminishing bone All these adjustments are regulated by PTH, 1,25-
strength and increasing the likelihood of fragility (OH)2 D and an array of systemic and local growth
fractures. Rapid bone loss is usually due to excessive factors.

resorption rather than diminished formation.
Calcium
Local regulation of bone remodelling
Calcium is essential for normal cell function and phys-
The coordinated interaction between osteoblastic iological processes such as blood coagulation, nerve
bone formation and osteoclastic resorption has been conduction and muscle contraction. An uncompen-
explained to a large extent by elucidation of the sated fall in extracellular calcium concentration
RANKL/RANK connection. However, another (hypocalcaemia) may cause tetany; an excessive rise
cytokine – osteoprotegerin (OPG) – comes into play (hypercalcaemia) can lead to depressed neuromuscu-
in the regulatory mechanism of this system. OPG, lar transmission.
which is also expressed by osteoblasts, is able to The main sources of calcium are dairy products,
inhibit the differentiation of osteoclast precursors by green vegetables and soya (or fortified foods). The
preferentially binding with RANKL (acting as a recommended daily intake for adults is 800–1000 mg
‘decoy’ receptor) and so reducing bone resorption by (20–25 mmol), and ideally this should be increased to
preventing RANKL from binding with its receptor on 1200 mg during pregnancy and lactation. Children
the osteoclast precursor. need less, about 200–400 mg per day.
Bone remodelling is, therefore, influenced continu- About 50 per cent of the dietary calcium is absorbed
ously by an array of hormones, cytokine systems, (mainly in the upper gut) but much of that is secreted
dietary elements, medication and signals from back into the bowel and only about 200 mg (5 mmol)
mechanical stresses that impinge on any part of the enters the circulation. The normal concentration in
RANKL/RANK/OPG triad. Already explanations plasma and extracellular fluid is 2.2–2.6 mmol/l (8.8–
involving this system have been advanced for the 10.4 mg/dL). Much of this is bound to protein; about
occurrence of osteoporosis in metastatic bone disease, half (1.1 mmol) is ionized and effective in cell metab-
myelomatosis, rheumatoid arthritis and other inflam- olism and the regulation of calcium homoeostasis.
matory conditions. There is promise also that down- Calcium absorption in the intestine is promoted by
regulation of osteoclastogenesis may offer an effective vitamin D metabolites, particularly 1,25-(OH)2 vitamin
treatment for age-related osteoporosis. D, and requires a suitable calcium/phosphate ratio.
It has been said, with good reason, that the Absorption is inhibited by excessive intake of phos-
RANKL/RANK/OPG signalling system is ‘…one of phates (common in soft drinks), oxalates (found in tea
the most important discoveries in bone biology in the and coffee), phytates (chapatti flour) and fats, by the
past decade’ (Boyce and Xing, 2007). administration of certain drugs (including corticos-
teroids) and in malabsorption disorders of the bowel.
Urinary excretion varies between 2.5 and 5 mmol
(100–200 mg) per 24 hours. If the plasma ionized cal-
MINERAL EXCHANGE AND BONE cium concentration falls, PTH is released and causes
TURNOVER (a) increased renal tubular reabsorption of calcium and
(b) a switch to increased 1,25-(OH)2 vitamin D pro-
Calcium and phosphorus have an essential role in a duction and enhanced intestinal calcium absorption. If
wide range of physiological processes. Over 98 per the calcium concentration remains low, calcium is
cent of the body’s calcium and 85 per cent of its phos- drawn from the skeleton by increased bone resorption,
phorus are tightly packed as hydroxyapatite crystals in which again is under the indirect influence of PTH.
bone and capable of only very slow exchange. A small
Hypocalcaemia The classic feature of hypocalcaemia is
amount exists in a rapidly exchangeable form, either
the development of tetany. Patients may complain of
in partially formed crystals or in the extracellular fluid
loss of sensation, paraesthesiae and muscle spasms.
and blood where their concentration is maintained
More severe signs are convulsions and laryngeal spasm.
within very narrow limits by an efficient homeostatic
mechanism involving intestinal absorption, renal Hypercalcaemia Clinical features vary with the degree
excretion and mineral exchange in bone. of hypercalcaemia: a mild elevation of serum calcium
The control of calcium is more critical than that of concentration may cause no more than general
124 phosphate. Transient alterations in blood levels are lassitude, polyuria and polydipsia. With plasma levels
between 3 and 3.5 mmol/L, patients may complain of and appear in high concentration in bone. Magne- 7
anorexia, nausea, muscle weakness and fatigue. Those sium is necessary for the efficient secretion and
with severe hypercalcaemia (more than 3.5 mmol/L) peripheral action of parathyroid hormone. Thus, if
have a plethora of symptoms including abdominal pain, hypocalcaemia is accompanied by hypomagnesaemia
nausea, vomiting, severe fatigue and depression. In it cannot be fully corrected until normal magnesium
longstanding cases patients may develop kidney stones concentration is restored.

or nephrocalcinosis due to chronic hypercalciuria;
some complain of joint symptoms, due to Vitamin D
chondrocalcinosis. The clinical picture is aptly (though
unkindly) summarized in the old adage ‘moans, Vitamin D, through its active metabolites, is princi-
groans, bones and stones’. pally concerned with calcium absorption and transport
There may also be symptoms and signs of the and (acting together with PTH) bone remodelling.
underlying cause, which should always be sought (in Target organs are the small intestine and bone.
the vast majority this will be hyperparathyroidism, Naturally occurring vitamin D (cholecalciferol) is
metastatic bone disease, myelomatosis, Paget’s disease derived from two sources: directly from the diet and
or renal failure). indirectly by the action of ultraviolet light on the pre-
cursor 7-dehydrocholesterol in the skin. For people
who do not receive adequate exposure to bright sun-
Phosphorus light, the recommended daily requirement for adults is
Apart from its role (with calcium) in the composition 400–800 IU (10–20 μg) per day – the higher dose for
of hydroxyapatite crystals in bone, phosphorus is people over 70 years of age. In most countries this is
needed for many important metabolic processes, obtained mainly from exposure to sunlight; those who
including energy transport and intracellular cell sig- lack such exposure are likely to suffer from vitamin D
nalling. It is abundantly available in the diet and is deficiency unless they take dietary supplements.
absorbed in the small intestine, more or less in pro- Vitamin D itself is inactive. Conversion to active
portion to the amount ingested; however, absorption metabolites (which function as hormones) takes place
is reduced in the presence of antacids such as alu- first in the liver by 25-hydroxylation to form 25-
minium hydroxide, which binds phosphorus in the hydroxycholecalciferol [25-OHD], and then in the
gut. Phosphate excretion is extremely efficient, but 90 kidneys by further hydroxylation to 1,25-dihydroxy-
per cent is reabsorbed in the proximal tubules. Plasma cholecalciferol [1,25-(OH)2D]. The enzyme responsi-
concentration – almost entirely in the form of ionized ble for this conversion is activated mainly by PTH, but
inorganic phosphate (Pi) – is normally maintained at also by other hormones (including oestrogen and pro-
0.9–1.3 mmol/L (2.8–4.0 mg/dL). lactin) or by an abnormally low concentration of phos-
The solubility product of calcium and phosphate is phate. If the PTH concentration falls and phosphate
held at a fairly constant level; any increase in the one remains high, 25-OHD is converted alternatively to
will cause the other to fall. The main regulators of 24,25-(OH)2D which is inactive. On the other hand,
plasma phosphate concentration are PTH and 1,25- during negative calcium balance production switches
(OH)2D. If the Pi rises abnormally, a reciprocal fall in to 1,25-(OH)2D in response to PTH secretion (see
calcium concentration will stimulate PTH secretion below); the increased 1,25-(OH)2D then helps to
which in turn will suppress urinary tubular reabsorp- restore the serum calcium concentration.
tion of Pi, resulting in increased Pi excretion and a fall
in plasma Pi. High Pi levels also result in diminished
1,25-(OH)2D production, causing reduced intestinal
absorption of phosphorus.
In recent years interest has centred on another group
of hormones or growth factors which also have the ef-
fect of suppressing tubular reabsorption of phosphate
independently of PTH. These so-called ‘phospho-
tonins’ are associated with rare phosphate-losing dis-
orders and tumour-induced osteomalacia. Their exact
role in normal physiology is still under investigation.
7.7 Vitamin D metabolism The active vitamin D

Magnesium metabolites are derived either from the diet or by
conversion of precursors when the skin is exposed to
Magnesium plays a small but important part in min- sunlight. The inactive ‘vitamin’ is hydroxylated, first in the
eral homeostasis. The cations are distributed in the liver and then in the kidney, to form the active metabolites
cellular and extracellular compartments of the body 25-HCC and 1,25-DHCC. 125
7 The terminal metabolite, 1,25-(OH)2D (calcitriol) In the intestine PTH has the indirect effect of
acts on the lining cells of the small intestine to increase stimulating calcium absorption by promoting the
the absorption of calcium and phosphate. In bone it conversion of 25-OHD to 1,25-(OH)2D in the
promotes osteoclastic resorption; it also enhances cal- kidney.
cium transport across the cell membrane and indi- In bone PTH acts to promote osteoclastic resorp-
rectly assists with osteoid mineralization. tion and the release of calcium and phosphate into the
Some antiepileptic drugs interfere with the vitamin blood. This it does not by direct action on osteoclasts
D metabolic pathway and may cause vitamin D defi- but by stimulating osteoblastic activity, increased
ciency. expression of RANKL and diminished production of
The concentration of all the active metabolites can OPG, thus leading to enhanced osteoclast differentia-
be measured in serum samples, the best indicator of tion and maturation (see page 124). Furthermore, the
vitamin D status being 25-OHD concentration PTH-induced rise in 1,25(OH)2D also has the effect
(serum 1,25-(OH)2D has a half-life of only 15 hours of stimulating osteoclastogenesis. The net effect of
and is therefore not as good an indicator). The rec- these complex interactions is a prolonged rise in
ommended serum concentration is 25–30 ng/L, a plasma calcium.
level which is often not achieved in elderly people,
especially in northern climes.
Calcitonin
Calcitonin, which is secreted by the C cells of the thy-
Parathyroid hormone roid, does more or less the opposite of PTH: it binds
Parathyroid hormone (PTH) is the fine regulator of to receptors on the osteoclasts, suppresses osteoclastic
calcium exchange, controlling the concentration of bone resorption and increases renal calcium excretion.
extracellular calcium between critical limits by either This occurs especially when bone turnover is high, as
direct or indirect action on the renal tubules, the renal in Paget’s disease. Its secretion is stimulated by a rise
parenchyma, the intestine and bone. in serum calcium concentration above 2.25 mmol/L
Production and release are stimulated by a fall and (9 mg/dL).
suppressed (up to a point) by a rise in plasma ionized
calcium. The active terminal fragment of the PTH
molecule can be readily estimated in blood samples.
Gonadal hormones
Acting on the renal tubules, PTH increases phos- In addition to their effects on bone growth, gonadal
phate excretion by restricting its reabsorption, and hormones have an important role in maintaining bone
conserves calcium by increasing its reabsorption. mass and trabecular integrity. Oestrogen appears to act
These responses rapidly compensate for any change in on both osteoblasts and osteoclasts and is now believed
plasma ionized calcium. to work via the RANKL/RANK/OPG system. It in-
Acting on the kidney parenchyma, PTH controls creases the production and activity of OPG, thereby
hydroxylation of the vitamin D metabolite 25-OHD; interfering with osteoclast differentiation and bone
a rise in PTH concentration stimulates conversion to resorption. Oestrogen is also thought to enhance
the active metabolite 1,25-(OH)2D and a fall in PTH calcium absorption by the intestine. It is well known
causes a switch towards the inactive metabolite 24,25- that bone loss accelerates after the menopause and a
(OH)2D. similar effect is seen in amenorrhoeic young women
Table 7.1 Regulation of mineral metabolism by PTH and 1,25-(OH)2 vitamin D
Source Secretion Secretion Effects on Effects on kidney Effects on bone Effect on

increased decreased by intestine serum Ca
by and Pi
PTH Parathyroid Fall in Rise in serum No direct effect Increase in No direct effect but Rise in serum
gland serum Ca Ca but Ca 1,25-(OH)2D resorption Ca
Increase in absorption Increased reabsorption increased via action Fall in serum
1,25-(OH)2D increased of Ca on 1,25-(OH)2D Pi
through Increased excretion of
1,25-(OH)2D phosphate
1,25(OH)2 Kidney Fall in Rise in serum Increased Osteoclastogenesis Rise in serum
vitamin D tubule serum Ca Ca absorption of Ca and increased bone Ca+Pi
Fall in Rise in serum Pi Increased resorption
serum Pi Fall in PTH absorption of
Rise in phosphate
serum PTH
126
who may actually lose bone at a time when their peers Electrical stimulation 7
are building up to peak bone mass.
Androgens also retard bone resorption, though the When bone is loaded or deformed, small electrical
signalling pathway is somewhat uncertain. Bone loss potentials are generated – negative on compressed
increases after the male climacteric, which occurs 15– surfaces and positive on surfaces under tension
20 years later than the female menopause. (Brighton and McCluskey, 1986). This observation

led to the idea that stress-generated changes in bone
mass may be mediated by electrical signals; from this
Glucocorticoids it was a logical step to suggest that induced electrical
Corticosteroids in excess cause a pernicious type of potentials can affect bone formation and resorption.
osteoporosis due to a combination of factors: dimin- How, precisely, this is mediated remains unknown.
ished osteoblastic bone formation (the most impor- Electromagnetic field potentials have been used for
tant effect), an adverse effect on collagen, decreased the treatment of delayed fracture union and regional
intestinal calcium absorption and increased calcium osteoporosis, so far with inconclusive results.
excretion. RANKL expression by osteoblasts is
enhanced and OPG expression is opposed, leading to Other environmental factors
increased osteoclastogenesis and bone resorption. Moderate rises in temperature or oxygen tension have
been shown experimentally to increase bone forma-
Thyroxine tion. Acid–base balance affects bone resorption, which
is increased in chronic acidosis and decreased in alka-
Thyroxine increases both formation and resorption, losis.
but more so the latter; hyperthyroidism is associated Increased dietary phosphates or pyrophosphates tend
with high bone turnover and osteoporosis. to inhibit bone resorption. Pyrophosphate analogues
(bisphosphonates) are used in the treatment of osteo-
Local factors porosis, where they appear to inhibit both resorption
and formation.
The intimate processes of signalling between Fluoride has complex effects on bone, the most
osteoblasts and osteoclasts, cell recruitment and acti- important being direct stimulation of osteoblastic
vation, spatial organization and mineral transport are activity, the formation of fluorapatite crystals (which
mediated by local factors derived from bone cells, are resistant to osteoclastic resorption) and an appar-
matrix components and cells of the immune system. ent increase in mineral density without a concomitant
Some serve as messengers between systemic and local gain in strength; there is also evidence of calcium
agents, or between the various cells that are responsi- retention and secondary hyperparathyroidism. Fluoro-
ble for bone remodelling; others are important in pro- sis occurs as an endemic disorder in India and some
moting bone resorption in inflammatory disorders other parts of the world due to an excess of fluoride
and fractures and may also account for the bone in the drinking water.
destruction and hypercalcaemia in metastatic bone
disease and myelomatosis.
AGE-RELATED CHANGES IN BONE

Mechanical stress
It is well known that the direction and thickness of tra- During childhood each bone increases in size and
beculae in cancellous bone are related to regional stress changes somewhat in shape. At the epiphyseal growth
trajectories. This is recognized in Wolff’s Law (1896), plate (physis), new bone is added by endochondral
which says that the architecture and mass of the skele- ossification; on the surface, bone is formed directly by
ton are adjusted to withstand the prevailing forces sub-periosteal appositional ossification; the medullary
imposed by functional need or deformity. Physiological cavity is expanded by endosteal bone resorption; bul-
stress is supplied by gravity, load-bearing, muscle action bous bone ends are re-formed and sculpted continu-
and vascular pulsation. If a continuous bending force is ously by coordinated formation and resorption.
applied, more bone will form on the concave surfaces Although during childhood each bone gets longer
(where there is compression) and bone will thin down and wider, the bone tissue of which it is made remains
on the convex surfaces (which are under tension). quite porous.
Weightlessness, prolonged bed rest, lack of exercise, Between puberty and 30 years of age the haversian
muscular weakness and limb immobilization are all canals and intertrabecular spaces are to some extent
associated with osteoporosis. How physical signals are filled in and the cortices increase in overall thickness; i.e.
transmitted to bone cells is not known, but they almost the bones become heavier and stronger. Bone mass in-
certainly operate through local growth factors. creases at the rate of about 3 per cent per year and dur- 127
7 trabecular bone. This steady depletion is due mainly
to excessive resorption – osteoclastic activity seeming
to be released from the restraining influence of
gonadal hormone. (Similar changes are seen in
younger women about 5 years after oophorectomy).
About 30 per cent of white women will lose bone to
the extent of developing postmenopausal osteoporo-

sis. For reasons that are not yet fully understood, the
degree of bone depletion is less marked in blacks than
in whites (Solomon 1968).
From the age of 65 or 70 years the rate of bone loss
in women gradually tails off and by the age of 75 years
(a) (b) it is about 0.5 per cent per year. This later phase of
depletion is due mainly to diminishing osteoblastic
activity (Parfitt, 1988).
Men are affected in a similar manner, but the phase
of rapid bone loss occurs 15 or 20 years later than in
women, at the climacteric.
Bone mass and bone strength

It is important to recognize that throughout life, and
regardless of whether bone mass increases or decreases,
the degree of mineralization in normal people varies
very little from age to age or from one person to an-
(c) (d) other.
7.8 Age-related changes in bone These fine-detail
With advancing years the loss of bone mass is ac-
x-rays of iliac crest biopsies and femoral head slices show companied by a disproportionate loss of bone strength,
the marked contrast between trabecular density in a which is explained in a number of ways. (1) The
healthy 40-year-old woman (a,b) and one of 75 years absolute diminution in bone mass is the most impor-
(c,d). tant, but not the only, factor. (2) With increased post-
menopausal bone resorption, perforations and gaps
ing the third decade each individual attains a state of appear in the plates and cross-spars of trabecular bone;
peak bone mass, the level of which is determined by ge- not all these defects are repaired and the loss of struc-
netic, hormonal, nutritional and environmental fac- tural connectivity further reduces the overall strength
tors. By the end of bone growth, mean bone mass is of the bone. (3) In old age the decrease in bone cell ac-
about 5–10 per cent greater in young men than in tivity makes for a slower remodelling rate; old bone
young women, due mainly to increased appositional takes longer to be replaced and microtrauma to be re-
bone formation when androgen levels rise after puberty paired, thus increasing the likelihood of stress failure.
(Seeman, 2003). At the other end of the scale, young This tendency to increased bone fragility with age is
women with amenorrhoea due to prolonged and in- counteracted to some extent, in tubular bones, by the
tensive exercise or anorexia nervosa tend to have lower fact that as their cortices become thinner they actually
than normal bone mass. The greater the peak bone increase in diameter; i.e. during each remodelling
mass, the less marked will be the effects of the inevitable cycle resorption exceeds formation on the endosteal
depletion which occurs in later life. surface while formation slightly exceeds resorption on
From 30 years onwards there is a slow but inexorable the periosteal surface. Simple mechanics can show
loss of bone; haversian spaces enlarge, trabeculae that, of two cylinders with equal mass, the one with a
become thinner, the endosteal surface is resorbed and greater diameter and thin walls is stronger than one
the medullary space expands, i.e. year by year the with thicker walls but lesser diameter.
bones become slightly more porous. The diminution The boundary between ‘normal’ age-related bone
in bone mass proceeds at a rate of about 0.3 per cent loss and a clinical disorder (osteoporosis) is poorly
per year in men and 0.5 per cent per year in women defined. Factors that have an adverse influence on bone
up to the menopause. mass are shown in Table 7.2. Ageing individuals also
From the onset of the menopause and for the next 10 often have some degree of osteomalacia due to lack of
years the rate of bone loss in women accelerates to dietary vitamin D and poor exposure to sunlight, and
128 about 3 per cent per year, occurring predominantly in this added to the normal age-related bone depletion
Table 7.2 Factors adversely affecting bone mass and their relationship to previous disease or trauma 7
Early onset of menopause
should be carefully considered, especially in older
people who may have suffered insufficiency fractures.
General nutritional deficiency and ill health
Other causal associations are retarded growth, malnu-
Lack of vitamin D, calcium and phosphate trition, dietary fads, intestinal malabsorption, alcohol
Chronic illness abuse and cigarette smoking.

High consumption of alcohol A careful family history may yield clues to heritable
Smoking disorders associated with osteoporosis and vulnerabil-
Inactivity
ity to fracture.
Long-term medication (anti-inflammatory drugs, diuretics,
glucocorticoids, antiepileptic drugs, thyroid hormone) Examination
The patient’s appearance may be suggestive of an
makes them more vulnerable than usual to insuffi- endocrine or metabolic disorder: the moon face and
ciency fractures (Schnitzler and Solomon, 1983). cushingoid build of hypercortisonism; the smooth, hair-
less skin of testicular atrophy; physical underdevelop-
ment and bone deformities in rickets. Thoracic kyphosis
is a non-specific feature of vertebral osteoporosis.
METABOLIC BONE DISORDERS
Patients with metabolic bone disorders usually appear
X-rays
to the orthopaedic surgeon in one of the following Decreased skeletal radiodensity is a late and unreliable
guises: a child with bone deformities (rickets); an elderly sign of bone loss; it becomes apparent only after a 30
person with a fracture of the femoral neck or a verte- per cent reduction in mineral or skeletal mass, and
bral body following comparatively minor trauma (post- even then one cannot tell whether this is due to osteo-
menopausal or post-climacteric osteoporosis); an elderly porosis (a decrease in bone mass) or osteomalacia
patient with bone pain and multiple compression frac- (insufficient mineralization of bone) or a combination
tures of the spine (osteomalacia); a middle-aged person of both. Sometimes the term osteopaenia is used to
with hypercalcaemia and pseudogout (hyperpara- describe a mild or moderate loss of radiodensity in
thyroidism); or someone with multiple fractures and a bone x-rays without implying whether this is patho-
history of prolonged corticosteroid treatment. logical or not.
X-rays may show stress fractures, vertebral compres- A more reliable sign of osteoporosis is a loss of the
sion, cortical thinning, loss of trabecular structure or horizontal trabeculae in vertebral bodies; the remain-
merely an ill-defined loss of radiographic density – ing vertical trabeculae seem, by contrast, to be more
osteopaenia – which can signify either osteomalacia or conspicuous and the vertebral cortices are sharply
osteoporosis. etched around the faded interiors. The presence of
These appearances are so common in old people obvious fractures – new and old – especially in the
that they seldom generate a call for detailed investiga- spine, ribs, pubic rami or corticocancellous junctions
tion. However, in patients under the age of 50, those of the long bones, is suggestive of severe osteoporo-
with repeated fractures or bone deformities and those sis. Small stress fractures are more difficult to detect:
with associated systemic features, a full clinical, radio- they may be found in the femoral neck, the proximal
logical and biochemical evaluation is essential. part of the femur or the upper end of the tibia.
In addition to these general signs of reduced bone
mass or defective mineralization, there may be specific
History
features of bone disorders such as rickets, hyper-
Children are likely to be brought for examination parathyroidism, metastatic bone disease or myelo-
because of failure to thrive, below-normal growth or matosis.
deformity of the lower limbs. Adults may complain of
back pain, the sudden onset of bone pain near one of
the large joints or symptoms suggesting a full-blown
Measurement of bone mass
fracture following some comparatively modest injury. The investigation of bone-losing disorders has been
Generalized muscle weakness is common in osteoma- greatly advanced by the development of methods for
lacia. measuring bone mineral density and bone mass. Meas-
Details such as the patient’s sex, age, race, onset of urement is based on the principle that a beam of energy
menopause, nutritional background, level of physical is attenuated as it passes through bone, and the degree
activity, previous illnesses, medication and operations of attenuation is related to the mass and mineral con-
are important. The onset and duration of symptoms tent of the bone. Bone mineral density (BMD) is ex- 129
7 through bone. The method is simple and not very
expensive. However, it is applicable only to the
appendicular skeleton, and measuring the BMD at the
wrist (for example) does not accurately reflect bone
density in the spine or femoral neck.
Dual-energy x-ray absorptiometry (DXA) This is now the

method of choice (see Fig. 1.23). Precision and
accuracy are excellent, x-ray exposure is not excessive
and measurements can be obtained anywhere in the
skeleton (Mirsky and Einhorn, 1998). Normative
graphs and tables are provided to show where the
obtained measurement falls in relation to age and
gender matched controls; a value of 2.5 standard
deviations or more below the norm is usually taken as
indicative of abnormal loss of bone mass.
Some investigators have reported good correlation
between measurements in the appendicular and axial
skeleton. However, the risk of fracture at any particu-
lar site is best gauged by measuring bone density at
(a) (b) the target site, though it should be noted that the
presence of vertebral spurs or osteophytes and inter-
vertebral bone bridges can make density measure-
ments less reliable for that region. DXA can also
provide a lateral view of the entire spine in one image;
while lacking the higher definition of conventional
x-rays, this is a helpful screening method for identify-
ing vertebral compression fractures.
Quantitative computed tomography (QCT) Quantitative
CT permits measurement of mineral content per unit
volume of bone, which is a three-dimensional
expression of bone density. It also provides separate
(c) values for cortical and cancellous bone. Its main
drawback is the high radiation exposure (compared to
7.9 Clinical and x-ray features An elderly patient
known to have spinal osteoporosis (a) presents with
DXA), and there is as yet no evidence that it is more
sudden onset of pain in the left groin. The plain x-ray accurately predictive of fracture than DXA.
(b) shows a suspicious feature at the base of the femoral
neck. This is enough to call for a bone scan (c) which
reveals increased activity at the suspicious site, confirming Indications for bone densitometry
the diagnosis of a spontaneous stress fracture.
The main indications for using bone densitometry
are: (a) to assess the degree and progress of bone loss
in patients with clinically diagnosed metabolic bone
pressed in grams per unit area (or unit volume in the disease or conditions such as hyperparathyroidism,
case of quantitative computed tomography) and is corticosteroid-induced osteoporosis, gonadal defi-
recorded in comparison to the sex and age specific dis- ciency or other endocrine disorders; (b) as a screening
tribution of these values in the general population. procedure for perimenopausal women with multiple
The measurements are specific for each location (lum- risk factors for osteoporotic fractures; and (c) to mon-
bar spine, femoral neck, distal radius, etc). itor the effect of treatment for osteoporosis. Other
indications are mentioned in Table 7.3.
Radiographic absorptiometry Density is measured using
standard radiographs and comparing the values against
those of an aluminium reference wedge. The method Biochemical tests
is applicable only to appendicular sites such as the hand
Serum calcium and phosphate concentrations should be
or calcaneum and values do not necessarily correlate to
measured in the fasting state, and it is the ionized cal-
findings in the femoral neck or vertebrae.
cium fraction that is important.
This measures the
Single-energy x-ray absorptiometry Serum bone alkaline phosphatase concentration is an
130 attenuation of a collimated photon beam as it passes index of osteoblastic activity; it is raised in osteomala-
Table 7.3 Indications for BMD measurement 7
All postmenopausal women under the age of 65
Young women following oophorectomy
Men with testosterone deficiency
Perimenopausal women with fractures of wrist, ribs, vertebral

bodies or hip
Women or men with x-ray features of osteopaenia
Patients with hyperparathyroidism, hyperthyroidism, renal
insufficiency or rheumatoid arthritis
Patients on long-term glucocorticoids, thyroid hormone,
thiazide diuretics
Patients with dietary deficiencies 7.10 Bone biopsy von Kossa stain showing the unusually
wide osteoid layer (in red) in a patient with osteomalacia.
Some experts would add all women over the age of 65
regardless of risk factors
decalcification) for histological bone volume, osteoid

formation and the relative distribution of formation
cia and in disorders associated with high bone and resorption surfaces. The rate of bone remodelling
turnover (hyperparathyroidism, Paget’s disease, bone can also be gauged by labelling the bone with tetracy-
metastases). cline on two occasions (2 weeks apart) before obtain-
Osteocalcin (Gla protein) is a more specific marker ing the biopsy. Tetracycline is taken up in new bone
of bone formation; elevated serum levels suggest and produces a fluorescent strip on ultraviolet light
increased bone turnover. microscopy. By measuring the distance between the
Parathyroid hormone activity can be estimated from two labels, the rate of new bone formation can be cal-
serum assays of the COOH terminal fragment. How- culated. Characteristically in osteomalacia there is a
ever, in renal failure the test is unreliable because there decrease in the rate of bone turnover and an increase
is reduced clearance of the COOH fragment. in the amount of uncalcified osteoid.
Vitamin D activity is assessed by measuring the
serum 25-OHD concentration. Serum 1,25-(OH)2D
levels do not necessarily reflect vitamin uptake but are
reduced in advanced renal disease. OSTEOPOROSIS
Urinary calcium and phosphate excretion can be
measured. Significant alterations are found in malab- Osteoporosis as a clinical disorder is characterized by
sorption disorders, hyperparathyroidism and other an abnormally low bone mass and defects in bone
conditions associated with hypercalcaemia. structure, a combination which renders the bone
Urinary hydroxyproline excretion is a measure of unusually fragile and at greater than normal risk of
bone resorption. It may be increased in high-turnover fracture in a person of that age, sex and race.
conditions such as Paget’s disease but it is not sensi- Although the cancellous regions are more porous and
tive enough to reflect lesser increases in bone resorp- the cortices thinner than normal, the existing bone is
tion. fully mineralized.
Excretion of pyridinium compounds and telopeptides Bone depletion may be brought about by predom-
derived from bone collagen cross-links is a much inant bone resorption, decreased bone formation or a
more sensitive index of bone resorption (Rosen et al., combination of the two. It seems self-evident that the
1994). This may be useful in monitoring the progress main reason for the loss of bone strength is the
of hyperparathyroidism and other types of osteoporo- reduction in bone mass; however, in the remaining
sis. However, excretion is also increased in chronic trabecular bone there may also be a loss of structural
arthritis associated with bone destruction. connectivity between bone plates, and this so alters
NB: Laboratory reports should always state the the mechanical properties that the loss of strength is
normal range for each test, which may be different for out of proportion to the diminution in bone mass. As
infants, children and adults. a consequence, the bone – particularly around the
diaphyseo-metaphyseal junctions in tubular bones and
in the mainly cancellous vertebral bodies – eventually
Bone biopsy
reaches a state in which a comparatively modest stress
Standardized bone samples are easily obtained from or strain causes a fracture. For reasons that are not
the iliac crest and can be examined (without prior fully understood, black African peoples are consider- 131
7 ably less prone to these effects and have a low inci- POSTMENOPAUSAL OSTEOPOROSIS
dence of ‘osteoprotic fractures’ (Solomon, 1968).
This section deals with generalized osteoporosis, but Symptomatic postmenopausal osteoporosis is an exag-
it should not be forgotten that osteoporosis is some- gerated form of the physiological bone depletion that
times confined to a particular bone or group of bones normally accompanies ageing and loss of gonadal
– regional osteoporosis (for example due to disuse, activity. Two overlapping phases are recognized: an
immobilization or inflammation) – which is usually

reversible once the local cause is addressed.
RISK FACTORS FOR POSTMENOPAUSAL
X-rays and bone densitometry OSTEOPOROSIS
The term osteopaenia is sometimes used to describe Caucasoid (white) or Asiatic ethnicity
bone which appears to be less ‘dense’ than normal on
x-ray, without defining whether the loss of density is Family history of osteoporosis
due to osteoporosis or osteomalacia, or indeed whether History of anorexia nervosa and/or amenorrhoea
it is sufficiently marked to be regarded as at all patho-
logical. More characteristic signs of osteoporosis are Low peak bone mass in the third decade
loss of trabecular definition, thinning of the cortices Early onset of menopause
and insufficiency fractures. Compression fractures of
the vertebral bodies, wedging at multiple levels or Unusually slim or emaciated build
biconcave distortion of the vertebral end-plates due to Oophorectomy
bulging of intact intervertebral discs are typical of
severe postmenopausal osteoporosis. Early hysterectomy
The clinical and radiographic diagnosis should be Nutritional insufficiency
backed up by assessment of BMD as measured by
DXA of the spine and hips, using the lower value of Chronic lack of exercise
the two. In otherwise ‘normal’ women over the age of Cigarette smoking
50 years, anything more than 2 standard deviations
below the average for the relevant population group Alcohol abuse
may be taken as indicative of osteoporosis.
(a) (b) (c) (d)
7.11 Osteoporosis – clinical features (a) This woman noticed that she was becoming increasingly round-shouldered;
she also had chronic backache and her x-rays (b) show typical features of postmenopausal osteoporosis: loss of bone
density in the vertebral bodies giving relative prominence to the vertebral end-plates, ballooning of the disc spaces
associated with marked compression of several vertebral bodies and obvious compression fractures of T12 and L1. An
additional feature commonly seen in osteoporotic patients is calcification of the aorta. (c) The next most common feature in
these patients is a fracture of the proximal end of the femur. (d) The incidence of fractures of the vertebrae, hip and wrist
132 rises progressively after the menopause.
early postmenopausal syndrome characterized by Women approaching the menopause should be 7
rapid bone loss due predominantly to increased osteo- advised to maintain adequate levels of dietary calcium
clastic resorption (high-turnover osteoporosis) and a and vitamin D, to keep up a high level of physical
less well-defined syndrome which emerges in elderly activity and to avoid smoking and excessive consump-
people and is due to a gradual slow-down in tion of alcohol. If necessary, the recommended daily
osteoblastic activity and the increasing effects of requirements should be met by taking calcium and

dietary insufficiencies, chronic ill health and reduced vitamin D supplements; these measures have been
mobility (low-turnover osteoporosis). shown to reduce the risk of low-energy fractures in
Around the menopause, and for the next 10 years, elderly women (Chapuy et al., 1994).
bone loss normally accelerates to about 3 per cent per
Hormone replacement therapy (HRT) Until the beginning
year compared with 0.3 per cent during the preceding
of the twenty-first century HRT was the most widely
two decades. This is due mainly to increased bone
used medication for postmenopausal osteoporosis.
resorption, the withdrawal of oestrogen having
Taking oestrogen (or a combination of oestrogen and
removed one of the normal restraints on osteoclastic
progesterone) for 5–10 years was shown convincingly
activity. Genetic influences play an important part in
to reduce the risk of osteoporotic fractures, though
determining when and how this process becomes
after stopping the medication the BMD gradually falls
exaggerated, but a number of other risk factors have
to the usual low level. Moreover there was growing
been identified (see Box on page 132).
concern about the apparent increased risks of
thromboembolism, stroke, breast cancer and uterine
Clinical features and diagnosis cancer. As more experience has been gained with other
antiresorptive drugs, the preference for HRT has
A woman at or near the menopause develops back
waned.
pain and increased thoracic kyphosis; she, or someone
in the family, may have noticed that her height has Bisphosphonates Bisphosphonates are now regarded as
diminished. X-rays of the spine may show wedging or the preferred medication for postmenopausal
compression of one or more vertebral bodies and osteoporosis. They act by reducing osteoclastic bone
often the lateral view also shows calcification of the resorption and the general rate of bone turnover. The
aorta. newer preparations have been shown to prevent bone
This is the typical picture, but sometimes the first loss and to reduce the risk of vertebral and hip
clinical event is a low-energy fracture of the distal fractures. Alendronate can be administered by mouth
radius (Colles’ fracture), the hip or the ankle. Women in once-weekly doses for both prevention and
who have had one low-energy fracture have twice the treatment of osteoporosis. Gastrointestinal side effects
normal risk of developing another. are a bother and suitable precautions should be taken;
DXA may show significantly reduced bone density for patients who cannot tolerate the drug, pamidronate
in the vertebral bodies or femoral neck. has been given intravenously at 3-monthly intervals.
The rate of bone turnover is either normal or
slightly increased; measurement of excreted collagen Parathyroid hormone Trials of parathyroid hormone,
cross-link products and telopeptides may suggest a either by itself or in combination with alendronate,
high-turnover type of bone loss. have shown good results in obtaining a rise in BMD in
Once the clinical diagnosis has been established, patients with postmenopausal osteoporosis (Black et
screening tests should be performed to rule out other al., 2005). This could be a way of managing patients
causes of osteoporosis (e.g. hyperparathyroidism, with severe osteoporosis who do not respond to
malignant disease or hypercortisonism). bisphosphonates alone.
Recent advances in drug treatment A novel way of
Prevention and treatment reducing osteoclastic activity and bone resorption is to
interrupt the RANKL–RANK interaction which is
Bone densitometry can be used to identify women who essential for prompting osteoclastogenesis (see page
are at more than usual risk of suffering a fracture at the 122). Phase 3 trials are now being conducted using
menopause, and prophylactic treatment of this group is denosumab, an antibody to RANKL, which holds out
sensible. However, routine DXA screening (even in the promise of an effective new line of treatment for
countries where it is available) is still not universally em- postmenopausal osteoporosis (McClung et al., 2006).
ployed; for practical purposes, it is usually reserved for
women with multiple risk factors and particularly those Management of fractures Femoral neck and other
with suspected oestrogen deficiency (premature or sur- long-bone fractures may need operative treatment.
gically induced menopause) or some other bone-losing Methods are described in the relevant chapters in
disorder, and those who have already suffered previous Section 3.
low-energy fractures at the menopause. Vertebral fractures are painful and patients will need 133
7 analgesic treatment, partial rest and assistance with menopausal women, only this occurs about 15 years
personal care for about 6 weeks. Physiotherapy should later unless there is some specific cause for testicular
initially be aimed at maintaining muscle tone and failure. Osteoporotic fractures in men under 60 years of
movement in all unaffected areas; if pain is adequately age should arouse the suspicion of some underlying dis-
controlled, patients should be encouraged to walk and order – notably hypogonadism, metastatic bone disease,
when symptoms allow they can be introduced to pos- multiple myeloma, liver disease, renal hypercalciuria,
tural training. Spinal orthoses may be needed for sup- alcohol abuse, malabsorption disorder, malnutrition,
port and pain relief, but they cannot be expected to glucocorticoid medication or anti-gonadal hormone
correct any structural deformity. Operative measures treatment for prostate cancer. Other causes of second-
are occasionally called for to treat severe compression ary osteoporosis are shown in Table 7.4.
fractures. Treatment is much the same as for postmenopausal
osteoporosis. Vitamin D and calcium supplementa-
tion is important; alendronate is the antiresorptive
INVOLUTIONAL OSTEOPOROSIS drug of choice. If testosterone levels are unusually
low, hormone treatment should be considered.
In advanced age the rate of bone loss slowly decreases
but the incidence of femoral neck and vertebral frac-
tures rises steadily; by around 75 years of age almost a SECONDARY OSTEOPOROSIS
third of white women will have at least one vertebral
fracture. For reasons that are not completely known, Among the numerous causes of secondary osteoporo-
age-related fractures are much less common in black sis, hypercortisonism, gonadal hormone deficiency,
people. hyperthyroidism, multiple myeloma, chronic alco-
BMD measurements in this age group show that holism and immobilization will be considered further.
there is considerable overlap between those who suf-
fer fractures and those who do not; the assumption is
that qualitative changes contribute increasingly to
Hypercortisonism
bone fragility in old age. Causes include a rising inci- Glucocorticoid overload occurs in endogenous Cush-
dence of chronic illness, mild urinary insufficiency, ing’s disease or after prolonged treatment with corti-
dietary deficiency, lack of exposure to sunlight, mus- costeroids. This often results in severe osteoporosis,
cular atrophy, loss of balance and an increased ten- especially if the condition for which the drug is
dency to fall. Many old people suffer from vitamin D administered is itself associated with bone loss – for
deficiency and develop some degree of osteomalacia example, rheumatoid arthritis.
on top of the postmenopausal osteoporosis Glucocorticoids have a complex mode of action.
(Solomon, 1973). The deleterious effect on bone is mainly by suppres-
sion of osteoblast function, but it also causes reduced
Treatment Initially, treatment is directed at manage-
calcium absorption, increased calcium excretion and
ment of the fracture. This will often require internal
stimulation of PTH secretion (Hahn, 1980). There is
fixation; the sooner these patients are mobilized and
now evidence that it also depresses OPG expression
rehabilitated the better. Patients with muscle weak-
and this would have an enhancing effect on osteoclas-
ness and/or poor balance may benefit from gait train-
togenesis and bone resorption.
ing and, if necessary, the use of walking aids and rail
Treatment presents a problem, because the drug may
fittings in the home.
be essential for the control of some generalized disease.
Thereafter the question of general treatment must
However, forewarned is forearmed: corticosteroid
be considered. Obvious factors such as concurrent ill-
dosage should be kept to a minimum, and it should not
ness, dietary deficiencies, lack of exposure to sunlight
be forgotten that intra-articular preparations and corti-
and lack of exercise will need attention. If the patient
sone ointments are absorbed and may have systemic
is not already on vitamin D and calcium as well as
effects if given in high dosage or for prolonged periods.
antiresorptive medication, this should be prescribed;
Patients on long-term glucocorticoid treatment
although bone mass will not be restored, at least fur-
should, ideally, be monitored for bone density.
ther loss may be slowed.
Preventive measures include the use of calcium sup-
plements (at least 1500 mg per day) and vitamin D
metabolites. In postmenopausal women and elderly
POST-CLIMACTERIC OSTEOPOROSIS IN men bisphosphonates may be effective in reducing
MEN bone resorption.
In late cases general measures to control bone pain
With the gradual depletion in androgenic hormones, may be required. Fractures are treated as and when
134 men eventually suffer the same bone changes as post- they occur.
Gonadal hormone insufficiency 7
Oestrogen lack is an important factor in postmenopausal
osteoporosis. It also accounts for osteoporosis in
younger women who have undergone oophorectomy,
and in pubertal girls with ovarian agenesis and primary

amenorrhoea (Turner’s syndrome). Treatment is the
same as for postmenopausal osteoporosis.
Amenorrhoeic female athletes, and adolescents with
anorexia nervosa, may become osteoporotic; fortu-
nately these conditions are usually self-limiting.
A decline in testicular function probably con-
tributes to the continuing bone loss and rising frac-
ture rate in men over 70 years of age. A more obvious
relationship is found in young men with overt hypog-
onadism; this may require long-term treatment with
testosterone.
Hyperthyroidism 7.12 Disuse osteoporosis X-ray of the knee after

prolonged immobilization. Note the extremely thin cortices
Thyroxine speeds up the rate of bone turnover, but re- and the loss of trabecular pattern in the metaphyses.
sorption exceeds formation. Osteoporosis is quite com-
mon in hyperthyroidism, but fractures usually occur
only in older people who suffer the cumulative effects Table 7.4 Causes of secondary osteoporosis
of the menopause and thyroid overload. In the worst
cases osteoporosis may be severe with spontaneous Nutritional Endocrine disorders
Malabsorption Gonadal insufficiency
fractures, a marked rise in serum alkaline phosphatase, Malnutrition Hyperparathyroidism
hypercalcaemia and hypercalciuria. Treatment is needed Scurvy Thyrotoxicosis
for both the osteoporosis and the thyrotoxicosis. Cushing’s disease
Inflammatory disorders Malignant disease
Rheumatoid disease Carcinomatosis
Multiple myeloma and carcinomatosis Ankylosing spondylitis Multiple myeloma
Generalized osteoporosis, anaemia and a high ESR are Tuberculosis Leukaemia
characteristic features of myelomatosis and metastatic Drug induced Other
bone disease. Bone loss is due to overproduction of Corticosteroids Smoking
Excessive alcohol Chronic obstructive
local osteoclast-activating factors. Treatment with bis-
consumption pulmonary disease
phosphonates may reduce the risk of fracture. Anticonvulsants Osteogenesis imperfecta
Heparin Chronic renal disease
Immunosuppressives
Alcohol abuse
This is a common (and often neglected) cause of os-
teoporosis at all ages, with the added factor of an in-
creased tendency to falls and other injuries. Bone Other conditions
changes are due to a combination of decreased calcium
absorption, liver failure and a toxic effect on osteoblast There are many other causes of secondary osteoporo-
function. Alcohol also has a mild glucocorticoid effect. sis, including hyperparathyroidism (which is consid-
ered below), rheumatoid arthritis, ankylosing
spondylitis and subclinical forms of osteogenesis
Immobilization imperfecta. The associated clinical features usually
point to the diagnosis.
The worst effects of stress reduction are seen in states
of weightlessness; bone resorption, unbalanced by
formation, leads to hypercalcaemia, hypercalciuria and
severe osteoporosis. Lesser degrees of osteoporosis RICKETS AND OSTEOMALACIA
are seen in bedridden patients, and regional
osteoporosis is common after immobilization of a Rickets and osteomalacia are different expressions of
limb. The effects can be mitigated by encouraging the same disease: inadequate mineralization of bone.
mobility, exercise and weightbearing. Osteoid throughout the skeleton is incompletely 135
7 calcified, and the bone is therefore ‘softened’ (osteo-
malacia). In children there are additional effects on
physeal growth and ossification, resulting in deformi-
ties of the endochondral skeleton (rickets).
The inadequacy may be due to defects anywhere
along the metabolic pathway for vitamin D: nutri-
tional lack, underexposure to sunlight, intestinal mal-

absorption, decreased 25-hydroxylation (liver disease,
anticonvulsants) and reduced 1α-hydroxylation (renal
disease, nephrectomy, 1α-hydroxylase deficiency).
The pathological changes may also be caused by cal-
cium deficiency or hypophosphataemia.
Pathology
The characteristic pathological changes in rickets arise
from the inability to calcify the intercellular matrix in
the deeper layers of the physis. The proliferative zone
is as active as ever, but the cells, instead of arranging
themselves in orderly columns, pile up irregularly; the
entire physeal plate increases in thickness, the zone of
calcification is poorly mineralized and bone formation (a) (b)
is sparse in the zone of ossification. The new trabecu-
lae are thin and weak, and with joint loading the 7.13 Rickets In countries with advanced health systems
nutritional rickets is nowadays uncommon. This 5-year-old
juxta-epiphyseal metaphysis becomes broad and cup- girl, after investigation, was found to have familial
shaped. hypophosphataemic rickets. In addition to the obvious
Away from the physis the changes are essentially varus deformities on her legs, (a) her lower limbs are
those of osteomalacia. Sparse islands of bone are lined disproportionately short compared to her upper body.
by wide osteoid seams, producing unmineralized ghost (b) X-ray of another child with classical nutritional rickets,
showing the well-marked physes, the flared metaphyses
trabeculae that are not very strong. The cortices also and the bowing deformities of the lower limb bones.
are thinner than normal and may show signs of new or
older stress fractures. If the condition has been present
formities increase, and stunting of growth may be ob-
for a long time there may be stress deformities of the
vious. In severe rickets there may be spinal curvature,
bones: indentation of the pelvis, bending of the femoral
coxa vara and bending or fractures of the long bones.
neck (coxa vara) and bowing of the femora and tibiae.
Remember that even mild osteomalacia can increase Adults Osteomalacia has a much more insidious
the risk of fracture if it is superimposed on post- course and patients may complain of bone pain, back-
menopausal or senile osteoporosis. ache and muscle weakness for many years before the
diagnosis is made. Vertebral collapse causes loss of
height, and existing deformities such as mild kyphosis
Clinical features of rickets and or knock knees – themselves perhaps due to child-
osteomalacia hood rickets – may increase in later life. Unexplained
In the past the vast majority of cases of rickets and pain in the hip or one of the long bones may presage
osteomalacia were due to dietary vitamin D deficiency a stress fracture.
and/or insufficient exposure to sunlight. These patients
still embody the classical picture of the disorder. X-rays
Children The infant with rickets may present with tetany Children In active rickets there is thickening and
or convulsions. Later the parents may notice that there widening of the growth plate, cupping of the meta-
is a failure to thrive, listlessness and muscular flaccidity. physis and, sometimes, bowing of the diaphysis. The
Early bone changes are deformity of the skull (cran- metaphysis may remain abnormally wide even after
iotabes) and thickening of the knees, ankles and wrists healing has occurred. If the serum calcium remains
from physeal overgrowth. Enlargement of the costo- persistently low there may be signs of secondary hyper-
chondral junctions (‘rickety rosary’) and lateral inden- parathyroidism: sub-periosteal erosions are at the sites
tation of the chest (Harrison’s sulcus) may also appear. of maximal remodelling (medial borders of the prox-
Distal tibial bowing has been attributed to sitting or ly- imal humerus, femoral neck, distal femur and proximal
136 ing cross-legged. Once the child stands, lower limb de- tibia, lateral borders of the distal radius and ulna).
7

(a) (b)
7.14 Rickets – x-rays X-rays obtained at two points during growth in a child with nutritional rickets. The typical features
such as widening of the physis and flaring of the metaphysis are well marked (a). After treatment the bones have begun to
heal but the bone deformities are still noticeable (b).
Adults The classical lesion of osteomalacia is the vertebrae (from disc pressure), lateral indentation of
Looser zone, a thin transverse band of rarefaction in the acetabula (‘trefoil’ pelvis) and spontaneous frac-
an otherwise normal-looking bone. These zones, tures of the ribs, pubic rami, femoral neck or the
seen especially in the shafts of long bones and the metaphyses above and below the knee. Features of
axillary edge of the scapula, are due to incomplete secondary hyperparathyroidism characteristically
stress fractures which heal with callus lacking in cal- appear in the middle phalanges of the fingers, and in
cium. More often, however, there is simply a slow severe cases so-called ‘brown tumours’ are seen in the
fading of skeletal structure, resulting in biconcave long bones.
(a)
(b) (c) (d)
7.15 Osteomalacia Four characteristic features of osteomalacia: (a) indentation of the acetabula producing the trefoil or
champagne glass pelvis; (b) Looser’s zones in the pubic rami and left femoral neck; (c) biconcave vertebrae; and
(d) fracture in the mid-diaphysis of a long bone following low-energy trauma (the femoral cortices in this case are egg-shell
thin). 137
7 Biochemistry ments are administered they have to be given in large
doses (50 000 IU per day).
Changes common to almost all types of vitamin D
related rickets and osteomalacia are diminished levels Surgery Established long-bone deformities may need
of serum calcium and phosphate, increased alkaline bracing or operative correction once the metabolic
phosphatase and diminished urinary excretion of cal- disorder has been treated.
cium. In vitamin D deficiency 25-OH D levels also are

low. The ‘calcium phosphate product’ (derived by
multiplying calcium and phosphorus levels expressed
in mmol/L), normally about 3, is diminished in rick- VITAMIN D RESISTANT RICKETS AND
ets and osteomalacia, and values of less than 2.4 are OSTEOMALACIA
diagnostic.
There are several types of rickets and osteomalacia that
do not respond to physiological doses of vitamin D.
Bone biopsy Although some are uncommon, they should be borne
With clearcut clinical and x-ray features the diagnosis in mind in dealing with resistant cases.
is obvious. In less typical cases a bone biopsy will pro-
vide the answer. Osteoid seams are both wider and
more extensive, and tetracycline labelling shows that Inadequacy of hepatic 25-OHD
mineralization is defective. Defective conversion to (or too-rapid breakdown of)
25-OHD in the liver may result from long-term admin-
Treatment istration of anticonvulsants or rifampicin, and if these
drugs are prescribed it is wise to give adequate amounts
Dietary lack of vitamin D (less than 100 IU per day) is of vitamin D at the same time. Occasionally the condi-
common in strict vegetarians, in old people who often tion is also seen in severe liver failure. Treatment in
eat very little and even in entire populations whose tra- these cases requires vitamin D in very large doses.
ditional foods contain very little vitamin D. If there is
also reduced exposure to sunlight, rickets or osteoma-
lacia may result. The use of sun-blocking lotions, or Abnormalities of 1,25-(OH)2D
overall cover by clothing, may seriously reduce expo- metabolism
sure to ultraviolet light. Some of these problems can be
corrected by simple social adjustments. Renal failure Patients with early renal failure some-
Treatment with vitamin D (400–1000 IU per day) times develop osteomalacia; this is thought to be due
and calcium supplements is usually effective; however, to reduced 1α-hydroxylase activity resulting in defi-
elderly people often require larger doses of vitamin D ciency of 1,25-(OH)2D. The condition can be
(up to 2000 IU per day). treated with 1,25-(OH)2D (or else with very large
Intestinal malabsorption – especially fat malabsorp- doses of vitamin D).
tion – can cause vitamin D deficiency (fat and vitamin Patients with advanced renal disease treated by
D absorption go hand in hand). If vitamin D supple- haemodialysis develop a more complex syndrome –
renal osteodystrophy. This is considered on page 141.
Vitamin D dependent rickets and osteomalacia Rare
OSTEOMALACIA AND OSTEOPOROSIS causes of 1,25-(OH)2D failure are two heritable
(autosomal recessive) disorders.
Common in ageing women Type I (pseudo vitamin D deficient rickets) is due to
Prone to pathological fracture deficiency of 1α-hydroxylase; children develop very
Decreased bone density severe rickets and secondary hyperparathyroidism
Osteomalacia Osteoporosis causing multiple fractures and generalized myopathy,
as well as dental enamel hypoplasia. They need life-
Unwell Well long treatment with 1-(OH) D.
Generalized chronic ache Pain only after fracture
Type II vitamin D dependent rickets and osteomala-
Muscles weak Muscles normal
cia is resistant to treatment with both vitamin D and
Looser’s zones No Looser’s zones
calcitriol (1,25-(OH)2D). Plasma 1,25-(OH)2D levels
Alkaline phosphatase Normal
are elevated but vitamin D receptors at the target
increased
organs (intestine and bone) are defective. Bone
Serum phosphorus Normal
decreased
changes usually appear during childhood but adults
Ca × P <2.4 mmol/L Ca × P >2.4 mmol/L also are affected. There is hypocalcaemia and second-
138 ary hyperparathyroidism. Neither vitamin D nor any
of its metabolites is curative and patients may need Table 7.5 Characteristics of different types of rickets 7
long-term parenteral calcium.
Vitamin D Renal Renal
NB: Patients treated with supra-physiological doses of deficiency tubular glomerular
calcitriol run the risk of developing hypercalcaemia,
Family history – + –
hypercalciuria and nephrocalcinosis; plasma calcium
concentration should be measured regularly and ideally Myopathy + – +

treatment should be conducted under the supervision of Growth defect ± ++ ++
a specialist in this field. Serum:
Ca ↓ N ↓
Hypophosphataemic rickets and P ↓ ↓ ↑
osteomalacia Alk. phos. ↑ ↑ ↑
Chronic hypophosphataemia occurs in a number of Urine:
disorders in which there is impaired renal tubular Ca ↓ ↓ ↓
reabsorption of phosphate. Calcium levels are normal P ↓ ↑ ↓
and there are no signs of hyperparathyroidism, but Osteitis fibrosa ± + ++
bone mineralization is defective.
Other Dietary Amino- Renal failure
Familial hypophosphataemic rickets In many countries deficiency or aciduria Anaemia
this is the commonest form of rickets seen today. It is malabsorption
an X-linked genetic disorder with dominant inheri- N = normal; Ca = calcium; P = phosphorus; Alk. Phos. = alkaline
tance, starting in infancy or soon after and causing phosphatase.
bony deformity of the lower limbs if it is not recog-
nized and treated.
During infancy the children look normal but defor- Treatment requires the use of phosphate (up to 3 g
mities of the lower limbs (genu valgum or genu per day, to replace that which is lost in the urine) and
varum) develop when they begin to walk, and growth large doses of vitamin D (to prevent secondary hyper-
is below normal. There is no myopathy. X-rays may parathyroidism due to phosphate administration). If
show marked epiphyseal changes but, because the calcitriol is given instead, plasma calcium concentra-
serum calcium is normal, there are no signs of section should be monitored in order to forestall the devel-
ondary hyperparathyroidism. opment of hypercalciuria and nephrocalcinosis.
During adulthood there is a tendency to develop Treatment is continued until growth ceases.
heterotopic bone formation around some of the larger Bony deformities may require bracing or osteo-
joints and in the longitudinal ligaments of the spinal tomy. If the child needs to be immobilized, vitamin D
canal (which may give rise to neurological symptoms). must be stopped temporarily to prevent hypercal-
(a) (b) (c)
7.16 Renal tubular rickets – familial hypophosphataemia (a) These brothers presented with knee deformities; their
x-rays (b) show defective juxta-epiphyseal calcification. (c) Another example of hypophosphataemic rickets; his growth
chart shows that he was well below the normal range in height, but improved dramatically on treatment with vitamin D
and inorganic phosphate. 139
7 caemia from the combined effects of treatment and Pathology
disuse bone resorption.
Overproduction of PTH enhances calcium conservation
Adult-onset hypophosphataemia Although rare, this by stimulating tubular absorption, intestinal absorption
must be remembered as a cause of unexplained bone and bone resorption. The resulting hypercalcaemia so
loss and joint pains in adults. The condition responds increases glomerular filtration of calcium that there is
dramatically to treatment with phosphate, vitamin D
hypercalciuria despite the augmented tubular reabsorp-

and calcium. tion. Urinary phosphate also is increased, due to sup-
More severe renal tubular defects can produce a pressed tubular reabsorption. The main effects of these
variety of biochemical abnormalities, including changes are seen in the kidney: calcinosis, stone forma-
chronic phosphate depletion and osteomalacia. If tion, recurrent infection and impaired function. There
there is acidosis, this must be corrected; in addition, may also be calcification of soft tissues.
patients may need phosphate replacement, together There is a general loss of bone substance. In severe
with calcium and vitamin D. cases, osteoclastic hyperactivity produces sub-
Oncogenic osteomalacia Hypophosphataemic vitamin D periosteal erosions, endosteal cavitation and replace-
resistant rickets or osteomalacia may be induced by cer- ment of the marrow spaces by vascular granulations
tain tumours, particularly vascular tumours like and fibrous tissue (osteitis fibrosa cystica). Haemor-
haemangiopericytomas, and also fibrohistiocytic lesions rhage and giant-cell reaction within the fibrous
such as giant cell tumours and pigmented villonodular stroma may give rise to brownish, tumour-like masses,
synovitis. The patient is usually an adult and osteomala- whose liquefaction leads to fluid-filled cysts.
cia may appear before the tumour is discovered. Clini-
cal and biochemical features are similar to those of other
types of hypophosphataemic disorder and (as in the lat- PRIMARY HYPERPARATHYROIDISM
ter) the condition is believed to be mediated by phos-
phatonin (Sundaram and McCarthy, 2000). Removal of Primary hyperparathyroidism is usually caused by a
the tumour will reverse the bone changes; if this cannot solitary adenoma in one of the small glands. Patients
be done, treatment is the same as outlined above. are middle-aged (40–65 years) and women are
affected twice as often as men. Many remain asymp-
tomatic and are diagnosed only because routine bio-
chemistry tests unexpectedly reveal a raised serum
HYPERPARATHYROIDISM calcium level.
Excessive secretion of PTH may be primary (usually

Clinical features
due to an adenoma or hyperplasia), secondary (due to
persistent hypocalcaemia) or tertiary (when second- Symptoms and signs are mainly due to hypercal-
ary hyperplasia leads to autonomous overactivity). caemia: anorexia, nausea, abdominal pain, depression,
(a) (b) (c) (d)
7.17 Hyperparathyroidism (a) This hyperparathyroid patient with spinal osteoporosis later developed pain in the right
arm; an x-ray (b) showed cortical erosion of the humerus; he also showed (c) typical erosions of the phalanges.
140 (d) Another case, showing ‘brown tumours’ of the humerus and a pathological fracture.
fatigue and muscle weakness. Patients may develop Treatment 7
polyuria, kidney stones or nephrocalcinosis due to
chronic hypercalciuria. Some complain of joint symp- Treatment is usually conservative and includes ade-
toms, due to chondrocalcinosis. Only a minority quate hydration and decreased calcium intake. The
(probably less than 10 per cent) present with bone indications for parathyroidectomy are marked and
disease; this is usually generalized osteoporosis rather unremitting hypercalcaemia, recurrent renal calculi,

than the classic features of osteitis fibrosa, bone cysts progressive nephrocalcinosis and severe osteoporosis.
and pathological fractures. Postoperatively there is a danger of severe hypocal-
caemia due to brisk formation of new bone (the ‘hun-
gry bone syndrome’). This must be treated promptly,
X-rays with one of the fast-acting vitamin D metabolites.
Typical x-ray features are osteoporosis (sometimes
including vertebral collapse) and areas of cortical ero-
sion. Hyperparathyroid ‘brown tumours’ should be SECONDARY HYPERPARATHYROIDISM
considered in the differential diagnosis of atypical
cyst-like lesions of long bones. The classical – and Parathyroid oversecretion is a predictable response to
almost pathognomonic – feature, which should always chronic hypocalcaemia. Secondary hyperparathy-
be sought, is sub-periosteal cortical resorption of the roidism is seen, therefore, in various types of rickets
middle phalanges. Non-specific features of hypercal- and osteomalacia, and accounts for some of the radi-
caemia are renal calculi, nephrocalcinosis and chon- ological features in these disorders. Treatment is
drocalcinosis. directed at the primary condition.
Biochemical tests
There may be hypercalcaemia, hypophosphataemia RENAL OSTEODYSTROPHY
and a raised serum PTH concentration. Serum alka-
line phosphatase is raised with osteitis fibrosa. Patients with chronic renal failure and lowered
glomerular filtration rate are liable to develop diffuse
bone changes which resemble those of other condi-
Diagnosis tions that affect bone formation and mineralization.
It is necessary to exclude other causes of hypercal- Thus the dominant picture may be that of secondary
caemia (multiple myeloma, metastatic disease, sar- hyperparathyroidism [due to phosphate retention,
coidosis) in which PTH levels are usually depressed. hypocalcaemia and diminished production of 1,25-
Hyperparathyroidism also comes into the differential (OH)2D], osteoporosis, osteomalacia or – in
diagnosis of all types of osteoporosis and osteomalacia. advanced cases – a combination of these. In older
7.18 Renal glomerular

osteodystrophy (a) This young
boy with chronic renal failure
developed severe deformities of
(b) the hips and (c) the knees.
Note the displacement of the
upper femoral epiphyses.
(b)
(a) (c) 141

7 patients the effects of postmenopausal osteoporosis cline histomorphometry. It is important that the
may be superimposed; in some there are concomitant underlying metabolic changes be established as this
changes due to glucocorticoid medication; and in will determine the choice of treatment.
patients with end-stage renal failure bone changes can
be aggravated by aluminium retention or contamina-
Treatment
tion of dialysing fluids.
Hyperphosphataemia and secondary hyperparathy-

roidism can be treated by restricting the intake of
Clinical features phosphorus (e.g. by taking phosphate binders) and
Renal abnormalities usually precede the bone changes by administering vitamin D or one of its analogues. More
several years. Children are more severely affected than recently a calcimimetic drug, cinacalcet, has been
adults: they are usually stunted, pasty-faced and have introduced; this acts directly on the parathyroid
marked rachitic deformities associated with myopathy. X- glands increasing the sensitivity of calcium receptors
rays show widened and irregular epiphyseal plates. and inducing a reduction in serum PTH levels. How-
In older children with longstanding disease there ever, the biochemical changes are usually more com-
may be displacement of the epiphyses (epiphyseoly- plex and treatment should always be managed by a
sis). Osteosclerosis is seen mainly in the axial skeleton specialist in this field.
and is more common in young patients: it may pro- Renal failure, if irreversible, may require haemodial-
duce a ‘rugger jersey’ appearance in lateral x-rays of ysis or renal transplantation.
the spine, due to alternating bands of increased and Epiphyseolysis may need internal fixation and resid-
decreased bone density. ual deformities can be corrected once the disease is
In all patients signs of secondary hyperparathy- under control.
roidism may be widespread and severe. Biochemical
features are low serum calcium, high serum phosphate
and elevated alkaline phosphatase levels. Urinary
excretion of calcium and phosphate is diminished. SCURVY
Plasma PTH levels may be raised.
Vitamin C (ascorbic acid) deficiency causes failure of
collagen synthesis and osteoid formation. The result is
Diagnosis osteoporosis, which in infants is most marked in the
Precise diagnosis, differentiation from other meta- juxta-epiphyseal bone. Spontaneous bleeding is com-
bolic bone disorders and attribution to a specific cat- mon.
egory of change requires painstaking biochemical The infant is irritable and anaemic. The gums may
investigation and bone biopsy for quantitative tetracy- be spongy and bleeding. Sub-periosteal haemorrhage
7.19 Scurvy (a,b) The

epiphyseal ring sign and
small sub-periosteal
haemorrhages; (c) the
femoral epiphysis has
displaced and the sub-
periosteal haemorrhage
has calcified.
142 (a) (b) (c)

causes excruciating pain and tenderness near the large together with hyperostosis at the bony attachments of 7
joints. Fractures or epiphyseal separations may occur. ligaments, tendons and fascia in these areas. Despite
X-rays show generalized bone rarefaction, most the apparent thickening and ‘density’ of the skeleton,
marked in the long-bone metaphyses. The normal tensile strength is reduced and the bones fracture
calcification in growing cartilage produces dense more easily under bending and twisting loads.
transverse bands at the juxta-epiphyseal zones and Patients complain of backache, bone pain and joint

around the ossific centres of the epiphyses (the ‘ring stiffness. Examination may show thickening of the
sign’). The metaphyses may be deformed or fractured. tubular bones. Sometimes the first clinical manifesta-
Sub-periosteal haematomas show as soft-tissue tion is a stress fracture. In the worst cases there may
swellings or peri-osseous calcification. be deformities of the spine and lower limbs; hyperos-
Treatment is with large doses of vitamin C. tosis can lead to vertebral canal encroachment and
resultant neurological defects.
The typical x-ray features are osteosclerosis, osteo-
phytosis and heterotopic ossification of ligamentous
HYPERVITAMINOSIS and fascial attachments. Changes are most marked in
the spine and pelvis, where the bones become densely
Hypervitaminosis A occurs in children following opaque.
excessive dosage; in adults it seldom occurs except in In a full-blown case the diagnosis should be obvious,
explorers who eat polar bear livers. There may be but the rarity of the condition leads to it being over-
bone pain, and headache and vomiting due to raised looked. X-ray features at individual sites can be mis-
intracranial pressure. X-ray shows increased density in taken for those of Paget’s disease, idiopathic skeletal hy-
the metaphyseal region and sub-periosteal calcifica- perostosis, renal osteodystrophy or osteopetrosis.
tion. There is no specific treatment for this condition.
Hypervitaminosis D occurs if too much vitamin D is After exposure ceases it still takes years for bone fluo-
given. It exerts a PTH-like effect and so, as in the ride to be excreted. If there is evidence of osteomala-
underlying rickets, calcium is withdrawn from bones; cia and secondary hyperparathyroidism, this can be
but metastatic calcification occurs. In treatment the treated with calcium and vitamin D.
dose of vitamin D must be properly regulated and the
infant given a low-calcium diet but plentiful fluids.
PAGET’S DISEASE (OSTEITIS

FLUOROSIS DEFORMANS)
Fluorine in very low concentration – 1 part per mil- Paget’s disease is characterized by increased bone
lion (ppm) or less – has been used to reduce the inci- turnover and enlargement and thickening of the
dence of dental caries. At slightly higher levels (2–4 bone, but the internal architecture is abnormal and
ppm) it may produce mottling of the teeth, a condi- the bone is unusually brittle. The condition has a curi-
tion which is fairly common in those parts of the ous ethnic and geographical distribution, being rela-
world where fluorine appears in the soil and drinking tively common (a prevalence of more than 3 per cent
water. In some areas – notably parts of India and in people aged over 40) in North America, Britain,
Africa where fluorine concentrations in the drinking western Europe and Australia but rare in Asia, Africa
water may be above 10 ppm – chronic fluorine intox- and the Middle East. There is a tendency to familial
ication (fluorosis) is endemic and widespread skeletal aggregation. The cause is unknown, although the dis-
abnormalities are occasionally encountered in the covery of inclusion bodies in the osteoclasts has sug-
affected population. Mild bone changes are also gested a viral infection (Rebel et al., 1980).
sometimes seen in patients treated with sodium fluo-
ride for osteoporosis.
Fluorine directly stimulates osteoblastic activity;
Pathology
fluoroapatite crystals are laid down in bone and these The disease may appear in one or several sites; in the
are unusually resistant to osteoclastic resorption. tubular bones it starts at one end and progresses
Other effects are thought to be due to calcium reten- slowly towards the diaphysis, leaving a trail of altered
tion, impaired mineralization and secondary hyper- architecture behind. The characteristic cellular change
parathyroidism. The characteristic pathological is a marked increase in osteoclastic and osteoblastic
features in severe cases are sub-periosteal new-bone activity. Bone turnover is accelerated, plasma alkaline
accretion and osteosclerosis, most marked in the ver- phosphatase is raised (a sign of osteoblastic activity)
tebrae, ribs, pelvis and the forearm and leg bones, and there is increased excretion of hydroxyproline in 143
7 increases in thickness but is structurally weak and
easily deformed. Gradually, osteoclastic activity abates
and the eroded areas fill with new lamellar bone, leav-
ing an irregular pattern of cement lines that mark the
limits of the old resorption cavities; these ‘tidemarks’
produce a marbled or mosaic appearance on micros-
copy. In the late, osteoblastic, stage the thickened

bone becomes increasingly sclerotic and brittle.
Clinical features
Paget’s disease affects men and women equally. Only
occasionally does it present in patients under 50, but
7.20 Paget’s disease – histology Section from pagetic from that age onwards it becomes increasingly com-
bone, showing the mosaic pattern due to overactive bone
resorption and bone formation. The trabeculae are thick
mon. The disease may for many years remain localized
and patterned by cement lines. Some surfaces are to part or the whole of one bone – the pelvis and tibia
excavated by osteoclastic activity whilst others are lined by being the commonest sites, and the femur, skull, spine
rows of osteoblasts. The marrow spaces contain and clavicle the next commonest.
fibrovascular tissue. Most people with Paget’s disease are asymptomatic,
the disorder being diagnosed when an x-ray is taken
for some unrelated condition or after the incidental
discovery of a raised serum alkaline phosphatase level.
When patients do present, it is usually because of pain
or deformity, or some complication of the disease.
The pain is a dull constant ache, worse in bed when
the patient warms up, but rarely severe unless a frac-
ture occurs or sarcoma supervenes.
Deformities are seen mainly in the lower limbs.
Long bones bend across the trajectories of mechanical
stress; thus the tibia bows anteriorly and the femur
anterolaterally. The limb looks bent and feels thick,
and the skin is unduly warm – hence the term ‘osteitis
deformans’. If the skull is affected, it enlarges; the
patient may complain that old hats no longer fit. The
skull base may become flattened (platybasia), giving
the appearance of a short neck. In generalized Paget’s
disease there may also be considerable kyphosis, so the
patient becomes shorter and ape-like, with bent legs
and arms hanging in front of him.
Cranial nerve compression may lead to impaired
vision, facial palsy, trigeminal neuralgia or deafness.
Another cause of deafness is otosclerosis. Vertebral
thickening may cause spinal cord or nerve root com-
pression.
7.21 Paget’s disease Paget’s original case compared with Steal syndromes, in which blood is diverted from
a modern photograph. internal organs to the surrounding skeletal circula-
tion, may cause cerebral impairment and spinal cord
ischaemia. If there is also spinal stenosis the patient
develops typical symptoms of ‘spinal claudication’ and
the urine (due to osteoclastic activity). lower limb weakness.
In the osteolytic (or ‘vascular’) stage there is avid
resorption of existing bone by large osteoclasts, the
excavations being filled with vascular fibrous tissue. In X-rays
adjacent areas osteoblastic activity produces new The appearances are so characteristic that the diagno-
woven and lamellar bone, which in turn is attacked by sis is seldom in doubt. During the resorptive phase
osteoclasts. This alternating activity extends on both there may be localized areas of osteolysis; most typical
144 endosteal and periosteal surfaces, so the bone is the flame-shaped lesion extending along the shaft of
7

(a) (b) (c) (d) (e)
7.22 Paget’s disease (a) Deformity of the tibia due to Paget’s disease. (b) X-ray shows that the bone is thickened,
coarsened and bent. Complications include (c) erosive arthritis in a nearby joint; (d) fracture; and (e) osteosarcoma of the
affected bone.
the bone, or a circumscribed patch of osteoporosis in telopeptide is a sensitive marker of bone resorption
the skull (osteoporosis circumscripta). Later the bone and is helpful in gauging the response to treatment.
becomes thick and sclerotic, with coarse trabecula-
tion. The femur or tibia sometimes develops fine
cracks on the convex surface – stress fractures that Complications
heal with increasing deformity of the bone. Occasion-
ally the diagnosis is made only when the patient pres- Fractures Fractures are common, especially in the
ents with a pathological fracture. Silent lesions are weightbearing long bones. In the femoral neck they
revealed by increased activity in the radionuclide scan. are often vertical; elsewhere the fracture line is usually
partly transverse and partly oblique, like the line of
section of a felled tree. In the femur there is a high
Biochemical investigations rate of non-union; for femoral neck fractures pros-
Serum calcium and phosphate levels are usually normal, thetic replacement and for shaft fractures early inter-
though patients who are immobilized may develop hy- nal fixation are recommended. Small stress fractures
percalcaemia. The most useful routine tests are meas- may be very painful; they resemble Looser’s zones on
urement of the serum alkaline phosphatase concentra- x-ray, except that they occur on convex surfaces.
tion (which reflects osteoblastic activity and extent of Osteoarthritis Osteoarthritis of the hip or knee is not
the disease) and 24-hour urinary hydroxyproline merely a consequence of abnormal loading due to
(which correlates with bone resorption). Urinary N- bone deformity; in the hip it seldom occurs unless the
7.23 Paget’s disease

(a,b) In this early case
the x-ray is almost
normal, but the
radionuclide scan of
the same femur
shows increased
activity. (c) Flame-
shaped area of
osteopaenia.
(a) (b) (c) 145

7 innominate bone is involved. The x-ray appearances inhibiting their rate of growth and dissolution. It is
suggest an atrophic arthritis with sparse remodelling, claimed that the reduction in bone turnover following
and at operation joint vascularity is increased. their use is associated with the formation of lamellar
rather than woven bone and that, even after treatment
Nerve compression and spinal stenosis Occasionally this
is stopped, there may be prolonged remission of dis-
is the first abnormality to be detected, and may call for
ease (Bickerstaff et al., 1990). Etidronate can be given
definitive surgical treatment. Local bone hypertrophy

orally (always on an empty stomach) but dosage
may cause hearing loss.
should be kept low (e.g. 5 mg/kg per day for up to 6
Bone sarcoma Osteosarcoma arising in an elderly months) and vitamin D and calcium should also be
patient is almost always due to malignant transforma- given lest impaired bone mineralization results in
tion in Paget’s disease. The frequency of malignant osteomalacia. The newer bisphosphonates (e.g. alen-
change is probably around 1 per cent. It should dronate or pamidronate) do not have this disadvan-
always be suspected if a previously diseased bone tage, so they should be used as the treatment of
becomes more painful, swollen and tender. Occasion- choice; they produce remissions even with short
ally it presents as the first evidence of Paget’s disease. courses of 1 or 2 weeks.
The prognosis is extremely grave.
Surgery The main indication for operation is a patho-
Though rare, this is an
High-output cardiac failure logical fracture, which (in a long bone) usually requires
important general complication. It is due to pro- internal fixation. When the fracture is treated the
longed, increased bone blood flow. opportunity should be taken to straighten the bone.
Other indications for surgery are painful osteoarthritis
Hypercalcaemia Hypercalcaemia may occur if the
(total joint replacement), nerve entrapment (decom-
patient is immobilized for long.
pression) and severe spinal stenosis (decompression).
Beware – blood loss is likely to be excessive in these cases.
In spite of all these complications, patients with
Paget’s disease usually come to terms with the condi-
An osteosarcoma, if detected early, may be resectable,
tion and live to a ripe old age.
but generally the prognosis is grave.
Treatment
Most patients with Paget’s disease never have any
symptoms and require no treatment. Sometimes pain
is due to an associated arthritis rather than bone dis-
ease, and this may respond to non-steroidal anti-
inflammatory therapy.
The indications for specific treatment are: (1) per-
sistent bone pain; (2) repeated fractures; (3) neuro-
logical complications; (4) high-output cardiac failure;
(5) hypercalcaemia due to immobilization; and (6) for
some months before and after major bone surgery
where there is a risk of excessive haemorrhage.
Drugs that suppress bone turnover, notably calci-
tonin and bisphosphonates, are most effective when
the disease is active and bone turnover is high.
Calcitonin is the most widely used. It reduces bone
resorption by decreasing both the activity and the num-
ber of osteoclasts; serum alkaline phosphatase and uri-
nary hydroxyproline levels are lowered. Salmon calci-
tonin is more effective than the porcine variety;
subcutaneous injections of 50–100 MRC units are
given daily until pain is relieved and the alkaline phos-
phatase levels are reduced and stabilized. Maintenance
injections once or twice weekly may have to be con-
tinued indefinitely, but some authorities advocate stop- (a) (b)
ping the drug and resuming treatment if symptoms re-
7.24 Endocrine disorders (a) Hypopituitarism: a boy of
cur. Calcitonin can also be administered in a nasal 12 with the unmistakable build of Frölich’s syndrome.
spray. (b) Hyperpituitarism: this 16-year-old giant suffered from a
146 Bisphosphonates bind to hydroxyapatite crystals, pituitary adenoma.
CLINICAL FEATURES 7
ENDOCRINE DISORDERS
Children In childhood and adolescence two distinct
clinical disorders are encountered. In the Lorain syn-
The endocrine system plays an important part in skele-
drome the predominant effect is on growth. The body
tal growth and maturation, as well as the maintenance
proportions are normal but the child fails to grow
of bone turnover. The anterior lobe of the pituitary

(proportionate dwarfism). Sexual development may
gland directly affects growth; it also controls the activ-
be unaffected. The condition must be distinguished
ities of the thyroid, the gonads and the adrenal cortex,
from other causes of short stature: hereditary or con-
each of which has its own influence on bone; and the
stitutional shortness, which is not as marked; child-
pituitary itself is subject to feedback stimuli from the
hood illness or malnutrition; rickets; and the various
other glands. The various mechanisms are, in fact, part
bone dysplasias, which generally result in dispropor-
of an interactive system in which balance is more
tionate dwarfism.
important than individual activity. For example: pitu-
In Fröhlich’s adiposogenital syndrome the effects
itary growth hormone stimulates cell proliferation and
include those of gonadal hormone deficiency. There is
growth at the physes. Gonadal hormone promotes
delayed skeletal maturation associated with adiposity
growth plate maturation and fusion. While pituitary
and immaturity of the secondary sexual characteris-
activity is in the ascendant, the bones elongate; after
tics. Weakness of the physes combined with dispro-
sexual maturation, the rise in gonadal hormone activ-
portionate adiposity may result in epiphyseal
ity simultaneously ‘feeds back’ on the pituitary and
displacement (epiphysiolysis or ‘slipped epiphysis’) at
also directly closes down further physeal growth.
the hip or knee.
When the system goes out of balance abnormalities
occur. They are often complex, with several levels of Adults Panhypopituitarism causes a variety of symp-
dysfunction, due to (a) the local effects of the lesion toms and signs, including those of cortisol and sex
which upsets the endocrine gland (e.g. pressure on hormone deficiency. The only important skeletal
cranial nerves from a pituitary adenoma); (b) over- effect is premature osteoporosis.
secretion or undersecretion by the gland affected; and
(c) over- or under-activity of other glands that are INVESTIGATIONS
dependent on the primary dysfunctional gland. Laboratory investigations should include direct assays
The descriptions which follow have been somewhat and tests for hormone function.
simplified. X-rays of the skull may show expansion of the pitu-
itary fossa and erosion of the adjacent bone.
CT and MRI may reveal the tumour.
PITUITARY DYSFUNCTION
TREATMENT
The posterior lobe of the pituitary gland has no influ- Treatment will depend on the cause and the degree of
ence on the musculoskeletal system. dwarfism. If a tumour is identified, it can be removed
The anterior lobe is responsible for the secretion of or ablated. A word of warning: the sudden reactiva-
pituitary growth hormone, as well as the thyrotropic, tion of pituitary function after removal of a tumour
gonadotropic and adrenocorticotropic hormones. may result in slipping of the proximal femoral epiph-
Abnormalities may affect the production of some of ysis. Awareness of this risk will make for early diagno-
these hormones and not others; thus there is no sin- sis and, if necessary, surgical treatment of the
gle picture of ‘pituitary deficiency’ or ‘pituitary epiphysiolysis.
excess’. Moreover, the clinical effects are determined Growth hormone deficiency has been successfully
in part by the stage in skeletal maturation at which the treated by the administration of biosynthetic growth
abnormality occurs. hormone (somatotropin). The response should be
checked by serial plots on the growth chart.
Hypopituitarism
Anterior pituitary hyposecretion may be caused by Hyperpituitarism
intrinsic disorders such as infarction or haemorrhage in
Oversecretion of pituitary growth hormone is usually
the pituitary, infection and intrapituitary tumours, or
due to an acidophil adenoma. However, there are rare
by extrinsic lesions (such as a craniopharyngioma)
cases of growth hormone secretion by pancreatic (and
which press on the anterior lobe of the pituitary. In
other) tumours. The effects vary according to the age
some cases there may also be features due to posterior
of onset.
lobe dysfunction (e.g. diabetes insipidus); and space-
occupying lesions are likely to have other intracranial Gigantism Growth hormone oversecretion in child-
pressure effects, such as headache or visual field defects. hood and adolescence causes excessive growth of the 147
7 entire skeleton. The condition may be suspected quite
early, and it is important to track the child’s develop-
ment by regular clinical and x-ray examination. In
addition to being excessively tall, patients may
develop deformity of the hip due to epiphyseal dis-
placement (epiphysiolysis). There may be mental
retardation and sexual immaturity.

Treatment is directed at early removal of the pitu-
itary tumour.
Acromegaly Oversecretion of pituitary growth hor-
mone in adulthood causes enlargement of the bones
and soft tissues, but without the very marked elonga-
tion which is seen in gigantism. The bones are thick-
ened, rather than lengthened, due to appositional
growth; there is also hypertrophy of articular carti-
lage, which leads to enlargement of the joints. Bones
such as the mandible, the clavicles, ribs, sternum and
scapulae, which develop secondary growth centres in
late adolescence or early adulthood, may go on grow-
ing longer than usual. Thickening of the skull, promi-
nence of the orbital margins, overgrowth of the jaw
(a) (b)
and enlargement of the nose, lips and tongue produce
the characteristic facies of acromegaly. The chest is 7.25 Cushing’s syndrome (a) A patient with rheumatoid
broad and barrel-shaped and the hands and feet are arthritis on long-term corticosteroid treatment. (b) On
x-ray, the bones look washed-out and there are
large. Thickening of the bone ends may cause sec- compression fractures at multiple levels.
ondary osteoarthritis. About 10 per cent of acrome-
galics develop diabetes and cardiovascular disease is
treatment with glucocorticoids (probably the com-
more common than usual.
monest cause). Whatever the cause, the clinical pic-
Treatment is sometimes possible; the indications for
ture is much the same and is generally referred to as
operation are the presence of a tumour in childhood
Cushing’s syndrome.
and cranial nerve pressure symptoms at any age.
Patients have a characteristic appearance: the face is
Trans-sphenoidal surgery has a high rate of success,
rounded and looks somewhat puffy (‘moon face’) and
provided the diagnosis is made reasonably early and
the trunk is distinctly obese, often with abdominal
the tumour is not too large. Mild cases of acromegaly
striae. However, the legs are quite thin and there may
can be treated by administering growth hormone sup-
be proximal wasting and weakness.
pressants (a somatostatin analogue or bromocriptine,
X-rays show generalized osteoporosis; fractures of
a dopamine agonist).
the vertebrae and femoral neck are common. A CT
scan may show an adrenal tumour.
Biochemical tests are usually normal, but there may
ADRENOCORTICAL DYSFUNCTION be a slight increase in urinary calcium.
Problems for the orthopaedic surgeon are manifold:
The adrenal cortex secretes both mineralocorticoids fractures and wounds heal slowly, bones provide little
(aldosterone) and glucocorticoids (cortisol). The lat- purchase for internal fixation, wound breakdown and
ter has profound effects on bone and mineral metab- infection are more common than usual, and the
olism, causing suppression of osteoblast activity, patients are generally less fit.
reduced calcium absorption, increased calcium excre- Prevention means using systemic corticosteroids
tion and enhanced PTH activity. Bone resorption is only when essential and in low dosage. If treatment is
increased and formation is suppressed. prolonged, calcium supplements (at least 1500 mg
per day) and vitamin D should be given. In post-
menopausal women and elderly men, hormone
Hypercortisonism (Cushing’s syndrome) replacement therapy is important. Bisphosphonates
Glucocorticoid excess may be caused by increased may also be effective in slowing the rate of bone loss
pituitary secretion of adrenocorticotropic hormone and preventing further fractures.
(ACTH) (the original Cushing’s disease), by inde- Treatment includes the management of fractures
pendent oversecretion by the adrenal cortex (usually and general measures to control bone pain. If a
148 due to a steroid-secreting tumour) or by excessive tumour is found, this will need surgical removal.
THYROID DYSFUNCTION tion should be avoided; symptoms can be controlled 7
with a wrist splint and the condition does not recur
Hypothyroidism after the end of pregnancy.
Rheumatic disorders respond in unusual ways.
Hypothyroidism takes various forms, depending on Patients with rheumatoid arthritis often improve dra-
the age of onset. matically, while those with systemic lupus erythe-

Congenital hypothyroidism (cretinism) may be caused matosus sometimes develop a severe exacerbation of
by developmental abnormalities of the thyroid, but it the disease.
also occurs in endemic form in areas of iodine defi-
ciency. Unless the condition is treated immediately
(and diagnosis at birth is not easy!) the child becomes
severely dwarfed and may have learning disabilities.
X-rays may show irregular epiphyseal ossification. REFERENCES AND FURTHER READING
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may reverse these changes. trial of effect of alendronate on risk of fracture in women
Adult hypothyroidism (myxoedema) may result from with existing vertebral fractures. Lancet 1996; 348:
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150
Genetic disorders,
skeletal dysplasias and 8
malformations
Deborah Eastwood, Louis Solomon
There can be few diseases in which genetic factors do phosphate molecule and either a purine base (adenine
not play a role – if only in creating a background or guanine) or a pyrimidine (thymine or cytosine)
favourable to the operation of some more proximate base. Some genes are comparatively large and some
pathogen. Sometimes, however, a genetic defect is the much smaller. They are the basic units of inherited
major – or the only – determinant of an abnormality biological information, each one coding for the syn-
that is either present at birth (e.g. achondroplasia) or thesis of a specific protein. Working as a set (or
evolves over time (e.g. Huntington’s chorea). Such genome) they ‘tell’ the cells how to develop, differen-
conditions can be broadly divided into three cate- tiate and function in specialized ways.
gories: chromosome disorders, single gene disorders and Chromosomes can be identified and numbered by
polygenic or multifactorial disorders. Various anom- microscopic examination of suitably prepared blood
alies may also result from injury to the formed embryo. cells or tissue samples; the cell karyotype defines its
Many of these conditions affect the musculoskeletal chromosomal complement. Somatic (diploid) cells
system, producing cartilage and bone dysplasia should have 46 chromosomes: 44 (numbers 1–22),
(abnormal bone growth and/or modelling), malfor- called autosomes, are disposed in 22 homologous pairs
mations (e.g. absence or duplication of certain parts) – one of each pair being derived from the mother and
or structural defects of connective tissue. In some a spe- one from the father, both carrying the same type of
cific metabolic abnormality has been identified. genetic information; the remaining 2 chromosomes
Genetic influences also contribute to the develop- are the sex chromosomes, females having two X chro-
ment of many acquired disorders. Osteoporosis, for mosomes (one from each parent) and males having
example, is the result of a multiplicity of endocrine, one X chromosome from the mother and one Y chro-
dietary and environmental factors, yet twin studies mosome from the father. Germ line cells (eggs and
have shown a significantly closer concordance in bone sperm) have a haploid number of chromosomes (22
mass between identical twins than between non-iden- plus either an X or a Y). This is the euploidic situation;
tical twins. abnormalities of chromosome number would lead to
Before considering the vast range of developmental an aneuploidic state.
disorders, it may be helpful to review certain general Gene studies are complicated and involve the map-
aspects of genetic abnormalities. ping of molecular sequences by specialized techniques
after fragmenting the chains of DNA by means of
restriction enzymes. Each gene occurs at a specific
point, or locus, on a specific chromosome. The chro-
THE HUMAN GENOME mosomes being paired, there will be two forms, or
alleles, of each gene (one maternal, one paternal) at
Each cell (apart from germ cells) in the human body each locus; if the two alleles coding for a particular
contains within its nucleus 46 chromosomes, each of trait are identical, the person is said to be homozygous
which consists of a single molecule of deoxyribonucleic for that trait; if they are not identical, the individual is
acid (DNA); unravelled, this life-imparting molecule heterozygous. Some chromosomes contain only a few
would be several centimetres long, a double-stranded genes (e.g. chromosomes 13, 18 and 21) whereas
chain along which thousands of segments are defined others contain many more (e.g. 17, 19 and 22).
and demarcated as genes. A small amount of DNA is The full genetic make-up of an individual is called the
also found within the mitochondria of the cell and this genotype. The finished person – a product of inherited
is termed the mitochondrial DNA. traits and environmental influences – is the phenotype.
Each gene consists of a group of nucleotides and An important part of the unique human genotype
every nucleotide contains a deoxyribose sugar, a is the major histocompatibility complex (MHC), also
8 known as the HLA system (after human leucocyte anti- extra chromosome 21 (trisomy 21), Turner’s syn-
gen). This is a cluster of genes on chromosome 6 that drome, in which one of the X chromosomes is lacking
is responsible for immunological specificity. The pro- (monosomy X), and Klinefelter’s syndrome, in which
teins for which they code are attached to cell surfaces there is one Y but several X chromosomes.
and act as ‘chaperones’ for foreign antigens which
Single gene disorders Gene mutation may occur by
have to be accompanied by HLA before they are rec-
insertion, deletion, substitution or fusion of amino-
ognized and engaged by the body’s T-cells. HLA pro-

acids or nucleotides in the DNA chain. This can have
teins can be identified by serological tests and are
profound consequences for cartilage growth, collagen
registered according to their corresponding genetic
structure, matrix patterning and marrow cell metabo-
loci on the short arm of chromosome 6. HLA typing
lism. The abnormality is then passed on to future gen-
is particularly important in tissue transplantation:
erations according to simple mendelian rules (see
acceptance or rejection of the transplant hinges on the
below). There are literally thousands of single gene
degree of matching between the HLA genes of donor
disorders, accounting for over 5 per cent of child
and recipient.
deaths, yet it is rare to see any one of them in a life-
time of orthopaedic practice.
Genetic mutation Polygenic and multifactorial disorders Many normal
A mutation is any permanent change in DNA traits (body build, for example) derive from the inter-
sequencing or structure. Such changes in a somatic action of multiple genetic and environmental influ-
cell are characteristic of malignancy. In a germ-line ences. Likewise, certain diseases have a polygenic
cell, mutations contribute to generational diversity. background, and some occur only when a genetic pre-
Some genes have many forms (or mutations) and the disposition combines with an appropriate environ-
Human Genome Project has identified thousands of mental ‘trigger’. Gout, for example, is more common
single nucleotide polymorphisms (SNPs). than usual in families with hyperuricaemia: the uric
acid level is a polygenic trait, reflecting the interplay of
Point mutations The substitution of one nucleotide
multiple genes; it is also influenced by diet and may be
for another is the most common type of mutation.
more than usually elevated after a period of overindul-
The effect varies from production of a more useful
gence; finally, a slight bump on the toe acts as the
protein to a new but functionless protein, or an inabil-
proximate trigger for an acute attack of gout.
ity to form any protein at all; the result may be com-
patible with an essentially normal life or it may be
NON-GENETIC DEVELOPMENTAL DISORDERS
lethal.
Many developmental abnormalities occur sporadically
Deletions/Insertions Deletion or insertion of a segment and have no genetic background. Most of these are of
in the gene chain can result in an unusual protein unknown aetiology, but some have been linked to
being synthesized, perhaps a more advantageous one specific teratogenic agents which damage the embryo
but maybe one that is non-functional or one that has or the placenta during the first few months of gesta-
a dire effect on tissue structure and function (e.g. pro- tion. Suspected or known teratogens include viral
duction of a shortened dystrophin protein in the infections (e.g. rubella), certain drugs (e.g. thalido-
Becker variant of muscular dystrophy). mide) and ionizing radiation. The clinical features are
usually asymmetrical and localized, ranging from mild
morphological defects to severe malformations such
as spina bifida or phocomelia (‘congenital amputa-
GENETIC DISORDER tions’).
Any serious disturbance of either the quantity or the

arrangement of genetic material may result in disease.
Three broad categories of abnormality are recog- PATTERNS OF INHERITANCE
nized: chromosome disorders, single gene disorders
and polygenic or multifactorial disorders. The single gene disorders have characteristic patterns
of inheritance, which may be autosomal or X-linked,
Chromosome disorders Additions, deletions and
and dominant or recessive.
changes in chromosomal structure usually have seri-
ous effects; affected fetuses are either still-born or Autosomal dominant disorders Autosomal dominant
become infants with severe physical and mental disorders are inherited even if only one of a pair of
abnormalities. In live-born children there are a few alleles on a non-sex chromosome is abnormal; the
chromosome disorders with significant orthopaedic condition is said to be heterozygous. A typical example
152 abnormalities: Down’s syndrome, in which there is one is hereditary multiple exostoses. Either parent may be
8
Genetic disorders, skeletal dysplasias and malformations

(a) (b)
Proband
Affected male
Affected female
Carrier
Unaffected male
Unaffected female
(c)
8.1 Patterns of inheritance (a) Autosomal dominant. (b) Autosomal recessive. (c) X-linked recessive.
affected and half the children of both sexes develop itance: several siblings in one generation are affected
exostoses. The pedigree shows a ‘vertical’ pattern of but neither their parents nor their children have the
inheritance, with several affected siblings in successive disease (Fig. 8.1b).
generations (Fig. 8.1a)
X-linked disorders These conditions are caused by a
Sometimes both parents appear to be normal: the
faulty gene in the X chromosome. Characteristically,
patient may be the first member of the family to suf-
therefore, they never pass directly from father to son
fer the effects of a mutant gene; or (as often happens)
because the father’s X chromosome inevitably goes to
the disease shows variable expressivity, some members
the daughter and the Y chromosome to the son. X-
of the family (in the above example) developing many
linked dominant disorders (e.g. hypophosphataemic
large exostoses and severe bone deformities, while
rickets) pass from an affected mother to half of her
others have only a few small and well-disguised
daughters and half of her sons, or from an affected
nodules.
father to all of his daughters but none of his sons. Not
Autosomal recessive disorders These disorders appear surprisingly, they are twice as common in girls as in
only when both alleles of a pair are abnormal – i.e. the boys. X-linked recessive disorders – of which the most
condition is always homozygous. Each parent con- notorious is haemophilia – have a highly distinctive
tributes a faulty gene, though if both are heterozy- pattern of inheritance (Fig. 8.1c): an affected male
gous they themselves will be clinically normal. will pass the gene only to his daughters, who will
Theoretically 1 in 4 of the children will be homozy- become unaffected heterozygous carriers; they, in
gous and will therefore develop the disease; 2 out of turn, will transmit it to half of their daughters (who
4 will be heterozygous carriers of the faulty gene. The will likewise be carriers) and half of their sons (who
typical pedigree shows a ‘horizontal’ pattern of inher- will be bleeders). 153
8 In-breeding of injury to the fetus and are therefore used only when
there is reason to suspect some abnormality. Indica-
All types of genetic disease are more likely to occur in tions are: (1) maternal age over 35 years (increased
the children of consanguineous marriages or in closed risk of Down’s syndrome) or an unduly high paternal
communities where many people are related to each age (increased risk of achondroplasia); (2) a previous
other. The rare recessive disorders, in particular, are history of chromosomal abnormalities (e.g. Down’s
seen in these circumstances, where there is an syndrome) or genetic abnormalities amenable to bio-
increased risk of a homozygous pairing between two chemical diagnosis (neural tube defects, or inborn
mutant genes. errors of metabolism) which will benefit from prompt
neonatal treatment; or (3) to confirm non-invasive
Genetic heterogenicity tests suggesting an abnormality.
The same phenotype (i.e. a patient with a characteris-
tic set of clinical features) can result from widely dif- Maternal screening
ferent gene mutations. For example, there are four Fetal neural tube defects are associated with increased
different types of osteogenesis imperfecta (brittle levels of alpha-fetoprotein (AFP) in the amniotic fluid
bone disease), some showing autosomal dominant and, to a lesser extent, the maternal blood. Women
and some autosomal recessive inheritance. Where this with positive blood tests may be given the option of
occurs, the recessive form is usually the more severe. further investigation by amniocentesis. It has also
Subtleties of this kind must be borne in mind when been noted that abnormally low levels of AFP are
counselling parents. associated with Down’s syndrome.
Fetal cells may be present in maternal plasma and in
Genetic markers the near future it is possible that genetic testing of
these cells will be possible.
Many common disorders show an unusually close as-
sociation with certain blood groups, tissue types or
other serum proteins that occur with higher than ex- Amniocentesis
pected frequency in the patients and their relatives. Under local anaesthesia, a small amount (about
These are referred to as genetic markers; they arise 20 ml) of fluid is withdrawn from the amniotic sac
from gene sequences that do not cause the disease but with a needle and syringe. (It is best to determine the
are either ‘linked’ to other (abnormal) loci or else ex- position of the fetus beforehand by ultrasonography.)
press some factor that predisposes the individual to a The procedure is usually carried out between the 12th
harmful environmental agent. A good example is anky- and 15th weeks of pregnancy. The fluid can be exam-
losing spondylitis: over 90 per cent of patients, and 60 ined directly for AFP and desquamated fetal cells can
per cent of their first-degree relatives, are positive for be collected and cultured for chromosomal studies
HLA-B27. In this case (as in other autoimmune dis- and biochemical tests for enzyme disorders. It is well
eases) the HLA marker gene may provide the necessary to remember that this procedure carries a small risk
conditions for invasion by a foreign viral fragment. (0.5–0.75 per cent of cases) of losing the fetus.
Gene mapping Chorionic villus sampling

With advancing recombinant DNA technology, the Under ultrasound screening, a fine catheter is passed
genetic disorders are gradually being mapped to specific through the cervix and a small sample of chorion is
loci. In some cases (e.g. Duchenne muscular dystrophy) sucked out. This is usually done between the 10th and
the mutant gene itself has been cloned, holding out the 12th weeks of pregnancy. Mesenchymal fibroblasts
possibility of effective treatment in the future. can be cultured and used for chromosomal studies, bio-
chemical tests and DNA analysis. Rapid advances in
DNA technology have made it possible to diagnose
sickle-cell anaemia and haemophilia (among other dis-
PRE-NATAL DIAGNOSIS orders) during early pregnancy, but spina bifida can-
not be tested for. The procedure-related fetal loss rate
Many genetic disorders can be diagnosed before is about 1 per cent.
birth, thus improving the chances of treatment or, at
worst, giving the parents the choice of selective abor-
tion. Ultrasound imaging is harmless and is now done
Pre-implantation genetic diagnosis
almost routinely. On the other hand, tests that involve With assisted reproductive technologies such as in
154 amniocentesis or chorionic villus sampling carry a risk vitro fertilization (IVF), genetic abnormalities in the
embryos can be detected prior to implantation, thus 8
allowing only ‘healthy’ embryos (as far as technology
can tell) to be implanted into the mother.
Fetal imaging

High resolution ultrasonography should provide
images of all the long bones and joint movements by
11 weeks of gestation. Bone lengths increase linearly
with time and by 18–23 weeks all three segments of
each limb are clearly visible; a single measurement of
one bone can be used to estimate growth. A femoral
length that is normal for the fetal age is very signifi-
cant in excluding many of the skeletal dysplasias or
malformations; even with mesomelic abnormalities
where the lower leg is most affected, the femur is
likely to be short. By the 18th week of pregnancy
anatomical abnormalities such as open neural tube
defects and short limbs should be visible.
8.2 Normal proportions Upper segment = lower
segment. Total height = span.
DIAGNOSIS IN CHILDHOOD
femora), mesomelia – short middle segments (fore-
arms and legs) and acromelia – stubby hands and feet.
Clinical features
Dysmorphism (a misshapen part of the body) is most
Tell-tale features suggesting skeletal dysplasia are: obvious in the face and hands. There is a remarkable
consistency about these changes, which makes for a
• retarded growth and shortness of stature
disturbing similarity of appearance in members of a
• disproportionate length of trunk and limbs
particular group.
• localized malformations (dysmorphism)
Local deformities – such as kyphosis, valgus or varus
• soft-tissue contractures
knees, bowed forearms and ulnar deviated wrists –
• childhood deformity.
result from disturbed bone growth.
All the skeletal dysplasias affect growth, although this
may not be obvious at birth. Children should be
measured at regular intervals and a record kept of
X-rays
height, length of lower segment (top of pubic symph- The presence of any of the above features calls for a
ysis to heel), upper segment (pubis to cranium), span, limited radiographic survey: a posteroanterior view of
head circumference and chest circumference. Failure the chest, anteroposterior views of the pelvis, knees
to reach the expected height for the local population and hands, additional views of one arm and one leg, a
group should be noted, and marked shortness of lateral view of the thoracolumbar spine and standard
stature is highly suspicious. views of the skull. Fractures, bent bones, exostoses,
Bodily proportion is as important as overall height. epiphyseal dysplasia and spinal deformities may be
The normal upper segment:lower segment ratio obvious, especially in the older child. Sometimes a
changes gradually from about 1.5:1 at the end of the complete survey is needed and it is important to note
first year to about 1:1 at puberty. Shortness of stature which portion of the long bones (epiphysis, metaph-
with normal proportions is not necessarily abnormal, ysis or diaphysis) is affected. With severe and varied
but it is also seen in endocrine disorders which affect changes in the metaphyses, periosteal new bone for-
the different parts of the skeleton more or less equally mation or epiphyseal separation, always consider the
(e.g. hypopituitarism). By contrast, small stature with possibility of non-accidental injuries – the ‘battered
disproportionate shortness of the limbs is characteristic baby’ syndrome.
of skeletal dysplasia, the long bones being more
markedly affected than the axial skeleton.
The different segments of the limbs also may be dis-
proportionately affected. The subtleties of dysplastic In many cases the diagnosis can be made without
growth are reflected in terms such as rhizomelia – laboratory tests; however, routine blood and urine
unusually short proximal segments (humeri and analysis may be helpful in excluding metabolic and 155
8 endocrine disorders such as rickets and pituitary or • local bone deformities or exostoses
thyroid dysfunction. Special tests are also available to • spinal stenosis
identify specific excretory metabolites in the storage • repeated fractures
disorders, and specific enzyme activity can be meas- • secondary osteoarthritis (e.g. due to epiphyseal dys-
ured in serum, blood cells or cultured fibroblasts. plasia)
Bone biopsy is occasionally helpful in disorders of • joint laxity or instability.
bone density.
The clinical approach is similar to that employed
Direct testing for gene mutations is already available
with children.
for a number of conditions and is rapidly being
extended to others. It is a useful adjunct to clinical
diagnosis. Still somewhat controversial is its applica-
tion to pre-clinical diagnosis of late-onset disorders
and neonatal screening for potentially dangerous con- PRINCIPLES OF MANAGEMENT
ditions such as sickle-cell disease.
Management of the individual patient depends on the
diagnosis, the pattern of inheritance, the type and
Previous medical history severity of deformity or disability, mental capacity and
Always ask whether the mother was exposed to ter- social aspirations. However, it is worth noting some
atogenic agents (x-rays, cytotoxic drugs or virus infec- general principles.
tions) during the early months of pregnancy.
Communication
The family history
Once the diagnosis has been made, the next step is to
A careful family history should always be obtained. explain as much as possible about the disorder to the
This should include information about similar dis- patient (if old enough) and the parents without
orders in parents and close relatives, previous deaths causing unnecessary distress. This is a skill that the
in the family (and the cause of death), abortions and orthopaedic surgeon must develop. Nowadays, with
consanguineous marriages. However, the fact that quick and easy access to the internet, it is relatively
parents or relatives are said to be ‘normal’ does not easy to obtain useful information about almost any
exclude the possibility that they are either very mildly condition, which the clinician can pass on in simple
affected or have a biochemical defect without any language.
physical abnormality. Many developmental disorders Rare developmental disorders are best treated in a
have characteristic patterns of inheritance which may centre that offers a ‘special interest’ team consisting of
be helpful in diagnosis. a paediatrician, medical geneticist, orthopaedic sur-
Racial background is sometimes important: some geon, psychologist, social worker, occupational thera-
diseases are particularly common in certain communi- pist, orthotist and prosthetist.
ties, for example, sickle-cell disease in Negroid
peoples and Gaucher’s disease in Ashkenazi Jews.
Counselling
Patients and families may need expert counselling
about (1) the likely outcome of the disorders; (2)
DIAGNOSIS IN ADULTHOOD what will be required of the family; and (3) the risk of
siblings or children being affected. Where there are
It is unusual for a patient to present in adulthood with severe deformities or mental disability, the entire
a condition that has been present since birth but in family may need counselling.
milder cases the abnormality may not have been rec-
ognized, particularly when several members of the
family are similarly affected. Maintaining an independent lifestyle
In the worst of the genetic disorders the fetus is
Parents are often anxious about having their child
still-born or survives for only a short time. Individuals
grow up as ‘normal’ as possible, yet ‘normality’ may
who reach adulthood, though recognizably abnormal,
mean something different for the child. For example,
may lead active lives, marry and have children of their
it is expected that children will become independently
own. Nevertheless, they often seek medical advice for
mobile only by learning to walk in a safe and effective
several reasons:
manner, but some children with genetic disorders may
• short stature – especially disproportionate shortness be equally independently mobile with the use of a
156 of the lower limbs wheelchair. Management must be influenced by goals
for adult life and not just the short-term goals of 8
childhood. CLASSIFICATION OF
DEVELOPMENTAL DISORDERS
Intrauterine surgery
There is no completely satisfactory classification of
The concept of operating on the unborn fetus is developmental disorders. The same genetic abnormal-

already a reality and is likely to be extended in the ity may be expressed in different ways, while a variety
future. At present, however, it is still too early to say of gene defects may cause almost identical clinical syn-
whether the advantages (e.g. prenatal skin closure for dromes. The grouping used in Table 8.1 lists only the
dysraphism) will outweigh the risks. least rare of the developmental disorders that come
within the sphere of orthopaedic surgery and offers
Prevention and correction of deformities no more than a convenient way of remembering and
dividing the various clinical syndromes.
Realignment of the limb, correction of ligamentous
laxity and/or joint reconstruction can improve the
stability and efficiency of gait and reduce the risk of
secondary joint degenerative change.
Anomalies such as coxa vara, genu valgum, club
foot, radial club hand or scoliosis (and many others
THE CHONDRO-
outside the field of orthopaedics) are amenable to cor- OSTEODYSTROPHIES
rective surgery. In recent years, with advances in
methods of limb lengthening, many short-limbed The chondro-osteodystrophies, or skeletal dysplasias,
patients have benefited from this operation; however, are a large group of disorders characterized by abnor-
the risks should be carefully explained and the mal cartilage and bone growth. Since the various con-
expected benefits should not be exaggerated. ditions are caused by different gene defects, it would
Several developmental disorders are associated with be scientifically correct to classify them according to
potentially dangerous spinal anomalies: for example, their basic molecular pathology. However, the
spinal stenosis and cord compression in achondro- orthopaedic surgeon faced with a patient will seek first
plasia; atlantoaxial instability, due to odontoid aplasia, to categorize the disorder according to recognizable
in any disorder causing vertebral dysplasia; or severe clinical and x-ray appearances; it is with this in mind
kyphoscoliosis, which occurs in a number of condi- that the conditions are presented here in clinical
tions. Cord decompression or occipitocervical fusion rather than etiological groups, as follows:
are perfectly feasible, but surgical correction of con-
genital kyphoscoliosis carries considerable risks and • those with predominantly epiphyseal changes
should be undertaken only in specialized units. • those with predominantly physeal and metaphyseal
When considering the need for surgery, it must be changes
remembered that some of these patients have a sig- • those with mainly diaphyseal changes; and
nificantly reduced walking tolerance and hence • those with a mixture of abnormalities.
improvements in limb alignment or length, for
example, may not bring about any significant func-
tional change. Conservative measures such as physio-
therapy and splinting still have an important role to
DYSPLASIAS WITH PREDOMINANTLY
play. EPIPHYSEAL CHANGES
This group of disorders is characterized by abnormal

Gene therapy development and ossification of the epiphyses. Limb
Gene therapy is still at the experimental stage. A car- length may be reduced, though not as severely as in
rier molecule or vector (often a virus that has been conditions where the physis is affected.
genetically modified to carry some normal human
genetic material) is used to deliver the therapeutic (i.e.
normal) material into the abnormal target cells where MULTIPLE EPIPHYSEAL DYSPLASIA
the DNA is ‘uploaded’ allowing, for example, func-
tional protein production to be resumed. There have Multiple epiphyseal dysplasia (MED) varies in severity
been considerable concerns that the viral ‘infection’ from a trouble-free disorder with mild anatomical
may trigger an immune reaction and this is one of sev- abnormalities to a severe crippling condition. There is
eral factors affecting the development of this line of widespread involvement of the epiphyses but the ver-
therapy in the human ‘model’. tebrae are not at all, or only mildly, affected. 157
8 Table 8.1 A practical grouping of generalized developmental disorders
1 Disorders of cartilage and bone growth 2 Connective tissue disorders

1.1 Dysplasias with predominantly physeal and 2.1 Generalized joint laxity
metaphyseal changes
2.2 Ehlers–Danlos syndrome
1.1.1 Hereditary multiple exostosis
2.3 Larsen’s syndrome

1.1.2 Achondroplasia
2.4 Osteogenesis imperfecta (brittle bones)
1.1.3 Hypochondroplasia
2.4.1 Mild
1.1.4 Metaphyseal chondrodysplasia
2.4.2 Lethal
1.1.5 Dyschondroplasia (enchodromatosis, Ollier’s
2.4.3 Severe
disease)
2.4.4 Moderate
1.2 Dysplasias with predominantly epiphyseal
changes 2.5 Fibrodysplasia ossificans progressive
1.2.1 Multiple epiphyseal dysplasia 3 Storage disorders and other metabolic defects
1.2.2 Spondyloepiphyseal dysplasia 3.1 Mucopolysaccharidoses
1.2.3 Dysplasia epiphysealis hemimlica (Trevor’s 3.1.1 Hurler’s syndrome (MPS I)

disease) 3.1.2 Hunter’s syndrome (MPS II)
1.2.4 Chondrodysplasia punctata (stippled epiphysis) 3.1.3 Morquio–Brailsford syndrome (MPS IV)
1.3 Dysplasias with predominantly metaphyseal and 3.2 Gaucher’s disease
diaphyseal changes
3.3 Homocystinuria
1.3.1 Metaphyseal dysplasia (Pyle’s disease)
3.4 Alkaptonuria
1.3.2 Craniometaphyseal dysplasia
3.5 Congenital hyperuricaemia
1.3.3 Diaphyseal dysplasia (Engelmann’s disease,
Cumurati’s disease) 4 Chromosome disorders
1.3.4 Craniodiaphyseal dysplasia 4.1 Down’s syndrome
1.3.5 Osteopetrosis (marble bones, Albers–Shönbert 4.2 Thoracospinal anomalies

disease) 4.3 Elevation of the scapula (Sprengel’s deformity)
1.3.6 Pyknodysostosis 4.4 Limb anomalies
1.3.7 Candle bones, spotted bones and striped bones
1.4 Combined and mixed dysplasias

1.4.1 Spondylometaphyseal
1.4.2 Pseudoachondroplasia
1.4.3 Diastrophic dysplasia
1.4.4 Cleidocranial dysplasia
1.4.5 Nail–patella syndrome
1.4.6 Craniofacial dysplasia
Clinical features In adult life, residual epiphyseal defects may lead to

joint incongruity and secondary osteoarthritis. If the
Children are below average height and the parents anatomical changes are mild, the underlying abnor-
may have noticed that the lower limbs are dispropor- mality may be missed and the patient is regarded as
tionately short compared to the trunk. They some- ‘just another case of OA’ (see Fig. 5.9 page 92).
times walk with a waddling gait and they may
complain of hip or knee pain. Some develop progres-
sive deformities of the knees and/or ankles. The
hands and feet may be short and broad. The face,
X-ray
skull and spine are normal. Changes are apparent from early childhood. Epiphyseal
In some cases only one or two pairs of joints are ossification is delayed, and when it appears it is irregu-
involved, while in others the condition is widespread; lar or abnormal in outline. In the growing child the epi-
these are probably expressions of several different dis- physes are misshapen; in the hips this may be mistaken
158 orders. for bilateral Perthes’ disease, but the symmetrical nature
8.3 Multiple 8
epiphyseal
dysplasia
(a,b) X-rays show
epiphyseal distortion
and flattening at
multiple sites, in this

case the hips, knees
and ankles. (c) The
ring epiphyses of the
(a)
vertebral bodies also
may be affected; in
spondyloepiphyseal
dysplasia this is the
dominant feature.
(b) (c)
of the changes and the presence of changes in other epi- typical cycle of changes from epiphyseal irregularity to
physes usually define the condition as MED. The ver- fragmentation, flattening and healing.
tebral ring epiphyses may be affected, but only mildly. Hypothyroidism, if untreated, causes progressive and
At maturity the femoral heads, femoral condyles and widespread epiphyseal dysplasia. However, these chil-
humeral heads are flattened; secondary osteoarthritis dren have other clinical and biochemical abnormali-
may ensue and, if many joints are involved, the patient ties and have learning difficulties.
can be severely crippled.
Management
Genetics Children may complain of slight pain and limp, but
This appears to be a heterogeneous disorder but most little can (or need) be done about this. At maturity,
cases have an autosomal dominant pattern of inheri- deformities around the hips, knees or ankles some-
tance. times require corrective osteotomy.
The abnormality identified in some cases is in the In later life, secondary osteoarthritis may call for
gene which codes for cartilage oligometric matrix pro- reconstructive surgery.
tein (COMP). In ways which are not fully under-
stood, this results in defective chondrocyte function.
SPONDYLOEPIPHYSEAL DYSPLASIA
Diagnosis
The term ‘spondyloepiphyseal dysplasia’ (SED) en-
MED is often confused with other childhood disor- compasses a heterogeneous group of disorders in which
ders which are associated with either lower-limb multiple epiphyseal dysplasia is associated with well-
shortness or Perthes-like changes in the epiphyses. marked vertebral changes – delayed ossification, flat-
Achondroplasia and hypochondroplasia should not tening of the vertebral bodies (platyspondyly), irregu-
be difficult to exclude. The former is marked by a lar ossification of the ring epiphyses and indentations of
more severe shortening in height and characteristic the end-plates (Schmorl’s nodes). The mildest of these
facial changes; the latter by the absence of epiphyseal disorders is indistinguishable from MED; the more se-
changes. Dyschondrosteosis, likewise, is associated with vere forms have characteristic appearances.
normal epiphyses.
Pseudoachondroplasia shows widespread epiphyseal
abnormalities. However, the skeletal deformities are
Clinical features
more severe than those of MED and they also involve SED CONGENITA
the spine. This autosomal dominant disorder can be diagnosed
Perthes’ disease is confined to the hips and shows a in infancy: the limbs are short, but the trunk is even 159
8 Management may involve corrective osteotomies
for severe coxa vara or knee deformities. Odontoid
hypoplasia increases the risks of anaesthesia; if there is
evidence of subluxation, atlantoaxial fusion may be
advisable.
SED TARDA
An X-linked recessive disorder, SED tarda is much less
severe and may become apparent only after the age of 5
(a) years when the child fails to grow normally and develops
a kyphoscoliosis. Adult men tend to be more severely
affected than women, showing a disproportionate
shortening of the trunk and a tendency to barrel chest.
They may develop backache or secondary osteoarthritis
of the hips.
X-rays show the characteristic platyspondyly and
abnormal ossification of the ring epiphyses, together
with more widespread dysplasia.
Treatment may be needed for backache or (in older
adults) for secondary osteoarthritis of the hips.
(b)
DYSPLASIA EPIPHYSEALIS HEMIMELICA
(TREVOR’S DISEASE)
This is a curious ‘hemidysplasia’ affecting just one half
(medial or lateral) of one or more epiphyses on one
8.5 Epiphyseal
dysplasia
(a) Trevor’s disease.
(b) Conradi’s
disease – the
(c) ‘spots’ disappeared
later.
8.4 Spondyloepiphyseal dysplasia (a,b) Adolescent
boys with marked lumbar lordosis, vertebral deformities,
flexed hips and epiphyseal dysplasia affecting all the limbs.
(c) Widespread deformities and barrel chest in adulthood.
X-rays show severe secondary osteoarthritis of the hips.
shorter and the neck hardly there. Older children

develop a dorsal kyphosis and a typical barrel-shaped
chest; they stand with the hips flexed and the lumbar
spine in marked lordosis. By adolescence they often
have scoliosis. (a)
X-rays show widespread epiphyseal dysplasia and
the characteristic vertebral changes. Odontoid
hypoplasia is common and may lead to atlantoaxial
subluxation and cord compression.
Diagnosis is not always easy; there are obvious sim-
ilarities to Morquio’s disease but, in the latter, short-
ening is in the distal limb segments and urinalysis
160 shows increased excretion of keratan sulphate. (b)
side of the body. It is a sporadic disorder which usu- Clinical Features 8
ally appears at the ankle or knee. The child (most
often a boy) presents with a bony swelling on one side The condition is usually discovered in childhood; hard
of the joint; several sites may be affected – all on the lumps appear at the ends of the long bones and along
same side in the same limb, but rarely in the upper the apophyseal borders of the scapula and pelvis. As
limb. the child grows, these lumps enlarge and some may

X-rays show an asymmetrical enlargement of the become hugely visible, especially around the knee.
bony epiphysis and distortion of the adjacent joint. At The more severely affected bones are abnormally
the ankle, this may give the appearance of an abnor- short; this is seldom very marked but on measurement
mally large medial malleolus. the lower body segment is shorter than the upper and
Treatment is called for if the deformity interferes span is less than height (Solomon, 1963). In the fore-
with joint function. The excess bone is removed, tak- arm and leg, the thinner of the two bones (the ulna or
ing care not to damage the articular cartilage or liga- fibula) is usually the more defective, resulting in typi-
ments. cal deformities: ulnar deviation of the wrist, bowing of
the radius, subluxation of the radial head, valgus knees
and valgus ankles. Bony lumps may cause pressure on
nerves or vessels. Occasionally one of the cartilage-
CHONDRODYPLASIA PUNCTATA (STIPPLED capped exostoses goes on growing into adult life and
EPIPHYSES) transforms to a chondrosarcoma; this is said to occur
in 1–2 per cent of patients.
Chondrodysplasia punctata (or Conradi’s disease) is a
generalized, multisystem disorder producing facial
abnormalities, vertebral anomalies, asymmetrical epi- X-RAY
physeal changes and bone shortening. In severe cases Typically the long-bone metaphyses are broad and
there may also be cardiac anomalies, congenital poorly modelled, with sessile or pedunculated exos-
cataracts and learning difficulties; some of these chil- toses arising from the cortices – almost as if longitu-
dren die during infancy. dinal growth has been squandered in profligate lateral
The characteristic x-ray feature is a punctate stip- expansion. A mottled appearance around a bony
pling of the cartilaginous epiphyses and apophyses. excrescence indicates calcification in the cartilage cap.
This disappears by the age of 4 years but is often fol- The distal end of the ulna is sometimes tapered or car-
lowed by epiphyseal irregularities and dysplasia. It is rot-shaped and the bone may be markedly reduced in
unlikely that these changes will be confused with length; in these cases the radius is usually bowed, or
those of MED, Down’s syndrome or hypothyroidism. the discrepancy in length may lead to subluxation of
Orthopaedic management is directed at the defor- the radiohumeral joint.
mities that develop in older children: joint contrac- The cuboidal carpal and tarsal bones show little or
tures, limb length inequality or scoliosis. no change on x-ray. This is simply because the ossified
parts of these bones (which is all that is visible on
x-ray) are completely surrounded by cartilage during
early development, and any cartilage irregularities are
subsumed in the overall expansion of the bone.
PHYSEAL AND METAPHYSEAL Pathology
CHANGES
The underlying fault in multiple exostosis is unre-
strained transverse growth of the cartilaginous physis
In these disorders there is abnormal physeal growth, (growth plate). The condition affects only the endo-
defective metaphyseal modelling and shortness of the chondral bones. Cartilaginous excrescences appear at
tubular bones. The axial skeleton is affected too, but the periphery of the physes and proceed, in the usual
the limbs are disproportionately short compared to way, to endochondral ossification. If the abnormal
the spine. physeal proliferation ceases at that point, but the bone
continues to grow in length, the exostosis is left behind
where it arose (now part of the metaphysis) but its car-
HEREDITARY MULTIPLE EXOSTOSIS tilage cap is still capable of autonomous growth. If the
(DIAPHYSEAL ACLASIS) physeal abnormality persists, further growth proceeds
in the new abnormal mould, without remodelling of
Multiple exostosis is the most common, and least dis- the broadened and misshapen metaphysis. The process
figuring, of the skeletal dysplasias. finally comes to a stop when endochondral proliferation 161
8 8.6 Hereditary multiple exostoses
Clinical presentation at (a) 3 years
(b) 6 years and (c) 28 years. In (c) note
the numerous small ‘bumps’, the one
large tumour near the right shoulder,
bowing of the left radius, shortening
of the left forearm and valgus
deformity of the right knee.
(a) (b) (c)
8.7 Hereditary multiple

exostoses – x-rays
(a) Typical x-ray appearances
of the knees. (b) Sessile
exostoses of the femoral
neck. (c) A large
pedunculated exostosis of the
distal femur. (d) Evolution of
the wide metaphysis during
growth.
(a) (b) (c)
(d)
ceases at the end of the normal period of growth for people are so mildly affected as to be unaware of the
that bone; any further growth of the exostotic cartilage disorder. In some cases the condition appears to be due
cap after that suggests neoplastic change. to a spontaneous mutation but this may be because the
parent is so mildly affected as to seem normal.
Abnormalities have been identified on chromo-
Genetics
somes 8, 11 and 19, referred to as EXT 1, 2 and 3,
The condition is acquired by autosomal dominant the differing sites being responsible for different phe-
transmission; half the children are affected, boys and notypes. The molecular basis of this condition is not
162 girls equally. However, expression is variable and some yet understood.
Management present in infancy but this almost always disappears in 8
a year or two. Mental development is normal.
Exostoses may need removal because of pressure on a By early childhood the trunk is obviously dispropor-
nerve or vessel, because of their unsightly appearance, tionately long in comparison with the limbs. Joint laxity
or because they tend to get bumped during everyday is common and contributes to the characteristic standing
activities. Care must be taken not to damage the phy- posture: flat feet, bowed legs, flexed hips, prominent

ses. Deformities of the legs or forearms may be severe buttocks, lordotic spine and elbows slightly flexed.
enough to warrant treatment by corrective osteotomy Relative stenosis of the foramen magnum can be a
or concomitant correction and lengthening by the problem in infancy. During adulthood, shortening of
Ilizarov technique (see Chapter 12). Physeal stapling or the vertebral pedicles may lead to lumbar spinal steno-
plating may be used to direct longitudinal growth. sis and disc prolapse (which is quite common) has
Exostoses should stop growing when the parent exceptionally severe neurological effects. Cervical spine
bone does; any subsequent enlargement suggests stenosis may cause typical features of cord compression.
malignant change and calls for advanced imaging and
wide local resection.
X-Rays
All bones that are formed by endochondral ossifica-
ACHONDROPLASIA tion are affected, so the facial bones and skull base are
abnormal but the cranial vault is not. The foramen
This is the commonest form of abnormally short magnum is smaller than usual. The tubular bones are
stature; adult height is usually around 122 cm (48 short but thick, the metaphyses flared and the physeal
inches). Disproportionate shortening of the limb lines somewhat irregular; sites of muscle attachment,
bones is detectable in utero by ultrasound scan. such as the tibial tubercle and the greater trochanter
of the femur, are prominent. Although the proximal
limb bones are disproportionately affected (rhi-
Clinical Features zomelia), changes are also seen in the wrists and
The abnormality is obvious in childhood: growth is hands, where the metaphyses are broad and cup-
severely stunted; the limbs – particularly the proximal shaped. The epiphyses are surprisingly normal and
segments – are disproportionately short (rhizomelic hence joint degeneration is uncommon.
shortening) and the skull is quite large with promi- The pelvic cavity is small (too small for normal
nent forehead and saddle-shaped nose. Frontal boss- delivery) and the iliac wings are flared, producing an
ing and mid-face hypoplasia contribute to the almost horizontal acetabular roof. The vertebral inter-
characteristic appearance of people with achondropla- pedicular distance often diminishes from L1 to L5 and
sia. The fingers appear stubby and somewhat splayed the spinal canal is reduced in size. These features are
(trident hands). A thoracolumbar kyphos is often best defined on CT or MRI.
(a) (b) (c) (d)
8.8 Achondroplasia (a) Mother and child with achondroplasia, showing the typical disproportionate shortening of the
tubular bones, particularly the proximal segments of the upper and lower limbs. (b) Other features are seen in this child:
lumbar lordosis, a prominent thoracolumbar gibbus and bossing of the forehead. (c,d) X-rays show the short, thick bones
(including the metacarpals). 163
8 Diagnosis Anaesthesia carries a greater than usual risk and
requires expert supervision.
Achondroplasia should not be confused with other
types of short-limbed ‘dwarfism’. In some (e.g.
Morquio’s disease) the shortening affects distal seg-
ments more than proximal and there may be wide- HYPOCHONDROPLASIA
spread associated abnormalities. Others (e.g.

This has been described as a very mild form of achon-
pseudoachondroplasia and the epiphyseal dysplasias)
droplasia. However, apart from shortness of stature
are distinguished by the fact that the head and face are
(with the emphasis on proximal limb segments) and
quite normal whereas the epiphyses show characteris-
noticeable lumbar lordosis, there is little to suggest any
tic changes on x-ray examination.
abnormality; the head and face are not affected and
many of those with hypochondroplasia pass for normal
Pathology stocky individuals. X-rays may show slight pelvic flat-
tening and thickening of the long bones. The condi-
This is essentially an abnormality of endochondral tion is transmitted as autosomal dominant, hence
longitudinal growth resulting in diminished length of several members of the same family may be affected.
the tubular bones. Membrane bone formation is unaf- Those affected sometimes ask for limb lengthening;
fected, hence the normal growth of the skull vault and after careful discussion, this may be done with a con-
the periosteal contribution to bone width. siderable chance of success.
Genetics
Achondroplasia occurs in about 1 in 30,000 births. DYSCHONDROSTEOSIS (LEHRI–WEILL
Inheritance is by autosomal dominant transmission; SYNDROME)
however, because few achondroplastic people have
children, over 80 per cent of cases are sporadic. In this disorder there is also disproportionate short-
The fault has been shown to be a gain-in-function ening of the limbs, but it is mainly the middle
mutation in the gene encoding for the growth- segments (forearms and legs) which are affected. It is
suppressing fibroblast growth factor receptor 3 the commonest of the mesomelic dysplasias and is
(FGFR-3) on chromosome 4. The effect on the transmitted as an autosomal dominant defect. Stature
proliferative zone of the physis is increased inhibition is reduced but not as markedly as in achondroplasia.
of growth, and the thickness of the hypertrophic cell The most characteristic x-ray changes are shortening
zone is reduced; this accounts for the diminution in of the forearms and leg bones, bowing of the radius
endochondral bone growth. and Madelung’s deformity of the wrist, which may
require operative treatment (see page 390).
Management
During childhood, operative treatment may be needed METAPHYSEAL CHONDRODYSPLASIA
for lower limb deformities (usually genu varum). Oc- (DYSOSTOSIS)
casionally the thoracolumbar kyphosis fails to correct it-
self; if there is significant deformity (angulation of more This term describes a type of short-limbed dwarfism in
than 40°) by the age of 5 years, there is a risk of cord which the bony abnormality is virtually confined to the
compression and operative correction may be needed. metaphyses. The epiphyses are unaffected but the
During adulthood, spinal stenosis may call for metaphyseal segments adjacent to the growth plates
decompression. Intervertebral disc prolapse superim- are broadened and mildly scalloped, somewhat resem-
posed on a narrow spinal canal should be treated as an bling rickets. There may be bilateral coxa vara and
emergency. bowed legs; patients tend to walk with a waddling gait.
Advances in methods of external fixation have made Apart from a lordotic posture, the spine is normal. The
leg lengthening a feasible option. This is achieved by main deformities are around the hips and knees.
distraction osteogenesis (see Chapter 12). However, There are several forms of metaphyseal chondrodys-
there are drawbacks: complications, including non- plasia. The best known (Schmid type) has the classic
union, infection and nerve palsy, may be disastrous; and features described above, with autosomal dominant
the cosmetic effect of long legs and short arms may be inheritance. Another group (McKusick type) is associ-
less pleasing than anticipated. It is essential that the de- ated with sparse hair growth and is sometimes compli-
tails of the operation, its aims and limitations and the cated by Hirschsprung’s disease; inheritance shows an
potential complications be fully discussed with the pa- autosomal recessive pattern. It is thought that these
164 tient (and, where appropriate, with the parents). cases may represent an entirely distinct entity. The
Clinical Features 8
Typically the disorder is unilateral; indeed only one
limb or even one bone may be involved. An affected
limb is short, and if the growth plate is asymmetrically
involved the bone grows bent; bowing of the distal

end of the femur or tibia is not uncommon and the
patient may present with valgus or varus deformity at
the knee and ankle. Shortening of the ulna may lead
to bowing of the radius and, sometimes, dislocation of
the radial head. The fingers or toes frequently contain
multiple enchondromata, which are characteristic of
the disease and may be so numerous that the hand is
crippled. A rare variety of dyschondroplasia is associ-
ated with multiple haemangiomata (Maffucci’s dis-
ease); this is described below.
The condition is not inherited; indeed, it is proba-
(a) (b) bly an embryonal rather than a genetic disorder.
8.9 Metaphyseal chondrodysplasia This boy with the
rare Jansen type shows the typical shortening of the lower X-Rays
limbs and metaphyseal enlargement of the long bones. The
x-rays show that the changes are confined to the The characteristic change in the long bones is radio-
metaphyses. lucent streaking extending from the physis into the
metaphysis – the appearance of persistent, incom-
pletely ossified cartilage columns trapped in bone. If
rarest (and most severe) of all (Jansen type) is usually only half the physis is affected, growth is asymmetri-
sporadic and may be associated with deafness. cally retarded and the bone becomes curved. With
Operative correction (osteotomy) may be needed maturation the radiolucent columns eventually ossify
for coxa vara or tibia vara. but the deformities remain. In the hands and feet the
cartilage islands characteristically produce multiple
enchondromata. Beware of any change in the appear-
ance of the lesions after the end of normal growth;
DYSCHONDROPLASIA this may be a sign of malignant change, which occurs
(ENCHONDROMATOSIS; OLLIER’S in 5–10 per cent of cases.
DISEASE)
Treatment
This is a rare, but easily recognized, disorder in which
there is defective transformation of physeal cartilage Bone deformity may need correction, but this should
columns into bone. No consistent inheritance pattern be deferred until growth is complete; otherwise it is
has been identified. likely to recur.
(a) (b) (c) (d) (e)
8.10 Dyschondroplasia (a,b) The bent femur in this boy is due to slow growth of half the lower femoral physis.
(c) Incomplete ossification of the cartilage columns accounts for the curious metaphyseal appearance. (d,e) Two patients
with multiple chondromas. 165
8 MAFFUCCI’S DISEASE occlusion may cause cranial nerve compression –
sometimes severe enough to require operative treat-
This rare disorder is characterized by the development ment.
of multiple enchondromas and soft-tissue haeman-
giomas of the skin and viscera. Lesions appear during
childhood; boys and girls are affected with equal fre-
quency.
There is a strong tendency for malignant change to DIAPHYSEAL CHANGES
occur in both soft-tissue and bone lesions; the inci-
dence of sarcomatous transformation in one of the Most of the ‘metaphyseal’ and ‘diaphyseal dysplasias’
enchondromas is probably greater than 50 per cent, appear to be the result of defective bone modelling.
but fortunately these tumours are not highly malig- Unlike the physeal and epiphyseal disorders, dwarfing
nant. is not a feature. There may be associated thickening of
Patients with Mafucci’s disease should be moni- the skull bones, with the risk of foraminal occlusion
tored regularly throughout life for any change in and cranial nerve entrapment.
either the bone or visceral lesions. Fibrous dysplasia is dealt with in Chapter 9.
METAPHYSEAL DYSPLASIA (PYLE’S OSTEOPETROSIS (MARBLE BONES,

DISEASE) ALBERS–SCHÖNBERG DISEASE)
The only significant clinical feature in this disorder is Osteopetrosis is one of several conditions which are
genu valgum – or rather valgus angulation of the characterized by sclerosis and thickening of the bones
bones on either side of the knee. X-rays show a typi- which appear with increased radiographic density.
cal ‘bottle shape’ of the distal femur or proximal tibia This is the result of an imbalance between bone for-
– the so-called Erlenmeyer flask deformity – suggest- mation and bone resorption; in the most common
ing a failure of bone modelling. Inheritance pattern is form, osteopetrosis, there is failed bone resorption
autosomal recessive. Treatment is seldom needed. due to a defect in osteoclast production and/or func-
Other conditions – notably Gaucher’s disease and tion.
thalassaemia – are also associated with Ehlenmeyer
flask deformities of the femur.
Osteopetrosis tarda
The common form of osteopetrosis is a fairly benign,
Craniometaphyseal dysplasia
autosomal dominant disorder that seldom causes
This condition, of autosomal dominant inheritance, is symptoms and may only be discovered in adolescence
similar to Pyle’s disease, but here the tubular defect is or adulthood after a pathological fracture or when an
associated with progressive thickening of the skull and x-ray is taken for other reasons – hence the designa-
mandible resulting in a curiously prominent forehead, tion tarda. Appearance and function are unimpaired,
a large jaw and a squashed-looking nose. Foraminal unless there are complications: pathological fracture
8.11 Marble bones Despite the remarkable density, the bones break easily; but, as in this humerus, union occurs,
166 although rather slowly.
or cranial nerve compression due to bone encroach- Milder cases usually clear up spontaneously by the age 8
ment on foramina. Sufferers are also prone to bone of 25 years.
infection, particularly of the mandible after tooth
extraction.
X-rays show increased density of all the bones: cor- CRANIODIAPHYSEAL DYSPLASIA
tices are widened, leaving narrow medullary canals;

sclerotic vertebral end-plates produce a striped This rare autosomal recessive disorder is characterized
appearance (‘football-jersey spine’); the skull is thick- by cylindrical expansion of the long bones and gross
ened and the base densely sclerotic. thickening of the skull and facial bones. Prominent
Treatment is required only if complications occur. facial contours may appear in early childhood and are
the most striking feature of the condition – giving rise
to the name ‘leontiasis’. Foraminal occlusion may
Osteopetrosis congenita cause deafness or visual impairment.
This rare, autosomal recessive form of osteopetrosis is
present at birth and causes severe disability. Bone
encroachment on marrow results in pancytopenia, PYKNODYSOSTOSIS
haemolysis, anaemia and hepatosplenomegaly. Foram-
inal occlusion may cause optic or facial nerve palsy. Interest in this rare disorder owes something to the
Osteomyelitis following, for example, tooth extrac- suggestion that the French impressionist, Toulouse-
tion or internal fixation of a fracture is quite common. Lautrec, was a victim. Clinical features are shortness
Repeated haemorrhage or infection usually leads to of stature, frontal bossing, underdevelopment of the
death in early childhood. mandible and abnormal dentition. The presence of
Treatment, in recent years, has focused on methods blue sclerae and propensity to fracture may cause con-
of enhancing bone resorption and haematopoeisis, fusion with osteogenesis imperfecta. The condition is
e.g. by transplanting marrow from normal donors and inherited as an autosomal recessive trait.
by long-term treatment with gamma-interferon. On x-ray the bones are dense; the skull is enlarged,
with wide suture lines and open fontanelles, but the
facial bones and mandible are hypoplastic, thus
DIAPHYSEAL DYSPLASIA (ENGELMANN’S accounting for the typical ‘triangular’ facies.
Despite appearances, it causes little trouble (apart
OR CAMURATI’S DISEASE) from the odd pathological fracture) and needs no
treatment.
This is another rare childhood disorder in which x-
rays show fusiform widening and sclerosis of the shafts
of the long bones, and sometimes thickening of the
skull. The condition is notable because of its associa- CANDLE, SPOTTED AND STRIPED BONES
tion with muscle pain and weakness. Children com-
Candle bones (melorheostosis, Leri’s disease) This rare,
plain of ‘tired legs’ and have a typical wide-based or
waddling gait. There may be muscle wasting and fail- non-familial, condition is sometimes discovered
ure to thrive. (almost accidentally) in patients who complain of pain
Muscle pain may need symptomatic treatment. and stiffness in one limb. X-rays show irregular
patches of sclerosis, usually distributed in a linear fash-
ion through the limb; the appearance is reminiscent of
wax that congeals on the side of a burning candle.
Some patients also develop scleroderma and joint
contractures.
Spotted bones (osteopoikilosis) Routine x-rays some-
times show (quite incidentally) numerous white spots
distributed throughout the skeleton. Closer examina-
tion occasionally reveals whitish spots in the skin (dis-
seminated lenticular dermatofibrosis). The condition
is inherited as an autosomal dominant trait.
Striped bones (osteopathia striata) X-rays show lines of
increased density parallel to the shafts of long bones,
8.12 Engelmann’s disease This patient had considerable but radiating like a fan in the pelvis. The condition is
discomfort from her long bones – all of which were wide symptomless. Some cases show autosomal dominant
and looked dense on x-ray. inheritance. 167
8 8.13 Candle bones, spotted
bones and striped bones
(a,b) Melorheostosis
(c,d) Osteopoikilosis
(e,f) Osteopathia striatia.
(a) (b) (c)
(d) (e) (f)
In contrast to achondroplasia, clinical features are not

COMBINED AND MIXED DYSPLASIAS evident at birth but become apparent only a year or two
later; the head and face look normal and spinal stenosis
A number of disorders show a mixture of epiphyseal, is not a feature.
physeal, metaphyseal and vertebral defects – i.e. Ligamentous laxity (particularly noticeable in the
dwarfism combined with epiphyseal maldevelopment, wrists) as much as anything has a significant effect on
abnormal modelling of the metaphyses and restricting function and also walking tolerance.
platyspondyly. The characteristic x-ray features are underdevelop-
ment and flattening of the epiphyses, widening of the
metaphyses, shortening of the tubular bones and oval-
SPONDYLOMETAPHYSEAL DYSPLASIA shaped vertebral bodies. By the end of growth, the
hips may be dysplastic and the vertebral bodies often
This is the commonest of the ‘mixed’ dysplasias. show defects of the bony end-plates. Spinal stenosis is
There may be severe vertebral flattening and not a feature.
kyphoscoliosis. Epiphyseal changes are usually mild Deformities sometimes require surgical correction.
but the metaphyses are broad and ill-formed. Patients In adults, secondary osteoarthritis may call for recon-
may need treatment for spinal deformity or malalign- structive surgery.
ment of the hip or knee.
PSEUDOACHONDROPLASIA DIASTROPHIC DYSPLASIA

This rare autosomal dominant disorder resembles This autosomal recessive disorder affects all types of
achondroplasia in that it is characterized by short- cartilage. Infants are severely dwarfed and distorted,
limbed dwarfism associated with ligamentous laxity, with deformities of the hands (‘hitch-hiker’s thumb’),
168 exaggerated lumbar lordosis and bow-leg deformities. club feet, joint contractures, dislocations, ‘cauliflower’
ears and cleft palate. Softening of the laryngeal carti- unduly wide and there may be some disproportion of 8
lage may produce respiratory distress. In older chil- the forearm or finger bones.
dren the main problems are scoliosis and joint X-rays show a brachycephalic skull and persistence
contractures. of wormian bones. Characteristically there is underde-
X-rays show epiphyseal hypoplasia and maldevelop- velopment of the clavicles, scapulae and pelvis. Much
ment, metaphyseal thickening, flattening of the pelvis of the clavicle may be missing, leaving a nubbin of

and kyphoscoliosis. Odontoid hypoplasia is usual. bone at the medial or lateral end. Scoliosis and coxa
Management involves early correction of joint con- vara are common.
tractures and treatment of club foot and hand defor- Treatment is unnecessary unless the patient devel-
mities. Scoliosis may require correction and spinal ops severe coxa vara or scoliosis; dental anomalies may
fusion. need attention.
CLEIDOCRANIAL DYSPLASIA NAIL–PATELLA SYNDROME

This disorder, of autosomal dominant inheritance, is This curious condition is relatively common and is
characterized by hypoplasia of the clavicles and flat inherited as an autosomal dominant trait. The nails
bones. In a typical case the patient is somewhat short, are hypoplastic and the patellae unusually small or
with a large head, frontal prominence, a flat-looking absent. The radial head is subluxed laterally and the
face and drooping shoulders. The teeth appear late elbows may lack full extension. Congenital nephro-
and develop poorly. Because the clavicles are pathy may be associated. The characteristic x-ray
hypoplastic or absent, the chest seems narrow and the features are hypoplastic or absent patellae and the
patient can bring his shoulders together anteriorly. presence of bony protuberances (‘horns’) on the
The pelvis is narrow but the symphysis pubis may be lateral aspect of the iliac blades.
8.14 Cleidocranial
dysplasia The ‘squashed
face’ and sloping shoulders
which can be brought
together anteriorly are
pathognomonic.
8.15 The nail–patella

syndrome The dystrophic
nails, minute patellae, pelvic
‘horns’ and subluxed radii
combine to give an
unmistakable picture.
169
8 CRANIOFACIAL DYSPLASIA 1. Passive hyperextension of the metacarpopha-
langeal joint of the fifth finger to beyond 90°
Many disorders – some inherited, some not – are dis- (score 2);
tinguished primarily by the abnormal appearance of 2. Passive stretching of the thumb to touch the
the face and skull. Other bones may be affected as radial border of the forearm (score 2);
well, but it is the odd facial appearance that is most 3. Hyperextension of the elbows (score 2);
striking. Premature fusion of the cranial sutures may 4. Hyperextension of the knees (score 2);
lead to exophthalmos and learning difficulties. 5. Ability to bend forward and place the hands flat
Orthopaedic problems arise from the associated on the floor with the knees held perfectly straight
anomalies of the hands and feet. (score 1).
The best-known of these conditions is Apert’s syn-
The trait runs in families and is inherited as a
drome (acrocephalosyndactyly). The head is somewhat
mendelian dominant. The condition is not in itself
egg-shaped: flat at the back, narrow anteroposteriorly,
disabling but it may predispose to congenital disloca-
with a broad, towering forehead, depressed face,
tion of the hip in the newborn or recurrent disloca-
bulging eyes and prominent jaw. The hands and feet are
tion of the patella or shoulder in later life. Transient
misshapen, with syndactyly or synostosis of the medial
joint pains are common and there is an increased risk
rays. The condition sometimes shows autosomal dom-
of ankle sprains.
inant inheritance, but most cases are sporadic.
A more florid hypermobility syndrome character-
Cerebral compression can be prevented by early
ized by lax connective tissues and joint subluxations
craniotomy and the facial appearance may be
may be associated with other conditions such as gas-
improved by maxillofacial reconstruction. Syndactyly
tro-oesophageal reflux, irritable bowel syndrome and
usually needs operative treatment.
bowel or uterine prolapse, sometimes merging with
variants of Ehlers–Danlos syndome (see below).
CONNECTIVE TISSUE
DISORDERS MARFAN’S SYNDROME
Collagen is the commonest form of body protein, mak- This is a generalized disorder affecting the skeleton,
ing up 90 per cent of the non-mineral bony matrix and joint ligaments, eyes and cardiovascular structures. It
70 per cent of the structural tissue in ligaments and ten- is thought to be due to a cross-linkage defect in colla-
dons. Some 20 types of collagen, produced by 30 or gen and elastin. The genetic abnormality has been
more genes, have been identified; those distributed mapped to the fibrillin gene on chromosome 15. It is
most abundantly in the musculoskeletal system are type transmitted as autosomal dominant but sporadic cases
I (in bone, ligament, tendon and skin), type II (in car- also occur. Males and females are affected equally.
tilage) and type III (in blood vessels, muscle and skin).
Heritable defects of collagen synthesis give rise to a Clinical Features
number of disorders involving either the soft connec-
tive tissues or bone, or both. In many cases the spe- Patients tend to be tall, with disproportionately long
cific collagen defect can now be identified. legs and arms, and often with flattening or hollowing
of the chest (pectus excavatum). Typically, the upper
body segment is shorter than the lower (a ratio of less
BENIGN JOINT HYPERMOBILITY than 0.8 is suggestive) and arm span exceeds height
(GENERALIZED FAMILIAL JOINT LAXITY) by 5 cm or more. The digits are unusually long, giv-
ing rise to the term ‘arachnodactyly’ (spider fingers).
About 5 per cent of normal people have joint hyper- Spinal abnormalities include spondylolisthesis and
mobility, as defined by a positive score of more than 5 scoliosis. There is an increased incidence of slipped
(the Beighton score) in the following tests: upper femoral epiphysis. Generalized joint laxity is
8.16 Generalized joint laxity Simple

tests for joint hypermobility.
170
8

8.17 Marfan’s syndrome The combination of spider fingers and toes with scoliosis is
characteristic; the high-arched palate is sometimes associated.
usual and patients may develop flat feet or dislocation on clinical findings, genetic cause and inheritance
of the patella or shoulder. pattern. Of the many types of EDS so far described over
Associated abnormalities include a high arched 90 per cent show autosomal dominant inheritance.
palate, hernias, lens dislocation, retinal detachment,
aortic aneurysm and mitral or aortic incompetence.
Cardiovascular complications are particularly serious
Clinical Features
and account for most of the deaths in severe cases. Babies may show marked hypotonia and joint laxity.
Hypermobility persists and older patients are often
capable of bizarre feats of contortion. The skin is soft
X-rays and hyperextensible; it is easily damaged and vascular
Bone structure appears normal (apart from excessive fragility may give rise to ‘spontaneous’ bruising. Joint
length), but x-rays may reveal complications such as laxity, recurrent dislocations and scoliosis are com-
scoliosis, spondylolisthesis or slipped epiphysis. mon.
Diagnosis Management
‘Marfanoid’ features are quite common and it is now Complications (e.g. recurrent dislocation or scoliosis)
thought that there are several variants of the underly- may need treatment. However, if joint laxity is
ing condition. Mild cases are easily missed or mistaken marked, soft-tissue reconstruction usually fails to cure
for uncomplicated joint laxity; it is important to look the tendency to dislocation. Beware! Blood vessel
for ophthalmic and cardiovascular defects. fragility may cause severe bleeding at operation or
Homocystinuria, an inborn error of methionine afterwards. Wound healing is often poor, leaving ‘cig-
metabolism; has in the past been confused with arette paper’ scars.
Marfan’s syndrome. Joint instability may lead to osteoarthritis in later
life.
Management
Patients occasionally need treatment for progressive LARSEN’S SYNDROME
scoliosis or flat feet. The heart should be carefully
checked before operation. This is a heterogeneous condition, the more severe
(recessive) forms presenting in infancy with marked
joint laxity and dislocation of the hips, instability of
EHLERS–DANLOS SYNDROME the knees, subluxation of the radial head, equinovarus
deformities of the feet and ‘dish-face’ appearance.
This syndrome comprises a collection of 6 major but Spinal deformities are common in older children.
heterogenous subtypes with a common phenotype of Mild forms of the same condition show autosomal
unusual skin elasticity, joint hypermobility and vascular dominant inheritance.
fragility, expressions of underlying abnormalities of Operative treatment may be needed for joint insta-
elastin and collagen formation. Sub-grouping is based bility and dislocation. 171
8
(a) (b)
8.18 Ehlers–Danlos syndrome (a) Typical features of

Ehlers–Danlos syndrome: marked joint hypermobility and skin
laxity. (b) Characteristic tissue-paper scarring of the knees and
(c) the usual remarkable skin hyperextensibility.
(c)
OSTEOGENESIS IMPERFECTA (BRITTLE type I collagen can lead to weakening of these tissues
and imperfect ossification in all types of bone. Bone for-
BONES) mation is initiated in the normal way but it progresses
abnormally, the fully formed tissue consisting of a mix-
Osteogenesis imperfecta (OI) is one of the common-
ture of woven and lamellar bone, and in the worst
est of the genetic disorders of bone, with an estimated
cases almost entirely of immature woven bone. There
incidence of 1 in 20 000. Abnormal synthesis and
is thinning of the dermis, laxity of ligaments, increased
structural defects of type I collagen result in abnor-
corneal translucency and (in some cases) loss of dentin
malities of the bones, teeth, ligaments, sclerae and
leading to tooth decay.
skin. The defining clinical features are (1) osteopenia,
(2) liability to fracture, (3) laxity of ligaments, (4)
blue coloration of the sclerae and (5) dentinogenesis Clinical features
imperfecta (‘crumbling teeth’). However, there are
The clinical features vary considerably, according to
considerable variations in the severity of expression of
the severity of the condition. The most striking abnor-
these features and in the pattern of inheritance and it
mality is the propensity to fracture, generally after
is now recognized that the condition embraces a het-
minor trauma and often without much pain or
erogeneous group of collagen abnormalities resulting
swelling. In the classic case fractures are discovered
from many different genetic mutational defects
during infancy and they recur frequently throughout
(Kocher and Shapiro, 1998).
childhood. Callus formation is florid, so much so that
the lump has occasionally been mistaken for an
osteosarcoma; however, the new bone is also abnor-
Pathology mal and it remains ‘pliable’ for a long time, thus pre-
The genetic abnormality in OI expresses itself as an al- disposing to malunion and an increased risk of further
teration in the structural integrity, or a reduction in the fracture. By the age of 6 years there may be severe
total amount of type I collagen, one of the major com- deformities of the long bones, and vertebral compres-
ponents of fibrillar connective tissue in skin, ligaments sion fractures often lead to kyphoscoliosis. After
172 and bone. Even small alterations in the composition of puberty fractures occur less frequently.
8

(a) (b) (c)
8.19 Osteogenesis imperfecta
(a) This young girl had severe
deformities of all her limbs, the result
of multiple mini-fractures of the long
bones over time. This is the classic
(type III) form of OI. (b,c) X-ray
features in a slightly older patient
with the same condition. (d) The
typical deep blue sclerae in type I
disease. (e) Faulty dentine in a
patient with type IV disease.
(d) (e)
The skin is thin and somewhat loose and the joints Diagnosis
are hypermobile. Blue or grey sclerae, when they
occur, are due to uveal pigment showing through the In most cases the clinical and radiological features are
hypertranslucent cornea. The teeth may be dis- so distinctive that the diagnosis is not in doubt. How-
coloured and carious. ever, mistakes have been made and rare disorders
In milder cases fractures develop a year or two after causing multiple fractures may have to be excluded by
birth – perhaps when the child starts to walk; they are laboratory tests. In hypophosphatasia, for example,
also less frequent and deformity is not a marked fea- the serum alkaline phosphatase level is very low. In
ture. older children with atypical features it is essential to
In the most severe types of OI, fractures are present look for evidence of physical abuse.
before birth and the infant is either stillborn or lives
only for a few weeks, death being due to respiratory
Classification
failure, basilar indentation or intracranial haemor-
rhage following injury. The clinical variants of OI can be divided into sub-
groups showing well-defined differences in the pat-
tern of inheritance, age of presentation and severity of
X-rays changes in the bones and extra-skeletal tissues. This is
There is generalized osteopenia, thinning of the long helpful in assessing the prognosis and planning treat-
bones, fractures in various stages of healing, vertebral ment for any particular patient.
compression and spinal deformity. The type of abnor- The most widely used classification is that of Sillence
mality varies with the severity of the disease. The skull (1981), which defines four clinical types of OI. The
may be enlarged and shows the presence of wormian principal features can be summarized as follows:
bones – areas of vicarious ossification in the calvarium.
After puberty, fractures occur less frequently, but in OI TYPE I (MILD)
those who survive the incidence rises again after the • The commonest variety; over 50 per cent of all cases.
climacteric. It is thought that very mild (‘subclinical’) • Fractures usually appear at 1–2 years of age.
forms of OI may account for some cases of recurrent • Healing is reasonably good and deformities are not
fractures in adults. marked. 173
8 • Sclerae deep blue
• Teeth usually normal but some have dentinogenesis
imperfecta.
• Impaired hearing in adults.
• Quality of life good; normal life expectancy.
• Autosomal dominant inheritance.
OI TYPE II (LETHAL)
• 5–10 per cent of cases.
• Intra-uterine and neonatal fractures.
• Large skull and wormian bones.
• Sclerae grey.
• Rib fractures and respiratory difficulty.
• Stillborn or survive for only a few weeks.
• Most due to new dominant mutations; some auto-
somal recessive. (a) (b)
8.20 Osteogenesis imperfecta (a) Moderately severe

OI TYPE III (SEVERE DEFORMING) (type IV) disease. These deformities can be corrected by
• The ‘classic’, but not the most common, form of multiple osteotomies and ‘rodding’ (b).
OI.
• Fractures often present at birth.
encountered in types III and IV. Fractures are treated
• Large skull and wormian bones; pinched-looking
conservatively, but immobilization must be kept to a
face.
minimum. Long-bone deformities are common, due ei-
• Marked deformities and kyphoscoliosis by 6 years.
ther to malunion of complete fractures or breaking of
• Sclerae grey, becoming white.
recurrent incomplete fractures; these may require op-
• Dentinogenesis imperfecta.
erative correction, usually by 4 or 5 years of age. Mul-
• Marked joint laxity.
tiple osteotomies are performed and the bone frag-
• Respiratory problems.
ments are then realigned on a straight intramedullary
• Poor quality of life; few survive to adulthood.
rod; the same effect can be achieved by closed osteo-
• Sporadic, or autosomal recessive inheritance.
clasis. The problem of the bone outgrowing the rod has
been addressed by using telescoping nails; however,
OI TYPE IV (MODERATELY SEVERE).
these carry a fairly high complication rate.
• Uncommon; less than 5 per cent of cases.
Spinal deformity is also common and is particularly
• Frequent fractures during early childhood.
difficult to treat. Bracing is ineffectual and progressive
• Deformities common.
curves require operative instrumentation and spinal
• Sclerae pale blue or normal.
fusion.
• Dentinogenesis imperfecta.
After adolescence, fractures are much less common
• Survive to adulthood with fairly good function.
and patients may pursue a reasonably comfortable and
• Autosomal dominant inheritance.
useful life.
Management
There is no medical treatment which will counteract FIBRODYSPLASIA OSSIFICANS
the effects of this abnormality, and genetic manipula- PROGRESSIVA
tion is no more than a promise for the future.
Conservative treatment is directed at preventing This rare condition, formerly known as myositis ossifi-
fractures – if necessary by using lightweight orthoses cans progressiva, is characterized by widespread ossifi-
during physical activity – and treating fractures when cation of the connective tissue of muscle, mainly in the
they occur. However, splintage should not be over- trunk. It starts in early childhood with episodes of
done as this may contribute further to the prevailing fever and soft-tissue inflammation around the shoulders
osteopenia. General measures to prevent recurrent and trunk. As this subsides the tissues harden and
trauma, maintain movement and encourage social plaques of ossification extend throughout the affected
adaptation are very important. Children with severe areas. In the worst cases movements are restricted and
OI may be treated medically with cyclical bisphos- the patient is severely disabled. Associated anomalies are
phonates to increase bone mineral density and reduce shortening of the big toe and thumb. The condition is
the tendency to fracture. probably transmitted as an autosomal dominant but,
174 Most of the long-term orthopaedic problems are since affected individuals seldom have children, most
8.21 Fibrodysplasia 8
ossificans progressive
(a) The lumps in this boy’s
back were hard and his back
movements were limited.
(b,c) This adult shows the
extensive soft-tissue

ossification.
(a) (b) (c)
cases result from new mutations. Treatment with bis- Type 2 (NF-2) is much less common, with an inci-
phosphonates may prevent progression. dence of 1 in 50 000 births. It is associated with the
gene which codes for schwannomin, located on chro-
mosome 22. Like NF-1, it is transmitted as autosomal
NEUROFIBROMATOSIS dominant. Unlike NF-1, intracranial lesions (e.g.
acoustic neuromas and meningiomas) are usual while
Neurofibromatosis is one of the commonest single musculoskeletal manifestations are rare.
gene disorders affecting the skeleton. Two types are
recognized:
Type 1 (NF-1) – also known as von Recklinghausen’s
Clinical features of NF-1
disease – has an incidence of about 1 in 3500 live Almost all patients have the typical widespread
births. The abnormality is located in the gene which patches of skin pigmentation and multiple cutaneous
codes for neurofibromin, on chromososme 17. It is neurofibromata which usually appear before puberty.
transmitted as autosomal dominant, with almost 100 Less common is a single large plexiform neurofi-
per cent penetrance, but more than 50 per cent of broma, or an area of soft-tissue overgrowth in one of
cases are due to new mutation. The most characteris- the limbs.
tic lesions are neurofibromata (Schwann cell tumours) The orthopaedic surgeon is most likely to encounter
and patches of skin pigmentation (café au lait spots), the condition in a child or adolescent who presents with
but other features are remarkably protean and muscu- scoliosis (the most suggestive deformity is a very short,
loskeletal abnormalities are seen in almost half of sharp curve) or with localized vertebral abnormalities
those affected. such as scalloping of the posterior aspects of the verte-
(a) (b) (c) (d)
8.22 Neurofirbromatosis (a) Café-au-lait spots; (b) multiple neurofibromata and slight scoliosis; (c,d) a patient with
scoliosis and soft-tissue overgrowth (‘elephantiasis’). 175
8 bral bodies, erosion of the pedicles, intervertebral have been defined. All except Hunter’s syndrome (an
foraminal enlargement and pencilling of the ribs at af- X-linked recessive disorder) are transmitted as autoso-
fected levels. Dystrophic spinal deformities, including de- mal recessive. As a group they have certain recogniz-
formities of the cervical spine, are also seen. able features: significantly short stature with vertebral
Congenital tibial dysplasia and pseudarthrosis are deformity, coarse facies, hepatosplenomegaly and (in
rare conditions, but almost 50 per cent of patients some cases) learning difficulties. X-rays show bone
with these lesions have some evidence of neurofibro- dysplasia affecting the vertebral bodies, epiphyses and
matosis (see page 185). metaphyses; typically the bones have a spatulate
Malignant change occurs in 2–5 per cent of affected appearance.
individuals and is the most common complication in There is a superficial similarity to spondyloepiphy-
older patients. seal and spondylometaphyseal dysplasia. However,
careful observation reveals several points of difference,
and the diagnosis can be confirmed by testing for
Treatment
abnormal GAG excretion or demonstrating the
The orthopaedic conditions associated with neurofi- enzyme deficiency in blood cells or cultured fibrob-
bromatosis are dealt with on page 184 of this chapter lasts.
and in the section on scoliosis in Chapter 18. At least 10 different disorders are recognized; here
only the three most common conditions will be
described.
STORAGE DISORDERS AND

METABOLIC DEFECTS HURLER’S SYNDROME (MPS I)
Infants look normal at birth but over the next 2–3
Many single gene disorders are expressed as under- years they gradually develop a typical appearance: they
secretion of an enzyme that controls a specific stage in are undersized, with increasing kyphosis, hepato-
the metabolic chain; the undegraded substrate splenomegaly, coarse facies, protruding tongue,
accumulates and may be stored, with harmful effects, defective hearing and learning difficulty. Speech is
in various tissues or be excreted in the urine. Condi- very poor. Joints are stiff and walking is delayed.
tions involving the musculoskeletal system are the There may be corneal opacities, respiratory difficulty
mucopolysaccharidoses (MPS), Gaucher’s disease, and cardiac anomalies.
homocystinuria, alkaptonuria and congenital hyper- X-rays usually show unmistakable features such as
uricaemia. All these inborn errors of metabolism are hypoplastic epiphyses and vertebral bodies, poorly
inherited as recessive traits. modelled metaphyses, short but wide metacarpals,
underdeveloped mandible, spatulate ribs and clavicles,
flared iliac blades, shallow acetabuli and coxa valga.
Cardiac or respiratory complications usually cause
MUCOPOLYSACCHARIDOSES death in later childhood.
The polysaccharide glycosaminoglycans (GAGs) form

the side-chains of macromolecular proteoglycans, a
major component of the matrix in bone, cartilage,
HUNTER’S SYNDROME (MPS II)
intervertebral discs, synovium and other connective This is also a recessive disorder, but X-linked – so all
tissues. Defunct proteoglycans are degraded by lyso- patients are male. Clinical features are similar to those
somal enzymes. Deficiency of any of these enzymes of Hurler’s syndrome, but less severe. Suspicious fea-
causes a hold-up on the degradative pathway. Partially tures usually appear at about 3 years, cardiorespiratory
degraded GAGs accumulate in the lysosomes in the complications gradually become more severe and
liver, spleen, bones and other tissues, and spill over in death usually occurs in the middle or late teens.
the blood and urine where they can be detected by
suitable biochemical tests. Confirmation of the
enzyme lack can be obtained by tests on cultured
fibroblasts or leucocytes. MORQUIO–BRAILSFORD SYNDROME
(MPS IV)
Clinical Features
Development seems normal for the first year or two, al-
Depending on the specific enzyme deficiency and the though walking may be delayed. Thereafter the child
176 type of GAG storage, at least six clinical syndromes beings to look dwarfed, with a moderate kyphosis,
8

(b) (c)
8.23 Mucopolysaccharidoses (a) Morquio-Brailsford

syndrome – note the manubriosternal angle.
(b) Platyspondyly in a similar patient, contrasted with
(c) the sabot appearance in Hurler’s syndrome. (d) A boy
with Hunter’s syndrome; his appearance is similar to that
in Hurler’s syndrome.
(a) (d)
short neck and protuberant sternum. There is marked unilateral subluxation may need femoral or acetabular
joint laxity and progressive genu valgum. Suitable tests osteotomy. Atlantoaxial instability may threaten the
will reveal a conductive hearing loss. However, the cord and require occipitocervical fusion. All the
face is unaffected and intelligence is normal. ‘spondylodysplasias’ carry a risk of atlantoaxial sub-
X-rays of the spine show the typical ovoid, luxation during anaesthesia and intubation, and spe-
hypoplastic vertebral bodies, which end up abnor- cial precautions are needed during operation.
mally flat (platyspondyly) and peculiarly pointed ante-
riorly. Odontoid hypoplasia is usual. A marked
manubriosternal angle (almost 90°) is pathogno-
monic. By the age of 5 years the femoral head epi- GAUCHER’S DISEASE
physes are underdeveloped and flat, and the acetabula
abnormally shallow. The long bones are of normal The genetic disorder first described by Gaucher over
width but the metacarpals may be short and broad, 100 years ago is now known to be caused by lack of a
and pointed at their proximal ends. specific enzyme which is responsible for the break-
down of and excretion of cell membrane products
from defunct cells. This is a classic example of a lipid
Management storage disease for which the pathogenesis has been
There is, as yet, no specific treatment for the painstakingly worked out, leading to the development
mucopolysaccharide disorder. However, enzyme of effective treatment.
replacement and gene manipulation are possible in Each time one of the cells in the body dies, a glu-
the future. cocerebroside is released from the cell membrane;
Bone marrow transplantation has been used for the before it can be excreted, the glycoside bond holding
last 20–30 years; when successful it halts progression the glucose molecule has to be split by a specific
of CNS disease and some of the clinical features of the enzyme – glucosylceramide β-glucosidase. If this
condition but it cannot reverse neurological damage enzyme is lacking, the glucocerebroside cannot be
that has already developed and it does not prevent excreted and instead is stored in the lysosomal bodies
progression of bone and joint disease. Enzyme of macrophages of the reticuloendothelial system,
replacement therapy is successful in mild cases of MPS notably in the marrow, spleen and liver. Accumulation
I but it does not cross the blood-brain barrier. of these abnormal macrophages leads to enlargement
Hurler’s syndrome has a very poor prognosis but of the spleen and liver, and secondary changes in the
the complications (e.g. respiratory infection) may marrow and bone.
need treatment. Most patients suffer from a chronic form of the
Morquio’s syndrome presents several orthopaedic disorder, with changes predominantly in the mar-
problems. Genu valgum may need correction by row, bone and spleen, and varying degrees of pan-
femoral osteotomy, though this should be delayed till cytopenia (Type I). A rare form of the disease
growth has ceased. Coxa valga and subluxation of affecting the central nervous system (Type II)
the hips, if symmetrical, may cause little disability; appears in infancy and usually causes death within a 177
8
(b)
(a) (c) (d) (e)
8.24 Gaucher’s disease (a) A distressed young boy during an acute Gaucher crisis. The right hip is intensely painful and
abduction is restricted. The x-ray (b) shows avascular necrosis of the right femoral head. (c) X-ray of an older patient with a
sclerotic left femoral head, the result of previous ischaemic necrosis. (d) Bilateral failure of femoral tubularization (the
Erlenmeyer flask appearance). (e) Pathological fractures sometimes occur and can be treated by internal fixation. The
sclerotic patches in the interior part of the bone are typical of old medullary infarcts.
year. Type III is a subacute disorder characterized disease predisposes to bone infection and this may be
by the appearance of hepatosplenomegaly in child- a source of confusion.
hood and skeletal and neurological abnormalities
during adolescence.
Like other storage disorders, Gaucher’s disease is
Imaging
acquired by autosomal recessive transmission. The X-rays show a variable pattern of radiolucency or
genetic abnormality accountable for the lack of the patchy density, more marked in cancellous bone. The
specific enzyme glucosylceramide b-glucosidase is distal end of the femur may be expanded, producing
located on the long arm of chromosome 1 (1q21) the Erlenmeyer flask appearance. A skeletal survey
where around 80 mutations of 3 basic types have been may reveal osteonecrosis of the femoral head, femoral
identified. condyles, talus or humeral head.
A radioisotope bone scan may help to distinguish a
crisis episode from infection: the former is usually
Clinical Features ‘cold’, the latter ‘hot’.
In the commonest form of the disease (Type I), MRI is the most reliable way of defining marrow
patients present in childhood or adult life with bone involvement.
pain and, sometimes, loss of movement in one of the
larger joints. The spleen may be enlarged, or it may
Treatment
already have been removed. Older patients may
develop back pain, due to vertebral osteopenia and Bone pain may need symptomatic treatment and bis-
compression fractures. Femoral neck fractures also are phosphonates have been used for osteoporosis. For
not uncommon; however, diaphyseal fractures are the acute crisis, analgesic medication and bed rest fol-
rare. The haematocrit and platelet count are usually lowed by non-weightbearing walking with crutches is
diminished. A suggestive finding (when positive) is recommended.
elevation of the serum acid phosphatase level. Specific therapy is available (albeit costly) in the
A common complication is osteonecrosis, usually of form of the replacement enzyme, alglucerase. This has
the femoral head but sometimes in the femoral been shown to reverse the blood changes and reduce
condyles, the proximal end of the humerus or the the size of the liver and spleen. The bone complica-
bones around the ankle. The patient (usually a child tions also are diminished.
or adolescent) may present with an acute ‘bone crisis’: Osteonecrosis of the femoral head usually results in
unrelenting pain, local tenderness and restriction of progressive deformity of the hip. However most
movement accompanied by pyrexia, leucocytosis and patients manage quite well with symptomatic treat-
an elevated ESR. The clinical features resemble those ment and surgery should be deferred for as long as
178 of osteomyelitis or septic arthritis; indeed, Gaucher’s possible (Katz et al, 1996).
HOMOCYSTINURIA DOWN’S SYNDROME (TRISOMY 21) 8
This rare disorder is due to deficiency of the enzyme This condition results, in 95 per cent of cases, from
cystathionine b-synthetase and accumulation of having an extra copy of chromosome 21. It is much
homocysteine and methionine. Patients are tall and more common than any of the skeletal dysplasias, with

thin and may develop features reminiscent of Marfan’s an overall incidence of 1 per 800 live births – and 1 in
disease (page 170). However, unlike Marfan’s disease, 250 if the mother is over 37 years of age.
homocystinuria is of autosomal recessive inheritance
and is associated with marked osteoporosis and learn-
ing difficulty. Joint laxity is unusual but there may be
Clinical features
muscle weakness. Thromboembolic disease is com-
mon and may be fatal. Homocysteine levels are raised Affected infants can be recognized at birth: the head
in the blood and urine. The enzyme deficiency may be is foreshortened and the eyes slant upwards, with
detected in fibroblast cultures. Though rare, the con- prominent epicanthic folds; the nose is flattened, the
dition should be diagnosed because it can be treated: lips are parted and the tongue protrudes. There may
about half the patients are ‘cured’ by pyridoxine (vit- be abnormal palmar creases, clinodactyly and spread-
amin B6) administered from early childhood. Others ing of the first and second toes. The babies are unusu-
may be helped by a low methionine, cysteine-supple- ally floppy (hypotonic) and skeletal development is
mented diet. delayed. Children are short and, because of their char-
acteristic facial appearance, they tend to resemble each
other. They show varying degrees of learning diffi-
culty. Joint laxity may lead to sprains or subluxation
ALKAPTONURIA (e.g. of the patella). Plano-valgus feet are common
and some children develop developmental dysplasia of
Deficiency of the enzyme homogentisic acid oxidase the hip. Up to 50 per cent of these children – and par-
leads to accumulation of homogentisic acid, which is ticularly those more severely affected – will develop
deposited in connective tissue and excreted in the urine. idiopathic scoliosis. Despite these physical drawbacks,
On standing the urine turns dark (hence the name, alka- functional performance is surprisingly good and over-
ptonuria); cartilage and other connective tissues are treatment must be resisted.
stained grey – a condition referred to as ochronosis. Adults have a significant incidence of atlantoaxial
Clinical problems arise from degenerative changes in instability, though fortunately this seldom causes neu-
articular cartilage with the development of osteoarthri- rological complications. Associated anomalies, partic-
tis, and from calcification of the intervertebral discs. ularly cardiac defects, are common, and there is
diminished resistance to infection. Life expectancy is
about 35 years.
CONGENITAL HYPERURICAEMIA
The Lesch–Nyhan syndrome is a rare, X-linked reces-

sive disorder causing absence of the enzyme hypoxan-
thine-guanine phosphoribosyltransferase (HGPRT).
This enzyme controls a ‘salvage pathway’ in the com-
plex purine metabolic chain; absence of HGPRT
results in excessive uric acid formation and gout. The
young boys have learning difficulties and are prone to
self-mutilation (gnawing the ends of their fingers).
Milder cases present simply as early-onset severe gout.
Diagnosis can be confirmed by measuring HGPRT in
red cell preparations.
CHROMOSOME DISORDERS
Chromosome disorders are common but usually
result in fetal abortion. Of the non-lethal conditions, 8.25 Down’s syndrome Head shape and facial features
several produce bone or joint abnormalities. in an eleven-month old child with Down’s syndrome. 179
8 Treatment VERTEBRAL ANOMALIES
There is no specific treatment but surgery can offer
considerable cosmetic improvement; there is now an These are of three main kinds of vertebral anomaly:
increasing trend towards offering these children max-
1. Agenesis – complete absence of one or more verte-
illo-facial surgery to alter their characteristic facial
brae;
appearance. Atlantoaxial fusion is occasionally needed

2. Dysgenesis – hemivertebrae or with vertebrae fused
for patients with neurological symptoms.
together (sometimes called errors of segmenta-
Attentive care will allow many of these people to
tion);
pursue a pleasant and productive life.
3. Dysraphism – deficiencies of the neural arch.
These are considered in the sections on spinal
deformity and spina bifida.
TURNER’S SYNDROME Corresponding sacral anomalies are also encoun-
tered and associated visceral anomalies (lower intes-
Congenital female hypogonadism is a rare abnormal- tinal and urogenital defects) are common in sacral
ity caused by a defective or non-functioning X chro- dysgenesis and dysraphism.
mosome. Those affected are phenotypically female,
with a normal vagina and uterus, but the ovaries are
markedly hypoplastic or absent. Patients are short, CONGENITAL SHORT NECK (KLIPPEL–FEIL
with webbing of the neck, barrel chest and increased
carrying angle of the elbows. Cardiovascular and renal SYNDROME)
abnormalities are common. They have primary amen-
In this condition there is a failure of vertebral seg-
orrhoea, and hypogonadism leads to early-onset
mentation. The patient has an unusually short neck,
osteoporosis. Treatment consists of oestrogen
replacement from puberty onwards.
KLINEFELTER’S SYNDROME
Klinefelter’s syndrome, a form of male hypogo-

nadism, occurs in about 1 per 1000 males. Those
affected have more than one X chromosome (as well
as the usual Y chromosome). They are recognizably
male, but they have eunuchoid proportions, with
gynaecomastia and underdeveloped testicles. The
condition should be borne in mind as a cause of
osteoporosis in men. Treatment with androgens may
improve bone mass.
LOCALIZED MALFORMATIONS
Localized congenital malformations of the vertebrae
or limbs are common. The majority cause no disabil-
ity and may be discovered incidentally during investi-
gation of some other disorder. Some have a genetic
background and similar malformations are seen in
association with generalized skeletal dysplasia. Most
are sporadic and probably non-genetic – i.e. caused by
injury to the developing embryo, especially during the
first 3 months of pregnancy. In some cases there is a
known teratogenic agent; for example, maternal infec-
tion or drug administration. Usually, however, the 8.26 Klippel–Feil syndrome The short neck and
180 exact cause is unknown. vertebral anomalies in a typical patient.
and neck movements are restricted or absent. Promi- represents a failure of scapular descent from the cervi- 8
nence of the trapezius muscles gives the appearance of cal spine. The high scapula may still be attached to the
webbing at the base of the neck. The posterior hair- spine by a tough fibrous band or a cartilaginous bar
line is much lower than normal. Associated anomalies (the omovertebral bar). Associated vertebral or rib
are common and include hemivertebra, posterior arch anomalies are quite common.
defects, cervical meningomyelocele, thoracic defects, Treatment is required only if shoulder movements

scapular elevation and visceral abnormalities involving are severely limited or if the deformity is particularly
the renal and cardio-respiratory systems. Occasionally, unsightly. Operation is best performed before the age
a familial pattern of inheritance is noted suggesting a of 6 years. The vertebroscapular muscles are released
genetic aetiology. from the spine, the supraspinous part of the scapula is
X-rays may show fusion of the lower cervical verte- excised together with the omovertebral bar and the
brae and various combinations of the associated disor- scapula is repositioned by tightening the lower mus-
ders, together with scoliosis or kyphosis. cles. Great care is needed as there is a risk of injury to
The natural history of the condition often depends the accessory nerve or the brachial plexus.
on the severity of the visceral anomalies.
Orthopaedic treatment is usually unnecessary. How-
ever cervical instability, with the risk of neurological
injury, may develop in the relatively hypermobile seg-
THORACOSPINAL ANOMALIES
ment adjacent to the fusion mass and surgical fusion Segmentation defects in the thoracic region usually
with or without cord decompression may be war- involve the ribs as well; for example, hemivertebrae
ranted. Contact sports should be avoided. may be associated with fusion of adjacent ribs or other
types of dysplasia. Some of these disorders are of auto-
somal dominant inheritance.
ELEVATION OF THE SCAPULA Clinically, patients present in childhood with scolio-
sis or kyphoscoliosis, sometimes leading to paraplegia.
(SPRENGEL’S DEFORMITY) X-rays may show various combinations of thoracic
Mild degrees of congenital elevation of the scapula are vertebral fusion or dysgenesis and rib anomalies,
common. In the full-blown Sprengel deformity the together with scoliosis and marked distortion of the
child has obvious asymmetry of the shoulders, with thorax.
elevation and underdevelopment of the affected side. Operative treatment may be needed for threatened
The scapula is abnormally small and too high. Some- cord compression.
times the clavicle is affected as well. Shoulder move-
ments may be restricted and on abduction or
elevation the scapula moves very little or not at all. SACRAL AGENESIS
Occasionally both sides are involved.
Sprengel’s deformity may be associated with other This term describes a group of conditions in which
defects of the cervical spine (e.g. Klippel–Feil syn- part or all of the distal spine is missing. Variable motor
drome), and high thoracic kyphosis or scoliosis is deficiencies are noted below the lowest level of nor-
quite common. mal spine but sensation is often preserved more dis-
This condition, which usually occurs sporadically, tally. Other deformities of the lower limb may be
(a) (b) (c)
8.27 Sacral agenesis This girl shows (a) the characteristic sitting posture and (b) the spinal hump. (c) The sacrum is
absent and the hips are dislocated. 181
8 present and, as with congenital scoliosis, there may be Some of the important and less rare disorders are
associated cardiac, visceral and renal abnormalities. described below and further details appear in the sec-
Some cases of sacral agenesis appear to be inherited in tion on Regional Orthopaedics.
either an autosomal or sex-linked dominant fashion.
UPPER LIMB
LIMB ANOMALIES When dealing with upper limb deficiencies it is impor-

tant to remember that hand function may be very sat-
Localized malformations of the limbs include extra isfactory (albeit not ideal) even if the appearance is
bones, absent bones, hypoplastic bones and fusions. not. Before any surgical treatment is considered, it is
Complete absence of a limb is called amelia, almost important to decide what the aims of treatment are,
complete absence (a mere stub remaining) phocomelia what side effects there might be and how to achieve
and partial absence ectromelia; defects may be trans- the most acceptable balance between function,
verse or axial. In the hands and feet brachydactyly, syn- appearance and pain. A hand that functions better but
dactyly, polydactyly and symphalangism are among the hurts more may not be more useful to a particular
many possibilities. patient. Hand dominance and whether or not the
The embryonal limb buds appear at about the 26th abnormality is bilateral are also important factors to
day of gestation; by the 30th day the upper limb has note when planning treatment.
started differentiating into its three segments (upper
arm, forearm and hand) and in the lower limb the
same process occurs shortly afterwards. By the end of
Radial deficiency
the 6th week the embryo has acquired a recognizable Absence or hypoplasia of the radius may occur alone
human form. The upper limb is fully formed by 12 or in association with visceral anomalies or (more
weeks and the lower limb by 14 weeks. During this rarely) certain blood dyscrasias. Two acronyms may
period the muscles and nerves also develop and by the help to keep this in mind. ‘VACTERLS’ refers to the
20th week joint movement is possible. systems involved and the defects identified: vertebral,
Most of the malformations involving limb reduc- anal, cardiac, tracheal, esophageal, renal, limb and sin-
tions are due to embryonal insults between the 4th gle umbilical artery. ‘TAR’ prompts one to remember
and 6th weeks of gestation. Some are genetically thrombocytopaenia with absent radius syndrome.
determined and these usually have an autosomal dom- Fanconi’s anaemia and the Holt–Oram syndrome are
inant pattern of inheritance. also sometimes associated with radial deficiency.
The forearm is short and bowed; the hand is under-
developed and markedly deviated towards the radial
Classification side (radial club hand) and the thumb may be miss-
Various classifications of limb deficiencies have been ing. The elbow too is often abnormal. In about half
proposed; none is completely satisfactory. Some veer the cases the condition is bilateral.
towards the purely descriptive; others go into almost The clinical deformity may look bizarre but chil-
obsessive detail based on topographical and morpho- dren often acquire excellent function. If this seems
logical features. Their usefulness lies in the elabora- unlikely, operative reconstruction may be advisable.
tion of an agreed terminology which will aid This could involve pollicisation of a digit and other
communication and permit sensible auditing of the complex reconstructive procedures. In the young
results of various forms of treatment. child simple stretching and splinting may help to
8.28 Radial dysplasia

(a) Bilateral. (b) X-ray
showing that the entire radius
is absent.
182 (a) (b)

improve and/or maintain hand and wrist position external compression from the subclavian artery or a 8
until further options have to be considered. failure of coalescence of the two intramembranous cen-
tres of ossification have been proposed.
Over time, the mobile pseudarthrosis may become
Ulnar deficiency
painful, particularly with overhead activities and on
Hypoplasia of the distal end of the ulna is usually seen direct pressure, but shoulder dysfunction itself is

as part of a generalized dysplasia, but occasionally it oc- unusual. Furthermore, the cosmetic appearance may
curs alone. The radius is bowed (as if growth is tethered be unacceptable.
on the ulnar side) and the radial head may dislocate; the Operative treatment is usually successful (in con-
wrist is deviated medially. Only if function is severely trast to the other ‘congenital’ pseudarthrosis that
disturbed should wrist stabilization be advised. affects the tibia) and involves excision of the
Congenital absence of the ulna is extremely rare. pseudarthrosis and internal fixation with or without
The forearm deformity is not as marked as in radial the use of bone graft.
deficiency but overall function is severely restricted.
Operative reconstruction may provide some improve- Digital anomalies
ment.
A wide variety of anomalies can occur ranging from
simple soft-tissue ‘extra digits’ (which are easy to
Radio-ulnar Synostosis excise) to complex syndactylies that restrict hand
This is often associated with a posterolateral disloca- function. They may occur alone or in conjunction
tion of the radial head. Clinically there is complete with more generalized skeletal dysplasias.
loss of pronation and supination, although some chil-
dren appear to maintain some forearm rotation due to Transverse deficiency of the arm
laxity of the wrist and elbow.
Transverse deficiency of the distal part of the arm will
Forearm rotation cannot be regained with surgery
leave a simple stump below a normal elbow. This can
but improvement in the resting position of the fore-
be managed by fitting a prosthesis with a mechanical
arm (and hence of the hand) can be achieved.
facility for grasp.
Cleft hand
A central defect of the hand is more common than an LOWER LIMB
ulnar post-axial deficiency. If associated with cleft Femoral deficiency (congenital short
foot, the ectrodactyly may be an autosomal dominant
condition but with variable penetrance affecting boys
femur)
more frequently than girls. Complex reconstructions In its most benign form, femoral dysplasia consists
can be considered but the balance between appear- merely of shortening of the bone with a normal hip and
ance and function must be remembered. knee. This can be dealt with by limb lengthening pro-
cedures or, if shortening is very marked, by adding a
distal orthosis. If this is associated with coxa vara a
Pseudarthrosis of the clavicle proximal osteotomy may be needed.
This almost always affects the right side (except in cases Dysplasia of the distal third – sometimes with synos-
of dextrocardia!) and the child presents with a lump tosis of the knee – is uncommon. Since the hip per-
over the mid-clavicular region. Often there is obvious mits normal weightbearing, this condition also can be
mobility at the pseudarthrosis site. Whilst occasional managed by limb lengthening operations.
familial autosomal dominant cases have been described, Proximal femoral dysplasia is more common – and
the true aetiology is unknown; other theories such as usually much more serious because it presents a two-
fold problem: shortening of the limb and defective
weightbearing at the hip.
Various grades of proximal femoral dysplasia are
encountered. The most widely used classification is
that of Aitkin, as illustrated in Figure 8.31.
Coxa vara with moderate shortening of the shaft
can be dealt with by corrective osteotomy and limb
lengthening. Severe degrees of coxa vara, sometimes
associated with pseudoarthrosis of the femoral neck,
8.29 Pseudoarthrosis of the clavicle It is always the may result in marked shortening of the femur.
right side which is affected. In the worst cases most of the femoral shaft is miss- 183
8
(a) (b) (c) (d)
8.30 Failure of formation and digital anomalies (a) Transverse failure of the hand; (b) transverse failure of the fingers;
(c) central failure of formation; (d) extra digit.
(a) (b) (c) (d)
8.31 Proximal femoral dysplasia The most widely used classification of proximal femoral focal deficiency is that of
Aitken. Type A: the child is born with a ‘gap’ between the proximal part of the femur and the diaphysis but this usually
ossifies by the end of growth. Type B: the femoral head is present (though hypoplastic) but there is a ‘gap’ which fails to
ossify. Type C: the femoral head and neck are absent and the acetebulum is under-developed. Type D: the acetabulum and
proximal femur are absent. Congenital coxa vara is not included in this classification although it may also be a variant of the
same disorder (see Chapter 19).
ing, the knee is situated at thigh level and the foot

hangs where the knee is normally expected to be. If
the deformity is bilateral and symmetrical, walking is
possible and some individuals acquire remarkable
agility; however, they may still seek treatment to over-
come the severe cosmetic problem. Unilateral defor-
mities are not only unsightly but also very disabling.
Effective limb lengthening is out of the question, and
fitting a prosthesis to a short limb with flexion defor-
mities of the ‘hip’ and knee and a foot jutting for-
wards where the knee-hinge of the prosthesis will lie
is a daunting prospect. In the past there was some
enthusiasm for the Van Nes operation: fusion of the
knee and 180 degree rotational osteotomy of the leg
bones to get the foot facing back-to-front and the
ankle substituting for the knee, followed by fitting an
(a) (b)
‘above-knee’ prosthesis. However, the trick is easier,
and looks better, in drawings than in real life and the 8.32 Coxa vara and shortening (a) This young boy has
procedure is seldom done nowadays. One alternative marked shortening of the right femur. (b) The x-ray shows
184 is to fuse the knee in a functional position, amputate severe coxa vara deformity.
8

(a) (b) (c)
8.33 Proximal femoral dysplasia (a) This man was born

with transverse deficiency of the right arm and bilateral
proximal femoral focal deficiency. Though unhappy with
his appearance, because the lower limb defects were
symmetrical he was able to get about remarkably well. (a) (b)
(b) By contrast, this young man with a similar but unilateral 8.34 Congenital pseudarthrosis The tibia is the most
dysplasia, was severely disabled. common site (a); in this case bone-grafting was successful
(c) X-ray showing the proximal femoral deficiency. (b).
the foot and fit a suitable prosthesis. The earlier this is

done the better. fibrous band in its place. Excision of this remnant may
permit correction of the valgus deformity.
In severe cases, management is dictated by the
Tibial deficiency
quality of the foot and by the percentage growth inhi-
Tibial dysplasia is very rare: several forms exist and the bition. This can be calculated by a variety of methods
condition may be associated with other limb anomalies. and allows good prediction of final limb length dis-
Prognosis, and hence treatment, depend on the qual- crepancy at skeletal maturity. Once this is known,
ity of the knee joint: if there is no ability for knee ex- treatment can be planned. Options range from partial
tension, a proximal amputation must be considered. If amputation and the use of a prosthetic limb to epi-
the ankle cannot be reconstructed a distal amputation physeodesis of the longer limb and one or more limb
may be required and a fibula transfer may extend the lengthening procedures involving distraction osteoge-
useful portion of the tibia. It is quite possible to con- nesis techniques and ring external fixators (the efficacy
struct a functioning one-bone leg by transposing the and longevity of new internal intramedullary length-
fibula and fusing it to the centre of the femoral articu- ening devices are as yet unproven).
lar surface; once fusion is achieved, a Syme’s amputa- Reconstructive techniques such as these are becom-
tion can be performed. In other cases reconstruction ing more successful but they rely on a high degree of
using limb lengthening techniques may be applicable. compliance from the child and their family over a long
This should be done as soon as the fibula has devel- time-span; the worse the initial problem the less likely
oped sufficiently to permit fusion at the knee. If the the child is to have a ‘normal’ limb.
procedure fails, or if the associated abnormalities turn In contrast, modern technology is allowing signifi-
out to be more severe than expected, proximal ampu- cant advances to be made in the field of amputation
tation can be undertaken at a later stage. prosthetics and it is possible that devices such as the
ITAP (intra-osseous transcutaneous amputation pros-
thesis) may turn out to be successful over the long
Fibular deficiency
term in allowing a cosmetically acceptable as well as
This is the most common long-bone deficiency. Mild more functional prosthetic limb to be worn. This may
fibular dysplasia causes little shortening or deformity; be more acceptable to the patient than the outcome
however, complete absence of the fibula leads to con- from limb lengthening treatment.
siderable shortening of the leg, bowing of the tibia
and valgus deformity of the unsupported ankle. There
may also be absence of the fourth and fifth rays of the
Congenital pseudarthrosis of the tibia
foot and underdevelopment of the entire limb. Some- This rare condition is usually diagnosed in early in-
times, if only the distal fibula is absent, there is a fancy. The child may be born with a fractured tibia, or 185
8 the bone may be attenuated and then fracture some Anterolateral tibial bowing with failure of normal
months later. In either case, the fracture fails to unite, tubularization may be the forerunner of localized os-
or heals very poorly only to fracture again shortly af- teolysis and eventual fracture with persistent non-union
terwards. By the age of two years the leg is noticeably and pseudarthrosis of the tibia. Corrective osteotomy
short and bowed anterolaterally. By then it has become should be avoided because of the high risk of non-
obvious that this is an intractable condition which will union. While the bone is intact, treatment consists of
not yield to ordinary forms of fracture treatment. bracing until the bone matures. If a fracture occurs,
X-ray shows a gap, or marked thinning, of the treatment is the same as for congenital pseudarthrosis.
tibial shaft. Sometimes the fibula also is affected.
Biopsy of the abnormal segment occasionally shows
histological features of neurofibromatosis, and other
stigmata of this condition are present in about half of REFERENCES AND FURTHER READING
those affected. They should always be looked for.
Treatment is likely to be prolonged and fraught Ainsworth SR, Aulicino PL. A survey of patients with
with difficulty. Simple immobilization will certainly Ehlers–Danlos syndrome Clin Orthop 1993; 286: 250–6.
fail, and internal fixation with bone grafting succeeds Crawford AH, Schorry EK. Neurofibromatosis in children:
only very occasionally. Better results have been the role of the orthopaedist. J Am Acad Orthop Surg
achieved by excising the affected segment of bone, 1999; 7: 217–30.
correcting the deformity and closing the gap gradually Evans CH, Robbins PD. Possible orthopaedic applications
by bone transport in a circular external fixator (the of gene therapy. J Bone Joint Surg 1995; 77A: 1103–14.
Ilizarov technique). Success has also been claimed for Jaffurs D, Evans CH. The human genome project: Implica-
excision of the abnormal segment and replacement by tions for the treatment of musculoskeletal disease. J Am
a vascularized fibular graft (Weiland et al, 1990). Acad Orthop Surg 1998; 6: 1–14.
The limb can be ‘stabilized’ and held in reasonable Katz K, Horev, G Grunebaum M et al. The natural history
alignment with a clamshell orthosis and an intra- of osteonecrosis of the femoral head in children and ado-
medullary device until the child is old enough to lescents who have Gaucher’s disease. J Bone Joint Surg
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Kocher MS, Shapiro F. Osteogenesis imperfecta. J Am
Acad Orthop Surg 1998; 6: 225–36.
Congenital tibial bowing Pastores GM, Sibille AR, Grabowski GA. Enzyme therapy
Congenital tibial bowing comprises a spectrum of disin Gaucher’s disease type 1: dosage efficacy and adverse
orders with significant differences in both aetiology effect in 33 patients treated for 6 to 24 months. Blood
and prognosis for the different types (Crawford and 1993; 82: 408–16.
Schorry, 1999). Pastores GM, Hermann G, Norton KI et al. Regression of
Posteromedial tibial bowing is a relatively benign skeletal changes in Type I Gaucher disease with enzyme
condition which usually resolves spontaneously as the replacement therapy. Skeletal Radiology 1996; 25: 485–8.
child grows. However, the leg may end up shorter Shiang R, Thompson LM, Zhu Y-Z et al. Mutations in the
than normal, requiring epiphysiodesis on the opposite transmembrane domain of FGFR3 cause the most com-
side or limb lengthening to counteract the limb mon genetic form of dwarfism, achondroplasia. Cell
length inequality. 1994; 78: 335–42.
Anteromedial bowing is almost always associated Sillence D. Osteogenesis imperfecta: an expanding panorama
with fibular deficiency and congenital defects of the of variants. Clin Orthop Relat Res 1981; 159: 11–25.
foot, or some type of femoral dysplasia. Treatment Solomon L. Hereditary multiple exostosis. J Bone Joint Surg
depends on the presence or absence (and severity) of 1963; 45B: 292–304.
the associated disorders and varies from reconstructive Weiland AJ, Weiss A-PC, Moore JR, Tolo VT. Vascularized
procedures of the ankle to – in the very worst cases – fibular grafts in the treatment of congenital pseudarthro-
amputation. sis of the tiba. J Bone Joint Surg 1990; 72A: 654–662.
186
Tumours
9
Will Aston, Timothy Briggs, Louis Solomon
Tumours, tumour-like lesions and cysts are considered

together, partly because their clinical presentation and
CLASSIFICATION
management are similar and partly because the defin-
itive classification of bone tumours is still evolving and Most classifications of bone tumours are based on the
some disorders may yet move from one category to recognition of the dominant tissue in the various
another. Benign lesions are quite common, primary lesions (Table 9.1). Knowing the cell line from which
malignant ones rare; yet so often do they mimic each the tumour has sprung may help with both diagnosis
other, and so critical are the decisions on treatment, and planning of treatment. There are, however, pit-
that a working knowledge of all the important condi- falls in this approach:
tions is necessary.
Table 9.1 A classification of bone tumours. Modified after Revised WHO Classification –
Schajowicz (1994)
Predominant tissue Benign Malignant

Bone forming Osteoma Osteosarcoma:
Osteoid osteoma central
Osteoblastoma peripheral
parosteal
Cartilage forming Chondroma Chondrosarcoma:
Osteochondroma central
Chondroblastoma peripheral
?Chondromyxoid fibroma juxtacortical
clear-cell
mesenchymal
Fibrous tissue Fibroma Fibrosarcoma
Fibromatosis
Mixed ?Chondromyxoid fibroma
Giant-cell tumours Benign osteoclastoma Malignant osteoclastoma
Marrow tumours Ewing’s tumour
Myeloma
Vascular tissue Haemangioma Angiosarcoma
Haemangiopericytoma Malignant haemangiopericytoma
Haemangioendothelioma
Other connective tissue Fibroma Fibrosarcoma
Fibrous histiocytoma Malignant fibrous histiocytoma
Lipoma Liposarcoma
Other tumours Neurofibroma Adamantinoma
Neurilemmoma Chordoma
9 • the most pervasive tissue is not necessarily the tissue Pathological fracture may be the first (and only)
of origin clinical signal. Suspicion is aroused if the injury was
• there is not necessarily any connection between slight; in elderly people, whose bones usually fracture
conditions in one category at the cortico-cancellous junctions, any break in the
• there is often no relationship between benign and mid-shaft should be regarded as pathological until
malignant lesions with similar tissue elements (e.g. proved otherwise.
osteoma and osteosarcoma)

• the commonest malignant lesions in bone –
metastatic tumours – are not, strictly speaking, EXAMINATION
‘bone’ tumours, i.e. not of mesenchymal origin.
If there is a lump, where does it arise? Is it discrete or
ill-defined? Is it soft or hard, or pulsatile? And is it ten-
der? Swelling is sometimes diffuse, and the overlying
CLINICAL PRESENTATION skin warm and inflamed; it can be difficult to distin-
guish a tumour from infection or a haematoma.
If the tumour is near a joint there may be an effu-
HISTORY sion and/or limitation of movement. Spinal lesions,
The history is often prolonged, and this unfortunately whether benign or malignant, often cause muscle
results in a delay in obtaining treatment. Patients may spasm and back stiffness, or a painful scoliosis.
be completely asymptomatic until the abnormality is The examination will focus on the symptomatic
discovered on x-ray. This is more likely with benign part, but it should include the area of lymphatic
lesions; and, since some of these (e.g. non-ossifying drainage and, often, the pelvis, abdomen, chest and
fibroma) are common in children but rare after the age spine.
of 30, they must be capable of spontaneous resolution.
Malignant tumours, too, may remain silent if they are
slow-growing and situated where there is room for IMAGING
inconspicuous expansion (e.g. the cavity of the pelvis).
Age may be a useful clue. Many benign lesions pres- X-RAYS
ent during childhood and adolescence – but so do Plain x-rays are still the most useful of all imaging
some primary malignant tumours, notably Ewing’s techniques. There may be an obvious abnormality in
tumour and osteosarcoma. Chondrosarcoma and the bone – cortical thickening, a discrete lump, a
fibrosarcoma typically occur in older people (fourth or ‘cyst’ or ill-defined destruction. Where is the lesion: in
sixth decades); and myeloma, the commonest of all the metaphysis or the diaphysis? Is it solitary or are
primary malignant bone tumours, is seldom seen there multiple lesions? Are the margins well-defined
before the sixth decade. In patients over 70 years of or ill-defined?
age, metastatic bone lesions are more common than all Remember that ‘cystic’ lesions are not necessarily
primary tumours together. hollow cavities: any radiolucent material (e.g. a
Pain is a common complaint and gives little indica- fibroma or a chondroma) may look like a cyst. If the
tion of the nature of the lesion; however, progressive boundary of the ‘cyst’ is sharply defined it is probably
and unremitting pain is a sinister symptom. It may be
caused by rapid expansion with stretching of sur-
rounding tissues, central haemorrhage or degeneration QUESTIONS TO ASK WHEN STUDYING AN
in the tumour, or an incipient pathological fracture. X-RAY
However, even a tiny lesion may be very painful if it is
encapsulated in dense bone (e.g. an osteoid osteoma).
Is the lesion solitary or are there multiple lesions?
Swelling, or the appearance of a lump, may be
alarming. Often, though, patients seek advice only What type of bone is involved?
when a mass becomes painful or continues to grow.
Where is the lesion in the bone?
A history of trauma is offered so frequently that it
cannot be dismissed as having no significance. Yet, Are the margins of the lesion well- or ill-defined?
whether the injury initiates a pathological change or
Are there flecks of calcification in the lesion?
merely draws attention to what is already there
remains unanswered. Is the cortex eroded or destroyed?
Neurological symptoms (paraesthesiae or numbness)
Is there any periosteal new-bone formation?
may be caused by pressure upon or stretching of a
peripheral nerve. Progressive dysfunction is more omi- Does the tumour extend into the soft tissues?
188 nous and suggests invasion by an aggressive tumour.
benign; if it is hazy and diffuse it suggests an invasive BIOPSY 9
tumour. Stippled calcification inside a cystic area is
characteristic of cartilage tumours. Needle biopsy Needle biopsy should be performed
Look carefully at the bone surfaces: periosteal new- either by the surgeon planning definitive treatment or
bone formation and extension of the tumour into the by an experienced radiologist. Often it is carried out
soft tissues are suggestive of malignant change. with the help of ultrasound or CT guidance (Stoker et
Tumours
Look also at the soft tissues: Are the muscle planes al., 1991; Saifuddin et al., 2000). A large bore biopsy
distorted by swelling? Is there calcification? needle, such as a Jamshidi or a Trucut needle, is used.
For all its informative detail, the x-ray alone can sel- It is important to ensure that a representative sample
dom be relied on for a definitive diagnosis. With some of the tumour is taken and that it is adequate to make
notable exceptions, in which the appearances are a histological diagnosis; a frozen section can be used
pathognomonic (osteochondroma, non-ossifying in order to confirm this. If infection is suspected then
fibroma, osteoid osteoma), further investigations will a sample should be sent for microbiology. It is also
be needed. If other forms of imaging are planned (bone essential that the biopsy is carried out in the line of
scans, CT or MRI), they should be done before under- any further surgical incision so that the tract can be
taking a biopsy, which itself may distort the appearances. excised at the time of definitive surgery.
RADIONUCLIDE SCANNING Open biopsy This is a more reliable way of obtaining

Scanning with 99mTc-methyl diphosphonate (99mTc- a representative sample, however it is associated with
MDP) shows non-specific reactive changes in bone; this significant morbidity (Mankin et al., 1982). It is often
can be helpful in revealing the site of a small tumour performed if a needle biopsy would place the neu-
(e.g. an osteoid osteoma) that does not show up clearly rovascular structures at risk or if a diagnosis has not
on x-ray. Skeletal scintigraphy is also useful for detect- been made after needle biopsy. The site is selected so
ing skip lesions or ‘silent’ secondary deposits. that it can be included in any subsequent operation.
As little as possible of the tumour is exposed and a
COMPUTED TOMOGRAPHY block of tissue is removed – ideally in the boundary
CT extends the range of x-ray diagnosis; it shows zone, so as to include normal tissue, pseudocapsule
more accurately both intraosseous and extraosseous and abnormal tissue. If bone is removed the raw area
extension of the tumour and the relationship to sur- is covered with bone wax or methylmethacrylate
rounding structures. It may also reveal suspected cement. If a tourniquet is used, it should be released
lesions in inaccessible sites, like the spine or pelvis; and and full haemostasis achieved before closing the
it is a reliable method of detecting pulmonary metas- wound. Drains should be avoided, so as to minimize
tases. the risk of tumour contamination.
An experienced histopathologist should be on hand
MAGNETIC RESONANCE IMAGING and the specimens should be delivered fresh, unfixed
MRI provides further information. Its greatest value is and uncrushed.
in the assessment of tumour spread: (a) within the For tumours that are almost certainly benign, an
bone, (b) into a nearby joint and (c) into the soft tis- excisional biopsy is permissible (the entire lesion is
sues. Blood vessels and the relationship of the tumour removed); with cysts that need operations, represen-
to the perivascular space are well defined. MRI is also tative tissue can be obtained by careful curettage. In
useful in assessing soft-tissue tumours and cartilagi- either case, histological confirmation of the diagnosis
nous lesions. is essential.
Biopsy should never be regarded as a ‘minor’ pro-
cedure. Complications include haemorrhage, wound
LABORATORY INVESTIGATIONS breakdown, infection and pathological fracture
(Mankin at al., 1982, 1996; Springfield and Rosen-
Blood tests are often necessary to exclude other con- berg, 1996). The person doing the biopsy should
ditions, e.g. infection or metabolic bone disorders, or have a clear idea of what may be done next and where
a ‘brown tumour’ in hyperparathyroidism. Anaemia, operative incisions or skin flaps will be placed. Errors
increased ESR and elevated serum alkaline phosphatase and complications are far less likely if the procedure is
levels are non-specific findings, but if other causes are performed in a specializing centre.
excluded they may help in differentiating between A last word of warning: When dealing with tumours
benign and malignant bone lesions. Serum protein that could be malignant, there is a strong temptation to
electrophoresis may reveal an abnormal globulin frac- perform the biopsy as soon as possible; as this may alter
tion and the urine may contain Bence Jones protein in the CT and MRI appearances, it is important to delay
patients with myeloma. A raised serum acid phos- the procedure until all the imaging studies have been
phatase suggests prostatic carcinoma. completed. 189
9
(a) (b) (c) (d) (e)
9.1 Tumours – differential diagnosis (a) This huge swelling was simply a clotted haematoma. (b) Bone infection with
pathological fracture. (c) Florid callus in an un-united fracture. (d) Large erosion in the calcaneum by a gouty tophus.
(e) Bone infarcts.
DIFFERENTIAL DIAGNOSIS The best known example is the tibial apophyseal stress
lesion of Osgood–Schlatter’s disease (see page 565),
A number of conditions may mimic a tumour, either but lesions at less familiar sites (the iliac crest, the ischial
clinically or radiologically, and the histopathology tuberosity, the lesser trochanter of the femur, the ham-
may be difficult to interpret. It is important not to be string insertions, the attachments of adductor magnus
misled by the common dissemblers. and longus and the distal humeral apophyses) may es-
cape immediate recognition.
Soft-tissue haematoma A large, clotted sub-periosteal
or soft-tissue haematoma may present as a painful Bone infection Osteomyelitis typically causes pain and
lump in the arm or lower limb. Sometimes the x-ray swelling near one of the larger joints; as with primary
shows an irregular surface on the underlying bone. bone tumours, the patients are usually children or
Important clues are the history and the rapid onset of young adults. X-rays may show an area of destruction
symptoms. in the metaphysis, with periosteal new bone. Systemic
features, especially if the patient has been treated with
Myositis ossificans Although rare, this may be a source
antibiotics, may be mild. If the area is explored, tissue
of confusion. Following an injury the patient develops
should be submitted for both bacteriological and his-
a tender swelling in the vicinity of a joint; the x-ray
tological examination.
shows fluffy density in the soft tissue adjacent to bone.
Unlike a malignant tumour, however, the condition Gout Occasionally a large gouty tophus causes a
soon becomes less painful and the new bone better painful swelling at one of the bone ends, and x-ray
defined and well demarcated. shows a large, poorly defined excavation. If it is kept
in mind the diagnosis will be easily confirmed – if nec-
Stress fracture Some of the worst mistakes have been
essary by obtaining a biopsy from the lump.
made in misdiagnosing a stress fracture. The patient is
often a young adult with localized pain near a large Other bone lesions Non-neoplastic bone lesions such
joint; x-rays show a dubious area of cortical ‘destruc- as fibrous cortical defects, medullary infarcts and
tion’ and overlying periosteal new bone; if a biopsy is ‘bone islands’ are occasionally mistaken for tumours.
performed the healing callus may show histological
features resembling those of osteosarcoma. If the pit-
fall is recognized, and there is adequate consultation
between surgeon, radiologist and pathologist, a seri- STAGING OF BONE TUMOURS
ous error can be prevented.
Tendon avulsion injuries Children and adolescents – In treating tumours we strive to reconcile two con-
especially those engaged in vigorous sports – are prone flicting principles: the lesion must be removed widely
to avulsion injuries at sites of tendon insertion, partic- enough to ensure that it does not recur, but damage
190 ularly around the hip and knee (Donnelly et al., 1999). must be kept to a minimum. The balance between
Table 9.2 Staging of benign bone tumours as adjacent ‘contaminated’ tissue are best shown by CT 9
described by Enneking and MRI; skip lesions can be detected by scintigraphy.
Latent Well-defined margin. Grows slowly and then
stops SURGICAL STAGE
Remains static/heals spontaneously
‘Staging’ the tumour is an important step towards
E.g. Osteoid osteoma
selecting the operation best suited to that particular
Tumours
patient, and carrying a low risk of recurrence. Locally
Active Progressive growth limited by natural barriers
recurrent sarcomas tend to be more aggressive, more
Not self-limiting. Tendency to recur often extracompartmental and more likely to metasta-
E.g. Aneurysmal bone cyst size than the original tumour.
Aggressive Growth not limited by natural barriers (e.g. giant Bone sarcomas are broadly divided as follows:
cell tumour)
• Stage I All low-grade sarcomas.
• Stage II Histologically high-grade lesions.
• Stage III Sarcomas which have metastasized.
these objectives depends on knowing (a) how the
tumour usually behaves (i.e. how aggressive it is), and
(b) how far it has spread. The answers to these two Table 9.3 Surgical stages as described by Enneking
questions are embodied in the staging system devel-
Stage Grade Site Metastases
oped by Enneking (1986).
IA Low Intracompartmental No
IB Low Extracompartmental No
AGGRESSIVENESS
IIA High Intracompartmental No
Tumours are graded not only on their cytological
IIB High Extracompartmental No
characteristics but also on their clinical behaviour, i.e.
the likelihood of recurrence and spread after surgical IIIA Low Intra- or Yes
removal. extracompartmental
Benign lesions, by definition, occupy the lowest IIIA High Intra- or Yes
grade, though even in this group there are important extracompartmental
differences in behaviour calling for further subdivision
into latent, active and aggressive lesions (Table 9.2).
The least aggressive tumours may disappear sponta- Following Enneking’s original classification, each
neously (e.g. non-osteogenic fibroma); the most category is further subdivided into Type A (intracom-
aggressive are difficult to distinguish from a low-grade partmental) and Type B (extracompartmental) (Fig.
sarcoma and sometimes undergo malignant change 9.3). Thus, a localized chondrosarcoma arising in a
(e.g. aggressive osteoblastoma). Most are amenable to cartilage-capped exostosis would be designated IA,
local (marginal) excision with little risk of recurrence. suitable for wide excision without exposing the
Malignant tumours are divided into ‘low-grade’ tumour. An osteosarcoma confined to bone would be
and ‘high-grade’: the former are only moderately IIA – operable by wide excision or amputation with a
aggressive and take a long time to metastasize (e.g. low risk of local recurrence; if it has spread into the
secondary chondrosarcoma or parosteal osteosar- soft tissues it would be IIB – less suitable for wide
coma), while the latter are usually very aggressive and excision and preferably treated by radical resection or
metastasize early (e.g. osteosarcoma or fibrosarcoma). disarticulation through the proximal joint. If there are
pulmonary metastases it would be classified as stage
III.
SPREAD
STAGING OF SOFT-TISSUE TUMOURS
Assuming that there are no metastases, the local Soft-tissue tumours are staged using the American
extent of the tumour is the most important factor in Joint Committee for Cancer Staging System, accord-
deciding how much tissue has to be removed. Lesions ing to their histological grade (G), size (T), lymph
that are confined to an enclosed tissue space (e.g. a node involvement (N) and whether they have metas-
bone, a joint cavity or a muscle group within its fascial tasized (M) (Russell et al., 1977). The main differ-
envelope) are called ‘intracompartmental’. Those that ences between this and the Enneking system are the
extend into interfascial or extrafascial planes with no increased number of histological grades (from low
natural barrier to proximal or distal spread (e.g. and high to 1, 2 and 3) and use of the size of the
perivascular sheaths, pelvis, axilla) are designated tumour (less than or greater than 5 cm), rather than
‘extracompartmental’. The extent of the tumour and whether it is intra- or extracompartmental. 191
9
(a) (b) (c) (d)
9.2 Staging (a) Plain x-ray shows a destructive lesion of the proximal tibia, almost certainly an osteosarcoma; but is it
locally resectable? (b,c) Coronal and sagittal MR images show the tumour extending medially, laterally and posteriorly into
the soft tissue. (d) Transectional MRI shows that the abnormal tissue extends posteriorly right up to the vascular
compartment (arrow). This tumour would be assessed as Stage IIB.
CT) and biopsy. This should allow a firm diagnosis

PRINCIPLES OF MANAGEMENT and staging to be established. The various treatment
options can then be discussed with the patient (or the
For all but the simplest and most obvious of benign parents, in the case of a young child). A choice needs
tumours, management calls for a multidisciplinary to be made between amputation, limb-sparing opera-
team approach and is best conducted in a tertiary tions and different types of adjuvant therapy, and the
centre specializing in the treatment of bone and soft- patient must be fully informed about the pros and
tissue tumours. Consultation and cooperation cons of each.
between the orthopaedic surgeon, radiologist, patho-
logist and (certainly in the case of malignant tumours)
the oncologist is essential in the initial management.
In many cases physiotherapists, occupational thera- METHODS OF TREATMENT
pists and prosthetists will also be involved.
TUMOUR EXCISION
Once clinical and radiological examination have
The more aggressive the lesion the more widely does
suggested the most likely diagnosis, further manage-
it need to be excised, in order to ensure that the
ment proceeds as follows.
tumour as well as any dubious marginal tissue is com-
Benign, asymptomatic lesions If the diagnosis is beyond pletely removed.
doubt (e.g. a non-ossifying fibroma or a small osteo- Intracapsular (intralesional) excision and curettage
chondroma) one can afford to temporize; treatment are incomplete forms of tumour ablation and there-
may never be needed. However, if the appearances are fore applicable only to benign lesions with a very low
not pathognomonic, a biopsy is advisable and this may risk of recurrence, or to incurable tumours which
take the form of excision or curettage of the lesion. need debulking to relieve local symptoms. Adjunctive
treatment such as the use of acrylic cement after
Benign, symptomatic or enlarging tumours Painful
curettage decreases the risk of local recurrence.
lesions, or tumours that continue to enlarge after the
Marginal excision goes beyond the tumour, but
end of normal bone growth, require biopsy and con-
only just. If the dissection of a malignant lesion is car-
firmation of the diagnosis. Unless they are unusually
ried through the reactive zone, there is a significant
aggressive, they can generally be removed by local
risk of recurrence (up to 50 per cent). For benign
(marginal) excision or (in the case of benign cysts) by
lesions, however, this is a suitable method; the result-
curettage.
ing cavity can be filled with graft bone.
Suspected malignant tumours If the lesion is thought Wide excision implies that the dissection is carried
to be a primary malignant tumour, the patient is out well clear of the tumour, through normal tissue.
admitted for more detailed examination, blood tests, This is appropriate for low-grade intracompartmental
192 chest x-ray, further imaging (including pulmonary lesions (grade IA), providing a risk of local recurrence
by amputation, if it is certain that there are no skip 9
lesions and if a functional limb can be preserved. The
ongoing debate around limb sparing versus amputa-
tion is addressed in an excellent paper by DiCaprio
and Friedlaender (2003).
Advanced surgical facilities for bone grafting and
Tumours
endoprosthetic replacement at various sites must be
available. The first step consists of wide excision of the
tumour with preservation of the neurovascular struc-
tures. The resulting defect is then dealt with in one of
several ways. Short diaphyseal segments can be
replaced by vascularized or non-vascularized bone
grafts. Longer gaps may require custom-made
implants. Osteo-articular segments can be replaced by
large allografts, endoprostheses or allograft–prosthetic
composites. It is recognized, however, that the use of
large allografts carries a high risk of infection and frac-
ture; this has led to them not being used as widely as
in the past. Endoprostheses used to be custom-made
but nowadays modular systems for tumour recon-
struction are available.
In growing children, extendible implants have been
used in order to avoid the need for repeated opera-
tions; however, they may need to be replaced at the
end of growth. Other procedures, such as grafting
and arthrodesis or distraction osteosynthesis, are suit-
able for some situations.
Sarcomas around the hip and shoulder present spe-
cial problems. Complete excision is difficult and
9.3 Tumour excision The more aggressive a tumour is, reconstruction involves complex grafting and replace-
and the wider it has spread, the more widely it needs to be ment procedures (O’Connor et al., 1996).
excised. Local excision is suitable only for low-grade
tumours that are confined to a single compartment. Outcome Tumour replacement by massive endopros-
Radical resection may be needed for high-grade tumours thesis carries a high risk of complications such as
and this often means amputation at a level above the wound breakdown and infection; the 10-year survival
compartment involved. rate of these prostheses with mechanical failure as the
end point is 75 per cent and for failure due to any
cause is 58 per cent. The limb salvage rate at 20 years
below 10 per cent. However, wide excision is also
is 84 per cent (Jeys et al., 2008).
used in conjunction with chemotherapy for grade IIA
lesions.
Radical resection means that the entire compart- AMPUTATION
ment in which the tumour lies is removed en bloc Considering the difficulties of limb-sparing surgery –
without exposing the lesion. It may be possible to do particularly for high-grade tumours or if there is
this while still sparing the limb, but the surrounding doubt about whether the lesion is intracompartmen-
muscles, ligaments and connective tissues will have to tal – amputation and early rehabilitation may be the
be sacrificed; in some cases a true radical resection can wisest option. Preoperative planning and the defini-
be achieved only by amputating at a level above the tive operation are best carried out in a specialized
compartment involved. This method is required for unit, so as to minimize the risk of complications and
high-grade tumours (IIA or IIB). permit early rehabilitation.
Amputation may be curative but it is sometimes
LIMB SALVAGE performed essentially to achieve local control of a
Amputation is no longer the automatic choice for tumour which is resistant to chemotherapy and radia-
grade II sarcomas. Improved methods of imaging and tion therapy.
advances in chemotherapy have made limb salvage the
treatment of choice for many patients. However, this MULTI-AGENT CHEMOTHERAPY
option should be considered only if the local control Multi-agent chemotherapy is now the preferred
of the tumour is likely to be as good as that obtained neoadjuvant and adjuvant treatment for malignant 193
9 bone and soft-tissue tumours. There is good evidence
BENIGN BONE LESIONS
to show that, for sensitive tumours, modern
chemotherapy regimens effectively reduce the size of
the primary lesion, prevent metastatic seeding and
NON-OSSIFYING FIBROMA (FIBROUS
improve the chances of survival. When combined with CORTICAL DEFECT)
surgery for osteosarcoma and Ewing’s tumours, the
long-term disease-free survival rate in the best series is This, the commonest benign lesion of bone, is a
now about 60 per cent. developmental defect in which a nest of fibrous tissue
Drugs currently in use are methotrexate, doxoru- appears within the bone and persists for some years
bicin (Adriamycin), cyclophosphamide, vincristine before ossifying. It is asymptomatic and is almost
and cis-platinum. Treatment is started 8–12 weeks always encountered in children as an incidental find-
preoperatively and the effect is assessed by examining ing on x-ray. The commonest sites are the metaphyses
the resected tissue for tumour necrosis; greater than of long bones; occasionally there are multiple lesions.
90 per cent necrosis is taken as a good response. If The x-ray appearance is unmistakable. There is a
there is little or no necrosis, a different drug may be more or less oval radiolucent area surrounded by a
selected for postoperative treatment. Maintenance thin margin of dense bone; views in different planes
chemotherapy is continued for another 6–12 months. may show that a lesion that appears to be ‘central’ is
actually adjacent to or within the cortex, hence the
RADIOTHERAPY alternative name ‘fibrous cortical defect’.
High-energy irradiation has long been used to destroy Pathology Although it looks cystic on x-ray, it is a
radiosensitive tumours or as adjuvant therapy before solid lesion consisting of unremarkable fibrous tissue
operation. Nowadays the indications are more with a few scattered giant cells.
restricted. For highly sensitive tumours (such as As the bone grows the defect becomes less obvious
Ewing’s sarcoma) it offers an alternative to amputa- and it eventually heals spontaneously. However, it
tion; it is then combined with adjuvant chemotherapy. sometimes enlarges to several centimetres in diameter
The same combination can be used as adjunctive and there may be a pathological fracture. There is no
treatment for high-grade tumours, for tumours in risk of malignant change.
inaccessible sites, lesions that are inoperable because
of their size, proximity to major blood vessels or Treatment Treatment is usually unnecessary. If the
advanced local spread, for marrow-cell tumours such defect is very large or has led to repeated fractures, it
as myeloma and malignant lymphoma, for metastatic can be treated by curettage and bone grafting. Recur-
deposits and for palliative local tumour control where rence is rare.
no surgery is planned. Radiotherapy may also be
employed postoperatively when a marginal or intrale-
sional excision has occurred, so as to ‘sterilize’ the FIBROUS DYSPLASIA
tumour bed.
The main complications of this treatment are the Fibrous dysplasia is a developmental disorder in which
occurrence of post-irradiation spindle-cell sarcoma areas of trabecular bone are replaced by cellular
and pathological fracture in weightbearing bones, par- fibrous tissue containing flecks of osteoid and woven
ticularly in the proximal half of the femur. bone. It may affect one bone (monostotic), one limb
9.4 Non-ossifying fibroma

(a) The x-ray shows a cortical
defect, although in some
projection planes this looks
deceptively like a medullary
lesion (b). The bone may
fracture through the weakened
area (c).
194 (a) (b) (c)

9
Tumours
(a) (b) (c)
9.5 Fibrous dysplasia Monostotic fibrous dysplasia of (a) the upper femur (with the so-called ‘shepherd’s crook’
appearance) and (b) of the tibia. (c) Polyostotic fibrous dysplasia.
(monomelic) or many bones (polyostotic). If the

lesions are large, the bone is considerably weakened
and pathological fractures or progressive deformity
may occur.
The most common sites of occurrence are the prox-
imal femur, tibia, humerus, ribs and cranio-facial
bones. Small, single lesions are asymptomatic. Large,
monostotic lesions may cause pain or may be discov-
ered only when the patient develops a pathological
fracture. Patients with polyostotic disease present in
childhood or adolescence with pain, limp, bony
enlargement, deformity or pathological fracture.
Untreated, the characteristic deformities persist
through adult life.
Occasionally the bone disorder is associated with 9.6 Fibrous dysplasia – histology Microscopic islands of
metaplastic bone lie scattered in a bed of cellular fibrous
café-au-lait patches on the skin and (in girls) preco- tissue. Occasional giant cells are seen. (x120)
cious sexual development (Albright’s syndrome).
X-rays show radiolucent ‘cystic’ areas in the meta-
physis or shaft; because they contain fibrous tissue
with diffuse spots of immature bone, the lucent tumour or hyperparathyroidism. However, detailed x-
patches typically have a slightly hazy or ‘ground-glass’ ray and laboratory studies will exclude these disorders.
appearance. The weightbearing bones may be bent, Malignant transformation to fibrosarcoma occurs in
and one of the classic features is the ‘shepherd’s 0.5 per cent of patients with monostotic lesions and
crook’ deformity of the proximal femur. Radioscintig- up to 5 per cent of patients with Albright’s syndrome.
raphy shows marked activity in the lesion.
Treatment Treatment depends on the extent of the
Pathology At operation the lesional tissue has a defect and the presence or absence of deformities.
coarse, gritty feel (due to the specks of immature Small lesions need no treatment. Those that are large
bone). The histological picture is of loose, cellular and painful or threatening to fracture (or have frac-
fibrous tissue with widespread patches of woven bone tured) can be curetted and grafted, but there is a strong
and scattered giant cells. tendency for the abnormality to recur. A mixture of cor-
Both clinically and histologically the monostotic tical and cancellous bone grafts may provide added
condition may resemble either a bone-forming strength even if the lesion is not eradicated. For very 195
9 large lesions, the grafts can be supplemented by methyl-
methacrylate cement. Deformities may need correction
by suitably designed osteotomies.
With large cysts, the bone often bleeds profusely at
operation: forewarned is forearmed.
OSTEOID OSTEOMA
This tiny bone tumour (less than 1 cm in diameter)
causes symptoms out of all proportion to its size.
Patients are usually under 30 years of age and males
predominate. Any bone except the skull may be
affected, but over half the cases occur in the femur or
tibia. The patient complains of persistent pain, some-
times well localized but sometimes referred over a 9.8 Osteoid osteoma – histology The histological
wide area. Typically the pain is relieved by salicylates. features are characteristic: the nidus consists of sheets of
If the diagnosis is delayed, other features appear: a pink-staining osteoid in a fibrovascular stroma. Giant cells
and osteoblasts are prominent. (×300)
limp or muscle wasting and weakness; spinal lesions
may cause intense pain, muscle spasm and scoliosis.
The important x-ray feature is a small radiolucent
area, the so-called ‘nidus’. Lesions in the diaphysis are Treatment The only effective treatment is complete
surrounded by dense sclerosis and cortical thickening; removal or destruction of the nidus. The lesion is
this may be so marked that the nidus can be seen only carefully localized by x-ray and/or CT and then
in fine cut CT scans. Lesions in the metaphysis show excised in a small block of bone or destroyed by CT-
less cortical thickening. Further away the bone may be localized radio-ablation. The specimen should be x-
osteoporotic. 99mTc-MDP scintigraphy reveals intense, rayed immediately to confirm that it does contain the
localized activity. little tumour. If the excision is likely to weaken the
It is sometimes difficult to distinguish an osteoid host bone (especially in the vulnerable medial cortex
osteoma from a small Brodie’s abscess without biopsy. of the femoral neck), prophylactic internal fixation
Ewing’s sarcoma and chronic periostitis must also be may be needed.
excluded.
Pathology The excised lesion appears as a dark-brown
or reddish ‘nucleus’ surrounded by dense bone; the OSTEOBLASTOMA (GIANT OSTEOID
central area consists of unorganized sheets of osteoid OSTEOMA)
and bone cells.
There is no risk of malignant transformation. This tumour is similar to an osteoid osteoma but it is
larger (more than 1 cm in diameter), more cellular
and sometimes more ominous in appearance. It is
usually seen in young adults, more often in men than
in women. It tends to occur in the spine and the flat
bones; patients present with pain and local muscle
spasm.
X-ray shows a well-demarcated osteolytic lesion
which may contain small flecks of ossification. There
is surrounding sclerosis but this is not always easy to
see, especially with lesions in the flat bones or the ver-
(b) tebral pedicle. A radioisotope scan will reveal the ‘hot’
area. Larger lesions may appear cystic, and sometimes
9.7 Osteoid osteoma The x-ray a typical aneurysmal bone cyst appears to have arisen
appearance depends on the site of the
lesion. (a) With cortical tumours there is in an osteoblastoma.
marked reactive bone thickening leaving a Pathology When the tumour is exposed it has a some-
small lucent nidus, which may itself have a
central speck of ossification. (b) Lesions in what fleshy appearance. Histologically it resembles an
cancellous bone produce far less periosteal osteoid osteoma, but the cellularity is more striking.
(a) reaction and are easily mistaken for a Occasionally the picture may suggest a low-grade
196 Brodie’s abscess. osteosarcoma.
Treatment Treatment consists of excision and bone 9
grafting. With lesions in the vertebral pedicle or the
floor of the acetabulum, this is not always easy and
removal may be incomplete; local recurrence is com-
mon and malignant transformation has been reported
(McLeod et al., 1976).
Tumours
COMPACT OSTEOMA (IVORY EXOSTOSIS)
This rare benign ‘tumour’ appears as a localized thick-
ening on the outer or inner surface of compact bone.
An adolescent or young adult presents with a painless,
ivory-hard lump, usually on the outer surface of the
skull, occasionally on the subcutaneous surface of the
tibia. If it occurs on the inner table of the skull it may
cause focal epilepsy; sometimes it protrudes into the
paranasal sinuses. On x-ray a sessile plaque of exceed-
ingly dense bone with a well-circumscribed edge is (a) (b)
seen. This might suggest a parosteal osteosarcoma,
but the long history, the absence of pain and the 9.9 Chondroma (a) The hand is a common site.
(b) Another chondroma before and after curettage and
smooth outline will dispel this suspicion.
bone grafting.
Treatment Unless the tumour impinges on important
structures, it need not be removed. However, the
patient may want to be rid of it; excision is easier if a patients with associated haemangiomas (Maffucci’s
margin of normal bone is taken with it. syndrome).
Signs of malignant transformation in patients over
30 years are: (1) the onset of pain; (2) enlargement of
the lesion; and (3) cortical erosion. Unfortunately,
CHONDROMA (ENCHONDROMA) biopsy is of little help in this regard as the cartilage
usually looks benign during the early stages of malig-
Islands of cartilage may persist in the metaphyses of nant transformation. If the other features are present,
bones formed by endochondral ossification; some- and especially in older patients, the lesion should be
times they grow and take on the characteristics of a treated as a stage IA malignancy; the biopsy then
benign tumour. Chondromas are usually asympto- serves chiefly to confirm the fact that it is a cartilage
matic and are discovered incidentally on x-ray or after tumour.
a pathological fracture. They are seen at any age (but
mostly in young people) and in any bone preformed Treatment Treatment is not always necessary, but if
in cartilage (most commonly the tubular bones of the the tumour appears to be enlarging, or if it presents as
hands and feet). Lesions may be solitary or multiple a pathological fracture, it should be removed as thor-
and part of a generalized dysplasia. oughly as possible by curettage; the defect is filled
X-ray shows a well-defined, centrally placed radi- with bone graft or bone cement. There is a fairly high
olucent area at the junction of metaphysis and diaph- recurrence rate and the tissue may be seeded in adja-
ysis; sometimes the bone is slightly expanded. In cent bone or soft tissues. Chondromas in expendable
mature lesions there are flecks or wisps of calcification sites are better removed en bloc.
within the lucent area; when present, this is a pathog-
nomonic feature.
Pathology When it is exposed the lesion is seen to con- PERIOSTEAL CHONDROMA
sist of pearly-white cartilaginous tissue, often with a cen- These are rare developmental lesions arising in the
tral area of degeneration and calcification. Histologically deep layer of the periosteum, usually around the prox-
the appearances are those of simple hyaline cartilage. imal humerus, femur or phalanges. A cartilaginous
Complications There is a small but significant risk of lump bulges from the bone into the soft tissues and
malignant change – probably less than 2 per cent (and causes some alarm when it is discovered by the
hardly ever in a child) for patients with solitary lesions patient.
but as high as 30 per cent in those with multiple Because the cartilage remains uncalcified, the lesion
lesions (Ollier’s disease) and up to 100 per cent in itself does not show on x-ray, but the surface of the 197
9 bone may be irregular or scalloped. MRI may reveal Treatment In children the risk of damage to the
the full extent of the tumour. Histologically the lesion physis makes one hesitate to remove the lesion. After
is composed of highly cellular cartilage. the end of the growth period the lesion can be
removed – by marginal excision wherever possible or
Treatment Because of its propensity to recur, it is best
(less satisfactorily) by curettage and alcohol or phenol
removed by marginal excision (taking a rim of normal
cauterization – and replaced with autogenous bone
bone). Recurrent lesions may look more aggressive

grafts. There is a high risk of recurrence after incom-
but the lesion probably does not undergo malignant
plete removal, and if this happens repeatedly there
change.
may be serious damage to the nearby joint. Occasion-
ally one is forced to excise the recurrent lesion with an
adequate margin of bone and accept the inevitable
CHONDROBLASTOMA need for joint reconstruction.
This benign tumour of immature cartilage cells is one of

the few lesions to appear primarily in the epiphysis, usu- CHONDROMYXOID FIBROMA
ally of the proximal humerus, femur or tibia. Patients
are affected around the end of the growth period or in Like other benign cartilaginous lesions, this is seen
early adult life; there is a predilection for males. The pre- mainly in adolescents and young adults. It may occur
senting symptom is a constant ache in the joint; the ten- in any bone but is more common in those of the lower
der spot is actually in the adjacent bone. limb.
X-ray shows a rounded, well-demarcated radiolu- Patients seldom complain and the lesion is usually
cent area in the epiphysis with no hint of central cal- discovered by accident or after a pathological fracture.
cification; this site is so unusual that the diagnosis X-rays are very characteristic: there is a rounded or
springs readily to mind. However, sometimes the ovoid radiolucent area placed eccentrically in the
lesion extends across the physeal line. Occasionally the metaphysis; in children it may extend up to or even
articular surface is breached. Like osteoblastoma, the slightly across the physis. The endosteal margin may
lesion sometimes expands and acquires the features of be scalloped, but is almost always bounded by a dense
an aneurysmal bone cyst. zone of reactive bone extending tongue-like towards
the diaphysis. The cortex may be asymmetrically
Pathology The histological appearances are fairly typ-
expanded. Sometimes there is calcification in the
ical – there are large collections of chondroblasts set
‘vacant’ area.
off by the surrounding matrix of immature fibrous tis-
sue. Within the stroma are scattered giant cells. In Pathology Although the lesion looks ‘cystic’ on x-ray,
expansile lesions, the edge may resemble that of an it contains mucinous material and bits of cartilage.
aneurysmal bone cyst. These tumours do not undergo Histologically three types of tissue can usually be
malignant change but they may be locally aggressive identified: patches of myxomatous tissue with deli-
and extend into the joint. cate, stellate cells; islands of hyaline cartilage; and
(a) (b)
9.10 Chondroblastoma (a) X-ray shows a cyst-like lesion occupying the epiphysis, and sometimes extending across the
physis into the adjacent bone. (b) The characteristic features in this photomicrograph are the more faintly staining islands
198 of chondroid tissue composed of round cells (‘chondroblasts’) and scattered multinucleated giant cells. (×300)
9.11 Chrondromyxoid 9
fibroma (a) The x-ray is quite
typical: there is an eccentric
cyst-like lesion with a densely
sclerotic endosteal margin
often extending like a tongue
towards the diaphysis. (b) The
Tumours
section shows predominantly
myxomatous cells and fibrous
tissue; elsewhere chondroid
tissue and giant cells are more
obvious. (×300)
(a) (b)
areas of fibrous tissue with cells of varying degrees of cartilage degeneration and calcification and then the
maturity. x-ray shows the bony exostosis surrounded by clouds
Malignant change has been recorded but this is of calcified material.
extremely rare. Multiple lesions may develop as part of a heritable
disorder – hereditary multiple exostosis – in which there
Treatment Where feasible, the lesion should be
are also features of abnormal bone growth resulting in
excised but often one can do no more than a thor-
characteristic deformities (see Chapter 8).
ough curettage followed by autogenous bone graft-
ing. There is a considerable risk of recurrence; if Pathology At operation the cartilage cap is seen sur-
repeated operations are needed, care should be taken mounting a narrow base or pedicle of bone. The cap
to prevent damage to the physis (in children) or the consists of simple hyaline cartilage; in a growing exos-
nearby joint surface. tosis the deeper cartilage cells are arranged in
columns, giving rise to the formation of endochondral
new bone. Large lesions may have a ‘cauliflower’
OSTEOCHONDROMA (CARTILAGE-CAPPED appearance, with degeneration and calcification in the
centre of the cartilage cap.
EXOSTOSIS)
Complications The incidence of malignant transfor-
This, one of the commonest ‘tumours’ of bone, is a mation is difficult to assess because troublesome
developmental lesion which starts as a small over- lesions are so often removed before they show histo-
growth of cartilage at the edge of the physeal plate and logical features of malignancy. Figures usually quoted
develops by endochondral ossification into a bony pro- are 1 per cent for solitary lesions and 6 per cent for
tuberance still covered by the cap of cartilage. Any multiple.
bone that develops in cartilage may be involved; the Features suggestive of malignant change are:
commonest sites are the fast-growing ends of long (1) enlargement of the cartilage cap in successive
bones and the crest of the ilium. In long bones, growth examinations; (2) a bulky cartilage cap (more than
leaves the bump stranded further down the metaphysis. 1 cm in thickness); (3) irregularly scattered flecks of
Here it may go on growing but at the end of the nor- calcification within the cartilage cap; and (4) spread
mal growth period for that bone it stops enlarging. Any into the surrounding soft tissues. MRI may be needed
further enlargement after the end of the growth period is to reveal these changes.
suggestive of malignant transformation.
The patient is usually a teenager or young adult Treatment If the tumour causes symptoms it should
when the lump is first discovered. Occasionally there be excised; if, in an adult, it has recently become big-
is pain due to an overlying bursa or impingement on ger or painful then operation is urgent, for these fea-
soft tissues, or, rarely, paraesthesia due to stretching of tures suggest malignancy. This is seen most often with
an adjacent nerve. pelvic exostoses – not because they are inherently dif-
The x-ray appearance is pathognomonic. There is a ferent but because considerable enlargement may, for
well-defined exostosis emerging from the metaphysis, long periods, pass unnoticed. If there are suspicious
its base co-extensive with the parent bone. It looks features, further imaging and staging should be car-
smaller than it feels because the cartilage cap is usually ried out before doing a biopsy. If the histology is that
invisible on x-ray; however, large lesions undergo of ‘benign’ cartilage but the tumour is known for 199
9
(a) (b) (c)
9.12 Osteochondroma (a) A young girl presented with this lump on her leg. It felt bony hard. (b) X-ray examination
showed the typical features of a large cartilage-capped exostosis; of course the cartilage cap does not show on x-ray unless
it is calcified. The bony part may be sessile, pedunculated or cauliflower-like. (c) Histological sections show that the
exostosis is always covered by a hyaline cartilage cap from which the bony excrescence grows.
(a) (b)
9.13 Osteochondroma – treatment (a) This 20-year-old man had known about the lump on his left scapula for many
years. He stopped growing at the age of 18 but the tumour continued to enlarge. (b) Despite the benign histology in the
biopsy, the tumour together with most of the scapula was removed; sections taken from the depths of the lesion showed
atypical cells suggestive of malignant change.
certain to be enlarging after the end of the growth X-rays show a well-demarcated radiolucent area in
period, it should be treated as a chondrosarcoma. the metaphysis, often extending up to the physeal
plate; the cortex may be thinned and the bone
expanded.
SIMPLE BONE CYST Diagnosis is usually not difficult but other cyst-like
lesions may need to be excluded. Non-osteogenic
This lesion (also known as a solitary cyst or unicameral fibroma, fibrous dysplasia and the benign cartilage
bone cyst) appears during childhood, typically in the tumours are solid and merely look cystic on x-ray. In
metaphysis of one of the long bones and most com- doubtful cases a needle can be inserted into the lesion
monly in the proximal humerus or femur. It is not a under x-ray control: with a simple cyst, straw-
tumour, it tends to heal spontaneously and it is sel- coloured fluid will be withdrawn. Very seldom will
dom seen in adults. The condition is usually discov- there be any need for biopsy. However, if curettage is
ered after a pathological fracture or as an incidental thought to be necessary, material from the cyst should
200 finding on x-ray. be submitted for examination.
9
Tumours
(a) (b) (c) (d)
9.14 Simple bone cysts (a) A typical solitary (or unicameral) cyst – on the shaft side of the physis and expanding the
cortex. (b) Injection with methylprednisolone, and (c) healing. (d) Fracture through a cyst.
Pathology The lining membrane consists of flimsy There is always the risk that the cyst will recur and
fibrous tissue, often containing giant cells. In an more than one operation may be needed.
actively growing cyst, there is osteoclastic resorption
of the adjacent bone.
Treatment Treatment depends on whether the cyst is ANEURYSMAL BONE CYST
symptomatic, actively growing or involved in a fracture.
Asymptomatic lesions in older children can be left alone Aneurysmal bone cyst may be encountered at any age
but the patient should be cautioned to avoid injury and in almost any bone, though more often in young
which might cause a fracture. ‘Active’ cysts (those in adults and in the long-bone metaphyses. Usually it
young children, usually abutting against the physeal arises spontaneously but it may appear after degener-
plate and obviously enlarging in sequential x-rays) ation or haemorrhage in some other lesion.
should be treated, in the first instance, by aspiration of With expanding lesions, patients may complain of
fluid and injection of 80–160 mg of methylpred- pain. Occasionally, a large cyst may cause a visible or
nisolone or autogenous bone marrow. This often stops palpable swelling of the bone.
further enlargement and leads to healing of the cyst. X-rays show a well-defined radiolucent cyst, often
If the cyst goes on enlarging, or if there is a patholog- trabeculated and eccentrically placed. In a growing
ical fracture, the cavity should be thoroughly cleaned tubular bone it is always situated in the metaphysis
by curettage and then packed with bone chips, but and therefore may resemble a simple cyst or one of the
great care should be taken not to damage the nearby other cyst-like lesions. Occasional sites include verte-
physeal plate. If the risk of fracture is thought to be brae and the flat bones. In an adult an aneurysmal
high, prophylactic internal fixation should be applied. bone cyst may be mistaken for a giant-cell tumour
(a) (b) (c) (d)
9.15 Cyst-like lesions (a) Simple bone cyst. Fills the medullary cavity but does not expand the bone. (b) Chondromyxoid
fibroma. Looks cystic but it is actually a radiolucent benign tumour; always in the metaphysis; hard boundary tailing off
towards the diaphysis. (c) Aneurysmal bone cyst. Expansile cystic tumour, always on the metaphyseal side of the physis.
(d) Giant-cell tumour. Hardly ever appears before epiphysis has fused, the pathognomonic feature is that it extends right up
to the subarticular bone plate; sometimes malignant. 201
9
(a) (b)
9.16 Aneurysmal bone cyst – histology (a) The cyst contained blood and was lined by loose fibrous tissue containing
numerous giant cells. (×120) (b) A high-power view of the same. (×300)
but, unlike the latter, it usually does not extend right hurry to re-operate; the lesion occasionally heals
up to the articular margin. Occasionally it causes spontaneously (Malghem et al., 1989).
marked ballooning of the bone end.
Pathology When the cyst is opened it is found to con-
tain clotted blood, and during curettage there may be GIANT-CELL TUMOUR
considerable bleeding from the fleshy lining mem-
Giant-cell tumour, which represents 5 per cent of all
brane. Histologically the lining consists of fibrous tis-
primary bone tumours, is a lesion of uncertain origin
sue with vascular spaces, deposits of haemosiderin and
that appears in mature bone, most commonly in the
multinucleated giant cells. Occasionally the appear-
distal femur, proximal tibia, proximal humerus and
ances so closely resemble those of giant-cell tumour
distal radius, though other bones also may be
that only the most experienced pathologists can con-
affected. It is hardly ever seen before closure of the
fidently make the diagnosis. Malignant transformation
nearby physis and characteristically it extends right up
does not occur.
to the subarticular bone plate. Rarely, there are multi-
Treatment The cyst should be carefully opened, thor- ple lesions.
oughly curetted and then packed with bone grafts. The patient is usually a young adult who complains
Sometimes the graft is resorbed and the cyst recurs, of pain at the end of a long bone; sometimes there is
necessitating a second or third operation. In these slight swelling. A history of trauma is not uncommon
cases, packing with methylmethacrylate cement may
be more effective. However, if the cyst is in a ‘safe’
area (i.e. where there is no risk of fracture) there is no
9.18 Giant-cell tumour – histology A low-power view

of the biopsy shows the abundant multinucleated giant
9.17 Giant-cell tumours The tumour always abuts cells lying in a stroma composed of round and polyhedral
202 against the joint margin. tumour cells. There are numerous mitotic figures.
and pathological fracture occurs in 10–15 per cent of Treatment Well-confined, slow-growing lesions with 9
cases. On examination there may be a palpable mass benign histology can safely be treated by thorough
with warmth of the overlying tissues. curettage and ‘stripping’ of the cavity with burrs and
X-rays show a radiolucent area situated eccentrically gouges, followed by swabbing with hydrogen perox-
at the end of a long bone and bounded by the sub- ide or by the application of liquid nitrogen; the cavity
chondral bone plate. The endosteal margin may be is then packed with bone chips. More aggressive
Tumours
quite obvious, but in aggressive lesions it is ill-defined. tumours, and recurrent lesions, should be treated by
The centre sometimes has a soap-bubble appearance excision followed, if necessary, by bone grafting or
due to ridging of the surrounding bone. The cortex is prosthetic replacement. Tumours in awkward sites
thin and sometimes ballooned; aggressive lesions (e.g. the spine) may be difficult to eradicate; supple-
extend into the soft tissue. The appearance of a ‘cys- mentary radiotherapy is sometimes recommended,
tic’ lesion in mature bone, extending right up to the but it carries a significant risk of causing malignant
subchondral plate, is so characteristic that the diagno- transformation.
sis is seldom in doubt. However, it is prudent to
obtain estimations of blood calcium, phosphate and
alkaline phosphatise concentrations so as exclude an
unusual ‘brown tumour’ associated with hyper-
parathyroidism.
Because of the tumour’s potential for aggressive
behaviour, detailed staging procedures are essential.
CT scans and MRI will reveal the extent of the
tumour, both within the bone and beyond. It is
important to establish whether the articular surface
has been breached.
Biopsy is essential. This can be done either as a
frozen section before proceeding with operative treat-
ment or (especially if a more extensive operation is
contemplated) as a separate procedure.
Pathology The tumour has a reddish, fleshy (a)
appearance; it comes away in pieces quite easily when
curetted but is difficult to remove completely from the
surrounding bone. Aggressive lesions have a poorly
defined edge and extend well into the surrounding
bone. Histologically the striking feature is an
abundance of multinucleated giant cells scattered on a
background of stromal cells with little or no visible
intercellular tissue. Aggressive lesions tend to show
more cellular atypia and mitotic figures, but histological
grading is unreliable as a predictor of tumour
behaviour. (b)
Rarely metastases are discovered in the lungs. The
9.19 Giant-cell tumour – treatment (a) Excision and
tumour has the potential to transform into an bone grafts. (b) Block resection and replacement with a
osteosarcoma. large allograft.
9.20 Cysts and cyst-like lesions of bone Thumb-nail sketches of lesions which appear as ‘cysts’ on x-ray examination. 203
9 GIANT-CELL SARCOMA Hand–Schüller–Christian disease is a disseminated
Giant-cell sarcoma is an unequivocally malignant form of the same condition. The patient is a child,
lesion with x-ray features like those of a highly usually with widespread lesions involving the skull,
aggressive benign giant-cell tumour. There is a high vertebral bodies, liver and spleen. There may be
risk of metastasis and treatment requires wide, or even anaemia and a tendency to recurrent infection.
radical, resection. Individual lesions can be treated by curettage or
radiotherapy; however, complete remission is very

unlikely.
EOSINOPHILIC GRANULOMA AND Letterer–Siwe disease is an extremely rare (and
severe) form of histiocytosis. It is seen in infants and
HISTIOCYTOSIS usually progresses rapidly to a fatal outcome.
Histiocytosis-X defines an unusual group of disorders
in which cells of the reticuloendothelial system (histi-
ocytes and eosinophils) form granulomatous collec- HAEMANGIOMA
tions which may cause osteolytic lesions resembling
bone tumours. Osseous haemangiomas consist of vascular channels
Eosinophilic granuloma is the commonest of these (capillary, venous or cavernous) and are usually seen
conditions, and the only one presenting as a pure in middle-aged patients, the spine being the com-
bone lesion. Marrow-containing bone is resorbed and monest site. They are usually symptomless and dis-
one or more lytic lesions may appear in the flat bones covered accidentally when the back is x-rayed for
or the metaphyses of long bones. The patient is usu- some other reason. However, if the patient does have
ally a child; there is seldom any complaint of pain and backache, the haemangioma is likely to be blamed.
the condition is discovered incidentally or after a The x-ray shows coarse vertical trabeculation (the
pathological fracture. so-called ‘corduroy appearance’) in the vertebral
X-ray shows a well-demarcated oval area of radiolu- body. Other sites include the skull and pelvis where
cency within the bone; sometimes this is associated with the appearance occasionally suggests malignancy, but
marked reactive sclerosis. There may be multiple lesions there is no associated cortical or medullary destruc-
and in the skull they have a characteristic punched-out tion. Rarely the presenting feature may be a patho-
appearance. Vertebral collapse may result in a flat wedge logical fracture.
(vertebra plana) which is pathognomonic. If operation is needed there is a risk of profuse bleed-
The condition usually heals spontaneously and is ing, and embolization may be a useful preliminary.
therefore rarely seen in adults. Occasionally, however,
a solitary lesion may herald the onset of one of the
generalized disorders (see below). Operation is usu- OSTEOLYSIS (‘DISAPPEARING BONES’)
ally done to obtain a biopsy; if the lesion is easily
accessible it may be completely excised or curetted; if In massive osteolysis (Gorham’s disease) there is pro-
not, radiotherapy is effective. gressive disappearance of bone, associated with hae-
(a) (b) (c) (d) (e)
9.21 Histiocytosis-X (a) An eosinophilic granuloma of the ischium which went on to spontaneous healing.
(b) Completely flattened vertebral body with discs of normal height, probably due to eosinophilic granuloma. (c,d) Two
stages in the development of vertebral flattening from an eosinophilic granuloma. (e) Hand–Schüller–Christian disease,
204 which typically affects the skull.
By far the majority of chondrosarcomas fall into 9
two well-defined categories: central tumours occupy-
ing the medullary cavity of the bone, and so-called
‘peripheral tumours’ growing out from the cortex.
Less common varieties are juxtacortical chondrosar-
coma, clear-cell chondrosarcoma and mesenchymal
Tumours
chondrosarcoma.
Central chondrosarcoma The tumour develops in the
medullary cavity of either tubular or flat bones, most
9.22 Haemangioma Most of these tumours are commonly at the proximal end of the femur or in the
symptomless and discovered accidentally during x-ray innominate bone of the pelvis. X-rays show an
examination for another reason, but in this case the
expanded, somewhat radiolucent area in the bone, with
vertebra collapsed and the patient presented with back
pain. flecks of increased density due to calcification within the
tumour. Aggressive lesions may take on a globular
appearance with scalloping or destruction of the cortex.
When a benign medullary chondroma (enchon-
mangiomatosis or multiple lymphangiectases. Usually droma) undergoes malignant transformation, it is dif-
the progression involves contiguous bones, but occa- ficult to be sure that the lesion was not a slowly
sionally multiple sites are affected. Patients may pres- evolving sarcoma from the outset.
ent with mild pain or with a pathological fracture. No Peripheral chondrosarcoma This tumour usually arises
effective treatment is known, but spontaneous arrest in the cartilage cap of an exostosis (osteochondroma)
has been described. Occasionally, however, the that has been present since childhood. Exostoses of
process spreads to vital structures and the outcome is the pelvis and scapula seem to be more susceptible
fatal. than others to malignant change, but perhaps this is
simply because the site allows a tumour to grow with-
out being detected and removed at an early stage. X-
PRIMARY MALIGNANT BONE rays show the bony exostosis, often surmounted by
clouds of patchy calcification in the otherwise unseen
TUMOURS lobulated cartilage cap. A tumour that is very large
and calcification that is very fluffy and poorly outlined
CHONDROSARCOMA are suspicious features, but the clearest sign of malig-
nant change is a demonstrable progressive enlarge-
Chondrosarcoma is one of the commonest malignant ment of an osteochondroma after the end of normal
tumours originating in bone. The highest incidence is bone growth. MRI is the best means of showing the
in the fourth and fifth decades and men are affected size and internal features of the cartilage cap.
more often than women.
Juxtacortical (periosteal) chondrosarcoma Here the lesion
These tumours are slow-growing and are usually
appears as an excrescence on the surface of one of the
present for many months before being discovered.
tubular bones – usually the femur. It arises from the
Patients may complain of a dull ache or a gradually
outermost layers of the cortex, deep to the periosteum.
enlarging lump. Medullary lesions may present as a
X-ray changes comprise features of both a
pathological fracture.
chondrosarcoma and a periosteal osteosarcoma: an
Although chondrosarcoma may develop in any of
outgrowth from the bone surface, often containing
the bones that normally develop in cartilage, almost
flecks of calcification, as well as ‘sunray’ streaks and
50 per cent appear in the metaphysis of one of the
new-bone formation at the margins of the stripped
long tubular bones, mostly in the lower limbs. The
periosteum. The dominant cell type is chondroblastic
next most common sites are the pelvis and the ribs.
but there may also be sparse osteoid formation, leading
Despite the relatively frequent occurrence of benign
one to doubt whether this is a cartilage tumour or a
cartilage tumours in the small bones of the hands and
non-aggressive osteosarcoma.
feet, malignant lesions are rare at these sites.
Chondrosarcomas take various forms, usually desig- Clear-cell chondrosarcoma There is some doubt as to
nated according to: (a) their location in the bone (cen- whether this rare tumour is really a chondrosarcoma.
tral or peripheral); (b) whether they develop without In some respects the tumour resembles an aggressive
precedent (primary chondrosarcoma) or by malignant chondroblastoma (e.g. its typical location in the head
change in a pre-existing benign lesion (secondary of the femur rather than the metaphysis). However,
chondrosarcoma); and (c) the predominant cell type in despite the fact that it is very slow-growing, it does
the tumour. eventually metastasize. 205
9
(c)
(a) (b) (d)
9.23 Central chondrosaroma (a) Typical x-ray of a central chondrosarcoma of the femur. (b) In this case the patient
presented with a pathological fracture of the humerus. X-rays showed rarefaction of the bone with central flecks of
calcification. At the fracture site the lesion extends into the soft tissues. (c) Radical resection was carried out. Pale glistening
cartilage tissue was found in the medullary cavity and, in several places, spreading beyond the cortex. Much of the bone is
occupied by haemorrhagic tissue. (d) The histological sections show lobules of highly atypical cartilage cells, including
binucleate cells.
(a) (b)
9.24 Chondrosarcoma At the age of 20 years, this young man complained of pain in the right groin; x-ray showed an
osteochondroma of the right inferior pubic ramus. (a) A biopsy showed ‘benign’ cartilage but a year later the tumour had
doubled its size (b), a clear sign that it was malignant.
206
Mesenchymal chondrosarcoma This is an equally metastases can be resected. The tumour does not 9
controversial entity. It tends to occur in younger respond to either radiotherapy or chemotherapy.
individuals and in about 50 per cent of cases the Prognosis is determined largely by the cellular
tumour lies in the soft tissues outside an adjacent bone. grade and the resection margin. There is a tendency
The x-ray appearances are similar to those of the for these tumours to recur late and the patient should
common types of chondrosarcoma but the clinical therefore be followed up for 10 years or longer.
Tumours
behaviour of the tumour is usually more aggressive.
Histology shows a mixture of mesenchymal cells and
chondroid tissue. OSTEOSARCOMA
In its classic (intramedullary) form, osteosarcoma is a
Staging highly malignant tumour arising within the bone and
If a chondrosarcoma is suspected, full staging proce- spreading rapidly outwards to the periosteum and sur-
dures should be employed. CT scans and MRI must rounding soft tissues. It is said to occur predominantly
be carried out before performing a biopsy. in children and adolescents, but epidemiological stud-
ies suggest that between 1972 and 1981 the age of
presentation rose significantly (Stark et al., 1990). It
Pathology may affect any bone but most commonly involves the
A biopsy is essential to confirm the diagnosis. How- long-bone metaphyses, especially around the knee and
ever, low-grade chondrosarcoma may show histologi- at the proximal end of the humerus.
cal features no different from those of an aggressive Pain is usually the first symptom; it is constant, worse
benign cartilaginous lesion. High-grade tumours are at night and gradually increases in severity. Sometimes
more cellular, and there may be obvious abnormal the patient presents with a lump. Pathological fracture
features of the cells, such as plumpness, hyperchroma- is rare. On examination there may be little to find
sia and mitoses. except local tenderness. In later cases there is a palpable
mass and the overlying tissues may appear swollen and
inflamed. The ESR is usually raised and there may be
Treatment an increase in serum alkaline phosphatase.
Since most chondrosarcomas are slow-growing and
metastasize late, they present the ideal case for wide
X-rays
excision and prosthetic replacement, provided it is
certain that the lesion can be completely removed The x-ray appearances are variable: hazy osteolytic
without exposing the tumour and without causing an areas may alternate with unusually dense osteoblastic
unacceptable loss of function; in that case amputation areas. The endosteal margin is poorly defined. Often
may be preferable. In some cases isolated pulmonary the cortex is breached and the tumour extends into
(a) (b) (c) (d)
9.25 Osteosarcoma (a) The metaphyseal site; increased density, cortical erosion and periosteal reaction are characteristic.
(b) Sunray spicules and Codman’s triangle; (c) the same patient after radiotherapy. (d) A predominantly osteolytic tumour. 207
9 9.26 Osteosarcoma –
pathology (a) After
resection this lesion was cut
in half; pale tumour tissue is
seen occupying the distal
third of the femur and
extending through the
cortex. (b) The dominant

features in the histological
sections were malignant
stromal tissue showing
osteoid formation (pink
(b) masses). (×480) (c) The same
tumour showed areas of
chondroblastic
differentiation. (×480)
(a) (c)
the adjacent tissues; when this happens, streaks of new Areas of bone loss and cavitation alternate with dense
bone appear, radiating outwards from the cortex – the patches of abnormal new bone. The tumour extends
so-called ‘sunburst’ effect. Where the tumour within the medulla and across the physeal plate. There
emerges from the cortex, reactive new bone forms at may be obvious spread into the soft tissues with ossifi-
the angles of periosteal elevation (Codman’s triangle). cation at the periosteal margins and streaks of new
While both the sunburst appearance and Codman’s bone extending into the extraosseous mass.
triangle are typical of osteosarcoma, they may occa- The histological appearances show considerable
sionally be seen in other rapidly growing tumours. variation: some areas may have the characteristic spin-
dle cells with a pink-staining osteoid matrix; others
may contain cartilage cells or fibroblastic tissue with
Diagnosis and staging
little or no osteoid. Several samples may have to be
In most cases the diagnosis can be made with confi- examined; pathologists are reluctant to commit them-
dence on the x-ray appearances. However, atypical selves to the diagnosis unless they see evidence of
lesions can cause confusion. Conditions to be osteoid formation.
excluded are post-traumatic swellings, infection, stress
fracture and the more aggressive ‘cystic’ lesions.
Other imaging studies are essential for staging pur-
Treatment
poses. Radioisotope scans may show up skip lesions, The appalling prognosis that formerly attended this
but a negative scan does not exclude them. CT and tumour has markedly improved, partly as a result of
MRI reliably show the extent of the tumour. Chest x- better diagnostic and staging procedures, and possibly
rays are done routinely, but pulmonary CT is a much because the average age of the patients has increased,
more sensitive detector of lung metastases. About 10 but mainly because of advances in chemotherapy to
per cent of patients have pulmonary metastases by the control metastatic spread. However, it is still impor-
time they are first seen. tant to eradicate the primary lesion completely; the
A biopsy should always be carried out before com- mortality rate after local recurrence is far worse than
mencing treatment; it must be carefully planned to following effective ablation at the first encounter.
allow for complete removal of the tract when the The principles of treatment are outlined on page
tumour is excised. 192. After clinical assessment and advanced imaging,
the patient is admitted to a special centre for biopsy.
The lesion will probably be graded IIA or IIB. Multi-
Pathology agent neoadjuvant chemotherapy is given for 8–12
The tumour is usually situated in the metaphysis of a weeks and then, provided the tumour is resectable
208 long bone, where it destroys and replaces normal bone. and there are no skip lesions, a wide resection is car-
9.27 Osteosarcoma – imaging (a,b) X-rays of a 9
distal femoral osteosarcoma in a child. (c,d,e) MRI
examination: coronal, sagittal and axial scans
showing the intra-and extra-osseous extensions of
the tumour and its proximity to the neurovascular
bundle.
Tumours
(a) (b)
(c) (d) (e)
9.28 Osteosarcoma – operative treatment Postoperative x-rays showing an endoprosthetic replacement following wide
resection of the lesion (Stanmore Implants Worldwide). 209
9 ried out. Depending on the site of the tumour, prepa-
rations would have been made to replace that segment
of bone with either a large bone graft or a custom-
made implant; in some cases an amputation may be
more appropriate.
The pathological specimen is examined to assess the
response to preoperative chemotherapy. If tumour

necrosis is marked (more than 90 per cent),
chemotherapy is continued for another 6–12 months;
if the response is poor, a different chemotherapeutic
regime is substituted.
Pulmonary metastases, especially if they are small
and peripherally situated, may be completely resected
with a wedge of lung tissue.
Outcome
Long-term survival after wide resection and
chemotherapy has improved from around 50 per cent (a) (b)
in 1980 (Rosen et al., 1982; Carter et al., 1991) to 9.29 Parosteal osteosarcoma (a,b) X-rays show an
over 60 per cent in recent years (Smeland et al., ill-defined extraosseous tumour – note the linear gap
2004). Tumour-replacement implants usually func- between cortex and tumour.
tion well. There is a fairly high complication rate
(mainly wound breakdown and infection) but, in
sionally the tumour has a much more aggressive
patients who survive, 10-year survival with mechani-
appearance (dedifferentiated parosteal osteosarcoma).
cal failure as the end point is 75 per cent and for fail-
ure for any cause is 58 per cent. The limb salvage rate Treatment For a low-grade parosteal osteosarcoma,
at 20 years is 84 per cent (Jeys et al., 2008) Aseptic wide excision without adjuvant therapy is sufficient to
loosening is more prevalent in younger patients. ensure a recurrence rate below 10 per cent. Dediffer-
entiated parosteal osteosarcoma should be treated in
the same way as intramedullary sarcoma.
VARIANTS OF OSTEOSARCOMA PERIOSTEAL OSTEOSARCOMA

This rare tumour is quite distinct from parosteal osteo-
PAROSTEAL OSTEOSARCOMA
sarcoma. It is more like an intramedullary osteosar-
This is a low-grade sarcoma situated on the surface of
coma, but situated on the surface of the bone. It occurs
one of the tubular bones, usually at the distal femoral
in young adults and causes local pain and swelling.
or proximal tibial metaphysis. The patient is a young
X-ray shows a superficial defect of the cortex, but
adult who presents with a slowly enlarging mass near
CT and MRI may reveal a larger soft-tissue mass. The
the bone end.
appearances sometimes suggest a periosteal chon-
X-ray shows a dense bony mass on the surface of the
droma and the diagnosis may not be certain until a
bone or encircling it; the cortex is not eroded and usu-
biopsy is performed.
ally a thin gap remains between cortex and tumour. The
picture is easily mistaken for that of a benign bone le- Pathology Histologically this is a true osteosarcoma,
sion and the diagnosis is often missed until the tumour but characteristically the sections show a prominent
recurs after local excision. CT and MRI will show the cartilaginous element.
boundary between tumour and surrounding soft tissues.
Treatment Treatment is the same as that of classic
Although the lesion is outside the bone, it does not
osteosarcoma.
spread into the adjacent muscle compartment until
fairly late. Staging, therefore, often defines it as a low-
PAGET’S SARCOMA
grade intracompartmental tumour (stage IA).
Paget’s disease affects about 2 per cent of western
Pathology At biopsy the tumour appears as a hard Europeans. Although malignant transformation is a
mass. On microscopic examination the lesion consists rare complication of this disease, most osteosarcomas
of well-formed bone but without any regular trabecu- appearing after the age of 50 years fall into this cate-
lar arrangement. The spaces between trabeculae are gory. Warning signs are the appearance of pain or
filled with cellular fibroblastic tissue; a few atypical swelling in a patient with longstanding Paget’s dis-
210 cells and mitotic figures can usually be found. Occa- ease. In late cases, pathological fracture may occur.
9
Tumours
(a) (b)
9.30 Parosteal osteosarcoma – histology (a) Histologically there are bony trabeculae and spindle-shaped, well-
differentiated fibrous tissue cells with occasional mitotic figures. (×120) (b) High-power view of the same. (×300)
X-ray shows the usual features of Paget’s disease, but X-ray shows an undistinctive area of bone destruc-
with areas of bone destruction and soft-tissue invasion. tion. CT or MRI will reveal the soft-tissue extension.
This is a high-grade tumour – if anything even Pathology Histologically the lesion consists of masses
more malignant than classic osteosarcoma. Staging of fibroblastic tissue with scattered atypical and mitotic
usually shows that extracompartmental spread has cells. Appearances vary from well-differentiated to
occurred; most patients have pulmonary metastases highly undifferentiated, and the tumours are some-
by the time the tumour is diagnosed. times graded accordingly.
Treatment Even with radical resection or amputation Treatment Low-grade, well-confined tumours (stage
and chemotherapy the 5-year survival rate is low. If the IA) can be treated by wide excision with prosthetic
lesion is definitely extracompartmental, palliative treat- replacement. High-grade lesions (IIA or IIB) require
ment by radiotherapy may be preferable; chemotherapy radical resection or amputation; if this cannot be
is usually difficult because of the patient’s age and un- achieved, local excision must be combined with radi-
certainty about renal and cardiac function. ation therapy. The value of adjuvant chemotherapy is
still uncertain.
FIBROSARCOMA OF BONE
MALIGNANT FIBROUS HISTIOCYTOMA
Fibrosarcoma is rare in bone; it is more likely to arise
in previously abnormal tissue (a bone infarct, fibrous Like fibrosarcoma, this tumour tends to occur in pre-
dysplasia or after irradiation). The patient – usually an viously abnormal bone (old infarcts or Paget’s dis-
adult – complains of pain or swelling; there may be a ease). Patients are usually middle-aged adults and
pathological fracture. x-rays may reveal a destructive lesion adjacent to an
9.31 Fibrosarcoma
(a) The area of bone
destruction in the femoral
condyle has no special
distinguishing features.
(b) The biopsy showed
highly atypical fibroblastic
tissue.
(a) (b) 211

9 9.32 Malignant fibrous
histiocytoma (a) X-ray
showing a large ‘cystic’
lesion in the distal femur.
The lesion may occur in an
area of old bone ‘infarct’,
which may account for the
flecks of increased density in

this x-ray. (b) Histology
shows abnormal
fibrohistiocytic cells, many of
which are unusually large
and some of which are
binucleate or multinucleate.
(×480)
(a) (b)
old area of medullary infarction. Staging studies Pathology

almost invariably show that the tumour has spread
beyond the bone. Macroscopically the tumour is lobulated and often
Histologically it is a fibrous tumour, but the fairly large. It may look grey (like brain) or red (like
arrangement of the tissue is in interweaving bundles, redcurrant jelly) if haemorrhage has occurred into it.
and the presence of histiocytes and of giant cells dis- Microscopically, sheets of small dark polyhedral cells
tinguishes it from the more uniform fibrosarcoma. with no regular arrangement and no ground sub-
stance are seen.
Treatment Treatment consists of wide or radical
resection and adjuvant chemotherapy. For inaccessible
lesions, local radiotherapy may be needed. Diagnosis
The condition which should be excluded as rapidly as
possible is bone infection. On biopsy the essential step
EWING’S SARCOMA is to recognize this as a malignant round-cell tumour,
distinct from osteosarcoma. Other round-cell
Ewing’s sarcoma is believed to arise from endothelial tumours that may resemble Ewing’s are reticulum-cell
cells in the bone marrow. It occurs most commonly sarcoma (see below) and metastatic neuroblastoma.
between the ages of 10 and 20 years, usually in a
tubular bone and especially in the tibia, fibula or
clavicle.
The patient presents with pain – often throbbing
in character – and swelling. Generalized illness and
pyrexia, together with a warm, tender swelling and
a raised ESR, may suggest a diagnosis of osteomyelitis.
Imaging
X-rays usually show an area of bone destruction
which, unlike that in osteosarcoma, is predominantly
in the mid-diaphysis. New bone formation may
extend along the shaft and sometimes it appears as
fusiform layers of bone around the lesion – the so-
called ‘onion-peel’ effect. Often the tumour extends
into the surrounding soft tissues, with radiating
streaks of ossification and reactive periosteal bone at
the proximal and distal margins. These features (the
‘sunray’ appearance and Codman’s triangles) are usu-
ally associated with osteosarcoma, but they are just as
common in Ewing’s sarcoma. (a) (b) (c)
CT and MRI reveal the large extraosseous compo- 9.33 Ewing’s tumour Examples of Ewing’s tumour in
nent. Radioisotope scans may show multiple areas of (a) the humerus, (b) the mid-shaft of the fibula and
212 activity in the skeleton. (c) the lower end of the fibula.
9
Tumours
(a) (b)
9.34 Ewing’s tumour – histology There is a
monotonous pattern of small round cells clustered around 9.35 Non-Hodgkin’s lymphoma (a) X-ray showing a
blood vessels. (×480) rather nondescript moth-eaten appearance of the ilium.
(b) MRI reveals the extent of the soft-tissue lesion.
Treatment
The prognosis is always poor and surgery alone does
little to improve it. Radiotherapy has a dramatic effect
on the tumour but overall survival is not much
enhanced. Chemotherapy is much more effective,
offering a 5-year survival rate of about 50 per cent
(Souhami and Craft, 1988; Damron et al., 2007).
The best results are achieved by a combination of
all three methods: a course of preoperative neoadju-
vant chemotherapy; then wide excision if the tumour
is in a favourable site, or radiotherapy followed by
local excision if it is less accessible; and then a further
course of chemotherapy for 1 year. Postoperative
radiotherapy may be added if the resected specimen is
found not to have a sufficiently wide margin of nor- 9.36 Non-Hodgkin’s lymphoma – histology There is
mal tissue. dense infiltration of abnormal lymphoid cells (a typical
‘round-cell tumour’), which is distinguished from Ewing’s
The prognosis for these tumours has improved dra- by the characteristic distribution of reticulin around
matically since the introduction of multi-agent collections of cells and between individual cells. (×200;
chemotherapy – from an erstwhile 10 per cent survival special reticulin stain)
rate to the current 70 per cent for patients with non-
metastatic Ewing’s sarcoma.
reticulin stains are needed to show the fine fibrillar
network that helps to distinguish the picture from
NON-HODGKIN’S LYMPHOMA that of Ewing’s sarcoma.
(RETICULUM-CELL SARCOMA) Treatment The preferred treatment is by chemother-
apy and radical resection; radiotherapy is reserved for
Like Ewing’s sarcoma, this is a round-cell tumour of
less accessible lesions.
the reticuloendothelial system. It is usually seen in
sites with abundant red marrow: the flat bones, the
spine and the long-bone metaphyses. The patient,
usually an adult of 30–40 years, presents with pain or MULTIPLE MYELOMA
a pathological fracture.
Multiple myeloma is a malignant B-cell lymphopro-
X-ray shows a mottled area of bone destruction in
liferative disorder of the marrow, with plasma cells
areas that normally contain red marrow; the radioiso-
predominating. The effects on bone are due to
tope scan may reveal multiple lesions.
marrow cell proliferation and increased osteoclastic
Pathology Histologically this is a marrow-cell tumour activity, resulting in osteoporosis and the appearance of
with collections of abnormal lymphocytes. Special discrete lytic lesions throughout the skeleton. A 213
9 particularly large colony of plasma cells may form sical’ lesions are multiple punched-out defects with
what appears to be a solitary tumour (plasmacytoma) ‘soft’ margins (lack of new bone) in the skull, pelvis
in one of the bones, but sooner or later most of these and proximal femur, a crushed vertebra, or a solitary
cases turn out to be unusual examples of the same lytic tumour in a large-bone metaphysis.
widespread disease.
Associated features of the marrow-cell disorder are
Investigations
plasma protein abnormalities, increased blood

viscosity and anaemia. Bone resorption leads to Mild anaemia is common, and an almost constant fea-
hypercalcaemia in about one-third of cases. Late sec- ture is a high ESR. Blood chemistry may show a raised
ondary features are due to renal dysfunction and creatinine level and hypercalcaemia. Over half the
spinal cord or root compression caused by vertebral patients have Bence Jones protein in their urine, and
collapse. serum protein electrophoresis shows a characteristic
The patient, typically aged 45–65, presents with abnormal band. A sternal marrow puncture may show
weakness, backache, bone pain or a pathological frac- plasmacytosis, with typical ‘myeloma’ cells.
ture. Hypercalcaemia may cause symptoms such as
thirst, polyuria and abdominal pain. Clinical signs
(apart from a pathological fracture) are often unre-
Diagnosis
markable. Localized tenderness and restricted hip If the only x-ray change is osteoporosis, the differen-
movements could be due to a plasmacytoma in the tial diagnosis must include all the other causes of bone
proximal femur. In late cases there may be signs of loss. If there are lytic lesions, the features can be simi-
cord or nerve root compression, chronic nephritis and lar to those of metastatic bone disease.
recurrent infection. Paraproteinaemia is a feature of other (benign)
gammopathies; it is wise to seek the help of a haema-
tologist before reaching a clinical diagnosis.
X-rays
X-rays often show nothing more than generalized
Pathology
osteoporosis; but remember that myeloma is one of the
commonest causes of osteoporosis and vertebral compres- At operation the affected bone is soft and crumbly.
sion fracture in men over the age of 45 years. The ‘clas- The typical microscopic picture is of sheets of plasma-
9.37 Myeloma The characteristic x-ray features are bone rarefaction, vertebral compression fractures, expanding lesions
214 (typically in the ribs and pelvis) and punched-out areas in the skull and the long bones.
The patient complains of longstanding backache. 9
The tumour expands anteriorly and, if it involves the
sacrum, may eventually (after months or even years)
cause rectal or urethral obstruction; rectal examina-
tion may disclose the presacral mass. In late cases
there may also be neurological signs.
Tumours
X-ray shows a radiolucent lesion in the sacrum. CT
and MRI reveal the extent of intrapelvic enlargement.
Treatment This is a low-grade tumour, though often
with extracompartmental spread. After wide excision
there is little risk of recurrence. However, attempts to
prevent damage to the pelvic viscera usually result in
inadequate surgery (intralesional or close marginal
excision) and consequently a greater risk of recur-
rence. If there are doubts in this regard, operation
9.38 Myeloma – histology There are dense sheets of
plasma cells with eccentric nuclei. (×480) should be combined with local radiotherapy.
cytes with a large eccentric nucleus containing a ADAMANTINOMA

spoke-like arrangement of chromatin.
This rare tumour has a predilection for the anterior
cortex of the tibia but is occasionally found in other
Treatment
long bones. The patient is usually a young adult who
The immediate need is for pain control and, if neces- complains of aching and mild swelling in the front of
sary, treatment of pathological fractures. General sup- the leg. On examination there is thickening and
portive measures include correction of fluid balance tenderness along the subcutaneous border of the
and (in some cases) hypercalcaemia. tibia.
Limb fractures are best managed by internal fixa- X-ray shows a typical bubble-like defect in the
tion and packing of cavities with methylmethacrylate anterior tibial cortex; sometimes there is thickening of
cement (which also helps to staunch the profuse the surrounding bone.
bleeding that sometimes occurs). Perioperative antibi- Adamantinoma is a low-grade tumour which metas-
otic prophylaxis is important as there is a higher than tasizes late – and usually only after repeated and inad-
usual risk of infection and wound breakdown. equate attempts at removal. Early on it is confined to
Spinal fractures carry the risk of cord compression bone; later, CT may show that the tumour has
and need immediate stabilization – either by effective extended inwards to the medullary canal or outwards
bracing or by internal fixation. Unrelieved cord pres- beyond the periosteum.
sure may need decompression.
Pathology The histological picture varies considerably
Solitary plasmacytomas can be treated by radio-
but the most typical features are islands of epithelial-
therapy.
like cells in a densely-populated stroma of spindle
Specific therapy is with alkylating cytotoxic agents
cells; the ‘epithelial’ nests may have an acinar arrange-
(e.g. melphalan). Corticosteroids are also used –
ment.
especially if bone pain is marked – but this probably
does not alter the course of the disease. Treatment Treatment If the diagnosis is made reasonably early,
should be carried out in a specialized unit where wide local excision with a substantial margin of nor-
dosages and response parameters can be properly mal bone is adequate. Preoperative CT and MRI are
monitored. essential to determine how deep the tumour pene-
The prognosis in established cases is poor, with a trates; if it is confined to the anterior cortex, the pos-
median survival of between 2 and 5 years. terior cortex can be preserved and this makes
reconstruction much easier. If the lesion extends to
the endosteal surface, a full segment of bone must be
CHORDOMA excised; the gap is filled with a vascularized graft or a
suitable endoprosthesis, or managed by distraction
This rare malignant tumour arises from primitive osteogenesis (see Chapter 12).
notochordal remnants. It affects young adults and If there has been more than one recurrence, or if
usually presents as a slow-growing mass in the sacrum; the tumour extends into the surrounding soft tissues,
however, it may occur elsewhere along the spine. radical resection or amputation is advisable. 215
9 9.39 Adamantinoma
(a) The bubble-like
appearance in the mid-shaft
of the tibia is typical.
(b) Histology shows clusters
of epithelial-like cells,
sometimes with an acinar
arrangement in a
moderately cellular fibrous
stroma. (×300)
(a) (b)
occasionally, visceral tumours spread directly to adja-

METASTATIC BONE DISEASE cent bones (e.g. the pelvis or ribs).
Metastases are usually osteolytic, and pathological frac-
The skeleton is one of the commonest sites of sec- tures are common. Bone resorption is due either to the
ondary cancer; in patients over 50 years bone metastases direct action of tumour cells or to tumour-derived factors
are seen more frequently than all primary malignant that stimulate osteoclastic activity. Osteoblastic lesions are
bone tumours together. The commonest source is car- uncommon; they usually occur in prostatic carcinoma.
cinoma of the breast; next in frequency are carcino-
mas of the prostate, kidney, lung, thyroid, bladder and
gastrointestinal tract. In about 10 per cent of cases no
primary tumour is found.
Clinical features
The commonest sites for bone metastases are the The patient is usually aged 50–70 years; with any
vertebrae, pelvis, the proximal half of the femur and destructive bone lesion in this age group, the differ-
the humerus. Spread is usually via the blood stream; ential diagnosis must include metastasis.
(a) (b) (c) (d)
9.40 Metastatic tumours (a,b) This patient presented with pain in the right upper thigh. X-ray showed what appeared
to be a single metastasis in the upper third of the femur. However, the radioisotope scan revealed many deposits in other
parts of the skeleton. (c) Patients over 60 with vertebral compression fractures may simply be very osteoporotic, but they
should always be investigated for metastatic bone disease and myelomatosis. (d) Prophylactic nailing for a femoral
216 metastasis which might otherwise have resulted in a pathological fracture.
Pain is the commonest – and often the only – clin- particularly to solitary renal cell, breast and thyroid 9
ical feature. The sudden appearance of backache or tumour metastases; but in the great majority of cases,
thigh pain in an elderly person (especially someone and certainly in those with multiple secondaries, treat-
known to have been treated for carcinoma in the past) ment is entirely symptomatic. For that reason, elabo-
is always suspicious. If x-rays do not show anything, a rate witch-hunts to discover the source of an occult
radionuclide scan might. primary tumour are avoided, though it may be worth-
Tumours
Some deposits remain clinically silent and are dis- while investigating for tumours that are amenable to
covered incidentally on x-ray examination or bone hormonal manipulation.
scanning, or after a pathological fracture. Sudden col-
lapse of a vertebral body or a fracture of the mid-shaft Prognosis
of a long bone in an elderly person are ominous signs;
if there is no history and no clinical clue pointing to a Bauer (1995) has suggested useful criteria for assess-
primary carcinoma, a biopsy of the fracture area is ing prognosis (see Box). In his series of patients, sur-
essential. vivorship at 1 year was as follows:
Symptoms of hypercalcaemia may occur (and are • of patients with 4 or 5 of Bauer’s criteria 50 per
often missed) in patients with skeletal metastases. cent were alive
These include anorexia, nausea, thirst, polyuria,
• of patients with 2 or 3 criteria 25 per cent were alive
abdominal pain, general weakness and depression.
• of patients with only 1 or none of the criteria, the
In children under 6 years of age, metastatic lesions
majority survived for less than 6 months and none
are most commonly from adrenal neuroblastoma. The
were alive at 1 year.
child presents with bone pain and fever; examination
reveals the abdominal mass.
Palliative care
Imaging Despite a poor prognosis, patients deserve to be made
comfortable, to enjoy (as far as possible) their remain-
X-rays Most skeletal deposits are osteolytic and ing months or years, and to die in a peaceful and dig-
appear as rarified areas in the medulla or produce a nified way. The active treatment of skeletal metastases
moth-eaten appearance in the cortex; sometimes contributes to this in no small measure. In addition,
there is marked bone destruction, with or without a patients need sympathetic counselling and practical
pathological fracture. Osteoblastic deposits suggest a assistance with their material affairs.
prostatic carcinoma; the pelvis may show a mottled
Control of pain and metastatic activity Most patients
increase in density which has to be distinguished from
require analgesics, but the more powerful narcotics
Paget’s disease or lymphoma.
should be reserved for the terminally ill.
Radioscintigraphy Bone scans with 99mTc-MDP are the Unless specifically contraindicated, radiotherapy is
most sensitive method of detecting ‘silent’ metastatic used both to control pain and to reduce metastatic
deposits in bone; areas of increased activity are growth. This is often combined with other forms of
selected for x-ray examination. treatment (e.g. internal fixation).
Secondary deposits from breast or prostate can
often be controlled by hormone therapy: stilboestrol
Special investigations for prostatic secondaries and androgenic drugs or
The ESR may be increased and the haemoglobin con- oestrogens for breast carcinoma. Disseminated sec-
centration is usually low. The serum alkaline phos- ondaries from breast carcinoma are sometimes treated
phatase concentration is often increased, and in pro- by oophorectomy combined with adrenalectomy or
static carcinoma the acid phosphatase also is elevated. by hypophyseal ablation.
Patients with breast cancer can be screened by
measuring blood levels of tumour-associated antigen
markers. BAUER’S POSITIVE CRITERIA FOR SURVIVAL
A solitary metastasis
Treatment
No pathological fracture
By the time a patient has developed secondary
deposits the prognosis for survival is poor. Occasion- No visceral metastases
ally, radical treatment (combined chemotherapy,
Renal or breast primary
radiotherapy and surgery) targeted at a solitary sec-
ondary deposit and the parent primary lesion may be No lung cancer
rewarding and even apparently curative. This applies 217
9 Hypercalcaemia may have serious consequences, Table 9.4 Mirel’s scoring system for metastatic bone
disease
including renal acidosis, nephrocalcinosis, uncon-
sciousness and coma. It should be treated by ensuring
Score 1 2 3
adequate hydration, reducing the calcium intake and,
if necessary, administering bisphosphonates. Site Upper limb Lower limb Peritrochanteric
Pain Mild Moderate Functional
Treatment of limb fractures Surgical timidity may con-

Lesion Blastic Mixed Lytic
demn the patient to a painful lingering death, so shaft
fractures should almost always be treated by internal Size* <1/3 1/3–2/3 >2/3
fixation and (if necessary) packing with methyl- *As seen on plain x-ray, maximum destruction of cortex in any view.
methacrylate cement. If there are multiple fractures, Maximum possible score is 12. If the lesion scores 8 or above, then
more than one bone may be fixed at the same sitting, prophylactic fixation is recommended prior to radiotherapy.
though one must bear in mind that the risk of fat

embolism increases with multiple intramedullary nail-
ing. Pain is immediately relieved, nursing is made eas- Between 41 and 70 per cent of all malignant tumours
ier and the patient can get up and about or attend for have a spinal metastasis, mostly in the thoracic spine
other types of treatment without unnecessary discom- and mainly in the vertebral body. The aims of inter-
fort. Shaft fractures usually unite satisfactorily. vention are to decrease pain, preserve the ability to
In most cases intramedullary nailing is the most walk, maintain urinary and faecal continence and pro-
effective method; fractures near joints (e.g. the distal long survival.
femur or proximal tibia) may need fixation with plates Pathological fractures usually require some form of
or blade-plates, and sometimes replacement by an support. If the spine is still completely stable, a well-
endoprosthesis. fitting brace may be sufficient. However, spinal insta-
Fractures of the femoral neck rarely, if ever, unite. bility may cause severe pain, making it almost
They are best treated by prosthetic replacement: a impossible for the patient to sit or stand – with or
hemiarthroplasty if the pelvis is intact, or total joint without a brace. For these patients, operative stabi-
replacement if the acetabulum is involved. If the lization is indicated – either posterior or anterior
pelvic wall is destroyed, it can be reconstructed by spinal fusion, depending on the individual need. Pre-
large bone grafts, a reconstruction cage or a custom- operative assessment should include CT or MRI to
made prosthesis; however, if such extensive surgery is establish whether the cord is threatened; if it is, spinal
contraindicated, one may have to settle for a simple decompression should be carried out at the same
excisional arthroplasty. time. If there are overt symptoms and signs of cord com-
Postoperative irradiation is essential to prevent fur- pression, treatment is urgent.
ther extension of the metastatic lesion. Other forms of surgery sometimes called for are
Prophylactic fixation Large deposits that threaten to
debulking of the tumour or removal of a solitary
result in fracture should be treated by internal fixation metastasis by vertebrectomy and reconstruction.
while the bone is still intact. As a rule of thumb, Operative intervention appears to provide a better
where 50 per cent of a single cortex of a long bone (in functional outcome than radiotherapy. Patients
any radiological view) has been destroyed, pathologi- remain ambulatory and continent for longer and the
cal fracture should be regarded as inevitable. In addi- 5-year survival rate is around 18 per cent. In general,
tion, avulsion of the lesser trochanter is an indication radiotherapy alone is reserved for patients with soft-
of imminent hip fracture. tissue compression and as palliation for inoperable
Mirels devised a scoring system (Table 9.4) to eval- cases.
uate fracture risk and therefore act as a guide as to It is well to remember that radiotherapy used as a
whether (and when) a fracture should be fixed or not. preoperative adjunct has been shown to increase the
A score of 8 or more indicates a high risk and a need postoperative infection rate (Jeys et al., 2005).
for internal fixation to be carried out prior to radio-
therapy (Mirels, 1989).
The principles of fixation are the same as for the
SOFT-TISSUE TUMOURS
management of fractures in general. A preoperative
radionuclide scan will show whether other lesions are
present in that bone, thus calling for more extensive Benign soft-tissue tumours are common, malignant
fixation and postoperative radiotherapy. ones rare. The distinction between these two groups
is not always easy, and some lesions, treated confi-
Treatment of metastatic spinal disease Metastatic spinal dently as ‘benign’, recur in more aggressive form after
disease is 40 times more common than all primary inadequate removal. Features suggestive of malig-
218 tumours of the spine together (Galasko et al., 2000). nancy are: pain in a previously painless lump; a rapid
increase in size; a lump deep to the fascia; size greater If the lump is troublesome it may be removed by 9
than 5 cm; poor demarcation; and attachment to the marginal excision. Prior biopsy is usually unnecessary;
surrounding structures. however, one should never be complacent about a
As with bone tumours, special imaging and staging ‘lipoma’ and if there are any atypical features, preop-
should be carried out before the field is disturbed by erative staging and biopsy are essential in order to
operation. Chest x-rays and blood investigations may avoid the risk of performing a marginal excision and
Tumours
also be necessary. If the imaging is conclusive then the then discovering that the lesion was malignant.
lesion can be removed with either a marginal or wide
excision biopsy, dependent on the diagnosis. Alterna- LIPOSARCOMA
tively, a biopsy to confirm the diagnosis should be Liposarcoma is rare but should be suspected if a fatty
undertaken prior to excision. tumour (especially in the buttock, the thigh or the
The role of chemotherapy for soft-tissue sarcomas is popliteal fossa) goes on growing and becomes painful.
uncertain, except in the treatment of rhabdomyosar- The lump may feel quite firm and is usually not
coma and synovial sarcoma. translucent. CT or MRI is essential to determine the
Radiotherapy is indicated for all high-grade lesions extent of the tumour.
and for tumours that are removed with poor margins Treatment depends on the degree of malignancy. Low-
or by intralesional excision. If margins are contami- grade lesions can be removed by wide excision; high-
nated then re-operation with wide resection of that grade tumours need radical resection. For liposarcomas
margin must be performed. in inaccessible sites, radiation therapy is often effective.
The account that follows is intended as a summary
of those soft-tissue tumours likely to be encountered
in orthopaedics. FIBROUS TUMOURS
FIBROMA
FATTY TUMOURS The common fibroma is a solitary, benign tumour of
fibrous tissue. It is usually discovered as a small
LIPOMA asymptomatic nodule or lump. Treatment is not
A lipoma, one of the commonest of all tumours, may essential; if it is removed, a marginal excision is ade-
occur almost anywhere; sometimes there are multiple quate.
lesions. The tumour usually arises in the subcutaneous
layer. It consists of lobules of fat with a surrounding FIBROMATOSIS
capsule which may become tethered to neighbouring This term encompasses a group of well-differentiated
structures. The patient, usually aged over 50, com- fibrous lesions that typically infiltrate the tissues,
plains of a painless swelling. The lump is soft and sometimes in an aggressive manner. They have a
almost fluctuant; the well-defined edge and lobulated strong tendency to recur after local excision but they
surface distinguish it from a chronic abscess. Fat is do not metastasize.
notably radiotranslucent, a feature that betrays the The lesions appear in various forms, divided broadly
occasional sub-periosteal lipoma. into superficial fibromatoses (comprising clinical
9.41 Fatty tumours

(a) Subcutaneous lipoma in the
thigh. Like so many lipomas,
this one felt almost fluctuant;
(b) intramuscular lipoma;
(c) liposarcoma – the cortex of
the fibula has been eroded.
(a) (b) (c) 219

9 entities as diverse as Dupuytren’s contracture, SYNOVIAL TUMOURS
Peyronie’s disease and thickened fibrous plaques
elsewhere in superficial mesenchymal tissues), and PIGMENTED VILLONODULAR SYNOVITIS AND GIANT-
more aggressive but comparatively rare deep fibro- CELL TUMOUR OF TENDON SHEATH
matoses, or desmoid tumours, which usually appear in These are two forms of the same condition – a benign
young adults as thick cords or plaques in the subcuta- disorder that occurs wherever synovial membrane is
neous tissues of the limbs or trunk where they grow found: in joints, tendon sheaths or bursae.
into featureless masses with ill-defined margins. CT Pigmented villonodular synovitis (PVNS) presents as
and MRI are useful to show the extent of this invasive a longstanding boggy swelling of the joint – usually the
tumour. hip, knee or ankle – in an adolescent or young adult. X-
After local excision, desmoid tumours often recur ray may show excavations in the juxta-articular bone on
in increasingly invasive form, threatening nearby neu- either side of the joint. When the joint is opened, the
rovascular structures. Pressure on nerves may cause synovium is swollen and hyperplastic, often covered
paraesthesiae. A particularly hazardous situation arises with villi and golden-brown in colour – the effect of
when the tumour, after several attempts at eradica- haemosiderin deposition. The juxta-articular excava-
tion, infiltrates into the axilla or pelvis; once this tions contain clumps of friable synovial material.
occurs, complete removal may be impossible. Tendon sheath lesions are seen mainly in the hands
and feet, where they cause nodular thickening of the
Pathology Microscopically these lesions vary from
affected sheath. X-ray may show pressure erosion of
those with clearly benign cells to some whose appear-
an adjacent bone surface – for example, on one of the
ance suggests malignancy (multinucleated cells with
phalanges. At operation the boggy synovial tissue is
many mitoses). Differentiation from fibrosarcoma
often yellow; this type of lesion is sometimes called
may be difficult and demands considerable histologi-
xanthoma of tendon sheath.
cal expertise, but it is important because fibromatosis
does not metastasize and can be eradicated if surgery Pathology Histologically, joint and tendon sheath
is sufficiently thorough. lesions are identical. There is proliferation and hyper-
trophy of the synovium, which contains fibroblastic
Treatment Although the tumour sometimes regresses
tissue with foamy histiocytes and multinucleated giant
spontaneously, the most predictable results are
cells. These features have engendered yet another
achieved by a combination of wide excision and radi-
name for the same condition: giant-cell tumour of ten-
ation therapy (Pritchard et al., 1996). The risk of local
don sheath.
recurrence is strongly related to the adequacy of the
margin of resection. Intralesional and marginal resec- Treatment The only effective treatment is synovec-
tions result in more than twice the recurrence rate fol- tomy. Although the tumour does not undergo malig-
lowing resection well beyond the tumour margins. nant change, the recurrence rate is high unless excision
Non-operative treatment has been tried for lesions is complete. This may be unattainable and subtotal
that are inaccessible or where several attempts at sur- synovectomy is then sometimes combined with local ra-
gical removal have failed. The most promising results diotherapy. If, despite such aggressive treatment, there
thus far reported have been achieved by the use of are repeated recurrences, it may be necessary to sacri-
hormonal agents (e.g. tamoxifen, an anti-oestrogen fice the joint and carry out arthroplasty or arthrodesis.
preparation) and cytotoxic chemotherapy (Janinis et
al., 2003). SYNOVIAL SARCOMA
This malignant tumour usually develops near synovial
FIBROSARCOMA joints in adolescents and young adults. However, only
Fibrosarcoma may occur in any area of connective about 20 per cent involve the joint itself and the term
tissue but is more common in the extremities. It pres- ‘synovioma’ is a misnomer because this is not a
ents as an ill-defined, painless mass and may grow to tumour of synovium, though the histological appear-
a considerable size. The diagnosis is usually made only ance may resemble that of synovium.
after biopsy and histological examination. Local The patient usually complains of rapid enlargement
extension can be shown on MRI. There may be of a lump around one of the larger joints – the hip, the
metastases in the lungs. knee or the shoulder. Occasionally the tumour
High-grade lesions showing atypical spindle cells presents as a small swelling in the hand or foot and the
are usually easy to diagnose. Low-grade lesions may histological diagnosis comes as a complete surprise.
be difficult to distinguish from fibromatosis. Pain is a common feature and many lesions are pres-
For low-grade lesions, wide excision is usually suffi- ent for years before they are diagnosed. X-rays show a
cient. For high-grade lesions, wide excision should be soft-tissue mass, sometimes with extensive calcifica-
supplemented by preoperative and postoperative radi- tion. MRI will help to outline the tumour.
220 ation therapy. Biopsy reveals a fleshy lesion composed of prolifera-
9
Tumours
(a) (b)
9.42 Pigmented villonodular synovitis (a) A farmer presented with pain in the hip. The x-rays showed cystic
excavations on both sides of the joint and at first suggested tuberculosis. However, there were no signs of
infection. At operation the synovium was thick and golden in colour. (b) The biopsy showed dense proliferation
of the synovium with scattered multinucleated giant cells and haemosiderin. (×120)
tive ‘synovial’ cells and fibroblastic tissue; characteris- BLOOD VESSEL TUMOURS
tically the cellular areas are punctured by vacant slits
that give the tissue an acinar appearance. Cellular HAEMANGIOMA
abnormality and mitoses reflect the degree of malig- This benign lesion, probably a hamartoma, is usually
nancy. seen during childhood but may be present at birth. It
Small, well-defined lesions can be treated by wide occurs in two forms. The capillary haemangioma is
excision. High-grade lesions, which usually have ill- more common; it usually appears as a reddish patch
defined margins, require radical resection – and this may on the skin, and the congenital naevus or ‘birthmark’
mean radical amputation. Resection may be combined is a familiar example. A cavernous haemangioma con-
with radiotherapy and occasionally chemotherapy. sists of a sponge-like collection of blood spaces; super-
ficial lesions appear as blue or purple skin patches,
sometimes overlying a soft subcutaneous mass; deep
lesions may extend into the fascia or muscles, and
occasionally an entire limb is involved. X-rays may
show calcified phleboliths in the cavernous lesions.
There is no risk of malignant change and treatment
is needed only if there is significant discomfort or dis-
ability. Local excision carries a high risk of recurrence,
but more radical procedures seem unnecessarily
destructive. Preoperative embolization of feeding
vessels may reduce intra-operative bleeding.
GLOMUS TUMOUR
This rare tumour usually occurs around fine periph-
eral neurovascular structures, and especially in the nail
9.43 Malignant synoviomas X-rays showing the beds of fingers or toes. A young adult presents with
so-called ‘snowstorm’ appearance. recurrent episodes of intense pain in the fingertip. A 221
9 small bluish nodule may be seen under the nail; the
area is sensitive to cold and exquisitely tender. X-rays
sometimes show erosion of the underlying phalanx. L1
L1
Treatment is excision; the tumour, never larger than a
pea, is easily shelled out of its fibrous capsule.
L2
L2
NERVE TUMOURS
NEUROMA
A neuroma is not a tumour but an overgrowth of
fibrous tissue and randomly sprouting nerve fibrils fol-
lowing injury to a nerve. It is often tender and local
percussion may induce distal paraesthesiae, thus indi-
cating the level of the lesion (Tinel’s sign).
Treatment can be frustrating. If the neuroma is (a) (b)
excised (or as a prophylactic measure during amputa-
tion) the epineural sleeve can be freed from the nerve 9.44 Neurofibromatosis (a) The anteroposterior x-ray
shows erosion of the pedicles of L1 and L2. Compare the
fascicles and sealed with a synthetic tissue adhesive. appearance with the well-marked pedicles (like staring
eyes) at L3 and L4. (b) The lateral view shows scalloping of
NEURILEMMOMA the backs of L1 and L2.
Neurilemmoma is a benign tumour of the nerve
sheath. It is seen in the peripheral nerves and in the
spinal nerve roots. The patient complains of pain or where it presumably originates in fine nerve fibrils. Oc-
paraesthesiae; sometimes there is a small palpable casionally it arises directly in bone; more often it causes
swelling along the course of the nerve. pressure erosion of an adjacent surface.
Growth on a spinal nerve root is a rare cause of Lesions may be solitary or multiple. Curiously, they
‘sciatica’, and x-rays of the spine may show erosion of the are sometimes associated with skeletal abnormalities
intervertebral foramen at that level. MRI will demon- (scoliosis, pseudarthrosis of the tibia) or overgrowth
strate the eccentric swelling on a peripheral nerve. of a digit or an entire limb, in which there is no obvi-
With careful dissection the tumour can be removed ous neural pathology.
from its capsule without damage to the nerve. The patient may present with a lump overlying one
of the peripheral nerves, or with neurological symp-
NEUROFIBROMA toms such as paraesthesiae or muscle weakness. If a
This is a benign tumour of fibrous and neural elements; nerve root is involved, symptoms can mimic those of
its origin in a peripheral nerve may be obvious, but it is a disc prolapse; x-rays may show erosion of a vertebral
also seen as a nodule in the skin or subcutaneous tissues pedicle or enlargement of the intervertebral foramen.
(a) (b) (c) (d)
9.45 Neurofibromatosis (a) Café-au-lait spots, (b) multiple fibromata and slight scoliosis; (c,d) a patient with scoliosis
222 and elephantiasis.
Multiple neurofibromatosis (von Recklinghausen’s a young adult – presents with ache and an enlarging, 9
disease) is transmitted by autosomal dominant inheri- ill-defined lump that moves with the affected muscle.
tance (see page 175). Patients (usually children) CT and MRI show that the mass is in the muscle, but
develop numerous skin nodules and café-au-lait the edge may be poorly demarcated because the
patches; there may be associated skeletal abnormali- tumour tends to spread along the fascial planes. At
ties. Malignant transformation is said to occur in 5–10 biopsy the tissue looks and feels different from normal
Tumours
per cent of cases. muscle and microscopic examination shows clusters of
highly abnormal muscle cells.
Pathology The pathological appearance of the indi-
This is a high-grade lesion which requires radical re-
vidual tumour is characteristic: on cross-section the
section of the affected muscle, i.e. from its origin to its
lesion consists of pale fibrous tissue with nerve ele-
insertion. If this cannot be assured or if the tumour has
ments running into and through the substance of the
spread beyond the fascial sheath, amputation is advis-
tumour. Microscopically, the fibrillar and cellular ele-
able. Recurrent lesions are also treated by amputation.
ments are arranged in a wavy pattern.
If complete removal is impossible, adjunctive radio-
Treatment Treatment is needed only if pain or paraes- therapy may lessen the risk of recurrence.
thesiae become troublesome, or if a tumour becomes
very large. However, the tumour cannot be completely
separated from intact nerve fibres; if it involves an
unimportant nerve, it can be excised en bloc; if nerve REFERENCES AND FURTHER READING
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224
Neuromuscular
disorders 10
Deborah Eastwood, Thomas Staunton, Louis Solomon
ble of receiving signals from other neuronal terminals.

NERVES AND MUSCLES A finer, longer branch – the axon – carries the action
potentials along its length to or from excitable target
NEURONS organs. Further signal transmission to the dendrites of
another neuron, or neuro-excitable tissue like muscle,
The neuron is the defining unit of the nervous system. occurs at a synapse where the axon terminal releases a
It is a specialized cell, capable of electrical excitation chemical neurotransmitter – typically acetylcholine.
and conduction of electrochemical impulses (action All motor axons and the larger sensory axons serv-
potentials) along its thread-like extensions. Its basic ing touch, pain and proprioception are covered by a
structure consists of a cell body, 5–25 μm in diameter, sheath – the neurilemma – and coated with myelin, a
with branching processes – dendrites – that are capa- multilayered lipoprotein substance derived from the
accompanying Schwann cells (or oligodendrocytes in
the central nervous system). Every few millimetres the
myelin sheath is interrupted, leaving short segments of
bare axon called the nodes of Ranvier. In these nerves
Dendrites the myelin coating serves as an insulator, which allows
the impulse to be propagated by electromagnetic con-
duction from node to node, much faster than is the
Cell body case in unmyelinated nerves. Consequently, depletion
of the myelin sheath causes slowing – and eventually
complete blocking – of axonal conduction.
Most axons, in particular the small-diameter fibres
Nucleus carrying crude sensation and efferent sympathetic
fibres, are not myelinated but wrapped in Schwann
Axon cell cytoplasm. Damage to these axons causes unpleas-
ant or bizarre sensations and abnormal sudomotor
and vasomotor effects.
Myelin sheath
NERVOUS PATHWAYS
Schwann cells
Anatomically, neurological structures can be divided
into the central nervous system (the CNS, comprising
the brain and tracts of the spinal cord) and the periph-
Ranvier’s node
eral nervous system (PNS) which includes the cranial
and spinal nerves. In terms of physiological function,
both the CNS and the PNS have a somatic compo-
nent and an autonomic component.
The somatic nervous system provides efferent motor
and afferent sensory pathways to and from peripheral
Terminal bouton
parts of the body serving, respectively, voluntary mus-
10.1 Diagram of a typical neuron cle contraction and sensibility.
10 Spinothalamic and enter the contralateral spinothalamic tracts run-
tract ning up the spinal cord to the brain. Fibres from sen-
sors in the joints, ligaments, tendons and muscle
Posterior Pyramidal
columns tracts carrying the sense of movement and bodily position in
Dorsal Dorsal space (proprioceptive sensation) join the ipsilateral pos-
root root terior columns in the spinal cord.
ganglion
Sensory areas (dermatomes) corresponding to the
spinal nerve roots are shown in Figure 10.5. However,
it should be remembered that there is considerable
overlap of the boundaries shown in these body maps;
furthermore, some parts, such as the hands and lips, are
more sensitive and discriminatory than others.
REFLEX ACTIVITY AND TONE

Sudden stretching of a muscle (e.g. by tapping sharply
Spinal Ventral Motor
nerve root neuron
over the tendon) induces an involuntary muscle con-
traction – the stretch reflex. The sharp change in mus-
10.2 Main nerve pathways Simplified diagram showing cle fibre length is detected by the muscle spindle; the
the main neurological pathways to and from a typical impulse is transmitted rapidly along myelinated affer-
thoracic spinal cord segment. Fibres carrying touch, sharp ent (sensory) neurons which synapse directly with the
pain and temperature impulses (-------) decussate, in some corresponding segmental α-motor neurons in the
cases over several spinal segments, and ascend in the
contralateral spinothalamic tracts; those carrying vibration spinal cord, triggering efferent signals which stimulate
and proprioceptive impulses (——) enter the ipsilateral the muscle to contract. This is the basis of the famil-
posterior columns. Motor neurons (——) arise in the iar clinical tests for tendon reflexes, and is also the
anterior horn of the grey matter and innervate ipsilateral mechanism for maintaining normal muscle tone.
muscles. Segmental reflex activity is normally regulated by
motor impulses passing from the brain down the
The autonomic system controls involuntary reflex spinal cord. Interruption of the UMN pathways
and homeostatic activities of the cardiovascular sys- results in undamped reflex muscle contraction (clini-
tem, visceral organs and glands. Its two components, cally hyperactive tendon reflexes) and spastic paralysis.
sympathetic and parasympathetic divisions, serve Damage to either afferent or efferent neurons in the
more or less opposing functions. reflex arc causes hypotonia; interruption of the LMN
pathway results in flaccid LMN paralysis.
SOMATIC MOTOR SYSTEM
Efferent impulses are conducted along axons in the AUTONOMIC SYSTEM
corticospinal or pyramidal tracts (upper motor neu- The autonomic system is involved with the regulation
rons – UMN) and along peripheral nerves from cell of involuntary activities of cardiac muscle and smooth
bodies in the anterior horn of the spinal cord to stri- (unstriated) muscle of the lungs, gastrointestinal tract,
ated muscle fibres (lower motor neurons – LMN). kidneys, bladder, genital organs, sweat glands and
The terminal synapses are situated at the neuromus- small blood vessels, with afferent (sensory) and effer-
cular junctions. Each large α-motor neuron inner- ent (motor) pathways constituting a continuously
vates from a few to several hundred muscle fibres active reflex arc (though there is also some input from
(together forming a motor unit) and stimulates mus- higher centres). In addition afferent fibres also convey
cle fibre contraction. In large muscles of the lower visceral pain sensation.
limb, power is adjusted by recruiting more or fewer The system is divided into sympathetic and parasym-
motor units. Smaller γ-motor neurons connect to sen- pathetic pathways, both of which comprise efferent
sors (muscle spindles) that control proprioceptive and afferent neurons.
feedback from muscle fibres. Preganglionic sympathetic neurons leave the spinal
cord with the ventral nerve roots at all levels from T1
SOMATIC SENSORY SYSTEM to L1, enter the paravertebral sympathetic chain of
Axons conveying afferent impulses from receptors in ganglia and synapse with postganglionic neurons that
the skin and other peripheral structures enter the dor- spread out to all parts of the body; they may also run
sal nerve roots, with their cell bodies in the dorsal up or down the sympathetic chain to synapse in other
root (or cranial nerve) ganglia, and end in synapses ganglia or pass on to become splanchnic nerves. Impor-
within the central nervous system. Myelinated fibres tant functions are the reflex control of heart rate, blood
carrying sensory stimuli from touch, pressure, pain flow and sweating, as well as other responses associated
226 and temperature (exteroceptive sensation) decussate with conditions of ‘fight and flight’.
Parasympathetic neurons leave the CNS (from the fibres, separated into bundles (or fascicles). Each fasci- 10
brain-stem) with cranial nerves III, VII, IX, X and cle is surrounded by a flimsy perimysium which
with the nerve roots of S2, 3 and 4 to reach ganglia envelops anything up to about 100 muscle fibres;
where they synapse with postganglionic neurons close large muscles concerned with mass movement, like
to their target organs. the glutei or quadriceps, have a large number of fibres
in each fascicle, while muscles used for precision
Neuromuscular disorders
movements (like those of the hand) have a much
PERIPHERAL NERVES smaller number in each bundle.
The muscle fibre is the important unit of all striated
Peripheral nerves are bundles of axons conducting muscle. Lying in a barely discernable connective tissue
efferent (motor) impulses from cells in the anterior cover, or endomysium, it is in actuality a single cell
horn of the spinal cord to the muscles, and afferent with a cell membrane (the sarcolemma), a type of
(sensory) impulses from peripheral receptors via cells cytoplasm (or sarcoplasm), mitochondria and many
in the posterior root ganglia to the cord. They also thousands of nuclei; its diameter is about 10 μm at
convey sudomotor and vasomotor fibres from gan- birth and 60–80 μm in mature adults.
glion cells in the sympathetic chain. Some nerves are The fibre itself consists of many tiny (1 μm diameter)
predominantly motor, some predominantly sensory; myofibrils, each of which is striated: dark bands con-
the larger trunks are mixed, with motor and sensory sisting of thick myosin filaments alternate with light
axons running in separate bundles. Detailed periph- bands of thin actin filaments (A and I bands respec-
eral nerve structure is described in Chapter 11. tively). In the middle of each A band is a lighter H zone
and in the middle of the I band there is a dark thin Z
line. The portion of the myofibril between two Z lines
SKELETAL MUSCLE is the sarcomere, representing a single contractile unit.
The α-motor neuron and the group of muscle
Each skeletal muscle belly, held within a connective fibres it supplies constitute a single motor unit; the
tissue epimysium, consists of thousands of muscle number of muscle fibres in the unit may be less than
five in muscles concerned with fine manipulatory
Ventral root
Dorsal root movements or more than 100 in those employed in
gross power movements.
Sensory ganglion
Nerve
Perimysium
Neuron Endomysium
Schwann cell
Fascicle Muscle
Myelin
Axon
Blood vessel
Muscle fibre
Endoneurium Muscle fibre
Perineurium
Sarcolemma
Fascicle Z band
Epineurium
Striated muscle
10.3 Nerve structure Diagram of the structural elements 10.4 Muscle structure Diagram showing the structural
of a peripheral nerve. elements of striated muscle. 227
10 Muscle fibres are also of different types, which can be not actually manifest themselves until later in child-
distinguished by histochemical staining. Type I fibres hood; cerebral palsy is the prime example. Conditions
contract slowly and are not easily fatigued; their prime such as poliomyelitis may affect anyone although chil-
function is postural control. Type II fibres are fast con- dren are most commonly afflicted. In contrast, spinal
tracting but they fatigue rapidly; hence they are ideally cord lesions and peripheral neuropathies are more
suited to intense activities of short duration. All mus- common in adults. The orthopaedic surgeon must be
cles consist of a mixture of fibre types, the balance ready to diagnose and treat neuromuscular disease
depending on anatomical site, basic muscle function, throughout life.
degree of training, genetic disposition and response to Past medical history may be relevant in terms of pre-
previous injury or illness. Long-distance runners have vious trauma (accidental or surgical), previous ill-
a greater proportion of type I fibres than the average nesses and their treatment (chemotherapy).
in age- and sex-matched individuals. Muscle weakness may be due to upper or lower
Muscle contraction is a complex activity. Individual motor neuron lesions (spastic versus flaccid paralysis)
myofibrils respond to electrical stimuli in much the but it may also be due to a primary muscle problem.
same way as do motor neurons. However, muscle The type and degree of weakness, the rate of onset,
fibres, and the muscle as a whole, are activated by whether it affects part of a limb, a whole limb, upper
overlap and summation of contractile responses. or lower limb, one side of the body or both sides – all
When the fibres contract, internal tension in the mus- these details should be enquired into and help to give
cle increases. In isometric contraction there is an insight into the aetiology.
increased tension without actual shortening of the Numbness and paraesthesiae may be the main com-
muscle or movement of the joint controlled by that plaints. It is important to establish their exact distri-
muscle. In isotonic contraction the muscle shortens bution to help localize the anatomical nature and level
and moves the joint, but tension within the muscle of the lesion accurately. The rate of onset and the rela-
fibres remains constant. tionship to posture may, similarly, suggest the cause.
Muscle tone is the state of tension in a resting mus- A history of trauma, including recent surgical proce-
cle when it is passively stretched; characteristically dures, or the use of a tourniquet must be noted.
tone is increased in upper motor neuron (UMN) Deformity is a common complaint in longstanding
lesions (spastic paralysis) and decreased in lower disorders. It arises as a result of muscle imbalances
motor neuron (LMN) lesions (flaccid paralysis). that may be very subtle and the deformity (such as
Muscle contracture (as distinct from contraction) is ‘claw toes’) may not be recognized until it is pointed
the adaptive change which occurs when a normally out to the patient.
innervated muscle is held immobile in a shortened Non-orthopaedic problems should also be discussed.
position for some length of time. If a joint is allowed It can be particularly important to note ‘throw-away’
to be held flexed for a long time, it may be impossible comments regarding problems such as headaches,
to straighten it passively without injuring the muscle. dizziness, falls, feeding problems, hearing difficulties
Active exercise will eventually overcome the muscle or visual disturbances in addition to the more obvious
contracture, unless the muscle has been permanently complaints of cognitive impairment, speech disorders
damaged. or incontinence. Some symptoms will only be dis-
Muscle wasting follows either disuse or denervation; closed on direct questioning as the patient may not
in the former, the fibres are intact but thinner; in the consider them relevant; other symptoms, such as
latter, they degenerate and are replaced by fibrous tis- incontinence or impotence, may be too embarrassing
sue or fat. to mention. Symptoms may also have been present for
Muscle fasciculation – or muscle twitch – is a local so long that they are considered to be ‘normal’.
involuntary muscle contraction of a small bundle of Family history may reveal clues to the underlying
muscle fibres. It is usually benign but can be due to aetiology of the patient’s symptoms.
motor neuron disease or dysfunction.
Examination
Neurological examination is described in Chapter 1.
CLINICAL ASSESSMENT Particular attention should be paid to the patient’s
mental state, natural posture, gait, sense of balance,
involuntary movements, muscle wasting, muscle tone
History
and power, reflexes, skin changes, the various modes
Age at presentation is important. Certain congenital of sensibility and autonomic functions such as sphinc-
or syndromic neuromuscular disorders are obvious at ter control, peripheral blood flow and sweating. The
birth (e.g. spina bifida and arthrogryposis). Others, back should always be carefully examined as it holds
228 while undoubtedly caused by perinatal problems, may the key to many causes of neurological disorder.
Table 10.1 Nerve root supply and actions of main 10
muscle groups
Sternomastoids Spinal accessory C2, 3, 4

Trapezius Spinal accessory C3, 4
Diaphragm C3, 4, 5
Deltoid C5, 6
Supra- and infraspinatus C5, 6
Serratus anterior C5, 6, 7
Pectoralis major C5, 6, 7, 8
Elbow flexion C5, 6
extension C7
Supination C5, 6
Pronation C6
Wrist flexion C6, (7)
extension C6, 7, (8)
Finger flexion C7, 8, T1
extension C7, 8, T1
ab- and adduction C8, T1
Hip flexion L1, 2, 3
extension L5,S1
adduction L2, 3, 4
abduction L4, 5, S1
Knee extension L(2), 3, 4
flexion L5, S1
Ankle dorsiflexion L4, 5
plantarflexion S1, 2
inversion L4, 5
eversion L5, S1
Toe extension L5
10.5 Examination Dermatomes supplied by the spinal
flexion S1 nerve roots.
abduction S1, 2
GAIT AND POSTURE • Antalgic gait – A markedly shortened stance phase

A single gait cycle consists of a stance phase (60 per on one side. Pain makes the patient move off the
cent) and a swing phase (40 per cent) and each full affected limb as quickly as possible.
cycle represents the stride length. Many parameters of • Spastic gait – A stiff-legged gait, often with a
each phase at each joint and in all three planes (coro- crouching posture (flexed hips and knees and feet in
nal, sagittal and transverse) can be analysed, often equinus) and ‘scissoring’ (legs crossing each other),
using a computerized gait analysis facility. However, due to muscle imbalance.
much can be learnt by carefully studying the way the • Drop-foot gait – During swing, the foot ‘drops’ into
patient walks and moves; clinical gait analysis equinus; if the foot was not lifted higher than usual
improves with experience and allows distinctive move- the toes would drag along the floor. This is caused
ment patterns to be recognized: by disorder or damage to the peripheral nerves sup-
plying the foot dorsiflexors.
• Dystonia – This term refers to abnormal posturing • High-stepping gait – This could be due to a bilateral
(focal or generalized) that may affect any part of the foot drop or it may signify problems with balance
body and is often aggravated when the patient is or proprioception.
concentrating on a particular motor task such as • Waddling (Trendelenburg) gait – The trunk is
walking. thrown from side to side with each step. The 229
10 motor neuron (spastic) type of weakness. Complete
MRC GRADING OF MUSCLE POWER loss of power is called hemiplegia.
Grade Description
• Diplegia (weakness in both upper or both lower
limbs) can be due to either UMN or LMN disor-
0 No muscle action – total paralysis der. In some cases the apparently unaffected limbs
1 Minimal muscle contraction
may show minimal degrees of weakness which
could easily be missed.

2 Power insufficient to overcome gravity • Quadriplegia (all four limbs affected) could be due
3 Anti-gravity muscle power
to either UMN or LMN pathology, e.g. cerebral
palsy, high cord damage or anterior horn cell
4 Less than full power pathology like poliomyelitis.
5 Full power
DEFORMITY
In unbalanced paralysis, one group of muscles is too
weak to balance the pull of the antagonists. At first
mechanics are similar to those that produce a posi- this produces a deformity that can be corrected pas-
tive Trendelenburg test (see page 493), as seen in sively (dynamic deformity); over time the active mus-
patients with functionally weak abductor muscles cles and the soft tissues of the joints contract and the
(or dislocation) of the hip. deformity becomes fixed or structural.
• Ataxic gait – Ataxia produces a more obvious and In balanced paralysis, the joint assumes the position
irregular loss of balance, which is compensated for imposed on it by gravity and it may feel floppy or flail.
by a broad-based gait, or sometimes uncontrollable In a dynamic deformity, rebalancing of the muscle
staggering. forces may be possible with a tendon transfer. If the
deformity is fixed, soft-tissue releases, and possibly
MOTOR POWER AND TONE osteotomies, may be needed to correct the deformity
It is important to examine not only individual muscles before rebalancing can be considered.
but also functional groups. In flaccid paralysis, grad- Paralysis occurring in childhood seriously affects
ing muscle power is important; in spastic paralysis, the growth. Bones are thinner and shorter than usual and
spasticity often obscures the inherent weakness and in the absence of normal mechanical stresses (imposed
testing specific muscles can be difficult due to the by normal muscle pull) bone modelling can be defec-
patient’s inability to isolate individual movements. tive (e.g. a valgus femoral neck–shaft angle, which is
Muscle power is usually graded as shown in the often seen in neuromuscular disorders).
accompanying Box. Repeated muscle charting allows
an objective measure of progressive disease or recov-
SENSATION
ery to be documented.
All sensory modalities must be tested over all der-
matomes. Any sensory disturbance must be mapped
WEAKNESS
to see if it fits a particular distribution pattern: der-
When patients complain of ‘weakness’ they often fail
matomal, glove and stocking or peripheral nerve dis-
to distinguish between true loss of muscle power and
tribution.
difficulties due to pain or instability. When testing for
muscle power it is essential to address individual mus-
cles and muscle groups as well as mass movements. AUTONOMIC SYSTEM
Different patterns of weakness will be encountered. A basic assessment of autonomic nervous system func-
Weakness may be partial (paresis) or complete (paral- tion is useful: colour, warmth and sudomotor function
ysis). can be assessed quickly and easily.
• Monoplegia (weakness of one limb) is usually
indicative of a lower motor neuron defect, most
commonly a peripheral nerve or nerve root; the
Imaging
movements affected on clinical testing will suggest Plain x-rays of the skull and/or spine are routine for
the likely anatomical location. However, if only the all disorders of the central nervous system. If the diag-
lower limb is affected the lesion could be in the dis- nosis is not obvious, further studies by CT or MRI
tal part of the spinal cord. may be necessary.
• Hemiparesis (weakness of either the right or the left Spinal imaging is usually directed at identifying
side of the body) usually denotes pathology some- compression of the cord or the nerve roots, the level
where between the cerebral cortex and the cervical of compression and its cause. Fractures and disloca-
230 segment of the spinal cord; this will be an upper tions usually show on the plain x-rays but a CT scan
will reveal the exact relationship of bone fragments to NERVE CONDUCTION STUDIES 10
nerve structures. Prolapsed intervertebral disc is
usually diagnosed on clinical examination, but myel-
ography, CT and MRI will help to establish the extent Motor nerve conduction
of the lesion and its exact site. Narrowing of the spinal The nerve under study (usually a mixed motor and
canal is best demonstrated by CT; the commonest sensory nerve) is stimulated electrically at an easily
cause is osteophytic overgrowth following disc degen- accessible subcutaneous site (e.g. the forearm or wrist
eration and osteoarthritis of the facet joints. This is for the median nerve or behind the medial malleolus
even worse when the spinal canal is congenitally nar- for the posterior tibial nerve), until it propagates an
row or trefoil-shaped (spinal stenosis). action potential which travels to the innervated mus-
Destructive lesions of the bones may be due to infec- cle where a surface electrode records the response.
tion or tumour (usually metastatic lesions). These may Measurements are displayed on an oscilloscope
show on plain x-rays but CT, MRI and myelography screen, the most informative being the time it takes in
are helpful. milliseconds (ms) for the impulse to reach the muscle,
Imaging of the brain is usually by MRI. Functional called the latency, and the magnitude of the response
scans such as positron emission tomography (PET in millivolts (mV), called the amplitude of the evoked
scan) that can isolate specific areas of brain activity are compound muscle action potential (CMAP). By
also gaining in popularity and are used in conjunction measuring the distance from the stimulating electrode
with MRI and CT. to the recording electrode, and setting this against the
latency, one can deduce the nerve conduction velocity
(NCV) in metres per second between those two
Other investigations points.
Blood and cerebrospinal fluid investigations may be In practice it is more useful (and more accurate) to
necessary, depending on the working diagnosis. stimulate the nerve at two points, first at a distal site
Muscle biopsy, to be reliable, calls for great care: the and then at a proximal site, and subtract the distal
biopsy must be taken from a muscle that is affected latency from the proximal latency to obtain a truer
but still functioning; local anaesthetic infiltration must measurement for the intervening segment of the
be avoided; the specimen must be handled gently; nerve. Thus, to measure the NCV of the median
and, depending on the tests required, it must be kept nerve in the carpal tunnel, one would take readings
at its resting fibre length. Biopsies must be placed in with the stimulating electrode first distal to the carpal
special transport medium or frozen immediately. tunnel and then in the upper forearm; this would
Audiological and ophthalmic testing and assessment allow one to deduce the NCV in the particular seg-
of mental capacity are also helpful in certain cases. ment of the nerve at the carpal tunnel.
Similarly with measurement of amplitude, which is
proportional to the number of motor units stimu-
lated: if a patient has lost one-half of the nerve fibres
in a peripheral nerve (e.g. due to compression, trauma
NEUROPHYSIOLOGICAL STUDIES
Neurodiagnostic techniques comprising nerve conduc-

tion studies and needle electromyography have an
important role in the investigation of peripheral nerve
and muscle disorders. Theoretically, any motor or sen-
sory nerve can be studied, but in everyday clinical
practice most of these investigations are involved in
studying the median, ulnar and radial motor and sen-
sory responses in the upper limb, and the sciatic nerve
as well as the posterior tibial and peroneal divisions,
motor and sensory, in the lower limbs.
Needle electromyography (EMG) of individual
muscles is used as a complementary technique, which
gives information about the nature and number of the
activated or denervated motor units from the specific
nerve root that innervates the muscle being tested.
10.6 Ulnar motor nerve conduction The ulnar nerve is
This can be used for anatomical clarification and sep- stimulated above the elbow, posterior to the medial
aration of radiculopathy from peripheral neuropathy epicondyle, and the CMAP is recorded from the abductor
and myopathy. digiti minimi. 231
10.7 Nerve conduction
10 5ms Stimulus site
Lat1 Dur Amp Area
velocity Oscillographic
ms ms mV mVms
recordings of nerve
A1: Wrist 2.9 5.2 7.7 21.1 conduction studies in a
case of acute ulnar nerve
A2: Below elbow 6.5 5.3 6.3 17.5
palsy due to compression
A3: Above elbow 9.5 3.0 0.6 1.1 of the patient’s arm while
A 19.2mA undergoing surgery

A4: Axilla
1 5mV under general
A5: Erb’s point anaesthesia. These
tracings show an acute
motor nerve conduction
block at the elbow, with
A 76.5mA normal distal CMAPs
2 5mV when stimulating below
the elbow (tracings A1
and A2) and a reduced
Dist Diff CV amplitude CMAP when
Segment
mm ms m/s
stimulating above the
A 76.5mA Wrist-Below elbow 220 3.6 61 elbow (A3). There is
3 5mV severe focal conduction
Below elbow-Above elbow 80 3.0 27
slowing across the elbow
Above elbow-axilla at 27 m/s, compared to
61 m/s in the segment
AxillaErb’s point-
below the elbow.
or vascular insufficiency) the size of the elicited NOTE: Clinical nerve conduction studies estimate
CMAP will be reduced by approximately 50 per cent the population of large myelinated sensory or motor
compared to the contralateral normal limb. When a nerves. Type C fibres (small myelinated fibres serving
nerve is stimulated at two sites, distally and then prox- pain and temperature appreciation) have an amplitude
imally, the evoked CMAPs should be of similar ampli- below the sensitivity of recording techniques, as well
tudes. However, if the CMAP on proximal as slowed velocity (5–10 metres/second) and cannot
stimulation is observed to be smaller than the CMAP be tested with standard clinical techniques.
on distal stimulation, one assumes that a reduced
number of motor units have conducted the action
potential over the intervening segment of the nerve: ELECTROMYOGRAPHY (EMG)
this is referred to as conduction block and is a feature
of a potentially recoverable neuropraxic lesion. To record the electrical discharge of motor units in a
Common investigations are measurement of the muscle, a concentric needle electrode, the shape of a
NCV for the median nerve at the wrist or the ulnar small hypodermic needle, is inserted into the muscle
nerve at the elbow in suspected cases of carpal tunnel and connected to an oscilloscopic screen and a loud-
syndrome or cubital tunnel syndrome respectively. In speaker. This will provide both a visual pattern on the
a focal entrapment neuropathy one will find focal
slowing with normal velocities on either side of the
lesion.
Conduction slowing of uniform degree along the
whole length of the nerve suggests a demyelinating
neuropathy, e.g. Charcot–Marie–Tooth syndrome.
Sensory nerve conduction

In a similar manner, a sensory nerve action potential
(SNAP) may be recorded by stimulating a suitable
subcutaneous sensory nerve and recording with sur-
face electrodes on the skin over a measured distance
along the same sensory nerve, e.g. from the index and
middle fingers of the median nerve. SNAP is much 10.8 Needle electromyography (EMG) The first dorsal
smaller in amplitude than CMAP and is measured in interosseus muscle (C8–T1 ulnar nerve) is being sampled
232 microvolts. during voluntary contraction against resistance.
1mV Foot Switch Status: / Run Trig: uV 10ms 10.9 Electromyography 10
Normal recruitment of motor units on needle EMG of
the biceps muscle, to full interference/recruitment
pattern. (Amplitude 1 mV/division)
Fibs: MUP Amp uV: Max Amp mV:
Pos Waves: MUP Dur ms:
Fascics: MUP Pattern:
Polyphasics: Max Effort:
1mV Foot Switch Status: / Run Trig: uV 10ms Myopathic recruitment pattern in a patient with
polymyositis. There are multiple small amplitude
motor units. (Amplitude 1 mV/division)

1mV Foot Switch Status: / Run Trig: uV 10ms Acute denervation pattern, characterized by florid
low amplitude fibrillation potentials recorded from
tibialis anterior (resting state).

1mV Foot Switch Status: / Run Trig: uV 10ms Severe neurogenic abnormality. Single rapidly firing
giant motor potential, typical of severe motor unit
loss in a patient with old poliomyelitis. A similar
pattern is seen in motor neuron disease. (Amplitude
1 mV/division)

screen and, simultaneously, crackling sounds from the the spinal cord, with its motor axon and the variable
loudspeaker. number of muscle fibres it innervates in the muscle).
At rest, a normal muscle is silent. As the patient This is reflected first as a progressive increase in the
slowly contracts the muscle there is recruitment of number and then also as increased amplitude of
one, then more and then multiple motor units (a motor unit action potentials, with recognizable pat-
motor unit being defined as the anterior horn cell in terns. A full recruitment pattern usually looks and 233
10 sounds like ‘white noise’, with so many motor units
firing that both the spikes on the screen and the crack- NEUROPHYSIOLOGICAL SIGNS OF
les from the speakers overlap each other – a so-called NEUROPATHIC DISORDER
‘interference pattern’.
In nerve disorders the muscle may not be silent at Reduced motor or sensory potentials reflect non-
rest and may manifest increased insertional activity functioning (perhaps transected) nerves
(activity during insertion of the needle electrode).

There are changes of active denervation, referred to as Loss of sensory responses (SNAP) reflects a disorder
fibrillation potentials and positive sharp waves, pro- distal to the spinal foramen (e.g. in the plexus);
duced by denervated muscle fibres firing sponta- intact SNAP in a hypaesthetic limb suggests disease
neously. This signifies motor nerve fibre loss or proximal to the foramen (e.g. a prolapsed disc)
disruption. It takes 7–12 days for the changes of Conduction block (i.e. intact distal motor response
active denervation to develop after axonal disruption. with focal conduction block) implies a neuropraxic
In a denervated muscle (e.g. the result of spinal recoverable injury
root entrapment) the number of motor units
recruited will be reduced proportional to disrupted Denervation changes on EMG more than 10 days
axons. Instead of the white noise of full recruitment after injury confirm significant nerve damage and
one sees a reduced pattern of muscle potentials. loss of motor nerve function
In muscle disease similar changes to the above may Any recruited volitional motor units in a weak limb
be seen, but the pattern of action potentials differs implies a potential for recovery
and the full interference pattern appears at lower lev-
els of active contraction.
A chronic neuropathy, with re-sprouting of remaining tion compared to distal stimulation, representing con-
viable nerve fibres, results in longer re-innervated motor duction block, or significant focal conduction slowing.
units with a polyphasic or higher amplitude profile.
Distinguishing nerve root disease from

peripheral entrapment
DIAGNOSTIC EVALUATION OF THE
The major anatomical defining characteristic of a
PATIENT proximal root entrapment (e.g. due to a prolapsed
disc) is the preservation of the sensory action poten-
Which nerves are studied in any patient, and the inter-
tial in the involved limb. This is because the lesion
pretation of the electrophysiological findings, will
interrupts the nerve root proximal to the dorsal root
depend upon the clinical presentation and the provi-
ganglion which is anatomically (and electrically) situ-
sional diagnosis. Appropriate nerve conduction stud-
ated outside the spinal cord where it is in continuity,
ies and EMG can confirm or refute the clinical
and maintains the integrity of the distal axon; hence
diagnosis. Comprehensive study of all nerves without a
the SNAP remains normal.
diagnostic plan is usually unhelpful.
The CMAP may be reduced as the motor nerve is
When investigating a specific nerve root syndrome,
separated from the anterior horn cell in the spinal
nerve conduction and EMG studies are concentrated
cord. For example, in a wrist drop from a C7 root
in the appropriate anatomical territory and the find-
entrapment, the radial motor potentials are reduced
ings are compared to those in other nerve root terri-
or even absent, there is gross denervation on EMG,
tories in the same as well as the contralateral (usually
but the radial sensory potentials are preserved and
asymptomatic) limb. For example, in a patient with a
entirely normal! The presence of an intact sensory poten-
weak arm and radial distribution paraesthesiae due to
tial is what distinguishes root and proximal disease from
a C5/6 disc prolapse, one would study the median
peripheral entrapment and plexus disease.
nerve motor and sensory potentials at the carpal tun-
nel, the radial sensory potentials at the wrist and EMG
of C6 innervated muscles (e.g. biceps and brachiora-
dialis). The findings are then compared to those in the INTRAOPERATIVE NEUROPHYSIOLOGICAL
C7 muscles such as extensor digitorum communis TECHNIQUES
and triceps.
In a mononeuropathy or plexopathy one needs to Spinal monitoring: somatosensory
compare conduction values (amplitude and velocity)
evoked responses (SSEP)
in one limb to those in the other.
In a disorder such as a focal entrapment one may Neurophysiological tests are sometimes necessary
234 demonstrate a reduced amplitude on proximal stimula- during corrective spinal operations to obviate injury
to the cord. These techniques use the basic principles mature babies and those of multiple births. Known 10
defined above, often combining them with techniques causal factors are maternal toxaemia, prematurity,
employed in electroencephalography (EEG), such as perinatal anoxia, kernicterus and postnatal brain infec-
averaging. A peripheral nerve in the upper or the tions or injury; birth injury, though often blamed, is a
lower limb (usually the median or posterior tibial) is distinctly unusual cause. These factors may also cause
stimulated but, instead of recording from the nerve or damage to other areas of the developing brain and
the muscle twitch, one records from the scalp overly- thus many children with cerebral palsy have associated
ing the patient’s sensory parietal cortex. problems such as epilepsy, perceptual problems,
The evoked responses from the recorded cortex are behavioural problems and learning difficulties.
miniscule and one must therefore average the The main consequence is the development of neu-
obtained responses from at least 100–200 stimuli in romuscular incoordination, dystonia, weakness and
order to differentiate the time-linked evoked response spasticity. Oro-facial motor incoordination may make
from the background brain EEG activity. Averaging speech and swallowing difficult and drooling is a fre-
200 or more responses at a stimulus rate of 3 per sec- quent problem; none of these defects, however,
ond to demonstrate a reproducible response may take implies a poor intellect although, even these days, far
2 minutes or longer, assuming all other factors are too frequently the wrong conclusions are drawn.
even and perfect. The surgeon should be aware of this
drawback. One can also measure potentials developed
in the cervical spinal cord at C7 level and the L1 level Classification
as well as distally in the brachial plexus at Erb’s point,
Cerebral palsy is usually classified according to the
resulting from peripheral nerve stimulation.
type of motor disorder, with subdivisions referring to
The important measured parameter is usually the
the topographical distribution of the clinical signs.
latency of the response, e.g. the N20 response from
median nerve stimulation (a brain response occurring
TYPE OF MOTOR DISORDER
at approximately 20 milliseconds after stimulating the
• Spasticity is the commonest muscle movement dis-
median nerve at the wrist). Accidental nerve injury
order and is associated with damage to the pyrami-
during surgery around the spinal cord will produce a
dal system in the CNS. It is characterized by
delay in the latency or a sudden loss of the evoked
increased muscle tone and hyper-reflexia. The
response.
resistance to passive movement may obscure a basic
weakness of the affected muscles.
Other intraoperative techniques • Hypotonia is usually a phase, lasting several years
during early childhood before the features of spas-
Various techniques are used, tailor-made according to
ticity become obvious.
the procedure involved. These may include nerve or
• Athetosis manifests as continuous, involuntary,
nerve root stimulation at various sites and measure-
writhing movements which may be exacerbated
ment of either the distal nerve or muscle impulse.
when the child is frightened. It is caused by damage
This can demonstrate conduction block or slowing or
to the extrapyramidal systems of the CNS. In pure
normal continuity of the nerve.
athetoid cerebral palsy, joint contractures are
Intraoperative EMG is performed with the needle
unusual and muscle tone is not increased.
in situ in the appropriate muscle (e.g. the quadriceps
• Dystonia may occur with athetosis. There is a more
for L4 root procedures, abductor hallucis for the S1
generalized increase in muscle tone and abnormal
root) to assess the muscle contraction when the nerve
positions induced by activity.
is stimulated, either intentionally or otherwise.
• Ataxia appears in the form of muscular incoordina-
Cord-to-cord stimulation and cord-to-cortical poten-
tion during voluntary movements. It is usually due
tial measurement are usually resolved as averaged
to cerebellar damage. Balance is poor and the
recordings to reveal intraoperative evidence of spinal
patient walks with a characteristic wide-based gait.
pathway disruption.
• Mixed palsy appears as a combination of spasticity
and athetosis. The presence of both types of motor
disorder can make the results of surgical interven-
CEREBRAL PALSY tion unpredictable.
NOTE: In some types of cerebral palsy there is con-
The term ‘cerebral palsy’ includes a group of disorders siderable variability in the ‘tone’ and ‘posture’ from day
that result from non-progressive brain damage during to day or situation to situation. If surgical treatment is
early development and are characterized by abnormal- being considered, it should never be based on a single
ities of movement and posture. The incidence is about assessment when, due to stress, the child appears to
2 per 1000 live births, with the highest rates in pre- have abnormally high tone and muscle contractures. 235
10
(a) (b)
10.10 Cerebral palsy – early diagnosis

By 6 months these twin brothers had
developed quite differently, the one being
smaller and showing (a) lack of head and
arm control, (b) lack of body control when
helped to the sitting position and
(c) inability to sit unaided.
(c)
TOPOGRAPHIC DISTRIBUTION (see Fig. 10.12) Diagnosis in infancy

• Hemiplegia is the commonest. This usually appears
as a spastic palsy on one side of the body with the The full-blown clinical picture may take months or
upper limb more severely affected than the lower. even years to develop. A history of prenatal toxaemia,
Most of these children can walk and they respond haemorrhage, premature birth, difficult labour, foetal
reasonably well to treatment. distress or kernicterus should arouse suspicion. A
• Diplegia involves both sides of the body, with the neonatal ultrasound scan of the head may identify
lower limbs always most severely affected. Some intracerebral bleeding that would increase the likeli-
disorder of upper limb function is invariably present hood of later problems.
but signs may be subtle. Side to side involvement Early symptoms include difficulty in sucking and
may be asymmetrical and the terms asymmetric swallowing, with dribbling at the mouth. The mother
diplegia and occasionally bilateral hemiplegia are may notice that the baby feels stiff or wriggles awk-
used. Many cases are secondary to prematurity and wardly. Gradually it becomes apparent that the motor
periventricular leucomalacia is seen on brain MRI. milestones are delayed. The normal child holds up its
Intelligence is often normal. The less severely head at 3 months, sits up at 6 months and begins
affected children can have reasonable mobility but walking at about 1 year.
the non-walking diplegic patient may be similar to
the total body involvement group discussed below.
Diagnosis in later childhood
• Total body involvement describes a general and often
more severe disorder affecting all four limbs, the Most children presenting to the orthopaedic surgeon
trunk, neck and face with varying degrees of sever- have already had the diagnosis made. Occasionally, for
ity. Patients usually have a low IQ, they may have example with a mild hemiplegia or a symmetrical mild
epilepsy, they are often unable to walk and the diplegia, the diagnosis has not been made and the
response to treatment is poor. child is simply referred for advice about their gait or
• Monoplegia occasionally appears in an upper limb; their tendency to trip and fall. A familiarity and
careful examination will often show that other areas knowledge of the normal developmental milestones
are involved as well. True monoplegia is so unusual and gait patterns helps the clinician identify the child
that other diagnoses should be considered, e.g. a who is outside the normal range.
236 neonatal brachial plexopathy. Bleck (1987) has described seven tests for children
10
(a) (b) (c) (d)
10.11 Cerebral palsy (a) Adductor spasm (scissor stance); (b) flexion deformity of hips and knees with equinus of
the feet; (c) general posture and characteristic facial expression; (d) ataxic type of palsy.
over 1 year; these give an idea of severity and of the lordosis may be obliterated and the child may have
prognosis for walking. The primitive neck-righting difficulty standing unsupported. Often attempts to
reflex, asymmetrical and symmetrical tonic neck correct one deformity may aggravate another and it is
reflexes, the Moro reflex and the extensor thrust important to establish which deformity is the primary
response should all have disappeared at 1 year of age. one and which are compensatory. Many patients show
Children who retain more than two primitive reflexes pelvic obliquity and a scoliosis. Asking the child to
after that age, cannot sit unsupported by 4 years and ‘stand tall’ and watching their response often gives
cannot walk unaided by 8 years are unlikely ever to some insight into the dynamic nature of the posture
walk independently. and muscle strength and, of course, intellectual abil-
Ideally the child should be reviewed by a multidis- ity.
ciplinary team so that speech, hearing, visual acuity, Balance reactions are often poor and a gentle push
intelligence and motivation can also be assessed. that would force a normal child to take a step in the
Since cerebral palsy is essentially a disorder of pos- appropriate direction to maintain his or her balance
ture and movement, the child should be carefully may simply knock over a child with cerebral palsy.
observed sitting, standing, walking and lying. His or
her condition should then be evaluated according to GAIT
the gross motor function classification system If a child can walk, the elements of gait are analysed
(GMFCS) which categorizes the child, relative to taking note of the use of walking aids and orthotic
their age, in terms of mobility and bases this on their devices. Gait should be observed with and without
average function, not the best that they can achieve
on a given occasion (Palisano et al., 2008). The sys-
tem is reliable and valid; it aids in communication
between members of the multidisciplinary team and is
a useful guide to management.
SITTING POSTURE
The child may find it difficult or impossible to sit
unsupported: children with a hypotonic trunk may
slump into a kyphotic posture and others may always
‘fall’ to one side. In attempting to sit, the lower limbs
may be thrust into extension. There may be an obvi-
ous scoliosis or pelvic obliquity.
STANDING POSTURE
In the typical case of a spastic diplegia, the child
stands with hips flexed, adducted and internally (a) (b) (c)
rotated, the knees are also flexed and the feet are in 10.12 Spastic palsy Common types of spastic palsy:
equinus. With tight hamstrings, the normal lumbar (a) hemiplegic, (b) diplegic, (c) whole body. 237
10 shoes or orthotic supports and the differences (if any) deformity present. The physiotherapist has often seen
noted. Dystonic, athetoid and ataxic movements may the child more often and in more relaxed circum-
become more noticeable during walking. Every stances than is the case in the orthopaedic clinic and
opportunity must be taken to observe gait so that dif- can therefore identify whether today’s examination is
ferences between ‘normal’ and ‘best behaviour’ walk- truly representative.
ing can be identified. In hemiplegics, best behaviour
walking may demonstrate a flat foot pattern with the DEFORMITY ASSESSMENT
heel coming down most of the time while the more It is important to assess the degree of deformity pres-
normal or representative pattern will highlight the ent at each joint and relate it to muscle-tendon
asymmetric flexed knee and toe-walking pattern. length. Deformity at one level may be markedly
Clinical gait analysis is difficult but improves with affected by the position of the joints above and below.
practice. Each limb must be observed in both the For example, ankle equinus with the knee extended
stance and swing phases of gait and in the coronal, often disappears when the knee is flexed; thus one can
sagittal and transverse planes. In the spastic diplegic differentiate between tightness in the soleus and
patient, the standing posture mentioned above is tightness in the gastrocnemius muscle. In the Silfver-
influential in defining their walking pattern too. The skiöld test, with the child lying supine on the exami-
lack of free rotation at the hip means that the trunk nation couch, the knee is flexed to a right angle and
has to move from side to side as each leg swings the ankle is dorsiflexed; this tests soleus tightness. The
through and with the adduction it leads to a ‘scissor- knee is then fully extended on the couch and ankle
ing’ action (one leg crossing in front of the other). dorsiflexion is repeated; now it is mainly gastrocne-
This results in a narrow walking base and, when com- mius tightness that is being tested. Similarly, tight
bined with the hip and knee flexion and foot equinus, hamstrings may limit knee extension more with the
there is a strong tendency to fall; this can be helped by hips flexed than when the hips are extended and hip
the use of walking aids such as crutches. adduction may be easier in flexion than in extension
Computerized gait analysis ideally supplements ob- due to a tight gracilis. If hip abduction is restricted,
servational gait analysis. Kinematics (joint and limb order an x-ray to look for subluxation of the joint.
segment movement), kinetics (joint moments and pow- In the upper limb, finger flexors may be tight with
ers), EMG (identification of the phases in which mus- the wrist extended but if the wrist is allowed to flex
cles are firing), pedobarography (foot pressures) and the fingers can extend. Children can use these fixed-
metabolic energy analysis (assessment of the ‘cost’ of length reactions to manipulate their hand and finger
walking) are all part of the analysis, as is a video record- function using ‘trick’ movements.
ing which can be viewed from any direction and at any In the patient with total body involvement, spinal
speed. Interpretation of all this data requires skill and deformity is common; usually this is a scoliosis, often
experience and the application of the information to an associated with pelvic obliquity. Kyphosis and lordosis
individual child also requires a degree of common also occur.
sense. Pattern recognition is important (in both forms
of gait analysis). Perhaps its main role is to help the cli- SENSATION
nician distinguish between dynamic and fixed tightness Sensation is often not entirely normal and problems
and in the identification of dyskinesia. with stereognosis (as well as with perception) may be
A good account of gait patterns in cerebral palsy is important factors contributing to upper limb disability.
given by Sutherland and Davids (1993).
MUSCLE CONTRACTURE
NEUROMUSCULAR EXAMINATION A degree of muscle contracture is almost inevitable
Examination of the limbs shows the typical features of with all forms of cerebral palsy where longstanding
upper motor neuron or spastic paresis. Passive move- spasticity leads to relative shortening of the muscles
ments are resisted, the reflexes are exaggerated and and hence fixed contractures and changes in joint
there is a positive Babinski response. However, spas- congruity. There is still some debate as to whether the
ticity may obscure the fact that muscle power is actu- changes are due to a true shortening of the muscle or
ally weak. By the end of the examination the clinician a failure to grow along with skeletal growth. Certainly
should have a clear idea of the muscle tone, muscle most of the effects are seen during the period of
power and range of movement at each joint. growth; after skeletal maturity the changes in muscle-
In children with cerebral palsy the physical signs tendon length and joint contracture are much less
often vary from day to day or even minute to minute progressive.
depending on factors such as the emotional state of
the patient and the temperature of the room. It takes BONY DEFORMITY
time to examine a child and get a representative ‘feel’ Normal bone growth is influenced by muscle pull.
238 for the tone, the muscle strength and the degree of Hence in children with persistent abnormal muscle pull
there may be a failure of normal modelling and new sleeping and participation in standing/swivel trans- 10
deformities can develop. The normal degree of femoral fers; (3) knees that are mobile enough for sitting,
neck anteversion persists and sometimes even increases sleeping and transferring; and (4) plantigrade feet that
with growth rather than improving – and significant fit into shoes and rest on the footplates of the wheel-
external tibial torsion may also be present. chair comfortably.
Bony deformities may, in turn, engender new prob- For all children good medical care is also essential
lems. Persistent adduction of the hip leads to valgus of as is access to good quality orthotic supports, walking
the femoral neck, acetabular dysplasia and subluxation aids and/or wheelchairs as appropriate. Unfortunately
of the joint. Flexion deformity of the knee is associ- these basic needs are still not met for children in many
ated with upward displacement of the patella and disadvantaged communities.
patello-femoral pain. External tibial torsion may give
rise to planovalgus deformity of the foot. TONE MANAGEMENT
Tone management is one of the most important
STRUCTURAL SCOLIOSIS aspects of patient care and it underpins all other forms
Flexible curves are common, but unfortunately many of treatment.
become structural; this is especially likely in patients Medical treatment The most generally effective med-
with total body involvement. ications are anticonvulsants for seizures, short-term
benzodiazepine use for postoperative pain and tri-
hexyphenadryl for dystonia.
Management Baclofen, an agonist of gamma-aminobutryic acid
There is no single ‘blueprint’ for the management of (GABA), acts by inhibiting reflex activity. In oral form
all patients with cerebral palsy; each patient and his or it does not cross the blood–brain barrier well. When
her family provides a different challenge. This section effective, it reduces muscle tone/spasticity generally.
will aim to discuss first some basic principles that are This may have a negative effect on head and trunk
applicable to all children and then some more specific control and combined with the side effects of drowsi-
principles that relate to various types of cerebral palsy. ness means that its use may be limited. Intrathecal
baclofen is administered via a refillable, subcutaneous
implanted pump and the dose administered can be
GOAL SETTING
titrated according to the child’s response. Long-term
It is human nature for a parent to want and indeed
studies of its use are not yet available but it appears
expect the best for their child and it is the role of the
that it may be most effective in those with severe spas-
healthcare professionals to support them in their
ticity or dystonia. It is not effective in all patients and
wishes. However, it is also important for the profes-
test doses and assessment of its benefits are required
sionals to ensure that the difference between hopeful
in all prospective patients.
optimism and pragmatic realism is understood by all
Dantrolene produces weakness without much
involved in the child’s care. Few patients with total
reduction in spasticity and hence it is rarely used in
body involvement will ever walk. The prognosis for
cerebral palsy.
walking in the patient with spastic diplegia may be
Analgesic medication is needed for the reduction of
assessed by looking at Bleck’s (1975) criteria and
pain associated with musculoskeletal problems, con-
those of Beals (1966). The definition of walking must
stipation and gastro-oesophageal reflux.
also be conveyed to the parents along with an expla-
nation that many children with cerebral palsy reach Botulinum toxin This potent neurotoxin is produced
their peak of physical function in late childhood and by Clostridium botulinum; it acts by blocking acetyl
with the increase in size and weight that comes with choline release at the neuromuscular junction. The
puberty weak muscles may no longer be able to main- preparation is injected into the ‘spastic’ muscle at (or
tain walking ability. as near as possible to) the motor end point. The usual
For all patients with cerebral palsy the priorities are: targets are the hip adductors, hamstrings, gastrocne-
(1) an ability to communicate with others; (2) an abil- mius and tibialis posterior. The weakness/paralysis
ity to cope with the activities of daily living (including that it causes takes a few days to become obvious; the
personal hygiene); and (3) independent mobility – effect is temporary and as new nerve terminals form
which may mean a motorized wheelchair rather than there is a return of muscle tone at around 10–12
walking. weeks.
For the child who from an early age is recognized to be Botulinum toxin must not be used on its own but
‘non-walking’ realistic goals should be: (1) a straight rather as part of a package of care in the overall tone
spine with a level pelvis; (2) located, mobile and pain- management programme. Thus injections are fol-
less hips that flex to 90 degrees (for comfortable sit- lowed by increased physiotherapy input and often an
ting) and extend sufficiently to allow comfortable alteration in orthotic/splinting regimens. This means 239
10 that the overall benefits attributed to the injections maximize the effects of surgery and overcome the
may last considerably longer than the 10–12 weeks of immediate pain, stiffness and weakness that follow
true neuromuscular blockade. surgery.
It is precisely because the toxin is never used on its
Positioning and splinting Care must be taken at all
own that it has been difficult to prove what the true
times to ensure that the child both sits and sleeps,
benefits of this form of treatment are but it is consid-
works and eats in a good position and with good pos-

ered useful as a focal treatment for a dynamic muscle
ture. Adjustments may need to be made to chairs,
imbalance that is interfering with function, producing
wheelchair and the child’s sleep system so as to limit
deformity or causing pain. It is perhaps more effective
disadvantageous positions such as hip adduction.
in younger children who are less likely to have fixed
Splints are used to prevent muscle contracture,
deformity. Multilevel injections may be required but
maintain joint position and improve movement and
the overall dose per child must be kept within safe
hence function. They also have an important role in
limits.
maintaining position following surgery. Splints may
There is also a role for botulinum toxin in the man-
be corrective – in that they aim to hold a passively cor-
agement of postoperative pain and spasm although for
rectable deformity – or ‘adoptive’, e.g. when the
optimal effect the injections need to be given some
splint adopts the shape of the foot and simply aims to
days prior to surgery.
prevent further loss of position. A badly fitting splint
Selective dorsal rhizotomy Division of selected dorsal at best does nothing and at worst provokes pain and
nerve roots from L1 to S2 has only recently gained spasm and increasing deformity.
wide acceptance, perhaps as the indications for its use
Manipulation and serial casting These methods may
have been refined and the techniques for performing
have a limited role in improving muscle/joint con-
the procedure have improved. In cerebral palsy, the
tractures, but relapse is frequent.
normal inhibitory influences on muscle tone from the
higher centres are deficient. This technique aims to
reduce spasticity and rebalance muscle tone by selec-
tively reducing the input from the muscle spindles, Operative treatment
thus leading to less excitation of the anterior horn
The indications for surgery are: (1) a spastic deformity
cells. Long-term studies are not yet available but good
which cannot be controlled by conservative measures;
results have been obtained in children aged 3–8 years
(2) fixed deformity that interferes with function; and
who meet the following criteria: they are walking but
(3) secondary complications such as bony deformities,
have significant spasticity; they were born prema-
dislocation of the hip and joint instability.
turely; they have good intellectual function and good
It is important to remember that in cerebral palsy
voluntary control. The presence of fixed contractures
all muscles are weak: thus, muscle-lengthening sur-
is a relative contraindication and may need surgical
gery is also muscle-weakening surgery unless by
correction.
improving the mechanical alignment of the limb, and
Physical therapy Cerebral palsy affects motor function hence the muscle, you allow it to work more effi-
in several ways. There is a dependence on immature or ciently. Correction of bony deformity may be impor-
primitive reflexes and a loss of selective muscle con- tant in this respect and although the surgery may be
trol. Physiotherapy attempts to reduce or prevent the more ‘aggressive’ it may actually be more appropriate.
problems arising from abnormal muscle tone, imbal- Weak muscles can be augmented by tendon trans-
ance between opposing muscle groups and abnormal fers but the muscle being transferred is weak already
body balance mechanisms. To this end various struc- and may have a limited ability to function in its new
tured approaches or ‘schools’ have been popularized. role; on the other hand it may produce an unwanted
No single method has been shown to be significantly overcorrection because of its increased tone. The role
better than another but all have good points and all of gravity plays an important part in guiding the
can work well in individual cases. In addition to these choice of tendon transfers.
programmed approaches, there is a philosophy that a The timing of surgical intervention is often crucial.
range of regular movement exercises will prevent or Development of the CNS and the gait pattern
(perhaps more realistically) reduce the degree of mus- matures around the age of 7–8 years and thus many
cle/joint contracture. orthopaedic surgeons advocate delaying surgery until
Physiotherapy is considered to be most helpful in this age and then doing all the necessary operations at
early childhood up to the age of 7 or 8 years but there one or two sittings. Our preferred approach is to
is surprisingly little evidence to guide us in knowing avoid ‘little and often’ surgery in favour of the ‘all or
what type of physiotherapy to prescribe and how none’ philosophy, but as always some patients require
often to do so in any particular case. However, post- the former and some the latter. Earlier operation may
240 operative physiotherapy is essential in order to be called for if the hip threatens to dislocate.
REGIONAL SURVEY thenar muscles, followed by tendon transfers to rein- 10
force abduction and extension. Here again the opera-
tions should be performed by a specialist in this field.
Upper limb
Upper limb deformities are seen most typically in the
child with spastic hemiplegia or total body involve- Lower limb
ment and consist of flexion of the elbow, pronation of
The functional effects of lower limb spasticity differ
the forearm, flexion of the wrist, clenched fingers and
considerably, depending on whether the patient has
adduction of the thumb. In the mildest cases, spastic
hemiplegia, diplegia or whole-body involvement; this
postures emerge only during exacting activities. Pro-
will obviously influence the lines of surgical treatment.
prioception is often disturbed and this may preclude
any marked improvement of function, whatever the
SPASTIC HEMIPLEGIA
kind of treatment. Operative treatment is usually
Four subtypes of hemiplegia have been identified and
delayed until after the age of 8 years and is aimed at
the most common lower limb problem is with foot
improving the resting position of the limb and restor-
deformity.
ing grasp.
Foot/ankle Tibialis anterior is invariably weak and the
Elbow flexion deformity Provided the elbow can patient develops an equinovarus foot deformity. Active
extend to a right angle, no treatment is needed. Occa- plantar flexion is required to assist knee extension dur-
sionally it may be necessary to treat a more marked ing the stance phase of gait so care must be taken
flexion contracture by fractional lengthening of the when considering a lengthening of the gastrocne-
biceps and brachialis tendons with release of the mius/soleus complex. The trend is to perform a mus-
brachialis origin. cle recession rather than a tendon lengthening
Forearm pronation deformity This is fairly common procedure.
and may give rise to subluxation or dislocation of the A dynamic varus deformity can be treated by a split
radial head. Simple release of pronator teres may tibialis anterior tendon transfer to the outer side of the
improve the position, or the tendon can be rerouted foot (only half the tendon is transferred so as to avoid
round the back of the forearm in the hope that it may the risk of overcorrection into valgus). In older chil-
act as a supinator. dren with fixed deformity, formal muscle lengthening
with or without a calcaneal osteotomy may be required.
Wrist flexion deformity Wrist flexion is usually in an Pes valgus (pronated foot deformity) may require
ulnar direction; it can be improved by lengthening or subtalar arthrodesis.
releasing flexor carpi ulnaris. If extension is weak, the
released flexor tendon is transferred into one of the Hip/knee Surgery is not usually required but if it is it
wrist extensors. In severe cases wrist arthrodesis with follows the principles outlined below for the walking
excision of the proximal carpal row may be of cos- diplegic patient.
metic rather than functional benefit. N.B. Before oper- Leg length discrepancy Due to discrepancies in
ating on the wrist it is essential to consider what effect growth, the hemiplegic limb is often short irrespective
this will have on finger movements. of any joint contractures. An epiphyseodesis of the
Flexion deformity of the fingers Spasticity of the long contralateral distal femoral and/or proximal tibial
flexor muscles may give rise to clawing. The flexor physes may be considered. This can improve some
tendons can be lengthened individually, but if the aspects of the gait pattern.
deformity is severe a forearm muscle slide may be
more appropriate. Ideally these operations should be SPASTIC DIPLEGIA
undertaken by a specialist in hand surgery. If the Most patients with cerebral palsy have a spastic diple-
fingers can be unclenched only by simultaneously flexing gia and treatment is concentrated on the lower limbs.
the wrist, it is obviously important not to extend the In the very young child, this consists of physiotherapy
wrist by tendon transfer or fusion. and splintage to prevent fixed contractures. Surgery is
indicated either to correct structural defects (e.g. a
Thumb-in-palm deformity This is due to spasticity of fixed contracture or hip subluxation) or to improve
the thumb adductors or flexors (or both), but later gait. By 3–4 years of age the sitting and walking pat-
there is also contracture of flexor pollicis longus. In terns can be observed, and particular attention should
mild cases, function can be improved by splinting the be paid to the interrelationship between the various
thumb away from the palm, or by operative release of postural defects, especially lumbar lordosis/hip flex-
the adductor pollicis and first dorsal interosseus mus- ion and knee flexion/ankle equinus.
cles. Resistant deformity may need combined length- Most children will walk but they are delayed in
ening of flexor pollicis longus and release of the learning to master this – a child who is not walking by 241
10 the age of 6 or 7 is unlikely to do so. Non-ambulant
children often have orthopaedic problems similar to
those with total body involvement (see below).
In walking diplegics, observational gait analysis is
important and computerized gait analysis may have a
role in guiding treatment. Affected children are often
relatively symmetrical in their gait pattern but in some

asymmetry is very marked with one limb maintaining
a hemiplegic posture and one more consistent with a
diplegic gait. Each limb must be assessed independ-
ently.
Hip adduction deformity The child walks with the
thighs together and sometimes even with the knees
10.13 Spastic hips X-ray of a boy with spastic adducted
crossing (‘scissors gait’). This may be combined with hips showing acetabular dysplasia and coxa valga, worse
spastic internal rotation. Adductor release is indicated on the left side.
if passive abduction is less than 20 degrees on each
side. If medial hamstring lengthening is planned (see
below) it should be done first because this alone may bined with acetabular reconstruction. Longstanding
restore some hip abduction. dislocation in a non-walker may be impossible to
For most patients open tenotomy of adductor reconstruct; if discomfort makes operation impera-
longus and division of gracilis will suffice. Only if this tive, the proximal end of the femur can be excised. In
fails to restore passive abduction (a rare occurrence) the adult walking diplegic patient, total hip replace-
should the other adductors be released. Anterior ment can be considered in selected cases where
branch obturator neurectomy should not be per- painful degenerative change is affecting function.
formed.
Knee flexion deformity This is one of the commonest
Hip flexion deformity This is often associated with fixed deformities; it is usually due to functional hamstring
knee flexion (the child walks with a ‘sitting’ posture) or tightness but is often aggravated by hip flexion or
else hyperextension of the lumbar spine. Operative weakness of ankle plantar flexion. Spastic flexion
correction is indicated if the hip deformity is more deformity may be revealed only when the hip is flexed
than 30 degrees. In the walking child, it is important to 90 degrees so that the hamstrings are tightened.
not to weaken hip flexion too much and thus intra-
muscular lengthening of the psoas tendon at the pelvic
brim is advocated. (In the non-walking child, psoas re-
lease at the level of the lesser trochanter is allowed). An
associated fixed flexion deformity of the knee may re-
quire medial hamstring lengthening as well.
Hip internal rotation deformity Internal rotation is usu-
ally associated with flexion and adduction. If so,
adductor release and psoas lengthening will be help-
ful. If, after a few years, rotation is still excessive, a
derotation osteotomy of the femur (subtrochanteric
or supracondylar) may be considered; however, be
warned that this may have to be followed by compen-
satory rotation osteotomy of the tibia.
Hip subluxation Subluxation of the hip occurs in
about 30 per cent of children with cerebral palsy. A
persistent flexion-adduction deformity leads to
femoral neck anteversion. If the abductors are weak
and the child is not fully weightbearing, there is a risk
of acetabular dysplasia and subluxation of the joint; in
non-walkers there may be complete dislocation. Cor-
rection of flexion and adduction deformities (see above) (a) (b)
before the age of 6 years may have a role in preventing 10.14 Spastic knee flexion deformity (a) This boy has
subluxation. Older children may need varus- spastic flexion of the knees due to tight hamstrings.
242 derotation osteotomy of the femur, perhaps com- (b) Here he is after hamstring release.
Capsular contracture of the knee joint is uncommon. 10
Gait analysis can be helpful in deciding whether the
hamstrings are truly short or only functionally short.
Fractional lengthening of the hamstrings (medial
more often than medial and lateral combined) reliably
improves gait mechanics but risks weakening hip
extension and exacerbating hip flexion/lumbar lordo-
sis; this is because the hamstrings normally assist with
hip extension. Fractional lengthening of semimem-
branosus can be combined with detachment and
transfer of semitendinosus to the adductor tubercle at
the distal end of the femur. Good results have been
reported by Ma et al. (2006) in children with bilateral
spastic flexion deformities of more than 15 degrees
combined with a flexed-knee posture when standing
or walking and ability to stand and walk only with (a) (b)
support. 10.15 Spastic equinus (a) Standing posture of a young
Severe flexion deformities (more than 25 or 30 girl with bilateral spastic equinus deformities. (b) Tendo
degrees) have also been treated by extension Achillis lengthening resulted in complete correction and a
osteotomy of the distal femur or by physeal plating balanced posture.
anteriorly.
Remember that knee extension is aided by plan-
tarflexion of the foot in walking, so it is important not
lengthening and plication of the medial structures
to weaken the triceps surae by overzealous lengthen-
when appropriate.
ing of the Achilles tendon (see below).
External tibial torsion may be corrected by a supra-
Spastic knee extension This can usually be corrected by malleolar osteotomy but remember that an externally
simple tenotomy of the proximal end of rectus femoris. rotated gait pattern may be compensating for an
inability of the foot to clear the ground when walking
External tibial torsion This is easily corrected by supra- because of weak muscles/stiff joints.
malleolar osteotomy, but before doing this first ensure
that the deformity is not actually advantageous in Single event multi-level surgery (SEMLS) The diplegic
compensating for an ankle/hindfoot deformity (see patient usually has problems at all levels and often the
below). most appropriate way to improve gait and overall
function is to enhance the mechanical efficiency of
Equinus of the foot The child with spastic diplegia gait by combining changes at hip, knee and ankle.
usually toe-walks. This triggers an excessive plan- Soft-tissue and bony surgery to both limbs can be per-
tarflexion–knee extension couple that may be mani- formed at one sitting or staged over a few weeks.
fested as knee hyperextension. In children with Postoperative rehabilitation is complex and time-con-
limited dorsiflexion, the gastrocnemius is often more suming but the results can be very rewarding.
affected than the soleus. Selective fractional lengthen- A good review of management of lower limb defor-
ing of the fascia/muscle is gaining favour but judi- mities in children with cerebral palsy is presented by
cious percutaneous lengthening of the Achilles Karol (2004).
tendon is still popular. Relative overlengthening is a
problem, particularly when associated knee flexion
contractures exist.
Total body involvement
If a varus deformity is present, treatment is as for All parts of the body are affected; function is generally
the hemiplegic patient described above. The more poor and the aims of surgical intervention differ sig-
common deformity is, however, one of equinovalgus nificantly from those for the hemiplegic or walking
and a ‘rocker-bottom’ foot. It makes the use of splints diplegic patient.
difficult and disrupts the plantarflexion–knee exten-
sion couple, exacerbating a knee flexion posture. It is HIP
important to note whether the hindfoot deformity is Hip subluxation progressing to dislocation is com-
reducible or not. Correction can be achieved by either mon. The adduction and flexion contractures out-
a calcaneal lengthening or displacement osteotomy lined above are more frequent and more severe in this
but often a subtalar fusion is required. Such surgery group of patients, leaving the hip at risk of developing
must be combined with a release of tight structures subluxation with acetabular dysplasia. Hips are often
(such as the Achilles tendon) and possibly peroneal ‘windswept’ (one hip lying adducted, flexed and 243
10 internally rotated while the other lies in abduction extending from the thoracic spine to the pelvis; there
and external rotation and often more extended). is an attempt to recreate a lumbar lordosis but in so
The hip at risk of subluxation must be watched doing it may, at least temporarily, exacerbate ham-
closely and, if necessary, treated by adductor and string tightness making sitting more difficult.
psoas releases as outlined above (a psoas tenotomy at Careful preoperative evaluation is essential to
the lesser trochanter is appropriate). Hip subluxation, ensure that the child is fit for a long and difficult oper-
defined as more than 30 per cent uncovering of the ation that is known to carry a high complication rate,
femoral head, may require a femoral varus derotation including neurological defects, problems with wound
(and shortening) osteotomy as well as an acetabular healing and implant failure. This type of spinal surgery
procedure for correction in addition to the soft-tissue has been shown to increase life expectancy, but
releases. If the hip has dislocated, open reduction, demonstrating a concurrent improvement in quality
release of soft tissues and bony realignment will be of life has been more difficult to prove.
necessary. The alternative is to consider a proximal A good review of this subject is presented by
femoral resection. McCarthy et al., 2006.
The opposite hip may require similar surgery, or in
the case of a windswept deformity, it may benefit from OTHER JOINTS
a release of the hip abductors and extensors, mainly Surgery to other joints may be required and follows
the gluteus maximus and the iliotibial band. the principles outlined above for the hemiplegic and
This is complex surgery and the complication rates diplegic patient.
are high. Some families, and indeed some surgeons,
opt for no active treatment of the subluxed or dislo-
cated hip particularly if it is relatively pain-free and
care of the child is not compromised significantly. ADULT ACQUIRED SPASTIC PARESIS
Others feel that hip subluxation/dislocation should
be prevented at all costs and although recent reports Cerebral damage following a stroke or head injury may
from Scandinavia suggest that hip dislocation is ‘pre- cause persistent spastic paresis in the adult; this can be
ventable’ this is only true with an aggressive regimen accompanied by disturbance of proprioception and
of tone management and surgery which many people stereognosis.
feel causes unnecessary suffering to the child con- In the early recuperative stage, physiotherapy and
cerned. Obviously, the management of such cases splintage are used to prevent fixed deformities; all
brings up moral dilemmas which are best dealt with affected joints should be put through a full range of
by maintaining good communication with the families movement every day. The use of botulinum toxin (as
and therapists at all stages and being clear about the for children with cerebral palsy) may be beneficial in
aims of any intervention. resistant cases (see page 239).
Deformities that are passively correctible should be
SPINE/PELVIS splinted in the neutral position until controlled mus-
Scoliosis is very common (probably appearing in more cle power returns; proprioception and coordination
than 50 per cent) in this group of patients. The defor- can be improved by occupational therapy. Yet even
mity is often a long C-shaped thoracolumbar curve with the best attention, these measures may fail to
and it frequently incorporates the pelvis which is tilted prevent the development of fixed deformities. Once
obliquely so that one hip is abducted and the other maximal motor recovery has been achieved – usually
adducted and threatening to dislocate. Of course the by 9 months after a stroke but more than a year after
adducted hip may be the primary problem with pelvic a brain injury – residual deformities or joint instability
obliquity and scoliosis following; in essence, trunk should be considered for operative treatment. The
muscle involvement due to the cerebral palsy must be patient should have sufficient cognitive ability, aware-
a major determinant of developing deformity. ness of body position in space and good psychological
Various forms of non-operative treatment (as impetus if a lasting result is to be expected.
described on page 239) have been used, and in some In the lower limbs the principal deformities requir-
cases patients opt for long-term use of an adapted ing correction are equinus or equinovarus of the foot,
wheelchair. flexion of the knee and adduction of the hip. In the
Where facilities and surgical expertise are available, upper limb (where the chances of regaining con-
operative correction and spinal stabilization are often trolled movement are less) the common residual
advocated. Indications are a progressive curve of more deformities are adduction and internal rotation of the
than 40 degrees in a child over 10 years, inability to shoulder (often accompanied by shoulder pain), and
sit without support, and a range of hip movement that flexion of the elbow, wrist and metacarpo-phalangeal
will allow the child to sit after spinal stabilization. joints. Treatment is similar to that of spastic deformity
244 Fixation is achieved with pedicle screws and rods in the child, and is summarized in Table 10.2.
Table 10.2 Treatment of the principal deformities of functional and neurological deterioration may be 10
the limbs rapid with the development of a cardiomyopathy and
Deformity Splintage Surgery
death in early to mid adulthood. In other more mild
cases, surgical correction of foot and spine deformities
Foot Equinus Spring-loaded Lengthen tendo may be worthwhile.
Equinovarus dorsiflexion Achillis
Bracing in Lengthen tendo
eversion and Achillis and transfer
dorsiflexion lateral half of tibialis
anterior to cuboid LESIONS OF THE SPINAL CORD
Knee Flexion Long caliper Hamstring release
The three major pathways in the spinal cord are the
Hip Adduction – Obturator
neurectomy
corticospinal tracts (in the anterior columns) carrying
Adductor muscle motor neurons, the spinothalamic tracts carrying sen-
release sory neurons for pain, touch and temperature, and the
Shoulder Adduction – Subscapularis release posterior column tracts serving deep sensibility (joint
position and vibration) (see Fig. 10.2).
Elbow Flexion – Release elbow flexors
Wrist Flexion Wrist splint Lengthen or release Clinical features

wrist flexors; may
need fusion or True lesions of the spinal cord present with a UMN
carpectomy spastic paresis and often a fairly precise sensory level
Fingers Flexion – Lengthen or release that suggests the level of cord involvement. However,
flexors extradural compressive lesions will often involve the
nerve roots as well resulting in a combination of
UMN and LMN signs.
Patients often complain of weakness and numbness
FRIEDREICH’S ATAXIA with loss of balance and possibly alteration in bowel or
bladder control and, in men, impotence. The symp-
Friedreich’s ataxia, though rare itself (1–2/50 000 in toms may be of variable severity and the speed of
the UK) is the most common of the hereditary atax- onset is similarly variable depending mainly on the
ias. It is an autosomal recessive condition which can aetiological factor.
be detected on genetic testing, the defect being a Several ‘classical’ patterns are recognized.
triplet expansion localized to chromosome 9. In the Cervical cord compression The patient typically pres-
USA, about 1 in 90 adults is a carrier for this condi- ents with UMN symptoms in the lower limbs (stiff-
tion. ness and a change in gait pattern) and LMN signs in
The condition presents in childhood (rarely adult- the upper limbs (complaints of numbness and clumsi-
hood) and all patients develop progressive ataxia of ness). Pain is a variable feature. Bladder symptoms are
the limbs and of their gait with associated extensor of frequency and incontinence more commonly than
plantar responses but absent knee and ankle reflexes retention.
and sensory disturbances such as loss of vibration A central cord syndrome may be caused by a hyper-
sense and two-point discrimination. Dysarthria extension injury in a middle-aged patient with long-
appears within 5 years of disease onset. standing cervical spondylosis, or may develop in
The neurological degeneration is seen in the spino- syringomyelia. In these cases there is disproportion-
cerebellar tracts, the corticospinal tracts, the posterior ately more UMN weakness in the upper limbs com-
columns of the spinal cord and parts of the cerebellum pared to the lower limbs with bladder dysfunction and
itself. Nerve conduction studies demonstrate slowed a variable sensory loss below the lesion.
motor velocities in both median and tibial nerves with
Thoracic cord compression This typically presents as a
absent sensory action potentials in the sural and digi-
tal nerves. UMN paralysis affecting the lower limbs, together
Painful muscle spasms occur in some patients and if with variable sensory loss depending on the degree of
so they tend to worsen with time. The more common involvement of the dorsal columns or the spinothala-
orthopaedic complaints are a progressive cavo-varus mic tracts.
foot deformity that is usually rigid, the development Lumbar cord compression The spinal cord terminates
of clawed toes and a scoliosis. In general, the earlier around the level of L1 so compression here may
the onset of the disease the greater is the risk of sig- involve the conus medullaris or the cauda equina or
nificant curve progression. In the more severe cases, both, giving a mixture of UMN and LMN signs. The 245
10 typical cauda equina syndrome consists of lower limb Table 10.3 Causes of spinal cord dysfunction
weakness, absent reflexes, impaired sensation and uri-
Acute injury
nary retention (with overflow perhaps mimicking
incontinence). Vertebral fractures
Fracture-dislocation
Brown-Séquard lesion The pure form of this syndrome
Infection
is very unusual but less pure forms are common and

serve as a reminder that careful assessment of the neu- Epidural abscess
rological symptoms and signs is important in helping Poliomyelitis
the clinician to localize the pathology and understand Intervertebral disc prolapsed
its aetiology. The pure lesion is defined as an incom-
Sequestrated disc
plete hemispherical cord lesion: below the lesion there
Disc prolapse in spinal stenosis
is ipsilateral UMN weakness and posterior column
dysfunction, with contralateral loss of skin sensibility; Vertebral canal stenosis
at the level of the lesion there is ipsilateral loss of sen- Congenital stenosis
sibility. Acquired stenosis
Spinal shock Acute cord lesions at any level may pres- Spinal cord tumours
ent with a flaccid paralysis which resolves over time, Neurofibroma
usually to reveal the more typical UMN signs associ- Meningioma
ated with cord injury. Intrinsic cord lesions
Tabes dorsalis
Diagnosis and management Syringomyelia
Other degenerative disorders
The more common causes of spinal cord dysfunction
are listed in Table 10.3. Traumatic and compressive Miscellaneous
lesions are the ones most likely to be seen by Spina bifida
orthopaedic surgeons. Plain x-rays will show struc- Vascular lesions
tural abnormalities of the spine; cord compression Multiple lesions
Multiple sclerosis
Haemorrhagic disorders
can be visualized by myelography, alone or combined

with CT. Intrinsic lesions of the cord require further
investigation by blood tests, CSF examination and
MRI.
Acute compressive lesions require urgent diagnosis
and treatment if permanent damage is to be pre-
vented. Bladder dysfunction is ominous: whereas
motor and sensory signs may improve after decom-
pression, loss of bladder control, if present for more
than 24 hours, is usually irreversible.
Spinal injury is dealt with in Chapter 25 but a few
important points deserve mention here.
• Any spinal injury may be associated with cord dam-
age, and great care is needed in transporting and
examining the patient.
• In the early period of ‘spinal shock’ the usual pic-
ture is one of flaccid paralysis, with or without pri-
apism.
• Plain x-rays seldom show the full extent of bone
displacement, which is much better displayed by
CT or MRI.
• Unstable injuries usually need operative decom-
pression and/or stabilization; stable injuries can be
246 10.16 The Brown-Séquard syndrome treated conservatively.
• Many centres consider the use of corticosteroids tabes dorsalis and syringomyelia – may present with 10
beneficial in terms of reducing the degree of per- orthopaedic problems because of neuropathic joint
manent neurological damage but the side effects of destruction.
gastrointestinal haemorrhage and avascular necrosis Tabes dorsalis is a late manifestation of syphilis caus-
are potentially serious. ing degeneration (‘tabes’ means wasting) of the pos-
terior columns of the spinal cord. A pathognomonic
Epidural abscess is a surgical emergency. The patient feature is ‘lightning pains’ in the lower limbs. Much
rapidly develops acute pain and muscle spasm, with later other neurological features appear: sensory
fever, leucocytosis and elevation of the ESR. X-rays may ataxia, which causes a stamping gait; loss of position
show disc space narrowing and bone erosion. Treat- sense and sometimes of pain sensibility; trophic
ment is by immediate decompression and antibiotics. lesions in the lower limbs; progressive joint instability;
Acute disc prolapse usually causes unilateral symp- and almost painless destruction of joints (Charcot
toms and signs. However, complete lumbar disc pro- joints). There is no treatment for the cord disorder.
lapse may present as a cauda equina syndrome with
Syringomyelia In syringomyelia a long cavity (the
urinary retention and overflow; spinal canal obstruc-
syrinx) filled with CSF develops within the spinal
tion is demonstrated by MRI.
cord, most commonly in the cervical region. Usually
Operative discectomy is urgent.
the cause is unknown but the condition is sometimes
Chronic discogenic disease is often associated with
associated with tumours, or spinal cord injury in
narrowing of the intervertebral foramina and com-
adults and congenital anomalies with hydrocephalus
pression of nerve roots (radiculopathy), and occasion-
and herniation of the cerebellar tonsils in children.
ally with bony hypertrophy and pressure on the spinal
Symptoms and signs are most noticeable in the
cord (myelopathy). Diagnosis is usually obvious on x-
upper limbs. The expanding cyst presses on the ante-
ray and MRI.
rior horn cells, producing weakness and wasting of the
Operative decompression may be needed.
hand muscles. Also, destruction of the decussating
Spinal stenosis produces a typical clinical syndrome,
spinothalamic fibres in the centre of the cord produces
due partly to direct pressure on the cord or nerve
a characteristic dissociated sensory loss in the upper
roots and partly to vascular obstruction and ischaemic
limbs: impaired response to pain and temperature but
neuropathy during hyperextension of the lumbar
preservation of touch. There may be trophic lesions in
spine. The patient complains of ‘tiredness’, weakness
the fingers and neuropathic arthropathy (‘Charcot
and sometimes aching or paraesthesia in the lower
joints’) in the upper limbs. CT may reveal an expanded
limbs after standing or walking for a few minutes,
cord and the syrinx can be defined on MRI.
symptoms that are relieved by bending forward, sit-
Deterioration may be slowed down by decompres-
ting or crouching so as to flex the lumbar spine.
sion of the foramen magnum.
Congenital narrowing of the spinal canal is rare,
except in developmental disorders such as achon-
droplasia, but even a moderately reduced canal may
be further narrowed by osteophytes, thus compromis-
ing the cord and nerve roots. SPINA BIFIDA
Treatment calls for bony decompression of the
nerve structures. Spina bifida is a congenital disorder in which the two
Vertebral disease, such as tuberculosis or metastatic halves of the posterior vertebral arch fail to fuse at one
disease, may cause cord compression and paraparesis. or more levels. This neural tube defect, or spinal dys-
The diagnosis is usually obvious on x-ray, but a needle raphism, which occurs within the first month of foetal
biopsy may be necessary for confirmation. life, usually affects the lumbar or lumbosacral seg-
Management is usually by anterior decompression ments of the spine. In its most severe form, the con-
and, if necessary, internal stabilization. However, in dition is associated with major neurological problems
metastatic disease, if the prognosis is poor it may be in the lower limbs together with incontinence.
wise also to use radiotherapy and corticosteroids, plus
narcotics for pain.
Spinal cord tumours are a comparatively rare cause
Pathology
of progressive paraparesis. X-rays may show bony Spina bifida occulta In the mildest forms of dys-
erosion, widening of the spinal canal or flattening of raphism there is a midline defect between the laminae
the vertebral pedicles. Widening of the intervertebral and nothing more; hence the term ‘occulta’. Most
foramina is typical of neurofibromatosis. Treatment cases are discovered incidentally on spine x-rays (usu-
usually involves operative removal of the tumour. ally affecting L5). However, in some cases – and espe-
Intrinsic lesions of the cord produce slowly progres- cially if several vertebrae are affected – there are
sive neurological signs. Two conditions in particular – telltale defects in the overlying skin, for example, a 247
10 dimple, a pit or a tuft of hair. Occasionally there are affected the risk for future siblings is significantly
associated intraspinal anomalies, such as tethering of higher.
the conus medullaris below L1, splitting of the spinal Neural tube defects are associated with high levels
cord (diastematomyelia) and cysts or lipomas of the of alpha-fetoprotein (AFP) in the amniotic fluid and
cauda equina. serum. This offers an effective method of antenatal
screening during the 15th to 18th week of pregnancy.
Spina bifida cystica In the more overt forms of dys-

Maternal blood testing is performed routinely at
raphism the vertebral laminae are missing and the
15–18 weeks and followed by an amniocentesis if nec-
contents of the vertebral canal prolapse through the
essary. A mid-term high resolution ultrasound scan
defect. The abnormality takes one of several forms.
will detect 95 per cent of cases of spina bifida and, in
The least disabling is a meningocele, which accounts
many countries, counselling regarding a termination
for about 5 per cent of cases of spina bifida cystica.
is offered. If the pregnancy is continued, arrange-
The dura mater is open posteriorly but the meninges
ments should be made to ensure that appropriate
are intact and a CSF-filled meningeal sac protrudes
services are available at birth and in the neonatal
under the skin. The spinal cord and nerve roots
period to minimize the risk of further neurological
remain inside the vertebral canal and there is usually
damage.
no neurological abnormality.
Folic acid, 400 micrograms daily taken before con-
The most common and most serious abnormality is
ception and continuing through the first 12 weeks of
a myelomeningocele, which usually occurs in the lower
pregnancy, has been shown to reduce the risk of neu-
thoracic spine or the lumbosacral region. Part of the
ral tube defects in the fetus.
spinal cord and nerve roots prolapse into the
meningeal sac. In some cases the neural tube is fully
formed and the sac is covered by a membrane and/or Clinical features
skin – a ‘closed’ myelomeningocele. In others the cord is
EARLY DIAGNOSIS
in a more primitive state, the unfolded neural plate
The major neural tube defects can easily be detected
forming the roof of the sac – an ‘open’ myelomeningo-
on antenatal scans or identified immediately at birth.
cele which is always associated with a neurological
Spina bifida occulta is often encountered in normal
deficit distal to the level of the lesion. If neural tissue
people, and can usually be ignored. However, a pos-
is exposed to the air, it may become infected, leading
terior midline dimple, a tuft of hair or a pigmented
to more severe abnormality and even death.
naevus signifies the potential for something more seri-
Hydrocephalus Distal tethering of the cord may cause ous. Children may present with mild neurological
herniation of the cerebellum and brain-stem through symptoms: enuresis, urinary frequency or intermittent
the foramen magnum, resulting in obstruction to CSF incontinence; neurological examination may reveal
circulation and hydrocephalus. The ventricles dilate weakness and some loss of sensibility in the lower
and the skull enlarges by separation of the cranial limbs. Plain x-rays may show the laminar defect and
sutures. Persistently raised intracranial pressure may any associated vertebral anomalies; a midline ridge of
cause cerebral atrophy and learning difficulties. bone suggests bifurcation of the cord (diastemato-
myelia). Intraspinal anomalies are best shown by
MRI.
Incidence and screening Spina bifida cystica is usually obvious at birth in the
Isolated laminar defects are seen in over 5 per cent of shape of a saccular lesion overlying the lumbar spine.
lumbar spine x-rays but cystic spina bifida is rare at 2– It may be covered only with membrane, or with
3 per 1000 live births. However, if one child is membrane and skin. In open myelomeningoceles the
(a) (b) (c) (d)
248 10.17 Dysraphism (a) Spina bifida occulta. (b) Meningocele. (c) Myelomeningocele. (d) Open myelomeningocele.
neural elements form the roof of the cyst, which defect or to conditions such as a diastematomyelia, 10
merges into plum-coloured skin at its base. Meningo- and with growth there is progressive damage to the
celes are covered by normal looking skin. cord and/or nerve roots. MRI with gadolinium
Hydrocephalus may be present at birth; with a com- enhancement is the investigation of choice and neu-
municating hydrocephalus the intracranial pressure rosurgical release the treatment of choice before any
may not be elevated until leakage from the spinal further neurological damage occurs.
lesion is arrested by surgical closure of the lesion. Older children with neurological lesions are liable
The baby’s posture may suggest some type of paral- to suffer fractures after minor injuries. These may not
ysis, or even the neurological level of the lesion. always be obvious but suspicion should be raised by
Deformities of the lower limbs such as equinovarus or the appearance of swelling, warmth and redness in the
calcaneovalgus of the feet, recurvatum of the knee and limb.
hip dislocation are common and probably due to a
combination of factors such as muscle imbalance, lack
Treatment
of movement and abnormal limb position in utero, or
to associated anomalies that are independent of the In recent years intrauterine surgery has been
paralysis. attempted: closure of the defect is possible but a
Muscle charting, although difficult, is possible in reduction in neurological disability has not yet been
the neonate and should be performed so that neuro- identified.
logical deterioration can be identified promptly. In After birth, care must be taken to dress the ‘wound’
about one-third of infants with myelomeningocele and prevent infection of these vulnerable tissues. For-
there is complete LMN paralysis and loss of sensation mal neurosurgical closure of the defect should take
and sphincter control below the affected level; in one- place within 48 hours of birth in order to prevent dry-
third there is a complete lesion at some level but a dis- ing and ulceration, or infection of the lesion. All neu-
tal segment of cord is preserved, giving a mixed ral tissue should be carefully preserved and covered
neurological picture with intact segmental reflexes with dura; the skin is then widely undercut to facilitate
and spastic muscle groups; in the remaining third the complete closure. However some centres avoid urgent
cord lesion is incomplete and some movement and operation if the neurological level is high (above L1),
sensation are preserved. if spinal deformities are very severe or if there is
X-rays and CT will show the extent of the bony marked hydrocephalus.
lesion as well as other vertebral anomalies. MRI may A few weeks later, when the back has healed, the
be helpful to define the neurological defects. degree of hydrocephalus is assessed. Almost all children
also have the Arnold–Chiari malformation with dis-
CLINICAL FEATURES IN OLDER CHILDREN placement of the posterior fossa structures through
The minor forms of spina bifida may present clinically the foramen magnum. Thus, around 90 per cent of
at any age. The physical signs mentioned above may children will require active management of their real
have been noted previously and the child (or or potential hydrocephalus in the form of a ven-
teenager) now presents with clawing of the toes, a triculo-peritoneal shunt (VP shunt) to reduce the risk
change in gait pattern, incontinence or abnormal sen- of further damage to their CNS. A chronically raised
sation. This delayed presentation is often attributed to intracranial pressure may be associated with learning
the tethered cord syndrome. Tethering may be second- difficulties and other problems. Similarly, if a child’s
ary to the early surgical reconstruction of the major neurological status changes unexpectedly, shunt
10.18 Spina bifida

(a) Baby with spina bifida
cystica (myelomeningocele).
(b) Tuft of hair over the
lumbosacral junction. X-ray in
this case showed a sacral
defect (c).
(a) (b) (c) 249

10 LEVELS EARLY MANAGEMENT-TIMING
Hip Knee Age Procedure

1 day Close skin defect
L1
Flexion L2 1 week Ventriculo-caval shunt

Adduction L3 Extension
L4 1 month Stretch and strap
Abduction L5 Flexion
S1 6 months to 3 years Orthopaedic operations
Extension S2
Whenever needed Urogenital operations
10.19 Spina bifida The diagram shows the root levels concerned with hip and knee movements. The table is a simple
guide to the timing of operations.
problems such as infection/blockage should be con- important for independence in the activities of daily
sidered. living than walking and, for many patients, the abil-
Ventriculo-peritoneal drainage can be maintained ity to sit comfortably is more important than the
(if necessary, by changing the valve as the baby grows) ability to stand awkwardly.
for 5 or 6 years, by which time the tendency to hydro- • The best predictor of walking ability and function is
cephalus usually ceases. the motor level of the paralysis. Children with
Management of neonatal deformities will vary lesions below L4 will have quadriceps control and
depending on the overall clinical picture, but physio- active knee extension and should be encouraged to
therapy and/or splinting will be the mainstays of early walk. Children with higher lesions may start off
treatment. It must be remembered that the skin is walking with the aid of orthotic devices but they are
likely to be insensate and pressure area care is essen- likely to opt for a wheelchair with time.
tial. • Immobilization and muscle imbalance both lead to
In the more severe forms of spina bifida, there must joint deformity and the risk of pathological fracture.
be a multidisciplinary approach to treatment from Physiotherapists working to correct, or indeed pre-
early infancy through to adulthood. Orthopaedic vent, joint deformity must understand the risk of
management is important but so is the management fracture, and orthotists must take into considera-
of the neurological lesion in terms of urological function the need for lightweight appliances and beware
tion and bladder/bowel control. The vast majority of the risk of pressure sores when using splints.
patients have urological problems necessitating the • Latex allergy is present in some children with spina
use of catheters or urinary diversion. Botulinum toxin bifida and a history of allergic reactions should be
injections may increase capacity and improve conti- noted. All treatment, including surgery, must be
nence. conducted in a latex-free environment. If a positive
The psychosocial aspects of the condition must also history is identified, antihistamines and/or corti-
be borne in mind; they can be overwhelming to the costeroids should be given.
child and his or her family and require patient atten-
tion.
ORTHOPAEDIC MANAGEMENT REGIONAL SURVEY

The orthopaedic surgeon, working as part of a team,
must identify the important treatment goals while Spine
bearing in mind some basic observations:
Spinal deformity (scoliosis and/or kyphosis) is com-
• Except in the mildest cases, the late functional out- mon in children with myelomeningocele, due to a
come cannot be predicted until the child is assessed combination of muscle weakness and imbalance, asso-
both intellectually and in terms of neuromuscular ciated congenital vertebral anomalies (in about 20 per
function around the age of 3–4 years. cent of cases) and the tethered cord syndrome.
• Most patients with myelomeningocele will never be Distal tethering of the cord or other neural ele-
functionally independent. ments is almost inevitable after repair of a
• The maintenance and development of intellectual myelomeningocele; this may be harmless, but it can
250 skills and upper limb function are often more cause pain and progression of neurological dysfunc-
tion during phases of rapid growth, and in some cases For children with ‘in between’ lesions, muscle 10
it gives rise to scoliosis. Diagnosis may be aided by CT imbalance is the main problem and many hips (up to
and MRI. Indications for operative release of the teth- 50 per cent) will sublux or dislocate by early child-
ered cord are increasing pain and neurological dys- hood. The effect of hip joint subluxation or disloca-
function or progressive spinal deformity. tion (and its associated pelvic obliquity) on spinal
Kyphosis may result in stretching and breakdown, or development is unclear, but the natural history of hip
chronic ulceration, of the overlying skin posteriorly joint function in these children can be surprisingly
and compression of the abdominal and thoracic vis- good. This has led to the recognition that retaining
cera anteriorly. Treatment is difficult and may require hip movement may be more useful than striving for
localized vertebral resection and arthrodesis. How- hip reduction by multiple operations, with their atten-
ever, the cord at the affected level is often non-func- dant complications and uncertain prognosis. There is,
tioning and therefore the risks of further neurological as yet, a lack of convincing evidence to suggest that
insult influencing the outcome are small. function is improved significantly by operative hip
Paralytic scoliosis appears as a long C-shaped curve relocation.
which is usually progressive and makes sitting particu-
larly difficult. It is unlikely to respond to a brace.
Molded seat inserts for the wheelchair are essential to
Knee
aid sitting balance and independence and may help re- Unlike the hip, the knee usually presents no problem,
duce the rate of curve deterioration. Surgery via an an- because the aim is simple – a straight knee suitable for
terior, a posterior or a combined approach is often wearing callipers and using gait-training devices. In
necessary and fusion to the pelvis may be required, al- older children fixed flexion may follow prolonged sit-
though this tends to reduce walking ability in ambulant ting. If stretching (by distraction) fails to correct this
patients – at least temporarily. The operation is always deformity, one or more of the hamstrings may be
difficult and carries a high risk of complications, par- lengthened, divided or reinserted into the femur or
ticularly postoperative infection and implant failure. patella; this may have to be combined with a posterior
capsular release. However, if the likely prognosis is
that the patient will be wheelchair dependent, flexion
Hip contractures are, of course, less of a problem.
Patients with spina bifida present a wide spectrum of Some children are born with a hyperextension con-
hip problems, the management of which is still being tracture and on occasion the hamstring tendons are
debated. In our approach the general aim is to secure subluxed anteriorly. Physiotherapy and sometimes
hips that have enough movement to enable the child serial casting are the treatments of choice initially but
both to stand up in calipers and to sit comfortably. a V–Y quadricepsplasty and hamstring lengthening
If the neurological level of the lesion is above L1, may be required in order to achieve enough knee flex-
all muscle groups are flaccid and splintage is the only ion to facilitate standing.
option; in the long term, the child will probably use a Walking patients often develop a valgus knee, in
wheelchair. With lesions below S1 a hip flexion con- some cases with torsional abnormalities in the lower
tracture is the most likely problem and this can be cor- limb. Secondary joint instability can further exacer-
rected by elongation of the psoas tendon combined bate the problems of walking, with patients relying
with detachment of the flexors from the ilium (the more and more on the use of forearm crutches and a
Soutter operation). swing-through gait.
Foot
Foot deformities are among the most common prob-
lems in children with spina bifida. The aim of treat-
ment is a mobile foot, with healthy skin and soft
tissues that will not break down easily, that can be
held or braced in a plantigrade position.
A flail foot or one that has a balanced paralysis or
weakness is relatively easy to treat and only requires
the use of accurately made orthoses (e.g. an ankle–
foot orthosis) or occasionally simply well-fitting ankle
boots.
Equinovarus deformity is likely to be more severe
10.20 Spina bifida Muscle imbalance may lead to (and more resistant to treatment) than the ‘ordinary’
bilateral hip dislocation. clubfoot. The standard treatment has been an 251
10 aggressive soft-tissue release, but increasingly there The acute illness Early symptoms are fever and
have been reports of success with the Ponseti tech- headache; in about one-third of cases the patient gives
nique of gentle manipulation towards progressive a history of a minor illness with sore throat, mild
correction, holding the feet in well-moulded plaster headache and slight pyrexia 5–7 days before. As the
casts which are changed every week for about 8–10 symptoms increase in severity, neck stiffness appears
weeks; in some cases a subcutaneous tendo achillis and meningitis may be suspected. The patient lies
tenotomy is needed to fully correct the equinus (Pon- curled up with the joints flexed; the muscles are
seti and Smoley, 1963). This primary treatment may painful and tender and passive stretching provokes
have to be followed later by further release of tight painful spasms.
tendons and/or a tendon transfer. Bony procedures
Paralysis Soon muscle weakness appears; it reaches a
are reserved for residual or recurrent deformity in the
peak in the course of 2–3 days and may give rise to
older child.
difficulty with breathing and swallowing. If the
A vertical talus deformity can be treated in a similar
patient does not succumb from respiratory paralysis,
way by a ‘reverse Ponseti’ regimen and transfer of the
pain and pyrexia subside after 7–10 days and the
tibialis anterior tendon to the neck of the talus, but
patient enters the convalescent stage. However, he or
surgical correction of this deformity is often required.
she should be considered to be infective for at least 4
Toe deformities sometimes cause concern because
weeks from the onset of illness.
of pressure points and difficulty fitting shoes.
‘Orthopaedic shoes’ with a high toe box may be Recovery and convalescence A return of muscle power
needed and could be more appropriate than surgical is most noticeable within the first 6 months, but there
intervention. may be continuing improvement for up to 2 years.
Residual paralysis In some patients the illness does not
progress beyond the early stage of meningeal irrita-
POLIOMYELITIS tion; some, again, who develop muscle weakness
recover completely; in others recovery is incomplete
Poliomyelitis is an acute infectious viral disease, spread
and they are left with some degree of asymmetric flac-
by the oropharyngeal route, that passes through sev-
cid (LMN) paralysis or unbalanced muscle weakness
eral distinct phases. Only around 10 per cent of
that in time leads to joint deformities and growth
patients exhibit any symptoms at all and involvement
defects. Although sensation is intact, the limb often
of the CNS occurs in less than 1 per cent of cases with
appears cold and blue.
effects on the anterior horn cells of the spinal cord
and brain-stem, leading to LMN (flaccid) paralysis of Post-polio syndrome Although it was generally held
the affected muscle groups. The poliomyelitis viruses that the pattern of muscle weakness became firmly
have varying virulence and in countries where vacci- established by 2 years, it is now recognized that in up
nation is encouraged it has become a rare disease; to 50 per cent of cases reactivation of the virus results
however, the effects of previous infection are still with in progressive muscle weakness in both old and new
us today. muscle groups, giving rise to unaccustomed fatigue. If
this occurs in patients with a confirmed history of
poliomyelitis and a period of neurological stability of
Clinical features at least 15 years then the diagnosis of post-polio syn-
Poliomyelitis typically passes through several clinical drome (PPS) must be considered. PPS is, however, a
phases, from an acute illness resembling meningitis to diagnosis of exclusion and care must be taken to
paralysis, then slow recovery or convalescence and investigate for other medical diagnoses that might
finally the long period of residual paralysis. The dis- explain the new symptoms. The older the child was at
ease strikes at any age but most commonly in children. the onset of disease, the more severe the disease was
10.21 Poliomyelitis
(a) Shortening and wasting of
the left leg, with equinus of
the ankle. (b) This long curve
is typical of a paralytic
scoliosis. (c) Paralysis of the
right deltoid and supraspinatus
makes it impossible for this
boy to abduct his right arm.
252 (a) (b) (c)

which makes weightbearing and walking impossible 10
without some type of brace) or loss of complex func-
tion (e.g. thumb opposition, which can be treated by
tendon transfer).
Passively correctible deformity Any unbalanced paraly-
sis (i.e. muscle weakness on one aspect of a joint and
greater power in the antagonists) can lead to defor-
mity. At first this is passively correctable and can be
(a) (b) (c) counteracted by a splint (a calliper or lightweight
10.22 Poliomyelitis – treatment Opponens paralysis has brace). However, an appropriate tendon transfer may
been treated by superficialis tendon transfer. In (b) the solve the problem permanently. It is here that mus-
tendon can be seen in action at the start of thumb cle charting is particularly important. A muscle usu-
opposition. (c) Full opposition achieved. ally loses one grade of power when it is transferred;
therefore, to be really useful, it should have grade 4
or 5 power, although a grade 3 muscle may act as a
likely to have been and the more likely is it that the sort of tenodesis and reduce the deformity caused by
adult would develop PPS. gravity.
Fixed deformity Fixed deformities cannot be corrected
Early treatment by either splintage or tendon transfer alone; it is
During the acute phase the patient is isolated and kept important also to restore alignment operatively and to
at complete rest, with symptomatic treatment for pain stabilize the joint, if necessary, by arthrodesis. This is
and muscle spasm. Active movement is avoided but especially applicable to fixed deformities of the ankle
gentle passive stretching helps to prevent contrac- and foot, but the same principle applies in treating
tures. Respiratory paralysis calls for artificial respira- paralytic scoliosis.
tion. Occasionally a fixed deformity is beneficial. Thus,
Once the acute illness settles, physiotherapy is an equinus foot may help to compensate mechanically
stepped up, active movements are encouraged and for quadriceps weakness; if so, it should not be cor-
every effort is made to regain maximum power. rected.
Between exercise periods, splintage may be necessary Flail joint Balanced paralysis, because it causes no
to maintain joint and limb alignment and prevent deformity, may need no treatment. However, if the
fixed deformities. joint is unstable or flail it must be stabilized, either by
Muscle charting (see page 230) is carried out at permanent splintage or by arthrodesis.
regular intervals until no further recovery is detected.
Shortening Normal bone growth depends on normal
muscle activity; thus many children who have been
Late treatment affected with poliomyelitis in their early years can be
expected to develop a difference in leg length. Dis-
Once the severity of residual paralysis has been estab-
crepancies of up to 3–5 cm can, in theory, be com-
lished, there are a number of basic problems that need
pensated for with a shoe raise although this tends to
to be addressed.
make the shorter (and weaker) leg clumsier. While leg
Isolated muscle weakness without deformity Isolated lengthening is always an option, the fact that the
muscle weakness, even in the absence of joint defor- increase in length discrepancy with growth can be cal-
mity, may cause instability (e.g. quadriceps paralysis culated fairly accurately from growth tables means it
10.23 Poliomyelitis –
arthrodesis (a) This patient
had paralysis of the left
deltoid: after arthrodesis
(b) he could lift his arm (c) by
using his scapular muscles.
(a) (b) (c) 253

10 can also be mitigated by a well-timed epiphyseodesis In the thumb, weakness of opposition can be over-
in the normal limb. come by a flexor superficialis transfer. The tendon
Disturbance of skeletal modelling As with all childhood
(usually of the ring finger) is wound round that of
paralytic disorders, the effects of muscle imbalance on flexor carpi ulnaris (which acts as a pulley), threaded
the growing skeleton must be anticipated. Changes across the palm and fixed to the distal end of the first
may become obvious with growth, appearing as tor- metacarpal.
sional deformities or angular deformities in either the

sagittal or the coronal plane. Moreover, muscle and Trunk
joint contractures may aggravate the effects of any
Unbalanced paralysis causes scoliosis, frequently a
bone distortion. Any changes that interfere with func-
long thoracolumbar curve which may involve the
tion should be prevented or treated as soon as possi-
lumbosacral junction, causing pelvic obliquity. Opera-
ble.
tive treatment is often needed, the most effective
Vascular dysfunction Sensation is intact but the paralysed being a combination of anterior and posterior instru-
limb is often cold and blue. Large chilblains sometimes mentation and fusion (see page 463).
develop and sympathectomy may be needed.
Hip
REGIONAL SURVEY Hip deformities are usually complex and difficult to
manage; the problem is often aggravated by the grad-
Treatment is often concentrated on the lower limbs ual development of subluxation or dislocation due
but this should not be at the expense of upper limb either to muscle imbalance (abductors weaker than
function. For children who are dependent on walking adductors) or pelvic obliquity associated with scolio-
aids and/or wheelchairs, obtaining and maintaining sis. Furthermore, since paralysis usually occurs before
bimanual function can be very important. the age of 5 years, growth of the proximal femur is
abnormal and this may result in secondary deformities
such as persistent anteversion of the femoral neck,
Shoulder coxa valga and underdevelopment of the acetabular
Provided the scapular muscles are strong, abduction socket – all of which will increase the tendency to
at the shoulder can be restored by arthrodesing the instability and dislocation.
gleno-humeral joint (50 degrees abducted and 25 The keys to successful treatment are: (1) to reduce
degrees flexed). Contracted adductors may need divi- any scoliotic pelvic obliquity by correcting or improv-
sion. ing the scoliosis; (2) to overcome or improve the mus-
cle imbalance by suitable tendon transfer; (3) to
correct the proximal femoral deformities by
Elbow and forearm intertrochanteric or subtrochanteric osteotomy; and
At the elbow, flexion can be restored in one of two (4) to deepen the acetabular socket, if necessary, by an
ways. If there is normal power in the anterior forearm acetabuloplasty which will prevent posterior displace-
muscles (wrist and finger flexors) the common flexor ment of the femoral head.
origin can be moved more proximally on the distal Fixed flexion can be treated by Soutter’s muscle
humerus to provide better leverage across the elbow. slide operation or by transferring psoas to the greater
Alternatively, if the pectoralis major is strong, the trochanter. For fixed abduction with pelvic obliquity
lower half of the muscle can be detached at its origin the fascia lata and iliotibial band may need division;
on the rib-cage, swung down and joined to the biceps occasionally, for severe deformity, proximal femoral
tendon. osteotomy may be required as well. With this type of
Pronation of the forearm can be strengthened by obliquity the ‘higher’ hip tends to be unstable and the
transposing an active flexor carpi ulnaris tendon across ‘lower’ hip to have fixed abduction; if the abducted
the front of the forearm to the radial border. Loss of hip is corrected first the pelvis may level and the other
supination may be countered by transposing flexor hip become normal.
carpi ulnaris across the back of the forearm to the dis-
tal radius.
Knee
Instability due to relative weakness of the knee exten-
Wrist and hand sors is a major problem. Unaided walking may still be
Wrist deformity or instability can be markedly possible provided the hip has good extensor power
improved by arthrodesis. Any active muscles can then and the foot good plantarflexion power (or fixed equi-
254 be used to restore finger movement. nus); with this combination the knee is stabilized by
being thrust into hyperextension as body weight 10
comes onto the leg. The patient has often learnt to
MOTOR NEURON DISORDERS
help this manoeuvre by placing a hand on the front of
the thigh and pushing the thigh backwards with every Rare degenerative disorders of the large motor neurons
stance phase of gait. If the hip or ankle joints are also may cause progressive and sometimes fatal paralysis.
weak, a full-length calliper will be required, or a
supracondylar extension osteotomy of the femur must Motor neuron disease (amyotrophic
be considered. lateral sclerosis)
Fixed flexion with flexors stronger than extensors is
more common and must be corrected. Flexor-to- This is a degenerative disease of unknown aetiology. It
extensor transfer (e.g. hamstring muscles to the affects both cortical (upper) motor neurons and the an-
patella or the quadriceps tendon) is feasible if the terior horn cells of the cord, causing widespread UMN
flexor muscles are normal; however, quadriceps power and LMN symptoms and signs. Patients usually present
is unlikely to be improved by more than one grade. If in middle age with dysarthria and difficulty in swallow-
the flexors are not strong enough, the deformity can ing or, if the limbs are affected, with muscle weakness
be corrected by supracondylar extension osteotomy. (e.g. clumsy hands or unexplained foot-drop) and wast-
Marked hyperextension (genu recurvatum) some- ing in the presence of exaggerated reflexes. Muscle
times occurs, either as a primary deformity or second- cramps are troublesome; muscle atrophy and fascicula-
ary to fixed equinus. It can be improved by tions may be obvious. Sensation and bladder control are
supracondylar flexion osteotomy; an alternative is to normal. Some of these features are also seen in spinal
excise the patella and slot it into the upper tibia where cord compression, which can be excluded by MRI.
it acts as a bone block (Hong-Xue Men et al., 1991). The disease is progressive and incurable. Patients
usually end up in a wheelchair and have increasing dif-
ficulty with speech and eating. Cognitive function is
Foot usually spared although some patients have associated
Instability and foot-drop can be controlled by an frontotemporal dementia or a pseudobulbar effect
ankle–foot orthosis or a below-knee calliper. Often causing emotional lability. Most of them die within 5
there is imbalance causing varus, valgus or calcaneo- years from a combination of respiratory weakness and
cavus deformity; fusion in the corrected position aspiration pneumonia.
should be combined with tendon re-routing to
restore balance, otherwise there is risk of the defor- Spinal muscular atrophy
mity recurring. In this rare group of heritable disorders (a defect on
For varus or valgus the simplest procedure is to slot the long arm of chromosome 5 has been identified)
bone grafts into vertical grooves on each side of the there is widespread degeneration of the anterior horn
sinus tarsi (Grice); alternatively, a triple arthrodesis cells in the cord, leading to progressive LMN weak-
(Dunn) of subtalar and mid-tarsal joints is performed, ness. The commonest form (Werdnig–Hoffman dis-
relying on bone carpentry to correct deformity. With ease) is inherited as an autosomal recessive and is
associated foot-drop, Lambrinudi’s modification is diagnosed at birth or soon afterwards. The baby is
valuable; triple arthrodesis is performed but the fully floppy and weak, feeding is difficult and breathing is
plantarflexed talus is slotted into the navicular with shallow. Death occurs, usually within a year.
the forefoot in only slight equinus: foot-drop is cor- A less severe form (Kugelberg–Welander disease), of
rected because the talus cannot plantarflex further, either dominant or recessive inheritance, is usually
and slight equinus helps to stabilize the knee. With seen in adolescents or young adults who present with
calcaneocavus deformity, Elmslie’s operation is useful: limb weakness, proximal muscle wasting and ‘para-
triple arthrodesis is performed in the calcaneus posi- lytic’ scoliosis. However, it sometimes appears in early
tion, but corrected at a second stage by posterior childhood as a cause of delayed walking. Patients may
wedge excision combined with tenodesis using half of live to 30–40 years of age but are usually confined to
the tendo achillis. a wheelchair. Spinal braces are used to improve sitting
There is a low incidence of secondary osteoarthritis ability; if this cannot prevent the spine from collaps-
in the joints adjacent to the arthrodesed joint because ing, operative instrumentation and fusion is advisable.
of the relatively low demands placed on the paralytic
limb.
Claw toes, if the deformity is mobile, are corrected
by transferring the toe flexors to the extensors; if the PERIPHERAL NEUROPATHIES
deformity is fixed, the interphalangeal joints should be
arthrodesed in the straight position and the long Disorders of the peripheral nerves may affect motor,
extensor tendons reinserted into the metatarsal necks. sensory or autonomic functions, may be localized to a 255
10 short segment or may involve the full length of the Table 10.4 Causes of polyneuropathy
nerve fibres including their cell bodies in the anterior
Hereditary
horn (motor neurons), posterior root ganglia (sen-
sory neurons) and autonomic ganglia. In some cases Hereditary motor and sensory neuropathy
spinal cord tracts are involved as well. There are over Friedreich’s ataxia
100 types of neuropathy; in this section we consider Hereditary sensory neuropathy
those conditions that are most likely to come within Infections

the ambit of the orthopaedic surgeon.
Viral infections
Herpes zoster
Classification Neuralgic amyotrophy
Leprosy
Classification by anatomical level and distribution is
probably the simplest. Although it does not fully Inflammatory
cover pathological causation, it does relate to clinical Acute inflammatory polyneuropathy
presentation and provides a framework for further Guillain–Barré syndrome
investigations. It is well to remember that in over 40 Systemic lupus erythematosus
per cent of cases no specific cause is found! Nutritional and metabolic
1. Radiculopathy – involvement of nerve roots, most Vitamin deficiencies
commonly by vertebral trauma, intervertebral disc Diabetes
herniation or bony spurs, space-occupying lesions Myxoedema
of the spinal canal and root infections like herpes Amyloidosis
zoster.
Neoplastic
2. Plexopathy – direct trauma (e.g. brachial plexus
traction injuries), compression by local tumours Primary carcinoma
(Pancoast’s tumour), entrapment in thoracic Myeloma
outlet syndrome, and viral infection such as Toxic
neuralgic amyotrophy. Alcohol
3. Distal neuronopathy – involvement of neurons in Lead
distinct peripheral nerves, which is usually
Drugs
subdivided into:
a. Mononeuropathy – involvement of a single Various
nerve, usually mixed sensorimotor (e.g.
nerve injury, nerve compression, entrapment
syndromes and nerve tumours).
mainly sensory neurons serving pain sensibility and
b. Multiple mononeuropathy – involvement of
autonomic neurons effecting vasomotor control, pilo-
several isolated nerves (e.g. leprosy and some
erection and neuroendocrine functions) are unmyeli-
cases of diabetes or vasculitis).
nated.
c. Polyneuropathy – widespread symmetrical
There are three basic types of peripheral neuronal
dysfunction (e.g. diabetic neuropathy, alco-
pathology: (1) acute interruption of axonal continu-
holic neuropathy, vitamin deficiency, Guillain–
ity; (2) axonal degeneration; and (3) demyelination.
Barré syndrome and a host of less common
In all three, conduction is disturbed or completely
disorders (see Table 10.4).
blocked, with consequent loss of motor and/or sen-
Abnormalities may be predominantly sensory (e.g. sory and/or autonomic functions.
diabetic polyneuropathy), predominantly motor
(e.g. peroneal muscular atrophy) or mixed. Chronic ACUTE AXONAL INTERRUPTION
motor loss with no sensory component is usually due This occurs most typically after nerve division and is
to anterior horn cell disease rather than more esoteric described in Chapter 11. Loss of motor and sensory
pathology like lead poisoning. functions is immediate and complete. The distal seg-
ments of axons that are crushed or severed will degen-
erate – as will the muscle fibres which are supplied by
Pathology motor neurons if nerve conduction is not restored
In general terms, large nerve fibres (those over 4 μm within two years. These changes are detectable at an
in diameter, which includes α-motor neurons, early stage by nerve conduction studies and EMG.
γ-motor neurons to the muscle spindles and sensory Axonal regeneration, when it occurs, is slow – the new
neurons serving touch and pressure) are myelinated axon grows by about 1 mm per day – and is often
256 whereas small fibres (less than 4 μm in diameter, incomplete.
CHRONIC AXONAL DEGENERATION Clinical features 10
In non-traumatic neuronal neuropathies the changes
are slower and progressive. Most large-fibre disorders af- Patients usually complain of sensory symptoms: ‘pins
fect both sensory and motor neurons causing ‘stocking’ and needles’, numbness, a limb ‘going to sleep’,
and ‘glove’ numbness, altered postural reflexes and ‘burning’, shooting pains or restless legs. They may
ataxia as well as muscle weakness and wasting, begin- also notice weakness or clumsiness, or loss of balance
ning distally and progressing proximally. Symptoms in walking. Occasionally (in the predominantly motor
tend to appear in the feet and legs before the hands and neuropathies) the main complaint is of progressive
arms. Some disorders are predominantly either motor deformity, for example, claw hand or cavus foot. The
or sensory. Nerve conduction studies show a reduction onset may be rapid (over a few days) or very gradual
in the size of CMAP and SNAP responses proportion- (over weeks or months). Sometimes there is a history
ate to the loss of peripheral nerve fibres, but relatively of injury, a recent infective illness, a known disease
little conduction slowing (in contrast to the demyeli- such as diabetes or malignancy, alcohol abuse or nutri-
nating neuropathies). EMG may demonstrate dener- tional deficiency.
vation changes in distal muscles and confirm the extent Examination may reveal motor weakness in a par-
and severity of nerve loss. ticular muscle group. In the polyneuropathies the
Small-fibre neuropathies may cause orthostatic hy- limbs are involved symmetrically, usually legs before
potension, cardiac arrhythmias, reduced peripheral limb arms and distal before proximal parts. Reflexes are
perfusion, ischaemia and a predisposition to limb in- usually depressed, though in small-fibre neuropathies
fection. Small nerve fibres also convey pain, heat and (e.g. diabetes) this occurs very late. In mononeuropa-
cold sensibility and when disturbed give rise to burn- thy, sensory loss follows the ‘map’ of the affected
ing dysaesthesias. Neurophysiological tests are not sen- nerve. In polyneuropathy, there is a symmetrical
sitive enough to distinguish small-fibre disturbances. ‘glove’ or ‘stocking’ distribution. Trophic skin
changes may be present. Deep sensation is also
DEMYELINATING NEUROPATHIES affected and some patients develop ataxia. If pain
Focal demyelination occurs most commonly in nerve sensibility and proprioception are depressed there may
entrapment syndromes and blunt soft-tissue trauma. be joint instability or breakdown of the articular sur-
The main effects are slowing of conduction and some- faces (‘Charcot’ joints).
times complete nerve block, causing sensory and/or Clinical examination alone may establish the diag-
motor dysfunction distal to the lesion. These changes nosis. Further help is provided by electromyography
are potentially reversible; recovery usually takes less (which may suggest the type of abnormality) and
than 6 weeks, and in some cases only a few days. nerve conduction studies (which may show exactly
Demyelinating polyneuropathies are rare, with the where the lesion is).
exception of Guillain–Barré syndrome. Other condi- The mononeuropathies – mainly nerve injuries and
tions are the heritable motor and sensory neu- entrapment syndromes – are dealt with in Chapter 11.
ropathies and some inherited metabolic disorders, but The more common polyneuropathies are listed in
most of these show a mixture of axonal degeneration Table 10.4 and some are described below. In over 40
and demyelination. per cent of cases no specific cause is found.
10.24 Peripheral
neuropathy Two typical
deformities in patients
with peripheral neuritis:
(a) ulnar claw hands and
(b) pes cavus and claw
toes.
(a) (b) 257

10 HEREDITARY NEUROPATHIES abling, operative correction may be indicated (see
Chapter 21). Claw toes (due to intrinsic muscle weak-
These rare disorders present in childhood and adoles- ness) can be corrected by transferring the toe flexors
cence, usually with muscle weakness and deformity. to the extensors, with or without fusion of the inter-
phalangeal joints. Clawing of the big toe is best cor-
Hereditary sensory neuropathy rected by the Robert Jones procedure – transfer of the
extensor hallucis longus to the metatarsal neck and

Congenital insensitivity to pain and temperature is fusion of the interphalangeal joint. The cavus defor-
inherited as either a dominant or a recessive trait. mity often needs no treatment, but if it causes pain it
Patients develop neuropathic joint disease and ulcera- can be improved by calcaneal or dorsal mid-tarsal
tion of the feet. The cycle of painless injury and pro- osteotomy or (in severe cases) triple arthrodesis.
gressive deformity can lead to severe disability.
Familial liability to pressure palsy (HNPP)

Hereditary motor and sensory
This is a relatively common, dominant disorder which
neuropathy (HMSN) often presents as multiple mixed entrapment
This is the preferred name for a group of conditions mononeuropathies (e.g. carpal tunnel syndrome and
which includes peroneal muscular atrophy, Charcot– ulnar nerve palsy), even in young patients.
Marie–Tooth disease and some benign forms of spinal
muscular atrophy. They are the commonest of the
Friedreich’s ataxia
inherited neuropathies, which are usually passed on as
autosomal dominant disorders. This autosomal recessive condition is the classic arche-
HMSN type I is seen in children who have difficulty type of a large group of genetic disorders – the spin-
walking and develop claw toes and pes cavus or cavo- ocerebellar ataxias – characterized by spinocerebellar
varus. There may be severe wasting of the legs and dysfunction, but there may also be degeneration of
(later) the upper limbs, but often the signs are quite the posterior root ganglia and peripheral nerves. Many
subtle. Spinal deformity may occur in severe cases. of these disorders have now been genotypically de-
This is a demyelinating disorder and nerve conduction fined. Patients generally present at around the age of 6
velocity is markedly slowed. The diagnosis can be con- years with gait ataxia, lower limb weakness and defor-
firmed by finding demyelination on sural nerve biopsy mities similar to those of severe Charcot–Marie–Tooth
or (if the facilities are available) by genetic testing of disease. The muscle weakness, which may also involve
blood samples. the upper limbs and the trunk, is progressive; by the age
HMSN type II occurs in adolescents and young of 20 years the patient has usually taken to a wheelchair
adults and is much less disabling than type I; it affects and is likely to die of cardiomyopathy before the age of
only the lower limbs, causing mild pes cavus and wast- 45. Despite the potentially poor prognosis, surgical
ing of the peronei. Nerve conduction velocity is only correction of deformities is worthwhile.
slightly reduced, indicating primary axonal degenera-
tion.
Treatment In the early stages foot and ankle orthoses METABOLIC NEUROPATHIES
are helpful. If the deformities are progressive or dis-
Diabetic neuropathy
10.25 Hereditary Diabetes is one of the commonest causes of peripheral
neuropathies – peroneal
neuropathy. The metabolic disturbance associated
muscular atrophy This
patient has the typical with hyperglycaemia interferes with axonal and
wasting of the legs, cavus Schwann cell function, leading to mixed patterns of
feet and claw toes associated demyelination and axonal degeneration. Autonomic
with peroneal muscular dysfunction and vascular disturbance also play a part.
atrophy.
The onset is insidious and the condition often goes
undiagnosed until patients start complaining of numb-
ness and paraesthesiae in the feet and lower legs. Even
at that early stage there may be areflexia and diminished
vibration sense. Another suspicious pattern is an in-
creased susceptibility to nerve entrapment syndromes.
Later, muscle weakness becomes more noticeable in
proximal parts of the limbs. In advanced cases trophic
258 complications can arise: neuropathic ulcers of the feet,
regional osteoporosis, insufficiency fractures of the foot Following an injury or intercurrent illness, the 10
bones, or Charcot joints in the ankles and feet. Another patient develops severe unilateral pain in the distribu-
late feature is loss of balance. Autonomic dysfunction tion of several adjacent nerve roots. Motor roots and
may produce postural hypotension and abnormal even the spinal cord may (rarely) be affected and
sphincter control, and may also account for an in- involvement of the lumbar roots can closely mimic sci-
creased susceptibility to infection. atica. Days or weeks later an irritating vesicular rash
appears; characteristically it trails out along the der-
Treatment It is vital to ensure that the underlying dis-
matomes corresponding to affected nerves. The condi-
order is properly controlled. Local treatment consists
tion usually subsides spontaneously but post-herpetic
of skin care, management of fractures and splintage or
neuralgia may persist for months or years.
arthrodesis of grossly unstable or deformed joints.
Treatment is symptomatic, though in severe cases
Management of the diabetic foot is discussed in
systemic antiviral therapy may be justified.
Chapter 21.
Alcoholic neuropathy Neuralgic amyotrophy (acute brachial

neuritis)
Axonal degeneration may be due to some toxic effect
of the alcohol, but the main cause is the accompany- This unusual cause of severe shoulder girdle pain and
ing nutritional deficiency, especially thiamine defi- weakness is believed to be due to a para-infectious dis-
ciency. order of one or more of the cervical nerve roots and
Presenting symptoms are, typically, ‘burning’ the brachial plexus, sometimes producing a pseudo-
paraesthesiae, numbness and muscle weakness in the mononeuropathic pattern (e.g. scapular winging or
feet and legs. The calf muscles are tender to pressure wrist-drop). There is often a history of an antecedent
and reflexes are depressed or absent. Men are likely to viral infection or antiviral inoculation; sometimes a
complain of urinary difficulty and impotence. small epidemic occurs among several inmates of an
institution.
Treatment Early cases may respond to nutritional sup- The history alone often suggests the diagnosis. Pain
plementation and administration of thiamine. Patients in the shoulder and arm is typically sudden in onset,
should be protected from trauma. Of course, steps intense and unabating; the patient can often recall the
should also be taken to deal with the alcohol abuse. exact hour when symptoms began. Pain may extend
into the neck and down as far as the hand; usually it
lasts for two or three weeks. Other symptoms are
INFECTIVE NEUROPATHY paraesthesiae in the arm or hand and weakness of the
muscles of the shoulder, forearm and hand.
Herpes zoster (shingles) Winging of the scapula (due to serratus anterior
weakness), wasting of the shoulder girdle muscles,
This common disorder is caused by the varicella (chick-
enpox) virus. The virus, having lain dormant for many
years in the dorsal root ganglia, is then reactivated and
migrates down the nerve. The exact cause of the reac-
tivation is unknown but immunocompromise, age and
stress are contributory factors; thus elderly or im-
munosuppressed patients are particularly susceptible.
10.26 Herpes
zoster This patient
was treated for
several weeks for
‘sciatica’ – then the
typical rash of
shingles appeared.
(a) (b)
10.27 Neuralgic amyotrophy A common feature of

neuralgic amyotrophy is winging of the scapula due to
serratus anterior weakness. Even at rest (a) the right
scapula is prominent in this young woman. When she
thrusts her arms forwards against the wall (b) the
abnormality is more pronounced. 259
10 and occasionally involvement of more distal arm mus- 10.28 Leprosy –
cles may be profound, becoming evident as the pain ulnar nerve paralysis
improves. Shoulder movement is initially limited by Ulnar nerve paralysis is
relatively common in
pain but this is superseded by weakness due to muscle longstanding leprosy.
atrophy. Sensory loss and paraesthesiae in one or more This patient has the
of the cervical dermatomes is not uncommon. typical ulnar claw-
Involvement of overlapping root territories of the hand deformity.

brachial plexus is a feature that helps to distinguish
neuralgic amyotrophy from an acute cervical disc her-
niation which is monoradicular.
There is no specific treatment; pain is controlled
with analgesics. The prognosis is usually good but full
neurological recovery may take months or years.
Guillain–Barré syndrome (acute

inflammatory demyelinating
polyneuropathy – AIDP)
Guillain–Barré syndrome describes an acute demyeli- Leprosy
nating motor and sensory (though mainly motor)
Although uncommon in Europe and North America,
polyneuropathy. It can occur at any age and usually
this is still a frequent cause of peripheral neuropathy
appears two or three weeks after an upper respiratory
in Africa and Asia.
or gastrointestinal infection – probably as an autoim-
Mycobacterium leprae, an acid-fast organism, causes
mune reaction.
a diffuse inflammatory disorder of the skin, mucous
The typical history is of aching and weakness in the
membranes and peripheral nerves. Depending on the
legs, often accompanied by numbness and paraesthe-
host response, several forms of disease may evolve.
siae, which steadily progresses upwards over a period
The most severe neurological lesions are seen in
of hours, a few days or a few weeks. Symptoms may
tuberculoid leprosy. Anaesthetic skin patches develop
stop when the thigh and pelvic muscles are reached,
over the extensor surfaces of the limbs; loss of motor
and then gradually retreat, or may go on ascending to
function leads to weakness and deformities of the
involve the upper limbs, facial muscles and diaphragm,
hands and feet. Thickened nerves may be felt as cords
resulting in quadriplegia and respiratory failure. In the
under the skin or where they cross the bones (e.g. the
established case there will be areflexia and loss of posi-
ulnar nerve behind the medial epicondyle of the
tion sense. In severe cases patients may develop fea-
elbow). Trophic ulcers are common and may predis-
tures of autonomic dysfunction. Unsurprisingly, the
pose to osteomyelitis.
condition is also known as ‘ascending paralysis’.
Lepromatous leprosy is associated with a symmetrical
Cerebrospinal fluid analysis may show a characteris-
polyneuropathy, which occurs late in the disease.
tic pattern: elevated protein concentration in the pres-
Treatment by combined chemotherapy (mainly ri-
ence of a normal cell count (unlike an infection, in
fampicin and dapsone) is continued for 6 months to 2
which the cell count would also be elevated).
years, depending on the response. Muscle weakness,
Nerve conduction studies may show conduction
particularly intrinsic muscle paralysis due to ulnar nerve
slowing or block; in severe cases there may be EMG
involvement, may require multiple tendon transfers.
signs of axonal damage.
The condition is discussed in greater detail in Chap-
Treatment Treatment consists essentially of bed rest, ter 2 and the peripheral nerve complications are dealt
pain-relieving medication and supportive management with in Chapter 11.
to monitor, prevent and deal with complications such
as respiratory failure and difficulty with swallowing. In
severe cases specific treatment with intravenous
immunoglobulins or plasmapheresis should be started PAIN
as soon as possible. Once the acute disorder is under
control, physiotherapy and splintage will help to pre- Many – perhaps most – musculoskeletal disorders are
vent deformities and improve muscle power. accompanied by pain. Whatever the nature of the
Most patients recover completely, though this may underlying condition, pain usually requires treatment
take 6 months or longer; about 10 per cent are left in its own right; sometimes it becomes the main focus
with long-term disability and about 3 per cent are of attention even after the initiating factors have dis-
260 likely to die. appeared or subsided.
Pain perception reduction of anxiety and general psychological sup- 10
port. The management of pain involves not only the
Pain is confounding. The same receptors that appreci- elimination of noxious stimuli, or the administration
ate discomfort also respond to tickling with feelings of of painkillers, but also the care of the whole person.
pleasure. The electrical discharge in ‘mild’ pain is no
different from that in ‘severe’ pain. That the degree of
Acute pain
discomfort is related to the magnitude of the physical
stimulus cannot be doubted, but ultimately both the Severe acute pain, as seen typically after injury, is ac-
severity of the pain and its character are experienced companied by an autonomic ‘fight or flight’ reaction:
subjectively and cannot be measured. increased pulse rate, peripheral vasoconstriction, sweat-
Pain receptors Nociceptors in the form of free nerve ing, rapid breathing, muscle tension and anxiety. Sim-
endings are found in almost all tissues. They are stim- ilar features are seen in pain associated with acute neu-
ulated by mechanical distortion, by chemical, thermal rological syndromes or in malignant disease. Lesser
or electrical irritation, or by ischaemia. Musculoskele- degrees of pain may have negligible side effects.
tal pain associated with trauma or inflammation is due Treatment is directed at: (1) removing or counter-
to both tissue distortion and chemical irritation (local acting the painful disorder; (2) splinting the painful
release of kinins, prostaglandins and serotonin). Vis- area; (3) making the patient feel comfortable and
ceral nociceptors respond to stretching and anoxia. In secure; (4) administering analgesics, anti-inflamma-
nerve injuries the regenerating axons may be hyper- tory drugs or – if necessary – narcotic preparations;
sensitive to all stimuli. and (5) alleviating anxiety.
Pain transmission Pain sensation is transmitted via both

myelinated axons (large-diameter fibres), which carry Chronic pain
well-defined and well-localized sensation, and the far Chronic pain usually occurs in degenerative and
more numerous unmyelinated fibres which are re- arthritic disorders or in malignant disease and is
sponsible for crude, poorly defined pain. From the accompanied by vegetative features such as fatigue
dorsal horn synapses in the cord, some fibres participate and depression. Treatment again involves alleviation
in ipsilateral reflex motor and autonomic activities while of the underlying disorder if possible and general anal-
others connect with axons in the contralateral gesic therapy, but there is an increased need for reha-
spinothalamic tracts that run to the thalamus and cor- bilitative and psychologically supportive measures.
tex (where pain is appreciated and localized) as well as
the reticular system, which may be responsible for re-
flex autonomic and motor responses to pain. Complex regional pain syndrome (CRPS)
Pain modulation Pain impulses may be suppressed or A number of clinical syndromes appear under this
inhibited by (1) simultaneous sensory impulses travel- heading, including Sudeck’s atrophy, reflex sympathetic
ling via adjacent axons or (2) impulses descending dystrophy, algodystrophy, shoulder–hand syndrome and –
from the brain. Thus, it is posited that pain impulses particularly after a nerve injury – causalgia. What they
are ‘sorted out’ – some of them blocked, some have in common is pain out of proportion (in both
allowed through – in the dorsal horn of the cord (the intensity and duration) to the precipitating cause,
‘gate-control’ theory of Melzak and Wall, 1965). This vasomotor instability, trophic skin changes, regional
could explain why counter-stimulation sometimes osteoporosis and functional impairment.
reduces pain perception. In addition, certain mor- Precipitating causes are trauma (often trivial), oper-
phine-like compounds (endorphins and enkephalins), ation or arthroscopy, a peripheral nerve lesion,
normally elaborated in the brain and spinal cord, can myocardial infarction, stroke and hemiplegia. The
inhibit pain sensibility. These neurotransmitters are incidence of post-traumatic CRPS is unknown, largely
activated by a variety of agents, including severe pain because there are no agreed criteria for diagnosing
itself, other neurological stimuli, psychological mes- mild cases. However, the condition is more common
sages and placebos. than is generally recognized and it has been suggested
that as many as 30 per cent of patients with fractures
Pain threshold The so-called ‘pain threshold’ is the of the extremities develop features of this condition –
level of stimulus needed to induce pain. There is no fortunately short-lived in the majority of cases. Adults
fixed threshold for any individual; pain perception is are the usual sufferers but the condition occasionally
the result of all the factors mentioned above, operat- occurs in children.
ing against a complex and changing psychological
background. The threshold is lowered by fear, anxiety, PATHOGENESIS
depression, lack of self-esteem and mental or physical The pathophysiology of this condition has been
fatigue; and it is elevated by relaxation, diversion, argued over since it was first described a hundred 261
10 years ago. Since many of the features involve auto- lumbar sympathetic ganglia, or by regional block with
nomic pathways it was usually regarded as a type of guanethidine given intravenously to the affected limb.
sympathetic ‘overactivity’ – hence the earlier name However, the effectiveness of these measures is unpre-
‘reflex sympathetic dystrophy’ – though this never dictable and somewhat doubtful.
explained why the abnormal activity was maintained A small percentage of patients go on complaining
for so long (sometimes indefinitely). It is now recog- of pain and impaired function almost indefinitely. Psy-
nized that multiple mechanisms are involved: abnor- chological treatment may help them to deal with the
mal cytokine release, neurogenic inflammation, emotional distress and anxiety and to develop better
sympathetic-mediated enhancement of pain responses coping strategies.
and as yet poorly understood cortical reactions to
noxious stimuli (Gibbs et al., 2000; Birklein, 2005).
‘Chronic pain syndrome’
For the time being, the purely descriptive term ‘com-
plex regional pain syndrome’ will have to suffice. In a minority of patients with chronic pain there is an
apparent mismatch between the bitterness of com-
plaint and the degree of physical abnormality. The
CLINICAL FEATURES
most common example is the patient with discogenic
Following some precipitating event, the patient com-
disease and prolonged, unresponsive, disabling low
plains of burning pain, and sometimes cold intoler-
back pain. Labels such as ‘functional overlay’, ‘com-
ance, in the affected area – usually the hand or foot,
pensitis’, ‘supratentorial reaction’ and ‘illness behav-
sometimes the knee or hip, and sometimes the shoul-
iour’ are introduced and both patient and doctor are
der in hemiplegia. In the mild or early case there may
overtaken by a sense of hopelessness. Sometimes there
be no more than slight swelling, with tenderness and
are well-marked features of depression, or complaints
stiffness of the nearby joints. More suspicious are local
of widespread somatic illness (pain in various parts of
redness and warmth, sometimes changing to cyanosis
the body, muscular weakness, paraesthesiae, palpita-
with a blotchy, cold and sweaty skin. X-rays are at first
tions and impotence).
usually normal but triple-phase radionuclide scanning
Treatment is always difficult and should, ideally, be
at this stage shows increased activity.
managed by a team that includes a specialist in pain
Later, or in more severe cases, trophic changes
control, a psychotherapist, a rehabilitation specialist
become apparent: a smooth shiny skin with scanty
and a social worker. Pain may be alleviated by a vari-
hair and atrophic, brittle nails. Swelling and tender-
ety of measures: (1) analgesics and anti-inflammatory
ness persist and there may be marked loss of move-
drugs; (2) local injections to painful areas; (3) local
ment. X-rays now show patchy osteoporosis, which
counter-irritants; (4) acupuncture; (5) transcutaneous
may be quite diffuse (Fig. 10.29).
nerve stimulation; (6) sympathetic block; and, occa-
In the most advanced stage, there can be severe
sionally, (7) surgical interruption of pain pathways.
joint stiffness and fixed deformities. The acute symp-
These methods, as well as psychosocial assessment and
toms may subside after a year or 18 months, but some
therapy, are best applied in a dedicated pain clinic.
degree of pain often persists indefinitely.
Causalgia is a severe form of regional pain, usually
seen after a nerve injury. Pain is intense, often ‘burn-
ing’ or ‘penetrating’ and exacerbated by touching, jar- FIBROMYALGIA
ring or sometimes even by a loud noise. Symptoms
may start distally and progress steadily up the limb to
Fibromyalgia is not so much a diagnosis as a descrip-
involve an entire quadrant of the body.
tive term for a condition in which patients complain
of pain and tenderness in the muscles and other soft
TREATMENT tissues around the back of the neck and shoulders and
Treatment should be started as early as possible; if the across the lower part of the back and the upper parts
condition is allowed to persist for more than a few of the buttocks. What sets the condition apart from
weeks it may become irreversible. other ‘rheumatic’ diseases is the complete absence of
Mild cases often respond to a simple regimen of demonstrable pathological changes in the affected tis-
reassurance, anti-inflammatory drugs and physiother- sues. Indeed, it is often difficult to give credence to
apy. Other conservative measures include the admin- the patient’s complaints, an attitude which is encour-
istration of corticosteroids, calcium channel blockers aged by the fact that similar symptoms are encoun-
and tricyclic antidepressants. tered in some patients who have suffered trivial
If there is no improvement after a few weeks, and as injuries in a variety of accidents; a significant number
a first measure in severe cases, sympathetic blockade also develop psychological depression and anxiety.
often helps. This can be done by one or more local The criteria for making the diagnosis were put for-
262 anaesthetic injections to the stellate or the appropriate ward by the American College of Rheumatology in
tions into the painful areas simply to reduce the level 10
of discomfort. Patients with more persistent and more
disturbing symptoms may benefit from various types
of psychotherapy.
ARTHROGRYPOSIS
‘Arthrogryposis’ is a broad term used to describe a large

(a)
group of congenital disorders – all of them rare – in
which children are born with multiple non-progressive
soft-tissue contractures and restriction of joint move-
ment. In other respects these conditions differ widely
in terms of pathological change and clinical appearance.
In the most common form – arthrogryposis multiplex
congenita (nowadays known as amyoplasia) – all joints
of the upper and lower limbs are involved; at the ex-
tremes of the range there are some patients in whom
only a few joints are affected (and not very severely at
that) and others in whom all joints are severely af-
fected. In the very rare myopathic form of the disease,
(b)
children may develop spinal deformities.
The incidence is said to be about 1 in 3000 live
10.29 Complex regional pain syndrome (a) A 53-year births; in some cases a genetic linkage has been
old woman suffered an undisplaced fracture of her right demonstrated. A more proximate cause may be an
tibia. The fracture healed but her foot became swollen,
intrauterine lack of sufficient room for movement (for
warm to the touch and tender, the skin reddish-purple and
sweaty. (b) X-rays showed an unusual degree of whatever reason) during foetal development. Joint
osteoporosis. capsules are often fibrotic.
The deformities are associated with unbalanced mus-
cle weakness which follows a neurosegmental distribu-
1990. These included symptoms of widespread pain
tion, and necropsy specimens show sparseness of anterior
in all four quadrants of the body, together with at least
horn cells in the cervical and lumbar cord. Deformities
9 pairs of designated ‘tender points’ on physical exam-
and contractures develop in utero and remain largely un-
ination. In practice, however, the diagnosis is often
changed throughout life. Myopathic and neuropathic
made in patients with much more localized symptoms
features may coexist in the same muscle.
and signs, and it is now quite common to attach this
label to almost any condition associated with myofas-
cial pain where no specific underlying disorder can be Classification
identified.
Considering arthrogryposis as a whole, the conditions
The cause of fibromyalgia remains unknown; no
can be placed in three major categories:
pathology has been found in the ‘tender spots’. It has
been suggested that this is an abnormality of ‘sensory 1. Those with total body involvement: typified by the
processing’, which is perhaps another way of saying condition formerly known as arthrogryposis multi-
that the sufferers have a ‘low pain threshold’; in fact plex congenita and now termed amyoplasia, but
they often do display increased sensitivity to pain in also including other congenital disorders showing
other parts of the body. There are also suggestions widespread joint contractures. In the rarer myo-
that the condition is related to stress responses which pathic form of the disease, children may develop
can be activated by sudden accidents or traumatic life spinal deformities.
events. This does not mean that such patients will 2. Those with predominantly hand or foot involvement:
necessarily show other features of psychological dys- conditions with joint features similar to those of
function and the condition cannot be excluded merely amyoplasia but usually limited to distal joints
by psychological testing. (wrists, hands, feet) and therefore termed distal
In mild cases, treatment can be limited to keeping arthrogryposis; included also are more severe types
up muscle tone and general fitness (hence the advice of distal myopathy such as the Freeman–Sheldon
to have physiotherapy and then continue with daily syndrome in which there are, in addition, abnor-
exercises on their own), perhaps together with injec- mal facial features (the ‘whistling face syndrome’). 263
10
(a) (b) (c) (d)
10.30 Arthrogryposis multiplex congenita (a,b) Severe deformities are present at birth. In this case all four limbs are
affected. (c,d) Operative treatment is often worthwhile. In this young boy the lower limbs were tackled first and the feet
and knees are held in splints. In the upper limbs, the minimum aim is to enable a hand to reach the mouth.
3. Pterygia syndromes: conditions characterized by splinting forming the mainstays of initial manage-
arthrogrypotic joint contractures with identifiable ment. A few cautionary words: check for neonatal
soft-tissue webs, usually across the flexor aspects fractures before starting treatment, and avoid forceful
of the knees and ankles. manoeuvres.
In the pterygia syndromes, physiotherapy can be
tried but early release of the popliteal contractures
Clinical features
should be considered. Great care is needed to avoid
Clinical examination is still the best way of making the injury to tight neurovascular structures.
diagnosis: involved joints are tubular and featureless In general, if progress is slow, tendon releases, ten-
and although the normal skin creases are missing don transfers and osteotomies may become necessary.
there are often deep dimples over the joints. Muscle Rigid equinovarus is particularly difficult to treat and
mass is markedly reduced. In some cases there is true operative correction is often necessary. Displacement
muscle weakness. or dislocation of the hip, likewise, often defies conser-
In the classic form of amyoplasia the shoulders are vative treatment and open reduction is then needed.
adducted and internally rotated, the elbows usually Unfortunately, recurrences of deformity are common.
extended and the wrists/hands flexed and deviated Before surgical intervention is considered, it should
ulnarwards. In the lower limbs, the hips are flexed and be noted that children often cope surprisingly well
abducted, the limbs externally rotated, the knees usu- with their deformities and a holistic approach to the
ally extended and the feet showing equinovarus or child is essential in order to ensure that the interaction
vertical talus deformities. Secondary problems include of all the involved joints is understood; changing the
feeding difficulties due to the stiff jaw and immobile position of one joint can have a significant adverse
tongue. effect on overall function. If both elbows are rigidly
Distal arthrogryposis often manifests an autosomal extended, function may be improved by leaving one
dominant pattern of inheritance. Common hand elbow in extension and the other in partial flexion.
deformities are ulnar deviation of the metacarpo-pha-
langeal joints, fixed flexion of the PIP joints and
tightly adducted thumbs. Foot deformities are likely
to be resistant forms of equinovarus or vertical talus. MUSCULAR DYSTROPHIES
The muscular dystrophies are a group of about 30

Treatment rare inherited disorders characterized by progressive
The condition is unlikely to improve spontaneously muscle weakness and wasting. Pathological changes
and it is essentially incurable. Treatment of the indi- include malformation of muscle fibres, death of mus-
vidual joint begins shortly after birth and may follow cle cells and replacement of muscle by fibrous tissue
264 basic principles with manipulation, stretching and and fat. They have been grouped according to their
various inheritance patterns, age of onset, distribution sis and, subsequently, further deterioration in lung 10
of affected musculature and severity of the muscle function. Cardiopulmonary failure is the usual cause of
weakness. Those most likely to be encountered in death, generally before the age of 30 years.
orthopaedic practice are:
• Duchenne’s muscular dystrophy – a severe, general- Investigations
ized sex-linked disorder affecting only boys in early
The diagnosis is usually based on the clinical features
childhood. Becker’s muscular dystrophy is similar
and family history and by testing for serum creatinine
but less severe, starts somewhat later and progresses
phosphokinase levels which are 200–300 times the
more slowly.
normal in the early stages of the disease (and also ele-
• Limb girdle dystrophies – a mixed group, usually of
vated, but less so, in female carriers). Confirmation is
autosomal recessive inheritance, with more local-
achieved by muscle biopsy and genetic testing with a
ized changes, affecting boys and girls in later child-
DNA polymerase chain reaction.
hood.
• Facioscapulohumeral dystrophy – an autosomal dom-
inant condition of variable severity, usually appear- Treatment
ing in early adulthood.
While the child can still walk, physiotherapy and splin-
tage or tendon operations may help to prevent or cor-
DUCHENNE MUSCULAR DYSTROPHY rect joint deformities and so prolong the period of
mobility.
This is a progressive disease of sex-linked inheritance Corticosteroids are useful in preserving muscle
with recessive transmission. It is therefore seen only in strength but there are significant side effects such as
boys (or in girls with sex chromosome disorders), osteoporosis, increased risk of fractures and cataract
affecting 1 in 3500 male births. Some women are formation.
‘manifesting carriers’ who have slight muscle weak- Research studies in which dystrophin in the form of
ness and cramps. myoblasts is introduced into diseased muscle have
A defect at locus p21 on the X chromosome results been successful in animal models but not so far in
in failure to code for the dystrophin gene, which is humans. Gene therapy has also been tried but there
essential for maintaining the integrity of cardiac and have been difficulties with the viral vectors and associ-
skeletal muscle cells. Absence of functional dystrophin ated immunological responses.
leads to cell membrane leakage, muscle fibre damage If scoliosis is marked (more than 30 degrees), instru-
and replacement by fat and fibrous tissue. mentation and spinal fusion helps to maintain pul-
monary function and improves quality of life although
not necessarily lifespan. Preoperative cardiac and pul-
Clinical features monary function evaluation should be performed.
The condition is usually unsuspected until the child Family counselling is important. Up to 20 per cent
starts to walk. He has difficulty standing and climbing of families already have a younger affected sibling by
stairs, he cannot run properly and he falls frequently. the time the proband is diagnosed.
Weakness begins in the proximal muscles of the lower
limbs and progresses distally, affecting particularly the
glutei, the quadriceps and the tibialis anterior, giving BECKER MUSCULAR DYSTROPHY
rise to a wide-based stance and gait with the feet in
equinus, the pelvis tilted forwards, the back arched in This condition, also an X-linked recessive disease, is
lordosis and the neck extended. The calf muscles look similar to but milder than Duchenne’s dystrophy.
bulky, but much of this is due to fat and the pseudo- Dystrophin is decreased and/or abnormal in charac-
hypertrophy belies the obvious weakness. A character- ter. Affected boys retain the ability to walk into their
istic feature is the child’s method of rising from the teens and patients may survive until middle age. The
floor by climbing up his own legs (Gowers’ sign); this muscles of facial expression are not affected and nei-
is due to weakness of the gluteus maximus and thigh ther are the muscles controlling bowel or bladder
muscles. function or swallowing.
Shoulder girdle weakness follows around 5 years
after the clinical onset of the disease, making it difficult
for the patient to use crutches. Facial muscle involve- LIMB GIRDLE DYSTROPHY
ment follows later. By the age of 10 years the child has
usually lost the ability to walk and becomes dependent This form of muscular dystrophy, characterized by
on a wheelchair; from then on there is rapid deteriora- weakness of the pelvic and shoulder girdle muscles,
tion in spinal posture with the development of scolio- represents a heterogeneous group of conditions, most 265
10 of which show an autosomal recessive inheritance pat- cle weakness; the face and tongue may be involved,
tern affecting both sexes. causing ptosis and difficulty with chewing. EMG
Symptoms usually start in late adolescence. Pelvic changes may be diagnostic. Enquiry will almost
girdle weakness causes a waddling gait and difficulty always reveal that a relative has been affected as well.
in rising from a low chair; pectoral girdle weakness With time, systemic features appear – diabetes,
makes it difficult to raise the arms above the head. cataracts and cardiorespiratory problems – and by
However, the muscles of facial expression are spared. middle age patients are often severely disabled.
Disease progression is usually slow. (NB: These fea- Treatment is essentially palliative but foot deformi-
tures can be mistaken for those of a mild form of ties may need manipulation and splintage. Affected
spinal muscular atrophy.) women who are planning to become pregnant should
Treatment consists of physiotherapy and splintage be warned that there is a risk of them giving birth to
to prevent contractures, and operative correction a floppy baby with feeding difficulties.
when necessary. Because the deltoid muscles are
spared, shoulder movements can sometimes be
improved by fixing the scapula to the ribs posteriorly, MYOTONIA CONGENITA
so improving deltoid leverage.
The usual form of congenital myotonia is inherited by
autosomal recessive transmission. Symptoms due to
FACIOSCAPULOHUMERAL DYSTROPHY ‘muscle stiffness’ appear in childhood and usually
progress slowly. Common complaints are that walking
This is an autosomal dominant condition with very and climbing stairs are difficult; typically this is worse
variable expression. In general, males are more after periods of inactivity and is relieved by exercise.
severely affected than females and from a younger Symptoms tend also to be triggered by exposure to
age. Characteristically, muscle weakness is first seen in cold and can cause pain (‘muscle cramps’). By adult-
the face (inability to purse the lips or close the eyes hood there may be muscle weakness, though the fore-
tightly). This is followed by weakness of scapular mus- arms and calves are unusually bulky. There is no
cles causing winging of the scapula and difficulty with specific treatment for this condition. Patients are
shoulder abduction. There may also be weakness of advised about avoiding aggravating activities.
the anterior tibial muscles. In a more rare subgroup, showing autosomal dom-
The condition is due to gene deletion on the long inant inheritance, symptoms appear in infancy or early
arm of chromosome 4; genetic testing to confirm the childhood but do not progress and are usually mild
diagnosis is highly sensitive and specific. enough not to need treatment. Other very rare sub-
groups have also been identified and their diagnosis
can be difficult. The best advice is that children with
‘atypical’ features of congenital myotonia should be
MYOTONIA referred to a centre specializing in muscle disorders.
Myotonia – persistent muscle contraction after cessa-

tion of voluntary effort – is a prominent feature in cer-
tain genetic disorders. The two least rare of these REFERENCES AND FURTHER READING
conditions are considered here: dystrophia myotonica,
in which myotonia is part of a more widespread sys- Banta JV, Lubicky JP. Orthopaedic aspects of
temic disorder, and myotonia congenita, in which myelomeningocele: spinal deformities. J Bone Joint Surg
myotonia is usually the only abnormal clinical feature. 1990; 72A: 628–9.
Beals RK. Spastic paraplegia and diplegia: an evaluation of
non-surgical and surgical factors influencing the progno-
DYSTROPHIA MYOTONICA sis for ambulation. J Bone Joint Surg 1966; 48A: 827–46.
Beaty, JH, Canale JT. Orthopaedic aspects of
Myotonic dystrophy is an autosomal dominant disor- myelomeningocele. Current concepts review. J Bone Joint
der with an incidence of about 1 in 7000. Patients Surg 1990; 72A: 626–30.
usually present in adult life with distal muscle weak- Birklein F. Complex regional pain syndrome. Neurology
ness and wasting. The defining feature, what the 2005; 252: 131–8.
patient perceives as ‘muscle stiffness’, may have been Bleck EE. Locomotor prognosis in cerebral palsy. Dev Med
present for some years; myotonia is most easily Child Neurol, 1975; 17: 18–25.
demonstrated by asking the patient to flex and extend Bleck EE. Orthopaedic Management in Cerebral Palsy.
the fingers rapidly. Some patients are only mildly Blackwell Scientific, Oxford; Lippincott, Philadelphia,
266 affected while others develop more widespread mus- 1987.
Gibbs GF, Drummond PD, Finch PM et al. Unravelling the management of high level spina bifida. J Bone Joint Surg 10
pathophysiology of complex regional pain syndrome: 1989; 71A: 56–61.
focus on sympathetically maintained pain. Clin Exp Phar- McCarthy JJ, D’Andrea LP, Betz RR, Clements DH. Scol-
macol Physiol 2000; 35: 717–24. iosis in the child with cerebral palsy. J Am Acad Orthop
Hoffer MM. Management of the hip in cerebral palsy. Surg 2006; 14: 367–75.
J Bone Joint Surg 1986; 68A: 629–31. Palisano RJ, Rosenbaum P, Bartlett D, Livingston MH.
Hong-Xue Men, Chan-Hua Bian, Chan-Dou Yang, et al. Gross Motor Function Classification System. Dev Med
Surgical treatment of the flail knee after poliomyelitis. Child Neurol 2008; 50(10): 744 –50.
J Bone Joint Surg 1991; 73B: 195–9. Ponseti IV, Smoley EN. Congenital club foot: The results
Karol LA. Surgical management of the lower extremity in of treatment. J Bone Joint Surg 1963; 45A: 261–75.
ambulatory children with CP. J Am Acad Orthop Surg Rang M, Wright J. What have 30 years of medical progress
2004; 12: 196–203. done for cerebral palsy? Clin Orthop Relat Res 1989;
Lau JHK, Parker JC, Hsu LCS, et al. Paralytic hip instabil- 247: 55–60.
ity in poliomyelitis. J Bone Joint Surg 1986; 68B: 528–33. Roper BA, Tibrewal SB. Soft tissue surgery in Charcot-
Louis DS, Hensinger RM, Fraser BA, et al. Surgical man- Marie-Tooth disease. J Bone Joint Surg 1989; 71B: 17–20.
agement of the severely multiply handicapped individual. Scrutton D. The early management of hips in cerebral palsy.
J Pediatr Orthop 1989; 9: 15–18. Dev Med Child Neurol 1989; 31: 108–16.
Ma FY, Selber P, Nattrass GR, et al. Lengthening and Sutherland DH, Davids JR. Common gait abnormalities of
transfer of the hamstrings for flexion a deformity of the the knee in cerebral palsy. Clin Orthop Rel Res 1993;
knee in children with bilateral cerebral palsy: Technique 288: 139–47.
and preliminary results. J Bone Joint Surg 2006; 88B: Sutherland DH, Ohlson R, Cooper L, Woo SK. The devel-
248–54. opment of mature gait. J Bone Joint Surg 1980; 62A:
Melzack R, Wall PD. Pain mechanisms: a new theory. 336–53.
Science 1965; 150: 971–9 Trail IA, Galasko CSB. The matrix seating system. J Bone
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267
Peripheral nerve
disorders 11
David Warwick, H. Srinivasan, Louis Solomon
predominantly motor, some predominantly sensory;

NERVE STRUCTURE AND FUNCTION the larger trunks are mixed, with motor and sensory
axons running in separate bundles.
Peripheral nerves are bundles of axons conducting Each axon is, in reality, an extension or elongated
efferent (motor) impulses from cells in the anterior process of a nerve cell, or neuron (see Chapter 10).
horn of the spinal cord to the muscles, and afferent The cell bodies of the motor neurons supplying the
(sensory) impulses from peripheral receptors via cells peripheral muscles are clustered in the anterior horn
in the posterior root ganglia to the cord. They also of the spinal cord; a single motor neuron with its axon
convey sudomotor and vasomotor fibres from gan- may, therefore, be more than a metre long. The cell
glion cells in the sympathetic chain. Some nerves are bodies of the sensory neurons serving the trunk and
(b)
(a)
11.1 Nerve structure (a) Diagram of the structural

elements of a peripheral nerve. (b) Histological section
through a large nerve. (c) High-power view of the same,
showing blood vessels in the perineurium. (c)
11 limbs are situated in the dorsal root ganglia and each strong where the nerve is subjected to movement and
neuron has one process (axon) extending from the traction, for example near a joint.
periphery to the cell body and another from the cell The nerve is richly supplied by blood vessels that run
body up the spinal cord. longitudinally in the epineurium before penetrating
The peripheral ends of all the neurons are the various layers to become the endoneurial capillar-
branched. A single motor neuron may supply any- ies. These fine vessels may be damaged by stretching
thing from 10 to several thousand muscle fibres, the or rough handling of the nerve; however, they can
ratio depending on the degree of dexterity demanded withstand extensive mobilization of the nerve, making
of the particular muscle (the smaller the ratio, the it feasible to repair or replace damaged segments by
finer the movement). Similarly, the peripheral operative transposition or neurotization. The tiny
branches of each sensory neuron may serve anything blood vessels have their own sympathetic nerve supply
from a single muscle spindle to a comparatively large coming from the parent nerve, and stimulation of
patch of skin; here again, the fewer the end receptors these fibres (causing intraneural vasoconstriction) may
served the greater the degree of discrimination. be important in conditions such as reflex sympathetic
The signal, or action potential, carried by motor dystrophy and other unusual pain syndromes.
neurons is transmitted to the muscle fibres by the
release of a chemical transmitter, acetylcholine, at the
terminal bouton of the nerve. Sensory signals are sim- PATHOLOGY
ilarly conveyed to the dorsal root ganglia and from
there up the ipsilateral column of the spinal cord,
Nerves can be injured by ischaemia, compression,
through the brain-stem and thalamus, to the opposite
traction, laceration or burning. Damage varies in
(sensory) cortex. Proprioceptive impulses from the
severity from transient and quickly recoverable loss of
muscle spindles and joints bypass this route and are
function to complete interruption and degeneration.
carried to the anterior horn cells as part of a local
There may be a mixture of types of damage in the var-
reflex arc. The economy of this system ensures that
ious fascicles of a single nerve trunk.
‘survival’ mechanisms like balance and sense of posi-
tion in space are activated with great speed.
In the peripheral nerves, all motor axons and the Transient ischaemia
large sensory axons serving touch, pain and proprio- Acute nerve compression causes numbness and tin-
ception are coated with myelin, a multilayered gling within 15 minutes, loss of pain sensibility after
lipoprotein membrane derived from the accompany- 30 minutes and muscle weakness after 45 minutes.
ing Schwann cells. Every few millimetres the myelin Relief of compression is followed by intense paraes-
sheath is interrupted, leaving short segments of bare thesiae lasting up to 5 minutes (the familiar ‘pins and
axon called the nodes of Ranvier. Nerve impulses leap needles’ after a limb ‘goes to sleep’); feeling is
from node to node at the speed of electricity, much restored within 30 seconds and full muscle power
faster than would be the case if these axons were not after about 10 minutes. These changes are due to
insulated by the myelin sheaths. Consequently, deple- transient endoneurial anoxia and they leave no trace
tion of the myelin sheath causes slowing – and even- of nerve damage.
tually complete blocking – of axonal conduction.
Most axons – in particular the small-diameter fibres Neurapraxia
carrying crude sensation and the efferent sympathetic
Seddon (1942) coined the term ‘neurapraxia’ to
fibres – are unmyelinated but wrapped in Schwann
describe a reversible physiological nerve conduction
cell cytoplasm. Damage to these axons causes unpleas-
block in which there is loss of some types of sensation
ant or bizarre sensations and various sudomotor and
and muscle power followed by spontaneous recovery
vasomotor effects.
after a few days or weeks. It is due to mechanical pres-
Outside the Schwann cell membrane the axon is
sure causing segmental demyelination and is seen typ-
covered by a connective tissue stocking, the
ically in ‘crutch palsy’, pressure paralysis in states of
endoneurium. The axons that make up a nerve are
drunkenness (‘Saturday night palsy’) and the milder
separated into bundles (fascicles) by fairly dense mem-
types of tourniquet palsy.
branous tissue, the perineurium. In a transected
nerve, these fascicles are seen pouting from the cut
surface, their perineurial sheaths well defined and Axonotmesis
strong enough to be grasped by fine instruments dur- This is a more severe form of nerve injury, seen typically
ing operations for nerve repair. The groups of fascicles after closed fractures and dislocations. The term means,
that make up a nerve trunk are enclosed in an even literally, axonal interruption. There is loss of conduc-
thicker connective tissue coat, the epineurium. The tion but the nerve is in continuity and the neural tubes
270 epineurium varies in thickness and is particularly are intact. Distal to the lesion, and for a few millimetres
open wound. It is now recognized that severe degrees 11
of damage may be inflicted without actually dividing
the nerve. If the injury is more severe, whether the
nerve is in continuity or not, recovery will not occur.
(a) As in axonotmesis, there is rapid wallerian degenera-
tion, but here the endoneurial tubes are destroyed
Peripheral nerve disorders

over a variable segment and scarring thwarts any hope
of regenerating axons entering the distal segment and
regaining their target organs. Instead, regenerating
fibres mingle with proliferating Schwann cells and
fibroblasts in a jumbled knot, or ‘neuroma’, at the site
(b) of injury. Even after surgical repair, many new axons
fail to reach the distal segment, and those that do may
not find suitable Schwann tubes, or may not reach the
correct end-organs in time, or may remain incom-
pletely myelinated. Function may be adequate but is
never normal.
(c)
The ‘double crush’ phenomenon

There is convincing evidence that proximal compres-
sion of a peripheral nerve renders it more susceptible
to the effects of a second, more peripheral injury. This
(d)
may explain why peripheral entrapment syndromes
11.2 Nerve injury and repair (a) Normal axon and are often associated with cervical or lumbar spondylo-
target organ (striated muscle). (b) Following nerve injury sis. A similar type of ‘sensitization’ is seen in patients
the distal part of the axon disintegrates and the myelin with peripheral neuropathy due to diabetes or alco-
sheath breaks up. The nerve cell nucleus becomes eccentric
and Nissl bodies are sparse. (c) New axonal tendrils grow
holism.
into the mass of proliferating Schwann cells. One of the
tendrils will find its way into the old endoneurial tube and
(d) the axon will slowly regenerate.
CLASSIFICATION OF NERVE INJURIES
retrograde, axons disintegrate and are resorbed by Seddon’s description of the three different types of
phagocytes. This wallerian degeneration (named after nerve injury (neurapraxia, axonotmesis and neu-
the physiologist, Augustus Waller, who described the rotmesis) served as a useful classification for many
process in 1851) takes only a few days and is accompa- years. Increasingly, however, it has been recognized
nied by marked proliferation of Schwann cells and that many cases fall into an area somewhere between
fibroblasts lining the endoneurial tubes. The dener- axonotmesis and neurotmesis. Therefore, following
vated target organs (motor end-plates and sensory Sunderland (1978), a more practical classification is
receptors) gradually atrophy, and if they are not re- offered here.
innervated within 2 years they will never recover.
First degree injury This embraces transient ischaemia
Axonal regeneration starts within hours of nerve
and neurapraxia, the effects of which are reversible.
damage, probably encouraged by neurotropic factors
produced by Schwann cells distal to the injury. From Second degree injury This corresponds to Seddon’s
the proximal stumps grow numerous fine unmyeli- axonotmesis. Axonal degeneration takes place but,
nated tendrils, many of which find their way into the because the endoneurium is preserved, regeneration
cell-clogged endoneurial tubes. These axonal can lead to complete, or near complete, recovery
processes grow at a speed of 1–2 mm per day, the without the need for intervention.
larger fibres slowly acquiring a new myelin coat. Even-
Third degree injury This is worse than axonotmesis. The
tually they join to end-organs, which enlarge and start
endoneurium is disrupted but the perineurial sheaths
functioning again.
are intact and internal damage is limited. The chances
of the axons reaching their targets are good, but fibro-
Neurotmesis sis and crossed connections will limit recovery.
In Seddon’s original classification, neurotmesis meant Fourth degree injury Only the epineurium is intact. The
division of the nerve trunk, such as may occur in an nerve trunk is still in continuity but internal damage is 271
11 severe. Recovery is unlikely; the injured segment should
be excised and the nerve repaired or grafted.
Fifth degree injury The nerve is divided and will have
to be repaired.
CLINICAL FEATURES
Acute nerve injuries are easily missed, especially if

associated with fractures or dislocations, the symp-
toms of which may overshadow those of the nerve
lesion. Always test for nerve injuries following any sig-
nificant trauma. If a nerve injury is present, it is cru-
cial also to look for an accompanying vascular injury.
Ask the patient if there is numbness, paraesthesia or
muscle weakness in the related area. Then examine
the injured limb systematically for signs of abnormal
posture (e.g. a wrist drop in radial nerve palsy), weak-
ness in specific muscle groups and changes in sensibil-
ity.
Areas of altered sensation should be accurately
mapped. Each spinal nerve root serves a specific der-
matome (see Fig. 11.3) and peripheral nerves have
more or less discrete sensory territories which are
illustrated in the relevant sections of this chapter.
Despite the fact that there is considerable overlap in
sensory boundaries, the area of altered sensibility is
usually sufficiently characteristic to provide an
11.3 Examination Dermatomes supplied by spinal nerve anatomical diagnosis. Sudomotor changes may be
roots. The sensory distribution of peripheral nerves is found in the same topographic areas; the skin feels dry
illustrated in the relevant sections.
due to lack of sweating. If this is not obvious, the
11.4 Examination Type of form used for

C5 C6 C7 C8 T1
recording muscle power in new and
recovering nerve lesions (after Merle
d’Aubigné). Power is recorded in individual
blocks on the MRC Scale 1–5.
Opponens
Serratus anterior pollicis,
Flexor digitorum
Pronator superficialis abductor
teres pollicis brevis,
flexor
Palmaris longus, pollicis brevis
flexor carpi radialis
Biceps
and Extensor Triceps
Deltoid Flexor
brachialis carpi Abductor
Abductor pollicis pollicis
radialis longus pollicis
longus longus, extensor
pollicis brevis
Extensor Extensor
carpi pollicis longus
radialis Flexor Hypothenar
Brachio- Extensor digitorum
brevis digitorum communis muscles
radialis profundus
External to index and
rotators Extensor Extensor
indicis carpi ulnaris middle fingers
of
shoulder proprius,
extensor Flexor Flexor digito- Interosseous
Supinator digiti minimi carpi ulnaris rum profundus muscles
to ring and
Latissimus dorsi little fingers
Pectoralis major
272
‘plastic pen test’ may help. The smooth barrel of the 11
pen is brushed across the palmar skin: normally there
is a sense of slight stickiness, due to the thin layer of
surface sweat, but in denervated skin the pen slips
along smoothly with no sense of stickiness in the
affected area.

The neurological examination must be repeated at
intervals so as not to miss signs which appear hours
after the original injury, or following manipulation or
operation.
In chronic nerve injuries there are other characteris-
tic signs. The anaesthetic skin may be smooth and
shiny, with evidence of diminished sensibility such as
cigarette burns of the thumb in median nerve palsy or 11.5 Two-point discrimination
foot ulcers with sciatic nerve palsy. Muscle groups will
be wasted and postural deformities may become fixed.
Beware of trick movements which give the appearance
of motor activity where none exists.
Assessment of nerve recovery

The presence or absence of distal nerve function can be
revealed by simple clinical tests of muscle power and
sensitivity to light touch and pin-prick. Remember that
after nerve injury motor recovery is slower than sensory
recovery. More specific assessment is required to answer
two questions: How severe was the lesion? How well is
the nerve functioning now?
THE DEGREE OF INJURY

The history is most helpful. A low energy injury is 11.6 Monofilament assessment
likely to have caused a neurapraxia; the patient should
be observed and recovery anticipated. A high energy
injury is more likely to have caused axonal and excludes neurapraxia but of course it does not distin-
endoneurial disruption (Sunderland third and fourth guish between axonotmesis and neurotmesis; this
degree) and so recovery is less predictable. An open remains a clinical distinction, but if one waits too long
injury, or a very high energy closed injury, will prob- to decide then the target muscle may have failed
ably have divided the nerve and early exploration is irrecoverably and the answer hardly matters.
called for.
Tinel’s sign – peripheral tingling or dysaesthesia ASSESSMENT OF NERVE FUNCTION
provoked by percussing the nerve – is important. In a Two-point discrimination is a measure of innervation
neurapraxia, Tinel’s sign is negative. In axonotmesis, density. After nerve regeneration or repair, a propor-
it is positive at the site of injury because of sensitivity tion of proximal sensory axons will fail to reach their
of the regenerating axon sprouts. After a delay of a appropriate sensory end-organ; they will either have
few days or weeks, the Tinel sign will then advance at regenerated down the wrong Schwann-cell tube or
a rate of about 1 mm each day as the regenerating will be entangled in a neuroma at the site of injury.
axons progress along the Schwann-cell tube. Motor Therefore, two-point discrimination (measured
activity also should progress down the limb. Failure of with a bent paper clip and compared with the oppo-
Tinel’s sign to advance suggests a fourth or fifth site normal side) gives an indication of how com-
degree injury and the need for early exploration. If the pletely the nerve has recovered. Static two-point
Tinel sign proceeds very slowly, or if muscle groups discrimination measures slowly adapting sensors
do not sequentially recover as expected, then a good (Merkel cells) and moving two-point discrimination
recovery is unlikely and here again exploration must measures rapidly adapting sensors (Meissner corpus-
be considered. cles and pacinian corpuscles). Moving two-point dis-
Electromyography (EMG) studies can be helpful. If a crimination is more sensitive and returns earlier.
muscle loses its nerve supply, the EMG will show Normal static two-point discrimination is about 6
denervation potentials by the third week. This mm and moving is about 3 mm. 273
11 Threshold tests measure the threshold at which a sen-
sory receptor is activated. They are more useful in
nerve-compression syndromes, where individual recep-
tors fail to send impulses centrally; two-point discrimi-
nation is preserved because the innervation density is
not affected. Fine nylon monofilaments of varying
widths are placed perpendicularly on the skin and the

size of the lightest perceptible filament is recorded.
Locognosia is the ability to localize touch and can be
tested with a standardized hand map.
The Moberg pick-up test measures tactile gnosis. The
patient is blindfolded and instructed to pick up and
identify nine objects as rapidly as possible.
Motor power is graded on the Medical Research
Council scale as:
0 No contraction.
1 A flicker of activity.
2 Muscle contraction but unable to overcome grav-
11.7 Nerve repair The stumps are correctly orientated
ity. and attached by fine sutures through the epineurium.
3 Contraction able to overcome gravity.
4 Contraction against resistance.
have not retracted much; their relative rotation is usu-
5 Normal power.
ally undisturbed; and there is no fibrosis.
A clean cut nerve is sutured without further prepa-
ration; a ragged cut may need paring of the stumps
PRINCIPLES OF TREATMENT with a sharp blade, but this must be kept to a mini-
mum. The stumps are anatomically orientated and
Nerve exploration fine (10/0) sutures are inserted in the epineurium.
Closed low energy injuries usually recover sponta- There should be no tension on the suture line. Opin-
neously and it is worth waiting until the most proxi- ions are divided on the value of fascicular repair with
mally supplied muscle should have regained function. perineurial sutures.
Exploration is indicated: (1) if the nerve was seen to Sufficient relaxation of the tissues to permit ten-
be divided and needs to be repaired; (2) if the type of sion-free repair can usually be obtained by positioning
injury (e.g. a knife wound or a high energy injury) the nearby joints or by mobilizing and re-routing the
suggests that the nerve has been divided or severely nerve. If this does not solve the problem then a pri-
damaged; (3) if recovery is inappropriately delayed mary nerve graft must be considered. A traction lesion
and the diagnosis is in doubt. – especially of the brachial plexus – may leave a gap
Vascular injuries, unstable fractures, contaminated too wide to close. These injuries are best dealt with in
soft tissues and tendon divisions should be dealt with specialized centres, where primary grafting or nerve
before the nerve lesion. The incision will be long, as transfer can be carried out.
the nerve must be widely exposed above and below If a tourniquet is used it should be a pneumatic
the lesion before the lesion itself is repaired. The one; it must be released and bleeding stopped before
nerve must be handled gently with suitable instru- the wound is closed.
ments. Bipolar diathermy and magnification are essen- The limb is splinted in a position to ensure minimal
tial. An operating microscope is ideal but magnifying tension on the nerve; if flexion needs to be excessive,
loupes are better than nothing. A nerve stimulator is a graft is required. The splint is retained for 3 weeks
essential if scarring makes recognition uncertain. If and thereafter physiotherapy is encouraged.
microsurgical equipment and expertise are not avail-
able, then the nerve lesion should be identified and
Delayed repair
the wound closed pending transferral to an appropri-
ate facility. Late repair, i.e. weeks or months after the injury, may
be indicated because: (1) a closed injury was left alone
but shows no sign of recovery at the expected time;
Primary repair (2) the diagnosis was missed and the patient presents
A divided nerve is best repaired as soon as this can be late; or (3) primary repair has failed. The options must
done safely. Primary suture at the time of wound be carefully weighed: if the patient has adapted to the
274 toilet has considerable advantages: the nerve ends functional loss, if it is a high lesion and re-innervation
is unlikely within the critical 2-year period, or if there 11
is a pure motor loss which can be treated by tendon
transfers, it may be best to leave well alone. Excessive
scarring and intractable joint stiffness may, likewise,
make nerve repair questionable; yet in the hand it is
still worthwhile simply to regain protective sensation.

The lesion is exposed, working from normal tissue
above and below towards the scarred area. When the
nerve is in continuity it is difficult to know whether
resection is necessary or not. If the nerve is only
slightly thickened and feels soft, or if there is conduc-
tion across the lesion, resection is not advised; if the
‘neuroma’ is hard and there is no conduction on
nerve stimulation, it should be resected, paring back
the stumps until healthy fascicles are exposed.
11.8 Nerve graft using fibrin polymer glue
How to deal with the gap? The nerve must be
sutured without tension. The stumps may be brought
together by gently mobilizing the proximal and distal in Volkmann’s ischaemia) a pedicle graft from the
segments, by flexing nearby joints to relax the soft tis- ulnar nerve may be used to bridge the gap in the
sues, or (in the case of the ulnar nerve) by transposing median. It is also possible to use free vascularized
the nerve trunk to the flexor aspect of the elbow. In this grafts for certain brachial plexus lesions.
way, gaps of 2 cm in the median nerve, 4–5 cm in the
ulnar nerve and 6–8 cm in the sciatic nerve can usually
Nerve transfer
be closed, the limb being splinted in the ‘relaxing’ posi-
tion for 4–6 weeks after the operation. Elsewhere, gaps In root avulsions of the upper brachial plexus, too
of more than 1–2 cm usually require grafting. proximal for direct repair, nerve transfer can be used.
The spinal accessory nerve can be transferred to the
suprascapular nerve, and intercostal nerves can be
Nerve guides transferred to the musculocutaneous nerve. If biceps
It is now apparent that nerve gaps can regenerate has failed because too much time has passed since the
through a tube which excludes the surrounding tissue injury, an entire muscle (gracilis or latissimus dorsi)
from each end. The tubes can be autogenous vein, can be transferred as a free flap, attached between
freeze-dried muscle, silicone or metal; soluble guides elbow and shoulder and then innervated by joining
(flexible at body temperature) which dissolve over
weeks or months are also used. This technology offers
a simple way of avoiding a nerve graft yet achieving PRINCIPLES OF TENDON TRANSFER
results which are at least as good in both digital nerves
and probably in main trunks. Assess the problem
Which muscles are missing?
Which muscles are available?
Nerve grafting
The donor muscle should be:
Free autogenous nerve grafts can be used to bridge
expendable
gaps too large for direct suture. The sural nerve is
powerful enough
most commonly used; up to 40 cm can be obtained
an agonist or synergist
from each leg. Because the nerve diameter is small,
several strips may be used (cable graft). The graft The recipient site should:
should be long enough to lie without any tension, and be stable
it should be routed through a well-vascularized bed. have mobile joints and supple tissues
The graft is attached at each end either by fine sutures
The transferred tendon should be:
or with fibrin glue.
routed subcutaneously
It is crucial that the motor and sensory fascicles are
placed in a straight line of pull
appropriately connected by the graft. There are various
capable of firm fixation
techniques which can help. Careful inspection of the
fascicular alignment, structure and vascular markings is The patient should be:
often helpful. Enzyme-staining techniques can be used. motivated
Vascularized grafts are used in special situations. If able to comprehend and attend hand therapy
the ulnar and median nerves are both damaged (e.g. 275
11 intercostal nerves or the spinal accessory nerve to the Size of gap Above the critical resection length, suture
stump of the original nerve supplying that muscle. is not successful.
Age Children do better than adults. Old people do
Care of paralysed parts poorly.
While recovery is awaited the skin must be protected Delay in suture This is a most important adverse fac-
from friction damage and burns. The joints should be tor. The best results are obtained with early nerve
moved through their full range twice daily to prevent repair. After a few months, recovery following suture
stiffness and minimize the work required of muscles becomes progressively less likely.
when they recover. ‘Dynamic’ splints may be helpful.
Associated lesions Damage to vessels, tendons and
other structures makes it more difficult to obtain recov-
Tendon transfers ery of a useful limb even if the nerve itself recovers.
Motor recovery may not occur if the axons, regener- Surgical techniques Skill, experience and suitable facil-
ating at about 1 mm per day, do not reach the mus- ities are needed to treat nerve injuries. If these are
cle within 18–24 months of injury. This is most likely lacking, it is wiser to perform the essential wound toi-
when there is a proximal injury in a nerve supplying let and then transfer the patient to a specialized cen-
distal muscles. In such circumstances, tendon transfers tre.
should be considered. The principles can be summa-
rized in the Box on the previous page.
Recommended transfers are discussed under the
individual nerve lesions. REGIONAL SURVEY OF NERVE
INJURIES
PROGNOSIS
BRACHIAL PLEXUS INJURIES
Type of lesion Neurapraxia always recovers fully;
axonotmesis may or may not; neurotmesis will not
unless the nerve is repaired.
Pathological anatomy
The brachial plexus is formed by the confluence of
Level of lesion The higher the lesion, the worse the
nerve roots from C5 to T1; the network and its
prognosis.
branches are shown diagrammatically in Figure 11.9.
Type of nerve Purely motor or purely sensory nerves The plexus, as it passes from the cervical spine
recover better than mixed nerves, because there is less between the muscles of the neck and beneath the clav-
likelihood of axonal confusion. icle en route to the arm, is vulnerable to injury –
Dorsal scapular nerve

C5
C6
Suprascapular nerve
C7
T1
T2
Radial nerve
Long thoracic nerve
Lateral and medial

Axillary nerve pectoral nerves
Musculocutaneous
nerve
Medial cutaneous nerve of arm
Medial cutaneous nerve of forearm
Median nerve
Ulnar nerve
11.9 Brachial plexus Diagram of the brachial plexus and its relationship to the clavicle (some of the less important nerve
276 branches and the posterior attachment of the second rib have been omitted).
either a stab wound or severe traction caused by a fall 11
on the side of the neck or the shoulder.
Traction injuries are generally classed as supraclav-
icular (65 per cent), infraclavicular (25 per cent) and
combined (10 per cent). Supraclavicular lesions typi-
cally occur in motorcycle accidents: as the cyclist col-

lides with the ground or another vehicle his neck and
shoulder are wrenched apart. In the most severe
injuries the arm is practically avulsed from the trunk,
with rupture of the subclavian artery. Infraclavicular
lesions are usually associated with fractures or disloca-
tions of the shoulder; in about a quarter of cases the 11.10 Brachial plexus injury Ischaemic insensate hand.
axillary artery also is torn. Fractures of the clavicle
rarely damage the plexus and then only if caused by a
direct blow. If the entire plexus is damaged, the whole limb is
The injury may affect any level, or several levels paralysed and numb.
within the plexus, often involving a mixture of nerve Sometimes the scapular muscles and one side of the
root(s), trunk(s) and nerve(s). An important distinc- diaphragm too are involved. By examining systemati-
tion is made between preganglionic and postgan- cally for each component of the brachial plexus (roots,
glionic lesions. Avulsion of a nerve root from the trunks, divisions, cords and branches) the exact site of
spinal cord is a preganglionic lesion, i.e. disruption the lesion may be identified. For instance, preserva-
proximal to the dorsal root ganglion; this cannot tion of the dorsal scapular nerve (rhomboids), long
recover and it is surgically irreparable. Rupture of a thoracic nerve (serratus anterior) and suprascapular
nerve root distal to the ganglion, or of a trunk or nerve (supraspinatus), but loss of musculocutaneous
peripheral nerve, is a postganglionic lesion, which is nerve function (biceps), radial nerve (triceps) and axil-
surgically reparable and potentially capable of recov- lary nerve (deltoid) suggest a lateral and posterior
ery. Lesions in continuity, from first to fourth degree, cord injury.
generally have a better prognosis than complete rup-
tures. Mild lesions (neurapraxia) are fairly common PRE- OR POST-GANGLIONIC?
and may be caused by comparatively trivial trauma It is crucial to establish how far from the cord the
such as sudden compression by a tight harness or lesion is. Preganglionic lesions (root avulsions) are
motor vehicle seatbelt; these recover spontaneously irreparable; postganglionic lesions may either recover
but mild residual symptoms may prove a nuisance for (axonotmesis) or may be amenable to repair. Features
many months. suggesting root avulsion are: (1) crushing or burning
pain in an anaesthetic hand; (2) paralysis of scapular
muscles or diaphragm; (3) Horner’s syndrome – pto-
Clinical features
sis, miosis (small pupil), enophthalmos and anhidro-
Brachial plexus injuries are often overshadowed by sis; (4) severe vascular injury; (5) associated fractures
other, life-threatening trauma which needs immediate of the cervical spine; and (6) spinal cord dysfunction
attention. Associated injuries, such as rupture of the (e.g. hyper-reflexia in the lower limbs).
subclavian or axillary artery, should be sought and The histamine test is intriguing. Intradermal injec-
attended to, otherwise a poor outcome is inevitable. tion of histamine usually causes a triple response in the
Neurological dysfunction soon becomes obvious. surrounding skin (central capillary dilatation, a wheal
Detailed clinical examination is directed at answering and a surrounding flare). If the flare reaction persists
specific questions: What is the level of the lesion? Is it in an anaesthetic area of skin, the lesion must be prox-
preganglionic or postganglionic? If postganglionic, imal to the posterior root ganglion, i.e. it is probably
what type of lesion is it? a root avulsion. With a postganglionic lesion the test
will be negative because nerve continuity between the
THE LEVEL OF THE LESION skin and the dorsal root ganglion is interrupted.
In upper plexus injuries (C5 and 6) the shoulder CT myelography or MRI may show pseudo-
abductors and external rotators and the forearm meningoceles produced by root avulsion. Note that
supinators are paralysed. Sensory loss involves the during the first few days a ‘positive’ result is unreliable
outer aspect of the arm and forearm. because the dura can be torn without there being root
Pure lower plexus injuries are rare. Wrist and finger avulsion.
flexors are weak and the intrinsic hand muscles are Nerve conduction studies need careful interpreta-
paralysed. Sensation is lost in the ulnar forearm and tion. If there is sensory conduction from an anaes-
hand. thetic dermatome, this suggests a preganglionic lesion 277
11 Progress of the neurological features is carefully
monitored. As long as recovery proceeds at the
expected rate, watchful conservation is the byword. If
recovery falters, or if special investigations show that
it is more than a second degree lesion, then the
patient should be referred to a special centre for sur-
gical exploration of the brachial plexus and nerve

repair, grafting or nerve transfer procedures. The
sooner this decision is made, the better: during the
early days operative exposure is easier and the
response to repair more reliable. Repairs performed
after 6 months are unlikely to succeed.
THE PATTERN OF INJURY

Surgical exploration reveals three typical patterns of
injury:
• C5,6(7) avulsion or rupture with C(7)8, T1 intact:
this group has the most favourable outcome as
11.11 Brachial plexus The myelogram shows leakage of hand function is preserved and muscles innervated
the contrast medium, indicating root avulsion. from the upper roots often recover after plexus
repair or nerve transfer.
• C5,6(7) rupture with avulsion of C7,8,T1: these
(i.e. the nerve distal to the ganglion is not inter-
may recover shoulder and elbow movement after
rupted). This test becomes reliable only after a few
repair and grafting of the upper levels, but hand
weeks, when wallerian degeneration in a postgan-
function is irretrievably lost.
glionic lesion will block nerve conduction.
• C5–T1 avulsion: these cases have a poor outcome.
There are few donor axons available to neurotize
THE TYPE OF LESION
the upper levels (shoulder and elbow function) and
Once a postganglionic lesion has been diagnosed, it
no recovery will take place in the hand.
becomes important to decide how severely the nerve
has been damaged. The history is informative: the The implication is that all efforts for nerve repair or
mechanism of injury and the impact velocity may sug- nerve transfer are directed towards lesions involving C5
gest either a mild (first or second degree) or a severe and 6. The objectives are to regain shoulder abduction,
(fourth or fifth degree) injury. With the former a elbow flexion, wrist extension, finger flexion, and sen-
period of observation is justified; a first or second sibility over the lateral (radial) side of the hand.
degree lesion may show signs of recovery by 6 or 8
weeks. If a neurotmesis seems likely then early opera- NERVE GRAFTING AND NERVE TRANSFER
tive exploration is called for. Since there may be dif- Nerve grafting is often necessary and the results for
ferent degrees of injury within the plexus, some restoration of shoulder and elbow function are quite
muscles may recover while others fail to do so. good; however, the outcome for lesions affecting the
forearm and hand is disappointing .
Nerve transfer is an alternative way of providing
Management functioning axons. If C5 and C6 are avulsed, then the
The patient is likely to be admitted to a general unit spinal accessory nerve can be transferred to the
where fractures and other injuries will be given prior- suprascapular nerve; or two or three intercostal nerves
ity. Emergency surgery is required for brachial plexus can be transferred to the musculocutaneous nerve.
lesions associated with penetrating wounds, vascular If one nerve root is available (e.g. C5) then this
injury or severe (high energy) soft-tissue damage should be grafted on to the lateral cord which will sup-
whether open or closed; clean cut nerves should be ply elbow flexion, finger flexion and sensation over the
repaired or grafted. This is best performed by a team radial side of the hand. If two roots are available (e.g.
specializing in this field of work. C5, C6) these can be grafted on to the lateral and pos-
All other closed injuries are left until detailed exam- terior cords. These procedures bypass the suprascapular
ination and special investigations have been com- nerve which is joined to the spinal accessory nerve.
pleted. Patients with root avulsion or severe, With complete preganglionic loss, the contralateral C7
mutilating injuries of the limb will be unsuitable for root can be extended across the chest with autologous
nerve surgery, at least until the prognosis for limb graft and then used as an axon source into the plexus.
278 function becomes clear. There is remarkably little deficit in the donor limb.
Two or three years must pass before the final results 11
of plexus reconstruction are apparent. OBSTETRICAL BRACHIAL PLEXUS
PALSY
LATER RECONSTRUCTION
Obstetrical palsy is caused by excessive traction on the
The best results of plexus reconstruction are obtained
brachial plexus during childbirth, e.g. by pulling the
after very early operation. If the patient is not seen

bay’s head away from the shoulder or by exerting trac-
until very late after injury, or if plexus reconstruction
tion with the baby’s arm in abduction. Three patterns
has failed, then there are a number of options:
are seen: (1) upper root injury (Erb’s palsy), typically in
Tendon transfer to achieve elbow flexion Various mus- overweight babies with shoulder dystocia at delivery;
cles can be transferred as elbow flexors: pectoralis (2) lower root injury (Klumpke’s palsy), usually after
major (Clarke’s transfer), the common flexor origin breech delivery of smaller babies; and (3) total plexus
(Steindler transfer), latissimus dorsi, or triceps. The injury.
nerve supply to these muscles must remain intact, so
they are suitable only for certain patterns of injury.
Clinical features
Free muscle transfer Gracilis, rectus femoris or the
The diagnosis is usually obvious at birth: after a diffi-
contralateral latissimus dorsi can be transferred as a
cult delivery the baby has a floppy or flail arm. Further
free flap and innervated with two or three intercostal
examination a day or two later will define the type of
nerves or contralateral C7. Elbow flexion and wrist
brachial plexus injury.
extension can be regained.
Erb’s palsy is caused by injury of C5, C6 and (some-
Shoulder arthrodesis Arthrodesis is usually reserved times) C7. The abductors and external rotators of the
for an unstable or painful shoulder, perhaps after fail- shoulder and the supinators are paralysed. The arm is
ure of re-innervation of the supraspinatus. The posi- held to the side, internally rotated and pronated.
tion must be tailored to the needs of the particular There may also be loss of finger extension. Sensation
patient. cannot be tested in a baby.
(a) (b)
11.12 Obstetrical brachial plexus palsy (a) Paralysis of the abductors and external rotators of the shoulder, as well as
the forearm supinators, results in the typical posture demonstrated in this baby with Erb’s palsy of the left arm. (b) Young
boy with Klumpke’s palsy of the right arm. 279
11 Klumpke’s palsy is due to injury of C8 and T1. The
baby lies with the arm supinated and the elbow flexed;
there is loss of intrinsic muscle power in the hand.
Reflexes are absent and there may be a unilateral
Horner’s syndrome.
With a total plexus injury the baby’s arm is flail and
pale; all finger muscles are parlysed and there may also
be vasomotor impairment and a unilateral Horner’s
syndrome.
X-rays should be obtained to exclude fractures of
the shoulder or clavicle (which are not uncommon
and which can be mistaken for obstetrical palsy).
Management
Over the next few weeks one of several things may
happen.
11.13 Long thoracic nerve palsy Winging of the
Paralysis may recover completely Many (perhaps most)
scapula is demonstrated by the patient pushing forwards
of the upper root lesions recover spontaneously. A against the wall. If the serratus anterior is paralysed, the
fairly reliable indicator is return of biceps activity by scapula cannot be held firmly against the rib-cage.
the third month. However, absence of biceps activity
does not completely rule out later recovery.
Paralysis may improve A total lesion may partially loads on the shoulder, and even viral illnesses or tox-
resolve, leaving the infant with a partial paralysis. oid injections.
Paralysis may remain unaltered This is more likely with
complete lesions, especially in the presence of a Clinical features
Horner’s syndrome.
Paralysis of serratus anterior is the commonest cause
While waiting for recovery, physiotherapy is applied
of winging of the scapula. The patient may complain
to keep the joints mobile.
of aching and weakness on lifting the arm. Examina-
tion shows little abnormality until the arm is elevated
OPERATIVE TREATMENT
in flexion or abduction. The classic test for winging is
If there is no biceps recovery by 3 months, operative
to have the patient pushing forwards against the wall
intervention should be considered. Unless the roots
or thrusting the shoulder forwards against resistance.
are avulsed, it may be possible to excise the scar and
bridge the gap with free sural nerve grafts; if the roots
are avulsed, nerve transfer may give a worthwhile Treatment
result. This is highly demanding surgery which should
Except after direct injury or division, the nerve usually
be undertaken only in specialized centres.
recovers spontaneously, though this may take a year or
The shoulder is prone to fixed internal rotation and
longer. Persistent winging of the scapula occasionally
adduction deformity. If diligent physiotherapy does
requires operative stabilization by transferring pec-
not prevent this, then a subscapularis release will be
toralis minor or major to the lower part of the scapula.
needed, sometimes supplemented by a tendon trans-
fer. In older children, the deformity can be treated by
rotation osteotomy of the humerus.
SPINAL ACCESSORY NERVE
The spinal accessory nerve (C2–6) supplies the ster-

LONG THORACIC NERVE nomastoid muscle and then runs obliquely across the
posterior triangle of the neck to innervate the upper
The long thoracic nerve of Bell (C5, 6, 7) may be half of the trapezius. Contrary to general belief, the
damaged in shoulder or neck injuries (usually an nerve appears also to have sensory functions, includ-
axonotmesis) or during operations such as first rib ing pain sensibility. Because of its superficial course, it
resection, transaxillary sympathectomy or radical mas- is easily injured in stab wounds and operations in the
tectomy. However, serratus anterior palsy is also seen posterior triangle of the neck (e.g. lymph node
280 after comparatively benign events, such as carrying biopsy). It is occasionally injured in whiplash injuries.
or sudden traction, or simply by carrying a heavy load 11
over the shoulder.
Clinical features
There may be a history of injury, but patients some-

times present with unexplained pain in the supras-
capular region and weakness of shoulder abduction –
symptoms readily mistaken for a rotator cuff syn-
drome. There is usually wasting of the supraspinatus
and infraspinatus, with diminished power of abduc-
tion and external rotation. Electromyography may
help to establish the diagnosis.
11.14 Accessory nerve The accessory nerve is embedded
in the fascia which covers the posterior triangle and is
easily damaged during lymph node biopsy or excision (and Treatment
in stab wounds).
This is usually an axonotmesis which clears up sponta-
neously after 3 months. If no recovery is seen at this
stage the nerve should be explored. In the absence of
trauma one might suspect a nerve entrapment syn-
Clinical features
drome, and decompression by division of the supras-
Following an open wound or operation, the patient capular ligament often brings improvement. The
complains of severe pain and ‘stiffness’ of the shoul- operative approach is through a posterior incision
der. Examination reveals asymmetry or drooping of above and parallel to the spine of the scapula.
the shoulder, reduced ability to hitch or hunch the
shoulder and weakness on abduction of the arm; typ-
ically there is mild winging of the scapula on attempt-
ing active abduction against resistance; unlike the AXILLARY NERVE
deformity in serratus anterior palsy, this disappears on
flexion or forward thrusting of the shoulder. Often The axillary nerve (C5, 6) arises from the posterior
the true nature of the problem is not appreciated and cord of the brachial plexus and runs along subscapu-
diagnosis is delayed for weeks or months. In late cases laris and across the axilla just inferior to the shoulder
there may be wasting of the trapezius. joint. It emerges behind the humerus, deep to the
deltoid; after supplying the teres minor, it divides into
a medial branch which supplies the posterior part of
Treatment
the deltoid and a patch of skin over the muscle and an
Stab injuries and surgical injuries should be explored
immediately and the nerve repaired. If the exact cause
of injury is uncertain, it is prudent to wait for about 8
weeks for signs of recovery. If this does not occur, the
nerve should be explored: (a) to confirm the diagno-
sis and (b) to repair the lesion by direct suture or
grafting. While waiting for recovery the arm is held in
a sling to prevent dragging on the neck muscles. The
results of early nerve repair are generally good but
some patients continue to complain of shoulder
fatigue during lifting and overhead activities.
SUPRASCAPULAR NERVE
The suprascapular nerve, which arises from the upper

trunk of the brachial plexus (C5, 6), runs through the
suprascapular notch to supply the supra- and infra-
spinatus muscles. It may be injured in fractures of the 11.15 Axillary nerve Surface marking of the axillary
scapula, dislocation of the shoulder, by a direct blow nerve. 281
11 anterior branch that curls round the surgical neck of
the humerus to innervate the anterior two-thirds of
the deltoid. The landmark for this important branch is
5 cm below the tip of the acromion.
The nerve is sometimes ruptured in a brachial
plexus injury. More often it is injured during shoulder
dislocation or fractures of the humeral neck. Iatro-

genic injuries occur in transaxillary operations on the
shoulder and with lateral deltoid-splitting incisions. It
is sometimes injured at the same time as the supras-
capular nerve in shoulder dislocation. Simultaneous
rupture of the rotator cuff can add to the diagnostic (a) (b)
confusion by causing weak or absent arm abduction 11.16 Radial nerve palsy (a) This man developed a
after shoulder dislocation. complete drop-wrist palsy following a severe open fracture
of the humerus and division of the radial nerve. (b) The
typical area of sensory loss.
Clinical features
The patient complains of shoulder ‘weakness’, and the
deltoid is wasted. Although abduction can be initiated cannot extend the metacarpophalangeal joints of the
(by supraspinatus), it cannot be maintained. hand. In the thumb there is also weakness of exten-
Retropulsion (extension of the shoulder with the arm sion and retroposition. Wrist extension is preserved
abducted to 90 degrees) is impossible. Careful testing because the branch to the extensor carpi radialis
will reveal a small area of numbness over the deltoid longus arises proximal to the elbow.
(the ‘sergeant’s patch’). High lesions occur with fractures of the humerus or
after prolonged tourniquet pressure. There is an obvi-
ous wrist drop, due to weakness of the radial extensors
Treatment of the wrist, as well as inability to extend the metacar-
Nerve injury associated with fractures or dislocations pophalangeal joints or elevate the thumb. Sensory loss
recovers spontaneously in about 80 per cent of cases. is limited to a small patch on the dorsum around the
If the deltoid shows no sign of recovery by 8 weeks, anatomical snuffbox.
EMG should be performed; if the tests suggest den- Very high lesions may be caused by trauma or oper-
ervation then the nerve should be explored through a ations around the shoulder. More often, though, they
combined deltopectoral and posterior (quadrilateral are due to chronic compression in the axilla; this is
space) approach. Excision of the nerve ends and graft- seen in drink and drug addicts who fall into a stupor
ing are usually necessary; a good result can be with the arm dangling over the back of a chair (‘Sat-
expected if the nerve is explored within 3 months of urday night palsy’) or in thin elderly patients using
injury. However, if the operation fails and the shoul- crutches (‘crutch palsy’). In addition to weakness of
der is painfully unstable, then provided that trapezius the wrist and hand, the triceps is paralysed and the tri-
and serratus anterior are functioning, shoulder ceps reflex is absent.
arthrodesis can provide both stability and some
degree of ‘abduction’.
Treatment
Open injuries should be explored and the nerve
repaired or grafted as soon as possible.
RADIAL NERVE Closed injuries are usually first or second degree
lesions, and function eventually returns. In patients
The radial nerve may be injured at the elbow, in the with fractures of the humerus it is important to exam-
upper arm or in the axilla. ine for a radial nerve injury on admission, before
treatment and again after manipulation or internal fix-
ation. If the palsy is present on admission, one can
Clinical features afford to wait for 12 weeks to see if it starts to recover.
Low lesions are usually due to fractures or dislocations If it does not, then EMG should be performed; if this
at the elbow, or to a local wound. Iatrogenic lesions shows denervation potentials and no active potentials
of the posterior interosseous nerve where it winds then a neurapraxia is excluded and the nerve should
through the supinator muscle are sometimes seen be explored. The results, even with delayed surgery
after operations on the proximal end of the radius. and quite long grafts, can be gratifying as the radial
282 The patient complains of clumsiness and, on testing, nerve has a straightforward motor function.
If it is certain that there was no nerve injury on claw hand deformity – with hyperextension of the 11
admission, and the signs appear only after manipula- metacarpophalangeal joints of the ring and little fin-
tion or internal fixation, then the chances of an iatro- gers, due to weakness of the intrinsic muscles.
pathic injury are high and the nerve should be Hypothenar and interosseous wasting may be obvious
explored and – if necessary – repaired or grafted with- by comparison with the normal hand. Finger abduc-
out delay. tion is weak and this, together with the loss of thumb

While recovery is awaited, the small joints of the adduction, makes pinch difficult. The patient is asked
hand must be put through a full range of passive to grip a sheet of paper forcefully between thumbs
movements. The wrist is splinted in extension. ‘Lively’ and index fingers while the examiner tries to pull it
hand splints are avoided as they tend to hold the away; powerful flexion of the thumb interphalangeal
metacarpophalangeal joints in extension with the joint signals weakness of adductor pollicis and first
proximal interphalangeal joints flexed and this will dorsal interosseous with overcompensation by the
lead to fixed contractures. flexor pollicis longus (Froment’s sign).
If recovery does not occur, the disability can be Entrapment of the ulnar nerve in the pisohamate
largely overcome by tendon transfers: pronator teres tunnel (Guyon’s canal) is often seen in long-distance
to the short radial extensor of the wrist, flexor carpi cyclists who lean with the pisiform pressing on the
radialis to the long finger extensors and palmaris handlebars. Unexplained lesions of the distal (motor)
longus to the long thumb abductor. branch of the nerve may be due to compression by a
deep carpal ganglion or ulnar artery aneurysm.
High lesions occur with elbow fractures or disloca-
tions. The hand is not markedly deformed because the
ULNAR NERVE ulnar half of flexor digitorum profundus is paralysed
and the fingers are therefore less ‘clawed’ (the ‘high
Injuries of the ulnar nerve are usually either near the ulnar paradox’). Otherwise, motor and sensory loss
wrist or near the elbow, although open wounds may are the same as in low lesions.
damage it at any level. ‘Ulnar neuritis’ may be caused by compression or
entrapment of the nerve in the medial epicondylar
(cubital) tunnel, especially where there is severe valgus
Clinical features deformity of the elbow or prolonged pressure on the
Low lesions are often caused by cuts on shattered glass. elbows in anaesthetized or bed-ridden patients. It is
There is numbness of the ulnar one and a half fingers. important to be aware of this condition in patients
The hand assumes a typical posture in repose – the who start complaining of ulnar nerve symptoms some
11.17 Ulnar nerve palsy (a) Clawing

of the ring and little fingers and wasting
of the intrinsic muscles. (b) A good test
for interosseous muscle weakness. Ask
the patient to spread his fingers (abduct)
as strongly as possible and then force his
hands together with the little fingers
apposed; the weaker side will collapse
(the left hand in this case). (c) Froment’s
sign: the patient is asked to grip a card
firmly between thumbs and index
(a) (b) fingers; normally this is done using the
thumb adductors while the
interphalangeal joint is held extended. In
the right hand, because the adductor
pollicis is weak, the patient grips the card
only by acutely flexing the
interphalangeal joint of the thumb
(flexor pollicis longus is supplied by the
median nerve). (d) Typical area of
sensory loss.
(c)
(d) 283
11 weeks after an upper limb injury; one can easily be
misled into thinking that the nerve lesion is due to the
original injury!
Treatment
Exploration and suture of a divided nerve are well

worthwhile, and anterior transposition at the elbow
permits closure of gaps up to 5 cm. While recovery is
awaited, the skin should be protected from burns.
Hand physiotherapy keeps the hand supple and
useful.
If there is no recovery after nerve division, hand
function is significantly impaired. Grip strength is (a)
diminished because the primary metacarpophalangeal
flexors are lost, and pinch is poor because of the weak-
ened thumb adduction and index finger abduction.
Fine, coordinated finger movements are also affected.
Metacarpophalangeal flexion can be improved by
extensor carpi radialis longus to intrinsic tendon trans-
fers (Brand), or by looping a slip of flexor digitorum
superficialis around the opening of the flexor sheath
(Zancolli procedure). Index abduction is improved by
transferring extensor pollicis brevis or extensor indicis
to the interosseous insertion on the radial side of the
finger.
(b) (c)
11.19 Median nerve lesions (a) Wasting of the thenar
eminence on the right side. (b) In high median nerve
MEDIAN NERVE lesions, the long flexors to the thumb and index fingers are
also paralysed and the patient shows the ‘pointing index
sign’. (c) Typical area of sensory loss.
The median nerve is most commonly injured near the
wrist or high up in the forearm.
abduct the thumb, and sensation is lost over the radial

Clinical features three and a half digits. In longstanding cases the
Low lesions may be caused by cuts in front of the wrist thenar eminence is wasted and trophic changes may
or by carpal dislocations. The patient is unable to be seen.
High lesions are generally due to forearm fractures
or elbow dislocation, but stabs and gunshot wounds
may damage the nerve at any level. The signs are the
same as those of low lesions but, in addition, the long
flexors to the thumb, index and middle fingers, the
radial wrist flexors and the forearm pronator muscles
are all paralysed. Typically the hand is held with the
ulnar fingers flexed and the index straight (the ‘point-
ing sign’). Also, because the thumb and index flexors
are deficient, there is a characteristic pinch defect:
instead of pinching with the thumb and index finger-
tips flexed, the patient pinches with the distal joints in
full extension.
Isolated anterior interosseous nerve lesions are
(a) (b) extremely rare. The signs are similar to those of a high
median nerve injury, but without any sensory loss.
11.18 Median nerve – testing for abductor power
(a) The hand must remain flat, palm upwards. (b) The
The usual cause is brachial neuritis (Parsonage–Turner
patient is told to point the thumb towards the ceiling Syndrome) which is associated with shoulder girdle
284 against the examiner’s resistance. pain after immunization or a viral illness.
11
(e)

(a) (b) (c) (d) (f) (g)
11.20 Two problems in sciatic nerve lesions are (a) trophic ulcers because of sensory loss and (b) foot drop. Sensory loss
following division of (c) complete sciatic nerve, (d) common peroneal nerve, (e) posterior tibial nerve and (f) anterior tibial
nerve. (g) Drop foot can be treated by rerouting tibialis posterior so that it acts as a dorsiflexor.
Treatment Clinical features

If the nerve is divided, suture or nerve grafting should Quadriceps action is lacking and the patient is unable
always be attempted. Postoperatively the wrist is to extend the knee actively. There is numbness of the
splinted in flexion to avoid tension; when movements anterior thigh and medial aspect of the leg. The knee
are commenced, wrist extension should be prevented. reflex is depressed. Severe neurogenic pain is common.
Late lesions are sometimes seen. If there has been
no recovery, the disability is severe because of sensory
loss and deficient opposition. If sensation recovers but
Treatment
not opposition, extensor indicis proprius or, less suit- This is a fairly disabling lesion and, where possible,
ably, abductor digiti minimi can be re-routed to the counter-measures should be undertaken. A thigh
insertion of abductor pollicis brevis. Extensor carpi haematoma may need to be evacuated. A clean cut of
radialis longus is available as a transfer for flexor digi- the nerve may be treated successfully by suturing or
torum profundus, brachioradialis for flexor pollicis grafting but results are disappointing. The alternative
longus and extensor indicis for abductor pollicis bre- would be a caliper to stabilize the knee, or tendon
vis. transfers of hamstrings to quadriceps.
LUMBOSACRAL PLEXUS SCIATIC NERVE
The plexus may be injured by massive pelvic trauma. Division of the main sciatic nerve is rare except in
These lesions are usually incomplete and often missed; gunshot wounds. Traction lesions may occur with
the patient may complain of no more than patchy traumatic hip dislocations and with pelvic fractures.
muscle weakness and some difficulty with micturition. Intraneural haemorrhage in patients receiving antico-
Sensation is diminished in the perineum or in one or agulants is a rare cause of intense pain and partial loss
more of the lower limb dermatomes. Some patients, of function.
however, have significant problems with incontinence, Iatropathic lesions are sometimes discovered after
impotence and neurogenic pain. Plexus injuries should total hip replacement – due either to inadvertent divi-
always be sought in patients with fractures of the pelvis. sion, compression by bone levers or possibly thermal
Surgery is rarely undertaken. injury from extruded acrylic cement; in most cases,
though, no specific cause can be found and injury is
assumed to be due to traction (see below).
FEMORAL NERVE
Clinical features
The femoral nerve may be injured by a gunshot In a complete lesion the hamstrings and all muscles
wound, by pressure or traction during an operation or below the knee are paralysed; the ankle jerk is absent.
by bleeding into the thigh. Sensation is lost below the knee, except on the medial 285
11 side of the leg which is supplied by the saphenous about the level of the lesion, EMG and nerve con-
branch of the femoral nerve. The patient walks with a duction tests will help.
drop foot and a high-stepping gait to avoid dragging X-rays may show a bone fragment or extruded
the insensitive foot on the ground. cement (with the possibility of thermal damage) in the
Sometimes only the deep part of the nerve is soft tissues; MRI may be needed to establish its prox-
affected, producing what is essentially a common per- imity to the sciatic nerve. However, in most cases no
oneal (lateral popliteal) nerve lesion (see below). This cause is identified and one is left guessing whether the
is the usual presentation in patients suffering foot- nerve was inadvertently injured by a scalpel point,
drop after hip replacement; however, careful examina- haemostat, electrocautery, suture knot or traction
tion will often reveal minor abnormalities also in the levers. Delayed onset palsy may be due to a
tibial (medial popliteal) division. Electrodiagnostic haematoma.
studies will help to establish the level of the injury. In about half the cases the lesion proves to be a first
If sensory loss extends into the thigh and the or second degree injury; some of these recover within
gluteal muscles are weak, suspect an associated lum- weeks, others take months and may not recover com-
bosacral plexus injury. pletely. Unless a definite cause is known or strongly
In late cases the limb is wasted, with fixed deformi- suspected, it is usually worth waiting for 6 weeks to
ties of the foot and trophic ulcers on the sole. see if the condition improves. During this time the
patient is fitted with a drop-foot splint and physio-
therapy is begun.
Treatment
There is no agreement about the indications for
If the nerve is known to be divided, suture or nerve immediate operation. Those who argue against it say
grafting should be attempted even though it may take they are unlikely to find any specific pathology and
more than a year for leg muscles to be re-innervated. anyway if they do discover evidence of nerve damage,
While recovery is awaited, a below-knee drop-foot the chances of functional recovery after nerve repair
splint is fitted. Great care is taken to avoid damaging are probably no better than those of waiting for spon-
the insensitive skin and to prevent trophic ulcers. taneous improvement. Our own indications for early
The chances of recovery are generally poor and, at operation are: (1) total sciatic palsy; (2) a partial lesion
best, will be long delayed and incomplete. Partial associated with severe burning pain; and (3) strong
lesions, in which there is protective sensation of the evidence of a local, and possibly reversible, cause such
sole, can sometimes be managed by transferring tib- as a bone fragment, acrylic cement or haematoma
ialis posterior to the front in order to counteract the near the nerve. If the exploratory operation reveals a
drop foot. The deformities should be corrected if they local cause, it should be corrected. If the nerve is
threaten to cause pressure sores. If there is no recov- divided or shows full thickness damage, repair or
ery whatever, amputation may be preferable to a flail, grafting may be worthwhile. At best, recovery will
deformed, insensitive limb. take several years and will be incomplete. Partial
lesions are better left alone and the resulting disability
managed by splintage and/or tendon transfers.
SCIATIC PALSY AFTER TOTAL HIP

REPLACEMENT
PERONEAL NERVES
The incidence of overt sciatic nerve dysfunction is
reported as 0.5–3 per cent following primary hip Injuries may affect either the common peroneal (lat-
replacement and about twice as high after revision. eral popliteal) nerve or one of its branches, the deep
However, subclinical EMG changes are quite com- or superficial peroneal nerves.
mon. The vast majority of these resolve fairly quickly
and do not manifest as postoperative nerve lesions.
The less fortunate patients present soon after opera-
Clinical features
tion with weakness of ankle dorsiflexion, or a foot- The common peroneal nerve is often damaged at the
drop, and abnormal sensibility in the distribution of level of the fibular neck by severe traction when the
the common peroneal nerve – a combination which is knee is forced into varus (e.g. in lateral ligament
readily mistaken for a peroneal nerve lesion (wishful injuries and fractures around the knee, or during
thinking in almost every case!). The reason for this is operative correction of gross valgus deformities), or
that the ‘peroneal’ portion of the sciatic nerve lies by pressure from a splint or a plaster cast, from lying
closest to the acetabulum and is most easily damaged. with the leg externally rotated, by skin traction or by
Careful examination will often show minor abnormal- wounds. A ganglion from the superior tibio-fibular
286 ities also in the tibial nerve. If there is any doubt joint can also present with this palsy. The patient has
a drop foot and can neither dorsiflex nor evert the Clinical features 11
foot. He or she walks with a high-stepping gait to
avoid catching the toes. Sensation is lost over the The tibial nerve supplies the flexors of the ankle and
front and outer half of the leg and the dorsum of the toes. With division of the nerve, the patient is unable to
foot. Pain may be significant. plantarflex the ankle or flex the toes; sensation is absent
The deep peroneal nerve runs between the muscles over the sole and part of the calf. Because both the long

of the anterior compartment of the leg and emerges at flexors and the intrinsic muscles are involved, there is
the lower border of the extensor retinaculum of the not much clawing. With time the calf and foot become
ankle. It may be threatened in an anterior compart- atrophic and pressure ulcers may appear on the sole.
ment syndrome, causing pain and weakness of dorsi- The posterior tibial nerve runs behind the medial
flexion and sensory loss in a small area of skin between malleolus under the flexor retinaculum, gives off a
the first and second toes. Sometimes the distal portion small calcaneal branch and then divides into medial
is cut during operations on the ankle, resulting in and lateral plantar nerves which supply the intrinsic
paraesthesia and numbness on the dorsum around the muscles and the skin of the sole. Fractures and dislo-
first web space. cations around the ankle may injure any of these
The superficial peroneal nerve descends along the branches and the resultant picture depends on the
fibula, innervating the peroneal muscles and emerging level of the lesion. Thus, posterior tibial nerve lesions
through the deep fascia 5–10 cm above the ankle to cause wide sensory loss and clawing of the toes due to
supply the skin over the dorsum of the foot and the paralysis of the intrinsics with active long flexors; but
medial four toes. The muscular portion may be injury to one of the smaller branches causes only lim-
involved in a lateral compartment syndrome. The ited sensory loss and less noticeable motor weakness.
patient complains of pain in the lateral part of the leg A compartment syndrome of the foot (e.g. following
and numbness or paraesthesia of the foot; there may metatarsal fractures) is easily missed if one fails to test
be weakness of eversion and sensory loss on the dor- specifically for plantar nerve function.
sum of the foot. The cutaneous branches alone may
be trapped where the nerve emerges from the deep Treatment
fascia, or stretched by a severe inversion injury of the
ankle, causing pain and sensory symptoms without A complete nerve division should be sutured as soon
muscle weakness. as possible. A peculiarity of the tibial nerve is that
injury or repair (especially delayed repair) may be fol-
lowed by causalgia.
Treatment While recovery is awaited, a suitable orthosis is
Direct injuries of the common peroneal nerve and its worn (to prevent excessive dorsiflexion) and the sole
branches should be explored and repaired or grafted is protected against pressure ulceration. In suitable
wherever possible. As usual, the earlier the repair, the cases, weakness of plantar flexion can be treated by
better the result. While recovery is awaited a splint hind-foot fusion or transfer of the tibialis anterior to
may be worn to control ankle weakness. Pain may be the back of the foot.
relieved and drop foot is improved in almost 50 per
cent of patients, especially those who are operated on
early. If there is no recovery, the disability can be NERVE COMPRESSION
minimized by tibialis posterior tendon transfer or by
hind-foot stabilization; the alternative is a permanent (ENTRAPMENT) SYNDROMES
splint.
Traction injuries from a knee dislocation may dam- Pathophysiology
age the nerve over a large length, needing a graft so
long that recovery is hopeless. Splintage and tendon Wherever peripheral nerves traverse fibro-osseous
transfers are required. tunnels they are at risk of entrapment and compres-
sion, especially if the soft tissues increase in bulk (as
they may in pregnancy, myxoedema or rheumatoid
arthritis) or if there is a local obstruction (e.g. a gan-
glion or osteophytic spur).
TIBIAL NERVES Nerve compression impairs epineural blood flow
and axonal conduction, giving rise to symptoms such
The tibial (medial popliteal) nerve is rarely injured as numbness, paraesthesia and muscle weakness; the
except in open wounds. The distal part (posterior tib- relief of ischaemia explains the sudden improvement in
ial nerve) is sometimes involved in injuries around the symptoms after decompressive surgery. Prolonged or
ankle. severe compression leads to segmental demyelination, 287
11 target muscle atrophy and nerve fibrosis; symptoms sis, axonal degeneration and end-organ decay; tunnel
are then less likely to resolve after decompression. decompression may then fail to give complete relief.
Peripheral neuropathy associated with generalized
disorders such as diabetes or alcoholism may render a
nerve more sensitive to the effects of compression.
There is evidence, too, that proximal compression MEDIAN NERVE COMPRESSION
(e.g. discogenic root compression) impairs the syn-

thesis and transport of neural substances, so predis- Three separate syndromes are recognized: (1) carpal
posing the nerve to the effects of distal entrapment – tunnel syndrome (far and away the most common);
the so-called ‘double-crush syndrome’. (2) proximal median nerve compression (the ‘prona-
Common sites for nerve entrapment are the carpal tor syndrome’); and (3) anterior interosseous nerve
tunnel (median nerve) and the cubital tunnel (ulnar compression.
nerve); less common sites are the tarsal tunnel (pos-
terior tibial nerve), the inguinal ligament (lateral
cutaneous nerve of the thigh), the suprascapular notch CARPAL TUNNEL SYNDROME
(suprascapular nerve), the neck of the fibula (common
peroneal nerve) and the fascial tunnel of the superficial This is the best known of all the entrapment syn-
peroneal nerve. A special case is the thoracic outlet, dromes. In the normal carpal tunnel there is barely
where the subclavian vessels and roots of the brachial room for all the tendons and the median nerve; con-
plexus cross the first rib between the scalenus anterior sequently, any swelling is likely to result in compres-
and medius muscles. In these cases there may be vas- sion and ischaemia of the nerve. Usually the cause
cular as well as neurological signs. eludes detection; the syndrome is, however, common
at the menopause, in rheumatoid arthritis, pregnancy
and myxoedema.
Clinical features
The patient complains of unpleasant tingling or pain
Clinical features
or numbness. Symptoms are usually intermittent and
sometimes related to specific postures which compro- The history is most helpful in making the diagnosis.
mise the nerve. Thus, in the carpal tunnel syndrome Pain and paraesthesia occur in the distribution of the
they occur at night when the wrist is held still in median nerve in the hand. Night after night the
flexion, and relief is obtained by moving the hand ‘to patient is woken with burning pain, tingling and
get the circulation going’. In ulnar neuropathy, symp- numbness. Hanging the arm over the side of the bed,
toms recur whenever the elbow is held in acute flex- or shaking the arm, may relieve the symptoms. In
ion for long periods. In the thoracic outlet syndrome, advanced cases there may be clumsiness and weakness,
paraesthesia in the distribution of C8 and T1 may be particularly with tasks requiring fine manipulation
provoked by holding the arms in abduction, extension such as fastening buttons.
and external rotation. The condition is far more common in women than
Areas of altered sensation and motor weakness are in men. The usual age group is 40–50 years; in
mapped out. In longstanding cases there may be obvi- younger patients it is not uncommon to find related
ous muscle wasting. The likely site of compression factors such as pregnancy, rheumatoid disease, chronic
should be carefully examined for any local cause. renal failure or gout.
Electromyography and nerve conduction tests help Sensory symptoms can often be reproduced by per-
to confirm the diagnosis, establish the level of com- cussing over the median nerve (Tinel’s sign) or by
pression and estimate the degree of nerve damage.
Conduction is slowed across the compressed segment
and EMG may show abnormal action potentials in
muscles that are not obviously weak or wasted, or fib-
rillation in cases with severe nerve damage.
Treatment
In early cases splintage may help (e.g. holding the
wrist or elbow in extension) and steroid injection into
the entrapment area can reduce local tissue swelling.
(a) (b)
If symptoms persist, operative decompression will
usually be successful. However, in longstanding cases 11.21 Median nerve compression (a) Thenar wasting in
288 with muscle atrophy there may be endoneurial fibro- the right hand, (b) sensory loss.
branches of the median nerve. Internal neurolysis is 11
not recommended. Endoscopic carpal tunnel release
offers an alternative with slightly quicker postopera-
tive rehabilitation; however, the complication rate is
higher.

PROXIMAL MEDIAN NERVE COMPRESSION
The median nerve can be (very rarely) compressed
beneath one of several structures around the elbow
including the ligament of Struthers (a connection
between the medial epicondyle and the humerus), the
bicipital aponeurosis or the arch-like origins of either
(a) pronator teres or flexor digitorum superficialis. This
variability is not well conveyed by the more common
term ‘pronator syndrome’. Symptoms are similar to
those of carpal tunnel syndrome, although night pain
is unusual and forearm pain is more common. Phalen’s
test will obviously be negative; instead, symptoms can
be provoked by resisted elbow flexion with the fore-
arm supinated (tightening the bicipital aponeurosis),
by resisted forearm pronation with the elbow extended
(pronator tension) or by resisted flexion of the middle
finger proximal interphalangeal joint (tightening the
superficialis arch). Pain may be felt in the forearm and
there may be altered sensation in the territory of the
palmar cutaneous branch of the median nerve (which
originates proximal to the carpal tunnel). Tinel’s sign
may be positive over the nerve proximally but not at
(b) the carpal tunnel. Nerve conduction studies may local-
11.22 Median nerve compression – treatment ize the level of the compression but are often negative,
(a) Carpal tunnel injection, (b) open carpal tunnel release. particularly in postural compression. X-ray examina-
tion may show a bony spur at the attachment of
Struthers’ ligament (a very rare association).
Surgical decompression involves division of the
holding the wrist fully flexed for less than 60 seconds bicipital aponeurosis and any other restraining struc-
(Phalen’s test). In late cases there is wasting of the ture (pronator teres, arch of flexor digitorum superfi-
thenar muscles, weakness of thumb abduction and cialis); great care is needed in the dissection.
sensory dulling in the median nerve territory.
Electrodiagnostic tests, which show slowing of ANTERIOR INTEROSSEOUS NERVE SYNDROME
nerve conduction across the wrist, are reserved for The anterior interosseous nerve can be selectively
those with atypical symptoms. Radicular symptoms of compressed at the same sites as the proximal median
cervical spondylosis may confuse the diagnosis and nerve. However, spontaneous (and usually tempo-
may coincide with carpal tunnel syndrome. rary) physiological failure (Parsonage–Turner syn-
drome) is a more likely cause. There is motor
weakness without sensory symptoms. The patient is
Treatment unable to make the ‘OK sign’ – pinching with the
Light splints that prevent wrist flexion can help those thumb and index finger joints flexed, like a ring –
with night pain or with pregnancy-related symptoms. because of weakness of the flexor pollicis longus and
Steroid injection into the carpal canal, likewise, pro- flexor digitorum profundus. Isolated loss of flexor
vides temporary relief. pollicis longus can occur. Pressure over the belly of
Open surgical division of the transverse carpal this muscle in the forearm will flex the thumb-tip,
ligament usually provides a quick and simple cure. thus excluding tendon rupture. The condition usually
The incision should be kept to the ulnar side of the settles spontaneously within a few months. If it
thenar crease so as to avoid accidental injury to the does not, surgical exploration and release or tendon
palmar cutaneous (sensory) and thenar motor transfer may be considered. 289
11 ULNAR NERVE COMPRESSION CUBITAL TUNNEL SYNDROME
The ulnar nerve is easily felt behind the medial epi-
This occurs most commonly at the elbow and less condyle of the humerus (the ‘funny bone’). It can be
commonly at the wrist. trapped or compressed within the cubital tunnel (by
bone abnormalities, ganglia or hypertrophied syn-
ovium), proximal to the cubital tunnel (by the fascial

arcade of Struthers) or distal to the cubital tunnel as
it passes through the two heads of flexor carpi ulnaris
to enter the forearm (Osbourne’s canal). Sometimes it
is ‘stretched’ by a cubitus valgus deformity or simply
by holding the elbow flexed for long periods.
Clinical features
The patient complains of numbness and tingling in
the little and the ulnar half of the ring finger; symp-
toms may be intermittent and related to specific
elbow postures (e.g. they may appear only while the
(a)
patient is lying down with the elbows flexed, or while
holding the newspaper – again with the elbows
flexed). Initially there is little to see but in late cases
there may be weakness of grip, slight clawing, intrin-
sic muscle wasting and diminished sensibility in the
ulnar nerve territory. Froment’s sign and weakness of
abductor digiti minimi can often be demonstrated.
Bone or soft-tissue abnormalities may be obvious.
Tinel’s percussion test, tenderness over the nerve
behind the medial epicondyle, reproduction of the
symptoms with flexion of the elbow, and weakness of
flexor carpi ulnaris and the flexor digitorum profun-
dus to the little finger all suggest compression at the
elbow rather than at the wrist.
The diagnosis may be confirmed by nerve conduc-
tion tests; however, since the symptoms are often pos-
(b) tural or activity related, a negative test does not
exclude the diagnosis.
Treatment
Conservative measures such as modification of pos-
ture and splintage of the elbow in mid-extension at
night should be tried.
If symptoms persist, and particularly if there is
intrinsic wasting, operative decompression is indicated.
Options include simple release of the roof of the
cubital tunnel, anterior transposition of the nerve into
a subcutaneous or submuscular plane, or medial epi-
condylectomy. Simple release is preferable as it avoids
the potential denervation associated with transposition
or the persisting epicondylar pain associated with epi-
(c) condylectomy. During the surgical approach, great
care is taken to avoid damaging the posterior branch of
11.23 Ulnar nerve compression at the elbow The
ulnar nerve may be compressed in the cubital tunnel by the medial cutaneous nerve of the forearm; otherwise
(a) tension in a valgus elbow or (b) osteoarthritic spurs. troublesome numbness, if not neurogenic pain or even
290 (c) Surgical release in situ. complex regional pain syndrome, may result.
COMPRESSION IN GUYON’S CANAL RADIAL (POSTERIOR INTEROSSEOUS) 11
The ulnar nerve can be compressed as it passes through NERVE COMPRESSION
Guyon’s canal at the ulnar border of the wrist. The
symptoms can be pure motor, pure sensory or mixed, The radial nerve itself is rarely the source of ‘entrap-
depending on the precise location of entrapment. A ment’ symptoms. Just above the elbow, it divides into

ganglion from the triquetrohamate joint is the most a superficial branch (sensory to the skin over the
common cause; a fractured hook of hamate and ulnar anatomical snuffbox) and the posterior interosseous
artery aneurysm (seen with overuse of a hammer) are nerve which dives between the two heads of the
much rarer causes. Preservation of sensation in the supinator muscle before supplying motor branches to
dorsal branch of the ulnar nerve (which leaves the extensor carpi ulnaris and the metacarpophalangeal
nerve proximal to Guyon’s canal) suggests entrapment extensors (branches to extensor carpi radialis longus
at the wrist rather than elbow; similarly, power to flexor and brevis arise above the elbow).
carpi ulnaris and flexor digitorum profundus to the lit- Posterior interosseous nerve compression may occur at
tle finger will be maintained. five sites, represented by the mnemonic FREAS
After electrophysiological localization of the lesion [Fibrous bands around radiocapitellar joint; Recurrent
to the wrist, further investigations should be consid- arterial branches; Extensor carpi radialis brevis, Arcade
ered: MRI may demonstrate a ganglion, CT a carpal of Frohse (a thickening at the proximal edge of supina-
fracture and Doppler studies an ulnar artery tor); distal edge of Supinator]. It may also be caused
aneurysm. Depending on the results of these investi- by a space-occupying lesion pushing on the nerve – a
gations, surgery can be planned. ganglion, a lipoma or severe radio-capitellar synovitis.
Two clinical patterns are encountered: the posterior
interosseous syndrome and the radial tunnel syndrome.
POSTERIOR INTEROSSEOUS SYNDROME

Clinical features
This is a pure motor disorder and there are no sensory
symptoms. Gradually emerging weakness of metacar-
pophalangeal extension affects first one or two and
then all the digits. Wrist extension is preserved (the
nerves to extensor carpi radialis longus arise proximal
to the supinator) but the wrist veers into radial devia-
tion because of the weak extensor carpi ulnaris. This
feature helps to distinguish posterior interosseous
(a) nerve entrapment from conditions such as neuralgic
amyotrophy, in which the more proximally supplied
muscles are often affected.
(b)
11.24 Ulnar nerve compression in Guyon’s canal

(a) Schwannoma pushing on the ulnar nerve. (b) Ulnar 11.25 Posterior interosseous nerve compression Wrist
artery aneurysm. in radial deviation; fingers dropped. 291
11 Compression usually occurs within the tunnel rior part of the glenohumeral joint, the acromioclav-
(FREAS) but it may also be caused by swellings (a icular joint, the subacromial bursa, the ligaments
lipoma, a ganglion or synovial proliferation) in or around the shoulder and (in a small proportion of
around the radial tunnel. MRI may help to pinpoint people) the skin on the outer, upper aspect of the arm.
the diagnosis. Compression or entrapment occurs at two sites: (a)
the suprascapular notch and (b) a fibro-osseous tun-
nel where the infraspinatus branch curves around the

Treatment
edge of the scapular spine. Causes are continuous
Surgical exploration is warranted if the condition does pressure or intermittent impact on the supraclavicular
not resolve spontaneously within three months or muscles (e.g. by carrying loads on the shoulder) or
earlier if MRI shows a swelling. Recovery after surgery repetitive traction due to forceful shoulder move-
is slow; if there is no improvement by the end of a ments (e.g. in games which involve pitching and
year, and if muscle weakness is disabling, tendon throwing). In some cases nerve compression may be
transfer is needed. produced by a soft-tissue mass such as a large ‘gan-
glion’ at the back of the shoulder joint.
RADIAL TUNNEL SYNDROME Clinical features

This syndrome is controversial; the symptoms resem- There may be a history of injury to the pectoral girdle;
ble those of ‘tennis elbow’ and the condition is some- more often patients present with unexplained pain in
times labelled ‘resistant tennis elbow’. However a the suprascapular region or at the back of the shoulder,
careful history and examination should distinguish and weakness of shoulder and upper arm movements –
between the two. symptoms readily mistaken for cervical radiculopathy or
Although a motor nerve is involved the patient a rotator cuff disorder. There is usually wasting of the
presents with pain, often work-related or at night, just supraspinatus muscle and diminished power of abduc-
distal to the lateral aspect of the elbow. Resisted wrist tion and external rotation. Tensing the nerve by force-
extension may precipitate the pain. Unlike posterior ful adduction (pulling the arm across the front of the
interosseous syndrome, there is no weakness and chest) causes increased pain.
there is not an association with a mass lesion. Electro-
diagnostic tests are not helpful.
If the symptoms do not resolve with prolonged non-
operative measures (modification of activities and splin- Electromyography and measurement of nerve con-
tage), then surgery is considered. The nerve is freed be- duction velocity may help to establish the diagnosis.
neath the extensor carpi radialis brevis and supinator Ultrasonography and MRI are useful in excluding a
muscle. However, the patient should be warned that soft-tissue mass.
surgery often fails to relieve the symptoms.
Treatment
The first step is to stop any type of activity which
might stress the suprascapular nerve; after a few
SUPRASCAPULAR NERVE weeks, graded muscle-strengthening exercises can be
COMPRESSION introduced. If the condition is likely to settle, it will
do so within 3–6 months.
Chronic or repetitive compression of the suprascapu- If there is no improvement, or if imaging studies
lar nerve and its branches is much more common than reveal a soft-tissue mass, operative decompression is
is generally recognized. The peculiar anatomy of the justified. The nerve is approached through a posterior
nerve makes it unusually vulnerable to both traction incision above and parallel to the spine of the scapula.
and compression. However, the symptoms of this Provided the diagnosis was correct, there is a good
condition closely mimic those of rotator cuff lesions chance that symptoms will be improved; however,
and cervical radiculopathy; unless the diagnosis is kept some muscle wasting will probably remain.
in mind in all such cases, it is likely to be missed.
The suprascapular nerve arises from the upper
trunk of the brachial plexus in the posterior triangle of
the neck and then courses through the suprascapular THORACIC OUTLET SYNDROME
notch beneath the superior transverse scapular liga-
ment to supply the supraspinatus and infraspinatus Neurological and vascular symptoms and signs in the
292 muscles. It also sends sensory branches to the poste- upper limbs may be produced by compression of the
lower trunk of the brachial plexus (C8 and T1) and ing. Unilateral Raynaud’s phenomenon should make 11
subclavian vessels between the clavicle and the first rib. one think ‘thoracic outlet’.
The subclavian artery and lower brachial trunk pass Symptoms and signs may be reproduced by various
through a triangle based on the first rib and bordered provocative manoeuvres. In Adson’s test the patient’s
by scalenus anterior and medius. These neurovascular neck is extended and turned towards the affected side
structures are made taut when the shoulders are while he or she breathes in deeply; this compresses the

braced back and the arms held tightly to the sides; an interscalene space and may cause paraesthesia and
extra rib (or its fibrous equivalent extending from a obliteration of the radial pulse. In Wright’s test the
large costal process), or an anomalous scalene muscle, arms are abducted and externally rotated; again the
exaggerates this effect by forcing the vessel and nerve symptoms recur and the pulse disappears on the
upwards. abnormal side. The examination is continued by ask-
These anomalies are all congenital, yet symptoms ing the patient to hold his or her arms high above
are rare before the age of 30. This is probably because, their head and then open and close the fingers rapidly;
with increasing age, the shoulders sag, thus putting this may cause cramping pain on the affected side
more traction on the neurovascular bundle; indeed (Roos’s test). Unfortunately these tests are neither sen-
drooping shoulders alone may cause the syndrome and sitive nor specific enough to clinch the diagnosis.
symptoms are characteristically posture-related.
Stretching or compression of the lower nerve trunk
produces sensory changes along the ulnar side of the
Investigations
forearm and hand, and weakness of the intrinsic hand X-rays of the neck occasionally demonstrate a cervi-
muscles. The subclavian artery is rarely compressed cal rib or an abnormally long C7 cervical process. X-
but the lumen may contract due to irritation of its rays should also be obtained of the lungs (is there an
sympathetic supply, or else its wall may be damaged apical tumour?) and the shoulders (to exclude any
leading to the formation of small emboli. Even more painful local lesion).
unusual are signs of venous compression – oedema, Angiography and venography are reserved for the
cyanosis or thrombosis. few patients with vascular symptoms.
Electrodiagnostic tests are helpful mainly to exclude
peripheral nerve lesions such as ulnar or median nerve
Clinical features compression which may confuse the diagnosis.
The patient, typically a woman in her 30s, complains
of pain and paraesthesia extending from the shoulder,
Diagnosis
down the ulnar aspect of the arm and into the medial
two fingers. Symptoms tend to be worse at night and In the absence of clear motor signs (which are rare!)
are aggravated by bracing the shoulders (wearing a the diagnosis of thoracic outlet syndrome is not easy.
back-pack) or working with the arms above shoulder Some of the symptoms occur as transient phenomena
height. Examination may show mild clawing of the in normal individuals, and ‘cervical ribs’ are some-
ulnar two fingers with wasting and weakness of the times discovered as incidental findings in patients
intrinsic muscles. If a female, the patient is often who are x-rayed for other reasons. Postural oblitera-
long-necked with sloping shoulders (like a tion of the radial pulse, likewise, may be quite nor-
Modigliani painting). mal; the provocative tests should be interpreted as
Vascular signs are uncommon, but there may be positive only if they affect the pulse and reproduce
cyanosis, coldness of the fingers and increased sweat- the sensory symptoms.
(a) (b)
11.26 Cervical rib (a) Unilateral on right side and (b) bilateral. 293
11 taken to prevent injury to the brachial plexus and
subclavian vessels, or perforation of the pleura.
Patients with arterial obstruction, distal embolism or
a local aneurysm will need vascular reconstruction as
well as decompression.
LOWER LIMB COMPRESSION

SYNDROMES
COMPRESSION OF LATERAL CUTANEOUS NERVE OF

(a) (b) THE THIGH
The lateral cutaneous nerve can be compressed as it
11.27 Thoracic outlet syndrome (a) Amadeo
Modigliani’s painting of Madame Zborowska (courtesy of runs through the inguinal ligament just medial to the
the Tate Gallery, London). (b) X-ray of a long-necked anterior superior iliac spine.
woman: all the vertebrae down to T1 are above the The patient complains of numbness, tingling or
clavicle. burning discomfort over the anterolateral aspect of
the thigh (meralgia paraesthetica). Testing for sensi-
bility to pinprick will reveal a patch of numbness over
the upper outer thigh.
The early symptoms and signs can be mistaken for If the symptoms are troublesome the nerve can be
those of ulnar nerve compression. In fact, ulnar neu- released.
ropathy may accompany thoracic outlet compression
as a manifestation of the double-crush syndrome.
There is pain and numbness over the medial side of TARSAL TUNNEL SYNDROME
the forearm and hand. In severe cases there will be
wasting of all the intrinsic muscles (T1) and weakness Pain and sensory disturbance over the plantar surface
of the long flexors (C8). of the foot may be due to compression of the poste-
Cervical spondylosis is sometimes discovered on x- rior tibial nerve behind and below the medial malleo-
ray. However, this disorder seldom involves the T1 lus. The pain may be precipitated by prolonged
nerve root. weightbearing. It is often worse at night and the
Pancoast’s syndrome, due to apical carcinoma of the patient may seek relief by walking around or stamping
bronchus with infiltration of the structures at the root his or her foot. Paraesthesia and numbness should fol-
of the neck, includes pain, numbness and weakness of low the characteristic sensory distribution, but these
the hand. A hard mass may be palpable in the neck symptoms are not as well defined as in other entrap-
and x-ray of the chest shows a characteristic opacity. ment syndromes. Tinel’s percussion test may be posi-
Rotator cuff lesions sometimes cause pain radiating tive behind the medial malleolus. The diagnosis is
down the arm. However, there are no neurological difficult to establish but nerve conduction studies may
symptoms and shoulder movement is likely to be show slowing of motor or sensory conduction.
abnormal.
Treatment
Treatment Tarsal tunnel entrapment may be relieved by fitting a
Most patients can be managed by conservative treat- medial arch support that holds the foot in slight varus.
ment: exercises to strengthen the shoulder girdle mus- If this fails, surgical decompression is indicated. The
cles, postural training and instruction in work nerve is exposed behind the medial malleolus and fol-
practices and ways of preventing shoulder droop and lowed into the sole; sometimes it is trapped by the
muscle fatigue. Analgesics may be needed for pain. belly of abductor hallucis arising more proximally
Operative treatment is indicated if pain is severe, if than usual. Unfortunately symptoms are not consis-
muscle wasting is obvious or if there are vascular dis- tently relieved by this procedure.
turbances. The thoracic outlet is decompressed by
removing the first rib (or the cervical rib). This is DIGITAL NERVE COMPRESSION IN THE FOOT
accomplished by either a supraclavicular approach or a Compression neuropathy of the digital nerve (Mor-
294 transaxillary approach; in the latter, care must be ton’s metatarsalgia) is dealt with in Chapter 21.
brachial plexus (during operations in the posterior 11
OTHER PERIPHERAL NERVE triangle of the neck), the axillary and musculocuta-
DISORDERS neous nerves (during operations for recurrent disloca-
tion of the shoulder), the posterior interosseous
branch of the radial nerve (during approaches to the
COMPARTMENT SYNDROMES proximal end of the radius), the median nerve at the

Capillary perfusion of a nerve may be markedly wrist (in tendon surgery), the palmar cutaneous
reduced by swelling within an osteofascial compart- branch of the median nerve (in carpal tunnel release),
ment. Direct trauma, prolonged compression or arte- the cutaneous branch of the radial nerve (when oper-
rial injury may result in muscle swelling and a critical ating for de Quervain’s disease), the digital nerves (in
rise in compartment pressure; if unrelieved, this causes operations for Dupuytren’s contracture), the sciatic
further impedance of blood flow, more prolonged nerve (in hip arthroplasty), the common peroneal
ischaemia and so on into a vicious circle of events end- nerve (in operations around the knee) and the sural
ing in necrosis of nerve and muscle. This may occur nerve (in operations on the calcaneum).
after proximal arterial injury, soft-tissue bleeding from Tourniquet pressure is an important cause of nerve
fractures or operations, circular compression by tight injury in orthopaedic operations. Damage is due to
dressings or plasters, and even direct pressure in a direct pressure rather than prolonged ischaemia;
comatose person lying on a hard surface. Lesser, self- injury is therefore more likely with very high cuff
relieving effects are sometimes produced by muscle pressure (it need never be more than 75 mmHg
swelling due to strenuous exercise. Common sites are above systolic pressure), a non-pneumatic tourniquet
the forearm and leg; less common are the foot, upper or a very narrow cuff. However, ischaemic damage
arm and thigh. may occur at ‘acceptable’ pressures if the tourniquet is
left on for more than 2 hours.
ACUTE COMPARTMENT SYNDROME Manipulative pressure or traction – e.g. during
Acute compartment syndrome and its late effects reduction of a fracture or dislocation – may injure a
(Volkmann’s contracture) are described in Chapter nerve coursing close to the bone or across the joint.
23. Shoulder abduction and varus angulation of the knee
under anaesthesia are particularly dangerous. Even
CHRONIC COMPARTMENT SYNDROME moderate pressure or traction can be harmful in
Long-distance runners sometimes develop pain along patients with peripheral neuropathy; this is always a
the anterolateral aspect of the calf, brought on by risk in alcoholics and diabetics.
muscular exertion. Swelling of the anterior calf mus- Injections are occasionally misdirected and delivered
cles contained within the inexpansile deep fascia into a nerve (usually the radial or sciatic during intra-
causes ischaemia of the deep peroneal nerve as it trav- muscular injection, the median nerve during non-
erses the compartment. The condition is diagnosed operative treatment of carpal tunnel syndrome or the
from the history and can be confirmed by measuring brachial plexus during axillary blockade).
the compartment pressure before and after exercise. Irradiation may cause irreparable nerve damage, a
Release of the fascia is curative. The same syndrome is mishap not always avoidable when treating cancer. The
very rarely seen in the forearm muscles. effects may not appear until a year or two after exposure.
Diagnosis
IATROPATHIC INJURIES Following operations in ‘high-risk’ areas of the body,
local nerve function should always be tested as soon as
Positioning the patient for diagnostic or operative pro- the patient is awake. Even then it may be difficult to
cedures needs careful attention so as to avoid com- distinguish true weakness or sensory change from the
pression or traction on nerves at vulnerable sites. The ‘normal’ postoperative discomfort and unwillingness
brachial plexus, radial nerve, ulnar nerve and common to move.
peroneal nerve are particularly at risk. Recovery may Initially it may be impossible to tell whether the
take anything from a few minutes to several months; lesion is a neurapraxia, axonotmesis or neurotmesis.
permanent loss of function is unusual. With closed procedures it is more likely to be a lesser
During operation an important nerve may be injury, with open ones a greater. If there is no recov-
injured by accidental scalpel or diathermy wounds, ery after a few weeks, EMG may be helpful. The
excessive traction, compression by instruments, snar- demonstration of denervation potentials suggests
ing by sutures or heating and compression by either axonotmesis or neurotmesis. Surgical explo-
extruded acrylic cement. Nerves most frequently ration at this early stage gives the best chance of a
involved are the spinal accessory or the trunks of the favourable outcome. 295
11 Prevention and treatment in loss of sensibility and muscle weakness affecting the
hands and feet (see Chapter 2). The former may result
Awareness is all. Knowing the situations in which in poor wound healing, ulceration and scarring –
there is a real risk of nerve injury is the best way to mainly affecting the hands. The latter may result in
prevent the calamity. The operative exposure should deformity and joint instability.
be safe and well rehearsed; important nerves should
be given a wide berth or otherwise kept under vision

and out of harm’s way; retraction should be gentle
and intermittent; hidden branches (such as the poste- THE HAND
rior interosseous nerve in the supinator muscle)
should be retracted with their muscular covering. It The ulnar nerve is most often affected; combined
goes without saying that self-retaining retractors ulnar and median nerve paralysis is less common and
should never be used to retract nerves. triple nerve (ulnar, median and radial) paralysis is rare.
If a nerve is seen to be divided during surgery, it Any other kind of paralysis is extremely rare. The clin-
should be repaired immediately; if this cannot be done, ical features associated with these conditions are sum-
the wound can be closed, help can be summoned and marized in Table 11.1 and typical deformities are
the nerve can be re-explored as soon as possible. shown in the accompanying figures.
If the injury is discovered only after the operation,
it is best to re-operate as soon as possible, referring
Claw-finger correction
the patient to a specialized centre if needed.
If nerve division is thought to be unlikely, then it is This deformity is improved, and the movements lost
wiser to wait for signs that might clarify the diagnosis. due to intrinsic muscle paralysis are restored, by rebal-
If there is marked loss of function and no flicker of ancing muscle pull at the metacarpophalangeal
recovery by 6 weeks, the nerve should be explored. (MCP) or proximal interphalangeal (PIP) joint or at
Even then, fibrosis may make diagnosis difficult; nerve both joints. A number of operations have been
stimulation will show whether there is conduction employed to achieve this end (Table 11.2). The oper-
across the injured segment. Partial lesions or injuries ation currently favoured by most surgeons is the ‘lasso
that cause only minor disability are probably best left operation’ of Zancolli in which one tendon of flexor
alone. More serious lesions may need excision and digitorum superficialis (FDS) is split into four slips
repair or grafting. and one slip each is looped around the A1 pulley of
each affected finger so as to provide an independent
flexor to MCP joints.
LEPROSY
The thumb in ulnar palsy
Long-term disabilities in patients with leprosy are due The severely unstable thumb due to flexor pollicis
mainly to peripheral nerve abnormalities which result brevis (FPB) paresis (Figs 11.30 and 11.31) can be
Table 11.1 Clinical features of paralytic hand deformities in leprosy

Pattern of paralysis Frequency Deformity Consequence Disability
Isolated (high or low) Most common Partial claw-hand Intrinsic muscle deficiency Poor precision handling
ulnar nerve paralysis Ulnar palsy thumb, Froment’s sign Weak grip
Z deformity
Combined ulnar and Less common Total claw-hand ‘Intrinsic zero’ hand Only thumb–index (lateral
low median nerve Claw fingers and claw All intrinsic muscles pinch or ‘key grip’) and hook
paralysis thumb paralysed grips possible. Power grip and
Fingers and thumb activated precision handing become
by long muscles only difficult or impossible
Ulnar, low median and Rare Drop-wrist and dropped ‘Long-flexor driven’ hand Severe loss of function
radial nerve paralysis digits All intrinsic muscles and long Cannot grasp or hold objects
extensors paralysed
Combined ulnar and Very rare Mild clawing ‘Extensor driven’ hand Very severe functional loss
high median nerve All intrinsic muscles and long Cannot grip
paralysis flexors paralysed
Ulnar, high median Very rare Drop-wrist Denervated hand Total loss of function
and radial nerve All muscles below elbow
paralysis paralysed
296
Table 11.2 Strategies and tactics for claw-finger 11
correction
Strategy Tactic Procedure
Restore Reduce extending Extensor diversion graft1
balance at force operation
MCP joint Increase flexing forces Capsulodesis2 Tenodesis3,4

Increase flexor Dermodesis5
moment arm Pulley advancement6
Restore Reduce flexing force FDS transfer of Bunnell7
balance at Increase extending FDS transfer of Bunnell7
PIP joint force
Restore Tendon transfer ECRL/ECRB transfers of
balance at operations in which Brand8
both joints the transfer is routed Palmaris longus transfer of
volar to MCP and Antia9
dorsal to PIP joints Fowler’s digital extensor (a) (b)
transfer and other similar
procedures 11.29 Total claw hand (a) Total claw-hand deformity in
combined paralysis of ulnar and median nerves. The flexed
1
Srinivasan, 2Zancolli, 3Parkes, 4Riordan, 5Srinivasan, 6Palande, 7Bunnell, terminal phalanges of the ring and little fingers indicate
8
Brand, 9Antia, 10Zancolli. that this is a case of ‘low’ ulnar paralysis, i.e. distal to the
ECRB, extensor carpi radialis brevis; ECRL, extensor carpi radialis longus; elbow beyond the points of origin of the motor branches
FDS, flexor digitorum superficialis; MCP, metacarpophalangeal; PIP, proximal of flexor digitorum profundus. (b) With intrinsic minus
interphalangeal. disability, isolated metacarpophalangeal flexion is not
possible. (Courtesy of Dr Santosh Rath.)
corrected by augmenting flexion at the MCP joint or
extension at the interphalangeal joint or both. In one
procedure, the radial half of flexor pollicis longus The thumb in combined ulnar and
(FPL) tendon is ‘dorsalized’ by bringing it over the median nerve paralysis
proximal phalanx distal to the MCP joint and fixing it
to extensor pollicis longus tendon, turning FPL into Complete paralysis of all thenar muscles (the
an MCP flexor. Alternatively, FPB can be substituted ‘intrinsic-zero’ thumb) results in loss of effective
by transferring the radial half of the index flexor power and precision-grip (Fig. 11.30). Correction
superficialis tendon. requires stabilization of the carpometacarpal joint in
the ‘opponens position’ (abduction, flexion and inter-
nal rotation) by opponensplasty using flexor superfi-
cialis of the middle or ring finger or extensor indicis
proprius.
(a) (b)
11.28 Partial claw hand (a) Partial claw-hand deformity
in ulnar nerve paralysis: ring and little fingers are clawed
more severely than index and middle fingers. The virtually (a) (b)
straight terminal phalanges of the clawed ring and little
fingers indicate that flexor digitorum profundus going to 11.30 Claw thumb (a) ‘Claw-thumb’ (hyperextended at
these two fingers is paralysed, so this must be a case of the basal and flexed at the middle and distal joints) in
‘high’ ulnar paralysis. (Courtesy of Dr G. N. Malaviya.) combined ulnar and median nerve paralysis. Note wasting
(b) ‘Intrinsic minus’ disability: isolated PIP extension. of the thenar eminence. (b) Illustrating pinch in thenar
Keeping the metacarpophalangeal joints in flexion is not paralysis. Only the lateral or ‘key-pinch’ is possible for these
possible. (Courtesy of Dr Santosh Rath.) hands. (Courtesy of Dr Santosh Rath.) 297
11
(a) (b)
(a) (b)
11.32 Right drop-foot (a) Preoperative deformity. The
11.31 Thumb in ulnar palsy with paralysis of flexor patient is attempting to lift both feet but can do so only on
pollicis brevis (a) While at rest the proximal phalanx is the left side. (b) Same patient one year after surgical
de-rotated and lies in line with the metacarpal instead of correction by two-tailed circumtibial transfer of tibialis
being flexed by about 25 degrees, and the distal phalanx is posterior to extensor hallucis longus and extensor
flexed by about 15 degrees. (Courtesy of Dr G. N. digitorum longus tendons over the dorsum of the foot.
Malaviya.) (b) Acting against resistance, the thumb (Courtesy of Dr Santosh Rath.)
collapses into hyperextension at the metacarpophalangeal
joint and hyperflexion at the interphalangeal joint
(Z deformity). (Courtesy of Dr Santosh Rath.)
walking. In the course of time, the foot becomes stiff
in varus, the weightbearing lateral part of the foot gets
damaged and ulcers develop here. In neglected cases
the outer part of the foot is destroyed by repeated
Triple paralysis ulceration.
Combined loss of ulnar, median and radial nerve A suitable drop-foot orthosis offers a temporary
function causes very severe disability. The patient has solution, only until corrective surgery is available. The
a ‘flexor driven’ hand as only the long flexors of the choice of operation depends on whether the defor-
fingers and the wrist flexors are active. Multiple ten- mity is mobile or fixed.
don transfers to stabilize the wrist, fingers and thumb
Mobile drop-foot This is corrected by transfer of tib-
in extension are needed; the resulting functionally
ialis posterior tendon, which is almost never paralysed
‘intrinsic zero’ hand is then corrected.
in leprosy. The tendon is re-routed to run in front of
the ankle and is fixed in the foot so that the muscle
now acts as a dorsiflexor (Fig. 11.32b). Skeletal fixa-
THE FOOT IN LEPROSY tion of the transferred tendon is not advised as that
might precipitate tarsal disorganization. Circumtibial,
Feet are involved less often than hands but the conse-
two-tailed tibialis posterior tendon transfer to exten-
quences are more serious. Problems include drop-foot,
sor hallucis and extensor digitorum longus tendons
claw toes, plantar ulceration and tarsal disorganiza-
over the dorsum of the foot is most commonly done;
tion.
it is usually combined with tendo calcaneus lengthen-
ing. When only the anterior compartment muscles are
Drop-foot paralysed, a similar transfer of peroneus longus is
done.
‘Drop-foot’ occurs in 1–2 per cent of leprosy patients,
because of paralysis of muscles in the anterior and lat- Fixed drop-foot deformity Fixed equinus or equino-
eral compartments of the leg consequent to damage varus usually requires triple arthrodesis of the hind-
to the common peroneal nerve. Sometimes only the foot (Lambinudi’s operation), which should provide
dorsiflexors or the evertors of the foot are paralysed. the patient with a plantigrade foot.
In dorsiflexor paralysis the patient has to lift the leg
higher than usual during walking for clearing the
Claw-toes
ground (high-stepping gait). If the condition is neg-
lected, the foot becomes stiff in equinus with This condition, due to plantar intrinsic muscle paraly-
intractable forefoot ulceration. sis, is more common than drop-foot. It increases the
In evertor paralysis the foot remains inverted when risk of plantar ulceration greatly. Treatment depends
298 striking the ground and during the push-off stage of on the severity of the deformity.
First degree (mild) claw-toes There is no joint stiffness NATURAL HISTORY 11
but the tips of the toes become ulcerated. The defor- The natural history of plantar ulcers is a dismal cycle of:
mity is corrected by transfer of the long flexor to the ulceration – infection – tissue loss – healing – break-
extensor expansion of each toe. down of scar – recurrent ulceration – spread of infec-
tion with further tissue loss – healing with deformity –
Second degree (moderate) claw-toes The interpha-
more frequent recurrences, and so on until the forefoot
langeal joints have fixed flexion but the metatarsopha-

is destroyed, tarsal sepsis supervenes and the foot is lost
langeal joints remain mobile. PIP arthrodesis, with or
or removed. Sometimes lethal complications (gas
without excision of the distal interphalangeal (DIP)
gangrene, septicaemia or malignancy) supervene.
joint, is needed.
Third degree (severe) claw-toes Fixed flexion of the IP MANAGEMENT
joints is associated with dorsal migration of the toes A necrosis blister should be treated promptly by com-
and fixed hyperextension of the metatarsophalangeal pression bandaging, rest and elevation for 3 days, fol-
(MTP) joints; the metatarsal heads are pushed down lowed by a below-knee walking plaster of Paris cast for
towards the sole of the forefoot (plunger effect). 3 weeks. If the blister is likely to burst, it is opened
Trans-metatarsal amputation is probably the treat- under aseptic conditions and dressed before applying
ment of choice, but patients usually reject this option. the cast.
A more conservative solution requires open reduction Simple ulcers present as chronic, shallow lesions.
of the MTP joints, proximalization of the long exten- They remain unhealed because they are subjected to
sor tendons and PIP arthrodesis. Surgical syndactyly the repetitive trauma of walking. A below-knee walk-
helps to fix a ‘floating toe’. ing-cast, which eliminates the forefoot stage of the
walking cycle, is applied and kept on for 6 weeks.
Split-thickness skin grafting hastens healing of large
simple ulcers. Walking is resumed gradually and only
Plantar ulceration (trophic ulcers)
with protective footwear.
Painless chronic ulcers that occur ‘spontaneously’ are Acute infected ulcers require bed rest, elevation of
commonly seen in the soles of neurologically com- the foot, frequent wet dressings and local irrigation.
promised feet. They heal with difficulty and recur eas- Systemic antibiotics are used if there are symptoms
ily. Loss of sensibility is the main predisposing cause and signs of general infection. Surgery is limited to
and the risk of ulceration increases greatly when plan- drainage procedures.
tar intrinsic muscles are paralysed or when there is Complicated ulcers are chronic ulcers associated
some deformity. Plantar ulcers are colonized by ‘street with additional factors such as infection of deeper
bacteria’; they remain chronic because they are not structures or deformity. The principles of manage-
treated properly. ment are ulcer debridement (which may have to be
About 80 per cent of the ulcers are located in the repeated many times) and protected weightbearing;
ball of the foot (the majority under the first MTP deformity correction and stabilizing operations (like
joint), about 8 per cent in the cubo-metatarsal joint arthrodesis) are performed, if needed, after sound
region, about 10 per cent in the heel, and about 2 per healing has been obtained. Sometimes chronic ulcers
cent over the tips of the toes. present as ‘cauliflower growths’ which commonly
turn out to be pseudo-epitheliomas or less commonly
PATHOGENESIS epitheliomas of low grade malignancy. Deep local
During walking the body-load shifts from the heel to excision is adequate as treatment and essential for his-
the forefoot and from the lateral to the medial side of tological confirmation.
the forefoot. In this process the subcutaneous tissues Recurrent plantar ulcers occur because the original
suffer significant compression, shear and stretch, causes (anaesthesia, muscle paralysis and walking) per-
which is normally countered by the intrinsic muscles. sist. Additional factors are: poor quality skin, excessive
These stresses are increased momentarily with each loading of the scar, deep-seated infection and poor
step when the intrinsic muscles are paralysed. Even blood supply. The risk can be minimized by constant
slightly increased stresses, if repetitive, eventually lead vigilance and attention to hydration of the sole, the
to tissue damage. A necrosis blister develops and that use of protective footwear, restricted walking and cor-
breaks down to form an ulcer. rection of stress-inducing deformities.
Injuries occurring in insensitive feet are often neg- Excessive pressures due to prominent metatarsal
lected because the patient does not experience pain. heads on the sole of the foot can be treated by: (a)
Wounds fester and develop into ulcers. Even in the plantar condylectomy and transfer of the long exten-
absence of any injury, the lack of sweating in the den- sor tendons to the metatarsal necks; (b) dorsal dis-
ervated sole predisposes to the development of cracks placement metatarsal osteotomies; or (c) excision of
and fissures and they easily become infected. an entire ray in the foot. 299
11 Intractable ulceration along the lateral border of the
foot, due to equinovarus deformity, will need an
appropriate triple arthrodesis or a more complicated
joint-sparing procedure to render the foot planti-
grade.
Heel scars may require plastic surgical flaps com-
bined with ‘bumpectomy’ to remove bony promi-

nences. Deformities of the calcaneum which produce
high-pressure areas should be treated by re-establish-
ing the posterior pillar of the arch of the foot, by (a)
doing an appropriately designed calcaneal osteotomy.
Sometimes subtotal resection of the calcaneum is
needed to get rid of persistent infection; after this type
of surgery the inside of the shoe heel will need to be
padded.
OTHER OPERATIONS
In suitably selected cases, posterior tibial neurovascu-
lar decompression behind and above the ankle
improves the blood supply to the sole and helps heal
a recurring or non-healing ulcer.
‘Flail foot’ after loss of the talus is corrected by (b)
tibio-calcaneal fusion. 11.33 The neuropathic foot (a) Neuropathic tarsal
disorganization (right foot). (b) Radiograph of the same
foot. There is disruption at the mid-tarsal level with
Neuropathic tarsal disorganization separation of the forefoot from the talus and calcaneum.
The talo-calcaneal articulation is intact, the talus is
ASEPTIC DISORGANIZATION plantarflexed and the calcaneum is in equinus. The head of
Aseptic tarsal disorganization is uncommon. It may the plantarflexed talus has ploughed through the mid foot
follow an inadequately treated fracture of a tarsal and has become directly weightbearing as may be seen
bone. In the early stages the patient may have mild from the clinical photograph. Because he could feel no
pain in the foot, this patient was able to walk on the foot
pain during walking and on examination there is local despite the severe damage. (Courtesy of Dr G. N.
swelling, warmth and tenderness. X-rays show the Malaviya.)
typical features of neuropathic bone necrosis and dis-
organization (Fig. 11.33)
Treatment consists of complete avoidance of all taken without careful consideration; amputation
weightbearing and movement, enforced bed rest and merely shifts the problem to a more proximal level
application of a total-contact cast that is renewed peri- where it will be even more difficult to manage because
odically until the soft-tissue swelling disappears (usu- the stump is often insensitive in these patients. More-
ally 8–12 weeks), and then for another 4 weeks. If the over, facilities for prostheses are scarce in many of the
foot is then found to be stable, a walking cast is areas where leprosy is endemic, and even where they
applied for a further 4–6 weeks, to be followed by the are available, hand deformities or poor vision in
use of an appropriate orthosis. If the foot is unstable, affected persons make their use difficult. The guiding
operative stabilization will be needed. principles are: amputate only if you must, amputate
conservatively and try to provide an end-bearing
SEPTIC TARSAL DISORGANIZATION stump where possible.
Infection may spread from a plantar ulcer to underly-
ing tarsal bones and joints and destroy these struc-
tures. Once the infection is controlled, the foot is
immobilized in a below-knee cast; the involved bones REFERENCES AND FURTHER READING
fuse together and a stable, rigid foot results. An
unstable foot will need surgical stabilization after Birch R. Brachial plexus injuries. J Bone Joint Surg, 1996;
clearing the infection. 78B: 986–92.
Birch R, Bonney G, Wyn Parry CB. Surgical Disorders of
the Peripheral Nerves. Churchill Livingstone, 1998.
Amputations Brand PW. Deformity in leprosy. Ch. XXI in Leprosy in
Occasionally amputation is necessary to keep the Theory and Practice, ed RG Cochrane, Bristol, John
300 patient ambulatory. However, this step should not be Wright, pp 265–319, 1959.
Brand PW. Deformity in leprosy. In Leprosy in Theory and Seddon HJ. A classification of nerve injuries. BMJ, 1942; 2: 11
Practice, Edn 2, eds RG Cochrane and TF Davy, Bristol, 237–239.
John Wright, pp 447–94, 1964. Sunderland S. Nerves and Nerve Injuries, 2nd ed. Edin-
Brand PW. Pressure sores – the problem. In Bed Sore Bio- burgh, Churchill Livingstone, 1978.
mechanics, ed Kenedi RM, Cowden JM & Scales JT, Lon- Srinivasan H. Disability, deformity and rehabilitation.
don, Macmillan, pp 19–23, 1976. Ch. 20 in Leprosy, 2nd edn. ed. Robert C Hastings,

Brandsma W, Schwarz R (eds). Surgical Reconstruction & Edinburgh, Churchill Livingstone, pp 411–448,
Rehabilitation in Leprosy and Other Neuropathies. Kath- 1994.
mandu (Nepal), Ekta Books, 2004. Srinivasan H. Atlas of Corrective Surgical Procedures Com-
Dong Li Wen. Microscopic surgical techniques in leprosy, monly Used in Leprosy. Published by the author, Chennai,
Published by Shanghai Skin Disease & STD Hospital, India, 2004.
Shanghai 2001, containing papers reprinted from Indian Srinivasan H, Desikan KV. Cauliflower growths in neuro-
J Lepr (1999) 71, pp 285–295, 297–309, 423–436, pathic plantar ulcers in leprosy patients. J Bone Joint Surg
437–450, vol. (2000) 72, pp 227–244, 431–436: and 1971; 53A: 123–32.
Lepr Rev (1992), 63, pp 141–144. Srinivasan H, Palande DD. Essential Surgery in Leprosy,
Landsmeer JMF. Functional considerations. Ch. 9 in Atlas Geneva, World Health Organization, 1997.
of Anatomy of the Hand. Edinburgh, Churchill Living- Srinivasan H, Mukherjee SM, Subramaniam RA. Two-
stone, pp 315–344, 1976. tailed transfer of tibialis posterior for correction of
Gravem PE. Role of flaps and skin grafts in the management of drop-foot in leprosy. J Bone Joint Surg 1968; 50B:
neuropathic plantar ulcers. Ch. 16 in Surgical Reconstruction 623–8.
& Rehabilitation in Leprosy and Other Neuropathies, Kath- Tsuge K, Hashizume C. Reconstruction of opposition in
mandu (Nepal), Ekta Books, pp 227–236, 2004. the paralyzed hand. Ch.23 in Surgical Rehabilitation in
McDowell F, Enna CD (eds.). Surgical Rehabilitation in Leprosy, ed. Frank McDowell and Carl D. Enna, Balti-
Leprosy, Baltimore, Williams Wilkins, 1974. more, Williams & Wilkins, pp 185–198, 1974.
Mulder JD, Landsmeer JMF. The mechanism of claw fin- Zancolli EA. Structural and Dynamic Basis of Hand Sur-
ger. J Bone Joint Surg 1968; 50B: 664–8. gery. 4th edn Philadelphia, JB Lippincott, 1979.
301
Orthopaedic
operations 12
Selvadurai Nayagam, David Warwick
The art and skill of orthopaedic surgery is directed ting cancellous bone), saws (for cutting cortical bone),
not simply to reshaping or constructing a particu- chisels (for shaping bone), gouges (for removing bone)
lar arrangement of parts but to restoring function and plates, screws and screwdrivers (for fixing bone).
to the whole. Many operations such as joint replacement, spinal
fusion and the various types of internal fixation
In this chapter principles applying to orthopaedic
require special implants and instruments to ensure
operations will be discussed and fundamental tech-
that the implants are correctly aligned and fixed. Sur-
niques of soft-tissue and bone repair will be described.
geons should familiarize themselves with the implants
For detailed descriptions of the various operative pro-
they plan to use, their advantages and disadvantages
cedures the reader is referred to standard textbooks
and the pitfalls encountered in their use. Most impor-
on operative orthopaedic surgery and monographs
tant of all, the surgeon is responsible for ensuring that
dealing with specific regional subjects.
the necessary instruments and implants (in appropri-
ate sizes!) are available in the operating theatre before
starting the operation; the explanation that a particu-
PREPARATION lar item ‘was not on the instrument table’ is no excuse
for failure.
PLANNING
Operations upon bone must be carefully planned in INTRAOPERATIVE RADIOGRAPHY
advance, when accurate measurements can be made
and bones can be compared for symmetry with those Intraoperative radiography is often helpful and some-
of the opposite limb. X-rays, magnetic resonance times essential for certain procedures. Fracture reduc-
imaging (MRI) and computed tomography (CT) (if tion, osteotomy alignments and the positioning of
necessary with three-dimensional re-formation) are implants and fixation devices can be checked before
helpful; transparent templates may be needed to help allowing the patient off the operating table. Angiog-
size and select the most appropriate implant. raphy may be needed to diagnose a vascular injury or
Corrective osteotomies and implant positioning can demonstrate the success of a vascular repair.
be simulated on x-ray or paper cut-outs before the X-ray cassettes must be wrapped in sterile drapes.
operation is undertaken. In today’s era of digital imag- Portable equipment must be positioned accurately and
ing, these methods have been superseded by image more time is lost while the plates are developed. How-
manipulation software, which allows measurement of ever, conventional x-ray films show excellent resolu-
angles and skeletal axes as well as ‘cutting’ and ‘rear- tion of bone architecture and provide a permanent
ranging’ of parts on digital files of x-ray images. Before record of the procedure. Image intensification and flu-
new or complex reconstructive operations are under- oroscopy are more efficient and, although fine features
taken they should, ideally, be rehearsed using artificial may not be seen in such detail, the resolution is usu-
bones and joints at a workbench. This is a facility that ally adequate. Some fluoroscopy machines are fitted
is now widely used in training programmes. with a printer, so that a permanent copy is available.
EQUIPMENT X-RAY GUIDANCE SYSTEMS

The minimum requirements for orthopaedic opera- By using a navigation system based on implanted
tions are drills (for boring holes), osteotomes (for cut- markers and intraoperative radiography with suitable
12
(a) (b) (c) (d)
12.1 Preoperative planning on digitized x-ray images The computer software allows the deformity to be analyzed
(a,b) and the correction simulated (c). The end result then mimics the simulation (d).
computer software, surgeons are able to improve their Total exposure varies with the type of procedure
accuracy and consistency in placing implants correctly. performed (operations on limb extremities produce
Examples are insertion of screws into vertebral pedi- the least, hip operations middling and spine opera-
cles and positioning of joint replacement components. tions the most) as well as the number of procedures
needing x-ray assistance and the protective measures
used. The latter influence the cumulative exposure
RADIATION EXPOSURE significantly and lead aprons are therefore compul-
sory; further attenuation of radiation exposure is
Intraoperative radiography involves the risk of expo- gained through the use of thyroid shields and, if prac-
sure to radiation; both the patient and surgeon are tical, eye goggles. Using a hip procedure as an exam-
affected. The dose limit for the general public is ple, lead aprons will reduce the effective dose received
1 mSv per year, which is the equivalent of 1000 chest by a factor of 16 for anteroposterior projections and
x-rays. Each chest x-ray in turn produces the same by a factor of 4–10 for lateral projections. Using a thy-
radiation dose as is endured during a 4-hour airline roid shield decreases the dose by another 2.5 times
flight. Fluoroscopic images acquired during opera- (Theocharopoulos et al., 2003).
tions are usually pulsed exposures rather than contin-
uous screening, so a few minutes of exposure to the
patient during a protracted operation would still MAGNIFICATION
amount to a negligible additional risk of developing
cancer. However, for the surgeon the risk is far greater Magnification is an integral part of peripheral nerve
304 because of the repeated use of fluoroscopy. and hand surgery. The improved view minimizes the
trauma of surgery and allows more accurate apposi- part of the limb. The ‘squeeze’ method, in which 12
tion of tissues during reconstruction. pressure on the palm or foot is followed by sequential
Operating loupes range in power from 2–6 × mag- squeezing of the limb in a proximal direction, is also
nification. As the magnification increases, the field of effective. If a clearer field is required then exsanguina-
view decreases and the interruption by unwanted tion can be achieved by pressure using an Esmarch or
head movements becomes more apparent. Most sur- gauze bandage wrapped from distal to proximal, or a
Orthopaedic operations
geons, therefore, choose between 2.5 and 3.5 × mag- rubber tubular exsanguinator. These methods reduce
nification. blood volume by an additional 20 per cent.
The operating microscope allows much greater
magnification with a stable field of view. It is particu-
larly important when very accurate apposition of tis- TOURNIQUET PRESSURE
sue is required, for example when aligning nerve
fascicles during nerve repair or nerve grafting, when A tourniquet pressure of 150 mmHg above systolic is
anastomosing small vessels or when operating in a recommended for the lower limb and 80–100 mmHg
narrow corridor of safety as in microdiscectomy of the above systolic for the upper limb. This may need to be
spine. increased in hypertensive, obese or very muscular
patients. Higher pressures are unnecessary and will
increase the risk of damage to underlying muscles and
nerves.
THE ‘BLOODLESS FIELD’
Tourniquet time
Many operations on limbs (and particularly the hand)
can be done more rapidly and accurately if bleeding is An absolute maximum tourniquet time of 3 hours is
prevented by the application of a tourniquet allowed, although it is safer (and more advisable) to
(Noordin et al., 2009). keep this under 2 hours; transient nerve-related
symptoms may occur with 3-hour tourniquet times
but full recovery is usual by the fifth day. Time can be
TOURNIQUET CUFF saved by ensuring that the limb is shaved, prepared,
draped and marked before inflating the cuff. The time
Only a pneumatic cuff should be used and it should of application of the tourniquet should be recorded
be at least as wide as the diameter of the limb. Wide and the surgeon should be informed of the elapsed
cuffs reduce the pressure needed for vascular occlu- time at regular intervals, particularly as the 2-hour
sion. A tied rubber bandage is a potentially dangerous period is approached.
substitute and should not be used; the pressure
beneath the bandage cannot be controlled and there Deflating and re-inflating the
is a real risk of damage to the underlying nerves and
muscle. A layer of wool bandage beneath the pneu- tourniquet
matic tourniquet will distribute the pressure and pre- This has serious local and systemic effects. Locally
vent wrinkling of the underlying skin. During skin deflation is followed by a hyperaemic response that
preparation, it is essential that the sterilizing fluid does reduces by half in 5, 12 and 25 minutes, respectively
not leak beneath the cuff as this can cause a chemical after ischaemic times of 1, 2 and 3 hours (Klenerman
burn. Isolating the tourniquet with a plastic drape et al., 1982). This information is useful to the surgeon
prevents this complication. trying to obtain haemostasis after tourniquet release.
There is also a variable amount of swelling, unrelated
to the length of the ischaemic period; it would there-
EXSANGUINATION fore be wise to omit tourniquet use for those limbs
where significant swelling is already evident so as not
Elevation of the lower limb at 60 degrees for 30 sec- to jeopardize wound healing. At the systemic level,
onds will reduce the blood volume by 45 per cent; tourniquet deflation induces a free radical-mediated
increasing the elevation time does not alter the per- reperfusion syndrome, which adds to any muscle
centage significantly. The same pattern is observed in damage already produced by the ischaemic period.
the upper limb (Blond et al., 2002; Blond and Mad- ‘Breathing periods’ (deflation followed after a pause
sen, 2002). This simple manoeuvre will therefore suf- by re-inflation), which were once popular to enable
fice to ‘drain’ the tissues if a truly bloodless field is not extended tourniquet times, are no longer recom-
essential, or when surgery is being undertaken for mended as the reperfusion effects are cumulative even
tumour or infection and forceful exsanguination though the local limb anoxia is relieved at each
might squeeze pathological tissue into the proximal tourniquet deflation (Bushell et al., 2002). If a 305
12 prolonged tourniquet time is required, and if this is where the limb has been wrapped in a cast or splint for
anticipated due to the complexity of surgery to be some time. Skin preparation prior to surgery should
undertaken, it is wise to warn the patient of the pos- be carried out with an alcohol-based preparation
sibility of transient nerve-related symptoms and to where safe; alcohol is not to be applied over open
obtain their consent to use the absolute maximum wounds, exposed joints or nerve tissue. Iodine or
period of 3 hours. chlorhexidine preparations are available but there is
evidence that chlorhexidine is more effective after a

single application, having longer residual activity and
Finger tourniquet
maintaining efficacy in the presence of blood and
This is suitable for relatively minor hand operations. A serum (Milstone et al., 2008). The use of colouring in
sterile rubber glove-finger makes a good cuff; the tip the preparation fluid will help to ensure that the limb
is cut and the margin is then rolled back proximally. is fully covered. However, use of red colouring should
This has the combined effect of exsanguinating the be avoided if a tourniquet is used since it may make it
finger and acting as a tourniquet. A stretched rubber difficult to determine whether blood flow has
catheter must not be used as this may damage the returned after releasing the tourniquet.
underlying structures. Always check that the finger
tourniquet has been removed at the end of the operation.
Drapes
These function to isolate the surgical field from the
Complications rest of the patient and reduce contamination from
Complications of tourniquet usage usually relate to outside. There are disposable and re-usable varieties
nerve injury (more often due to compression than and, as yet, none has been shown to be superior as
duration of ischaemia), skin burns from leakage of long as they have the following qualities: (1) barrier
alcoholic antiseptic solutions beneath the tourniquet effectiveness throughout the length of the procedure;
cuff and a failure to diagnose peripheral vascular dis- (2) quality that is maintained if it is re-used (for re-
ease before surgery (Klenerman, 2003). These usable varieties); (3) configurability to cover different
mishaps can be prevented, or the risk minimized, by areas of body or limb; (4) tear-resistance and does not
always using a wide cuff, sealing the cuff against seep- lint; (5) no cause for skin reactions through allergy or
ing fluids and avoiding excessive tourniquet pressures. abrasiveness; (6) reasonable cost (Rutala and Weber,
A wise precaution is to not employ a bloodless field at 2001). Plastic adhesive coverings, some impregnated
all in patients with impaired peripheral circulation or with iodine, function primarily to secure the drapes,
those with arterial prostheses or stents that may not especially if the limb is moved during surgery. This
expand sufficiently after tourniquet deflation to re- method of skin isolation was thought to reduce
establish an adequate blood flow. wound contact with some of the resident bacteria
around the skin incision; however there is no evidence
that they reduce surgical site infections and they may
even increase them! (Webster and Alghamdi 2007).
MEASURES TO REDUCE RISK OF
INFECTION
SURGICAL ATTIRE
SKIN PREPARATION AND DRAPING
Gowns
Hair removal Gowns need to share the requisite qualities of drapes
Shaving the limb is more likely to be harmful than but should also be comfortable.
helpful. Shaving before surgery causes superficial skin
damage and leads to local bacterial proliferation.
Depilatory creams have been shown to remove hair
Gloves
effectively and, if hair removal is deemed necessary, Gloves are available in latex and non-latex varieties.
they can be used the day before surgery without an The latter are needed if either the surgeon or the
increase in wound problems. patient has a latex hypersensitivity. This could apply to
patients who are constantly exposed to latex devices,
e.g. urinary catheters. Latex allergy is second only to
Skin cleaning muscle relaxants for inducing anaphylaxis during sur-
The limb may benefit from washing with soap to gery (Lieberman, 2002). Double gloving, with a
remove particulate matter and grease. This is particu- coloured inner glove (so-called indicator glove)
306 larly useful in managing open fractures and in cases reduces the number of inner glove perforations and
allows outer glove perforations to be picked up more Human immunodeficiency virus 12
quickly, but a difference in surgical site infections has
yet to be established (Tanner and Parkinson, 2006). The risk of human immunodeficiency virus (HIV) in-
fection after accidental injury is very low (less than 0.5
per cent) (Ippolito et al., 1999), although this may vary
Face mask between individuals. Vaccination is not available but
This hallmark of the surgeon in theatre has been ques- post-exposure treatment with antivirals is essential.
tioned in its ability to reduce surgical site infections.
As studies provide conflicting views (Lipp and
Edwards, 2002), for the time being at least, face
masks should continue to be used if only for protec- THROMBOPROPHYLAXIS
tion of the surgical staff. Modern face masks incorpo-
rate visors (eye shields), which substantially reduce the Venous thromboembolism (VTE) is the commonest
risk of contact with blood. complication of lower limb surgery. It comprises three
associated disorders: deep vein thrombosis (DVT), pul-
monary embolism (PE) and the later complication of
VACCINATION chronic venous insufficiency. Approximately one in
30–40 patients operated on for hip fractures or hip
There is a risk of transmission of blood-borne infec- and knee replacements will develop a symptomatic
tions to orthopaedic surgeons, not least because of thromboembolic complication despite the use of
the nature of surgery but also due to frequent han- prophylaxis during their hospital stay. The most
dling of instruments and bone fragments with sharp important risk factors are increasing age, obesity and
edges. Transfer of infectious agents through blood a history of previous thrombosis.
occurs mainly by contact (percutaneous or mucocuta-
neous) and through aerosols (Wong and Leung,
2004). The face and neck may become contaminated PATHOPHYSIOLOGY
and this may go unnoticed until after the procedure;
splashes and aerosol sprays often happen during the According to Virchow, thrombosis results from an in-
use of power tools and irrigation fluids (Quebbeman teraction between vessel wall damage, alterations in
et al, 1991). Exposure is more likely if the operation blood components and venous stasis. All of these occur
continues for over 3 hours or when blood loss is in major orthopaedic operations. The surgery is highly
greater than 300 mL (Gerberding et al., 1990). A bar- thrombogenic. Soft-tissue exposure, bone cutting and
rier created by surgeon attire must be coupled to the reaming induce a systemic hypercoagulable state and
correct etiquette for handling and passing instruments fibrinolytic inhibition. Blood flow in the femoral vein
between staff. This reduces the likelihood of acciden- is obstructed by the torsion needed to expose the
tal needle-stick injury but will need augmenting by femoral canal and the acetabulum in hip replacements;
prophylaxis through vaccination. this damages the endothelium, both in the proximal
femoral vein (by torsion) and in the distal veins (by dis-
tension). Furthermore, venous obstruction allows a
Hepatitis B concentration of clotting factors. In knee replacement,
Transmission may occur through inoculation or even the anterior subluxation of the tibia and vibration from
from contact with a contaminated surface (the virus is the saw may cause local endothelial damage. In addi-
able to survive for a week in dried blood). There is a tion, the relative immobility that follows lower limb
30 per cent risk of transmission from a single inocula- operations induces some degree of venous stasis.
tion of an unvaccinated person (Alter et al., 1976). DVT occurs most frequently in the veins of the calf
Vaccination is safe, effective and immunity, for those and less often in the proximal veins of the thigh and
who respond after a course of injections, indefinite. pelvis. It is from the larger and more proximal
Those who do not respond to immunization will need thrombi that fragments sometimes get carried to the
post-exposure prophylaxis using a combination of lungs, where they may give rise to symptomatic pul-
hepatitis B immunoglobulin and the vaccine. monary embolism (PE) and, in a small percentage of
cases, fatal pulmonary embolism (FPE).
Hepatitis C
The risk of accidental transmission is lower than for CLINICAL FEATURES AND DIAGNOSIS
hepatitis-B (less than 7 per cent). Unfortunately
neither effective vaccines nor post-exposure protec- Thromboembolic events can be represented as a
tion is available. pyramid; most of these events are asymptomatic but a 307
12 Fatal PE
Symptomatic PE
on passive dorsiflexion of the foot), although still fre-
quently employed, is now regarded as unreliable.
Symptomatic DVT
Pulmonary embolism (PE)
Asymptomatic DVT Patients may develop pleuritic pain in the chest and
shortness of breath, but other conditions, such as

myocardial infarction or fulminant pneumonia can be
mistaken for PE. In most cases PE is completely
asymptomatic and fatal PE usually presents as a sud-
den collapse without prior symptoms in the legs or
No DVT
chest; in such cases the diagnosis is confirmed by post-
mortem examination.
Imaging studies help to confirm the diagnosis in
patients who have a moderate or high clinical proba-
bility of thromboembolism. Ultrasound or venogra-
phy is important for demonstrating DVT and
12.2 The thromboembolism pyramid
computer tomographic pulmonary angiography or
ventilation-perfusion (VQ) scans are helpful in the
proportion is revealed clinically (Fig. 12.2). Hence diagnosis of PE.
DVT is, in the main, an occult disease and consider-
ably more common than the symptoms and signs sug- Post-thrombotic syndrome
gest.
Post-thrombotic syndrome (PTS) presents with leg
discomfort, swelling, skin changes and even ulcera-
Deep venous thrombosis
tion. This is a debilitating condition that directly
DVT is usually asymptomatic, although some patients influences quality of life (Kahn et al., 2008). Approx-
present with pain in the calf or thigh. A sudden slight imately one-third of patients with symptomatic DVT
increase in temperature and pulse rate may develop. will develop features of PTS within 2 years but it is
There are usually no signs but there may be calf not yet established whether the much more frequent
swelling and tenderness. Homans’ test (increased pain asymptomatic DVT after joint replacement predis-
(a)
(b) (c) (d)
12.3 Venous thromboembolism (a) Venous thrombosis – embolism from the deep veins of the leg, extracted from the
lung at post mortem. (b) Fatal pulmonary embolism at post mortem (c) chronic venous insufficiency
308 (d) acute thrombophlebitis.
Table 12.1 Risk of venous thromboembolism (VTE) 12
Procedure or condition Fatal PE Symptomatic VTE Asymptomatic DVT
Hip fracture 1 per cent 4 per cent 60 per cent

Hip replacement 0.2–0.4 per cent 3–4 per cent 55 per cent
Knee replacement 0.2 per cent 3–4 per cent 60 per cent
Isolated lower limb trauma Unknown 0.4–2 per cent 10–35 per cent
Spinal surgery Unknown 6 per cent 18 per cent
Knee arthroscopy Unknown 0.2 per cent 7 per cent
Major trauma Unknown Unknown 58 per cent
Spinal cord injury Unknown 13 per cent 35 per cent
Upper limb surgery Unknown Vv rare Vv rare
Minor lower limb surgery Vv rare Vv rare Vv rare
DVT, deep vein thrombosis; PE, pulmonary embolism; VTE, venous thromboembolism. Derived from the International Consensus
Statement (Nicolaides et al., 2006) and ACCP Guidelines (Geerts et al., 2008).
poses to this long-term outcome (Pesavento et al., which can be simplified by including an active
2006). reminder or checklist prior to surgery. This ensures
that safe, effective prophylaxis is routinely given
according to a protocol that has been accepted by the
Chronic pulmonary hypertension
surgeons and anaesthetists (Tooher et al., 2005;
This is a potential sequel for those who survive a Warwick et al, 2008).
symptomatic PE but the incidence is unknown.
General measures
INCIDENCE OF THROMBOEMBOLIC • Neuraxial anaesthesia – Spinal or epidural anaes-
thesia reduces mortality, enhances peri-operative
EVENTS analgesia and reduces the risk of VTE by about 50
It is generally accepted that the risk of symptomatic per cent through enhancing blood flow. It is wise to
thromboembolism and fatal PE is less now than it was avoid giving neuraxial anaesthesia and chemical
20 or 30 years ago, due to more efficient surgery and prophylaxis too close together to avoid a spinal
anaesthesia as well as earlier mobilization and the haematoma. Local guidelines should be followed.
widespread use of prophylaxis. • Surgical technique – Rough surgical technique will
Much of the information used to calculate risk potentiate thromboplastin release. Prolonged tor-
reduction with prophylaxis is derived from studies sion of a major vein, when maintaining a dislocated
using a venographic surrogate. Venograms are sensi- hip for purposes of replacement or during aggres-
tive and specific in identifying venous thrombi but the sive dorsal retraction of the tibia during knee
relationship between ‘venographic DVT’ and sympto- replacement, inhibits venous return and damages
matic events has not been fully defined. Many asymp- the endothelium.
tomatic venographic thrombi resolve without • Tourniquet – A tourniquet probably does not
untoward effects. However, there is reasonable evi- change the risk; clotting factors that accumulate
dence to show that a reduction in venographic DVT whilst the tourniquet is inflated are flushed out by
would produce a proportionate reduction in sympto- the hyperaemia on tourniquet deflation.
matic DVT or even fatal PE, thus shrinking the pyra- • Early mobilization – This is a simple physiological
mid of risk (Table 12.1). means of improving venous flow.
Physical methods
PREVENTION
• Graduated compression stockings can halve the inci-
The overall risk of DVT and PE can be reduced by dence of DVT when compared to no prophylaxis;
prophylaxis. Patients admitted for surgery, whether there is a suggestion that below-knee stockings may
electively or in emergency, need a risk assessment, be just as effective as above-knee types, as long as 309
12 the stockings are properly woven and well-fitted ready bio-availability and a wide window of safety;
(Phillips et al., 2008). therefore monitoring is not required. They are safe
• Intermittent plantar venous compression takes if used properly (with an adequate time between
advantage of the fact that blood from the sole of the administration and surgery or regional anaesthesia,
foot is normally expressed during weightbearing by and a reduced dose for those with impaired renal
intermittent pressure on the venous plexus around function). They are more effective than placebo or
the lateral plantar arteries; this, in turn, increases unfractionated heparin and at least as effective as
venous blood flow in the leg. A mechanical foot- warfarin, compression devices and foot pumps.
pump can reproduce this physiological mechanism Randomized studies have shown that it effectively
in patients who are confined to bed. It should not reduces the prevalence of venographic DVT in hip
be used in combination with compression stockings and knee replacement surgery, and the effect may
as these impair refill of the venous plexus after emp- be amplified when coupled to physical methods.
tying by the foot pump. There is some evidence • Pentasaccharide – This synthetic injectable anti-
that this technique provides effective thrombopro- thrombotic drug (fondaparinux) precisely inhibits
phylaxis in hip fracture, hip arthroplasy and knee activated Factor X. It is at least as effective as
arthroplasty, especially if combined with a chemical LMWH but must not be given too close to surgery
method (Pellegrini et al., 2008). (it is best given 6–8 hours after surgery) or bleed-
• Intermittent pneumatic compression of the leg has ing may become a significant problem. The drug is
also been shown to reduce the risk of ‘radiological excreted by the kidneys rather than metabolized by
DVT’ after hip replacements and in trauma. It is, the liver and so must be used carefully or avoided in
however, impractical for patients undergoing oper- those with poor renal function.
ations at or below the knee. • Direct anti-Xa inhibitors and direct thrombin
• Inferior vena cava filters resemble an umbrella and inhibitors – These drugs are likely to transform
are percutaneously passed through the femoral vein thromboprophylaxis. They are given orally and
and lodged in the inferior vena cava. They merely have a broad therapeutic and safety window (so that
catch an embolus to prevent it from reaching the no monitoring is required). They are given after
lungs. They have a specific role in the occasional case surgery and should be continued for as long as the
where the risk of embolism is high yet anticoagula- patient is at risk of VTE. There is good evidence of
tion is contra-indicated, e.g. in a patient with a pelvic equivalence of efficacy with LMWH in hip and knee
fracture who has already developed a DVT but needs replacement surgery. They provide a pragmatic
a major surgical reconstruction. The complication solution for after-hospital prophylaxis, requiring
rate, which includes death from proximal coagula- neither injections nor complex monitoring. Drug
tion, should restrict use of these devices. activity is difficult to reverse. Presently, two are
available: a direct thrombin inhibitor (dabigatran)
and an anti-Xa inhibitor (rivaroxaban).
Chemical methods • Warfarin – Warfarin has been used fairly widely,
particularly in North America. It reduces the preva-
These are generally safe, effective, easy to administer
lence of DVT after hip and knee replacement and
(tablet or injection) and can be used for extended
FPE is extremely rare. Drawbacks are the difficulty
periods. They are relatively inexpensive compared
in establishing appropriate dosage levels and the
with the overall cost of surgery. However, all chemical
need for constant monitoring. If it is used at all it
methods incur a risk of bleeding, which is a natural
must be maintained at an international normalized
concern for both the orthopaedic surgeon and the
ratio (INR) level of 2–3.
anaesthetist. Methods include:
• Aspirin – Whilst some authorities still recommend
the use of aspirin, others (NICE in the United
Timing and duration of prophylaxis
Kingdom, the American College of Chest Physi- Risk factors for thromboembolism are most pro-
cians, The International Consensus Statement) nounced during surgery but, in some patients (partic-
advise against its use because of its relatively poor ularly those with hip or major long-bone fractures of
efficacy, the risk of bleeding and the tendency to the lower limb), immobility and a hypercoagulable
cause gastrointestinal irritation. state may begin before the operation. In general pro-
• Unfractionated heparin – This carries a risk of phylaxis it is given on admission to hospital in this
increased bleeding after operation and is contraindi- group, particularly if surgery is delayed beyond 24
cated in elderly people. hours. Chemical prophylaxis should not be given too
• Low molecular weight heparin (LMWH) – This class close to surgery otherwise there is a risk of provoking
of drug has haematological and pharmacokinetic a bleeding complication. If it is given too long before
310 advantages over unfractionated heparin including surgery, metabolism or excretion may reduce its
potency; if given too long after surgery, the thrombo- deformity analysis and osteotomy are well described in 12
genic process will be established and the drug is now the monograph by Paley (2002).
therapeutic instead of prophylactic. Knowledge of the limb axes and their relation to
The ideal duration of thromboprophylaxis is not the joints is the foundation for analyzing skeletal
known (Warwick et al., 2007). Traditional recommen- deformity. ‘Corrective’ surgery is an exercise in bal-
dations suggesting that it should be continued until the ancing the extent of operative interventions needed to
patient is fully mobile have been superseded by evi- produce anatomical ‘normality’ with the anticipated
dence that the cumulative risk for VTE lasts for up to gain in function. ‘Anatomical’ correction, whilst
1 month after knee replacement surgery and 3 months desirable in most cases, is not always necessary. An
with hip surgery (Bjornara et al., 2006). Half of the appropriate example is a skeletal deformity due to a
VTE events after knee replacement and two-thirds after neuromuscular disorder where correction to achieve
hip replacement occur beyond hospital discharge. The maximal functional gain has to be greater than that
duration of risk in other orthopaedic conditions is not for anatomical accuracy.
known. Therefore, thromboprophylaxis should be pro- Modern deformity analysis recognizes the three-
longed for some time after discharge from hospital. dimensional basis of most deformities, whether the
Randomized clinical trials have shown that the risk origin of the problem is within a bone or a joint or a
of after-discharge symptomatic DVT can be reduced combination of both. Deformity of bone exists as a
by two-thirds by prolonging thromboprophylaxis. deviation in the coronal or sagittal plane (or any plane
The precise period depends on many factors, includ- in between) where it can be measured in degrees of
ing individual patient factors, which are difficult to angulation or millimetres of translation, or in the axial
quantify, but current evidence supports 14 days for plane, where it exists as degrees of rotation or milli-
knee replacement and 4–5 weeks for hip replacement metres of length abnormality. The lower limb is used
and hip fracture. Whilst many of the chemical meth- to illustrate the principles as applied to the coronal
ods may be appropriate, oral agents that do not plane.
require monitoring (e.g. anti-Xa and anti-thrombin
inhibitors) facilitate effective and practical extended
duration prophylaxis (NICE, 2010).
LIMB AXES AND REFERENCE ANGLES
Multimodal prophylaxis The mechanical axis of a limb is defined by an imagi-
nary line connecting the centre of the most proximal
Risk assessment of patients may determine that a com-
major joint to the centre of the most distal, e.g. in the
bination of physical and chemical prophylaxis is
lower limb from the centre of the hip to the centre of
needed. This form of multimodal prophylaxis is gain-
the ankle. In most individuals this line passes close to
ing popularity and some studies point to increased
the centre of the knee joint, usually 8(±7) mm medial
efficacy. For patients at particularly high risk of bleed-
to it. If a deformity is present the line may be dis-
ing, the mechanical method should be used until the
placed away from its usual position (Fig. 12.4a).
bleeding risk has resolved and until the device is no
Interestingly, if a deformity should exist at two or
longer tolerated. It is then safely replaced by a chem-
more levels in the limb, the resulting displacements
ical product, which is continued for as long as there is
may cancel each other out, so that the limb axis ends
a risk of thrombosis. For patients with a particularly
up in the ‘normal’ position (Fig. 12.4b). It follows the
high risk of thrombosis, the mechanical device is
observed position of the mechanical axis of the lower
started immediately after surgery and continued for as
limb in relation to the knee joint is a ‘screening’
long as tolerated; the chemical is started as close to
assessment and does not rule out the presence of
surgery as is safe (e.g. 6 hours postoperatively) and
deformity. A further step would be to compare refer-
continued for as long as the risk of VTE persists.
ence angles subtended by the mechanical axes of the
individual bone segments to joints. It is usual to com-
pare these angles with those of the contralateral ‘nor-
mal’ side but in the event the other is also affected
OPERATIONS ON BONES some reference ranges are available (Fig. 12.5):
1. At the hip – the angle between the anatomical axis
OSTEOTOMY of the femur and the axis of the femoral neck is
approximately 128 degrees (±3 degrees).
Osteotomy may be used to correct deformity, to 2. At the knee – the angle between the anatomical
change the shape of the bone, or to redirect load axis of the femur and a tangent to the joint line of
trajectories in a limb so as to influence joint function. the knee is, on the lateral aspect, approximately
Preoperative planning is essential; principles of 80 degrees (±2 degrees). 311
12
(a) (b)
12.4 (a) Deformity in the lower limb It may be

sufficient to alter the mechanical axis of the limb – here it
is shifted laterally due to changes at the hip joint. (b) If 12.5 Analysis of coronal plane deformity This can be
there are two deformities, the mechanical axis may be based on a contralateral normal limb or use of reference
normal if the effect of each is to shift the axis in equal and angles in relation to the anatomical (or mechanical) axes
opposite directions – a compensated deformity. (Paley, 2002).
3. At the knee – the angle between the anatomical If an abnormal value is encountered, it suggests a
axis of the tibia and a tangent to the joint line of deformity is present within that bone. However, when
the knee is, on the medial aspect, approximately drawing out these reference angles and seeking to
87 degrees (±2 degrees). identify a source of deformity, it is easy to be carried
4. At the ankle – the angle between the anatomical away by abnormal values that differ from the reference
axis of the tibia and a tangent to the tibial plafond ranges by a few degrees. The clinical significance of
is, on the lateral aspect, approximately 89 degrees these ‘abnormalities’ must be taken in context; an
312 (±2 degrees). intrinsic (naturally present) varus angulation of a few
degrees at the distal femur matters little if the main the rotation axis further along the bisector 12
source of deformity is a larger varus malunion of a tib- increases or decreases the size of the opening, i.e.
ial fracture further distally – in which case the correc- achieves simultaneous lengthening or shortening
tion should be in the tibia. with the angular correction (Fig. 12.7b and c). If
the rotation axis is not placed on the bisector, a
translation deformity will ensue despite satisfactory
RULES FOR OSTEOTOMY correction of angulation.
2. It reveals the presence of translation as well as
Most surgeons are familiar with the simple method of
angulation as components of the deformity and
drawing the anatomical axes of the bone segments
can also indicate the presence of multi-apical
proximal and distal to a deformity and measuring the
deformities.
size of the deformity (in degrees) at the intersection
(a) When the CORA is identified and is found to
of these axes. In modern deformity analysis this inter-
lie within the boundaries of the bone involved
section of anatomical axes is referred to as a centre of
as well as coinciding in level with the apex of
rotation of angulation (CORA) and can also be deter-
the deformity, this indicates only an angular
mined by noting the intersection of the mechanical
component to the deformity. The rotation
axes of the segments proximal and distal to the defor-
axis to correct the deformity can be sited on
mity (Fig. 12.6). The CORA is important for the fol-
the bisector and the osteotomy performed at
lowing reasons:
the same level – this is equivalent to classic
1. It indicates where an axis of rotation, named correction through opening or closing wedge
angulation correction axis or ACA (Paley, 2002), methods (Fig. 12.7b).
should be placed about which the two intersecting (b) When the CORA lies within the boundaries of
axes of the CORA can be brought in line and the bone involved but is at a different level to
hence the deformity corrected. This axis of that of the apex of deformity, it indicates the
rotation, which enables appropriate realignment of presence of translation and angulation within
the intersecting axes, should be positioned on the deformity (Fig. 12.8a). The rotation axis
either side of the CORA but along a line termed to enable correction should be maintained on
‘the bisector’. This is, as is implied in its name, the the bisector of the CORA but the osteotomy
line that bisects the angle described by the can be sited at either of the two levels
deformity (Fig. 12.7a). The effect of placing the (coincident with the apex of deformity or at
axis of rotation on the convex side of the the CORA): (1) when positioned on the
deformity is to envisage an opening wedge former, correction of both translation and
correction, and conversely if it is placed on the angulation is simultaneously accomplished at
concave side – a closing wedge correction. Moving the site of original deformity (Fig. 12.8b); (2)
12.6 Location of the

CORA It is found at the
intersection of the
anatomical (or
mechanical) axes of the
segments proximal and
distal to the deformity.
The bisector is the line
that divides the
supplement to the angle
of deformity. Whilst the
apex of angulation and
CORA coincide in this
example, that is not
always so.
(a) (b) (c)
12.7 Axis of rotation It is placed along the bisector of

the CORA on the convex side. This achieves an open
wedge correction (a,b). If the rotation axis is moved further
along the bisector, lengthening – in addition to the open
wedge realignment – is obtained (c). 313
12 Undercorrection and overcorrection Under- and over-
correction of the deformity can be avoided by careful
preoperative planning. In difficult cases, intra-operative
x-ray or fluoroscopy is essential. If the fault is
recognized while the patient is still under the
anaesthetic, it should be corrected straightaway. If
discovered on a postoperative x-ray check, the impact

of the mistake will need to be gauged and, if
significant, it may still be advisable to re-do the
procedure.
Nerve tension Correction of severe deformities may put
excessive tension on a nearby nerve. The commonest
example is peroneal nerve palsy after corrective
osteotomy for a marked valgus deformity of the knee. In
(a) (b) (c)
general, acute long-bone corrections greater than 20
12.8 If the CORA is found to be proximal or distal to the degrees should be avoided and if there is a known risk of
apex of angulation, but within the boundaries of the bone, nerve injury it should be limited to 10 degrees. If greater
this suggests the presence of translation as an additional
correction is needed it can be done gradually in an
component to the deformity (a). Simultaneous correction
can be achieved by placing the axis of rotation on the appropriate external fixator (see on page 319, under the
CORA or its bisector; the osteotomy can be either at the Ilizarov method).
apex of angulation (b) or at the same level as the CORA
(c). Compartment syndrome Osteotomy of the tibia or
forearm bones is at risk of this rare but potentially
limb-threatening complication. The limb should be
checked repeatedly for signs and prompt action taken
if danger signals appear (see Chapter 23).
when sited on the latter, a new deformity is
created which correctly ‘balances’ the Non-union Non-union may occur if fixation is
malalignment produced from the original site inadequate or if the soft tissues are damaged by
(Fig. 12.8c). excessive stripping during surgical exposure. Gentle
(c) When the CORA lies outside the boundaries handling of tissues and respect for the blood supply to
of the involved bone, a multi-apical deformity bone together with sound fixation techniques will
is likely to be present (and the deformity minimize the risk.
more akin to a curve). The deformity would
need to be resolved through multiple
osteotomies.
These features of the CORA are, in essence, the BONE FIXATION
rules of osteotomy as described by Paley (2002). It
explains why it is permissible to perform osteotomies Stabilizing two segments or fragments of bone is usu-
away from the apex of the deformity as long as the ally by internal or external fixation methods. In inter-
correction is achieved through a rotation axis placed nal fixation, this may involve screws, wires, plates or
on the CORA or on its bisector. Many examples in intramedullary rods. External fixators come in a vari-
orthopaedics illustrate this principle, e.g. performing ety of types. There are basic rules for choosing and
an intertrochanteric or subtrochanteric osteotomy to using either method.
correct malalignment of the femoral neck in a child
with a slipped capital femoral epiphysis, or inducing
translation in correcting a genu valgum arising from INTERNAL FIXATION BY SCREWS
the femoral joint line – both of which are examples of
the 2(b) scenario above. Screws can be used simply by holding two fragments
in close proximity or to fix a plate to the bone. They
may also be used to compress two fragments together
COMPLICATIONS OF OSTEOTOMY AND through what is called the ‘lag principle’. By over-
drilling the near fragment, the threads of the screw
DEFORMITY CORRECTION only engage the far fragment and, when the screw is
tightened, it draws the two parts together in com-
General As with all bone operations, thrombo- pression. The lag screw works best if passed at right
314 embolism and infection are calculated risks. angles to the plane between the bone fragments. If
there is a long fracture line, several screws can be INTERNAL FIXATION BY PLATES AND 12
inserted at different levels with each screw at right
angles to the fracture plane at their respective sites. A SCREWS
similar lag effect is achieved if the screw is threaded
Plates of varying design may be incorporated: (1) sim-
only near its tip – a partially threaded screw.
ple straight compression plates, which will allow com-
The pull-out strength of a screw fixed in bone
pression along the axis of the plate; (2) contoured
depends on factors involving both the screw and the
plates to fit specific bones; (3) low-profile plates that
bone: it increases (1) with the size of screw and the
reduce the ‘footprint’ on the bone so as to preserve
length of screw embedded; (2) with the thickness and
local vascularity; (4) locked plates where the screw also
density of the bone in which it is embedded; (3) if
engages the plate by a secure mechanism so as to cre-
both cortices are engaged by the screw.
ate a stable construct and prevent toggling.
Most screws are inserted after drilling a pilot-hole
The plate may be applied subperiosteally by a for-
and tapping, although self-drilling and self-tapping
mal exposure of the fracture or osteotomy, or extrape-
varieties are available. In cancellous bone, and partic-
riosteally (in the submuscular plane) so as to span the
ularly if it is osteoporotic, it may be preferable not the
site. These are internal splints that should not be used
tap after pre-drilling; tapping removes additional bone
as loadbearing devices. The ability to control loads
that would help anchor the screw.
across the bone will depend on the degree of contact
12.9 Lag screw fixation This is

accomplished through design of the
screw (being unthreaded for part of the
shank) or through overdrilling the near
fragment (a,b). Lag screws are thus
used individually or in conjunction with
a plate (c,d). Plates can be applied to
control twisting forces (here they are
used in conjunction with lag screws) or
simply as long internal splints, as in
indirect submuscular plating of fractures
(e).
(a) (b)
(c) (d) (e) 315

12 between the bone ends; it is important that this anatomically contoured; therefore intramedullary
should be accomplished, usually by compression reaming to a diameter greater than the nail to be used
across the bone ends by a lag screw or through the allows unimpeded insertion of the device. Insufficient
plate itself (Fig. 12.9). reaming potentially risks the bone splitting during nail
In addition to improving contact between the bone insertion as a result of hoop stresses (expansile forces)
ends, compression through the plate can be utilized as generated.
part of the tension-band concept. Curved long bones Unlocked intramedullary nails are increasingly used
have a compression side and tension side when axially in the treatment of long-bone shaft fractures in chil-
loaded; plate application on the tension side will con- dren. These flexible rods are inserted so as not to
vert the loading forces that attempt to separate the damage the physes at either end of the long bone and
fracture ends into compressive ones and thereby function as internal splints until callus formation takes
maintain bone contact. over (Fig. 12.10).
INTERNAL FIXATION BY EXTERNAL FIXATION

INTRAMEDULLARY DEVICES External fixators are useful for open fractures and for
reconstruction of limbs using the Ilizarov method.
Two major design types are used: those with and They can also be used as temporary fracture stabiliza-
those without interlocking capabilities. Interlocking tion devices when the local soft tissue conditions need
nails have become a standard fixation method for improving before open surgery, or during emergency
most shaft fractures of the tibia and femur in adults. fixation of multiple long-bone fractures (Fig. 12.11).
Stability from these nails is due to a combination of an The fixator functions as an exoskeleton through
interference (frictional) fit within the medullary canal which the patient’s own skeleton can be supported
and the capture of bone to nail by means of the inter- and adjusted. The basic components are wires or pins
locking screws. Interlocked intramedullary nails offer inserted into bone to which rods or rings are attached
better control of length and torsion than the unlocked and interconnected. Pin- or wire-related problems
varieties of this device. Older nail designs had an open have limited widespread adoption of this method;
cross-section but these are being replaced by closed newer pin designs, and some with hydroxyapatite
section devices, which provide greater torsional stiff- coating have reduced the frequency of problems. The
ness. mechanics of pin-hold in bone is governed by similar
The medullary canals of the femur and tibia are not factors to that of screws.
simple curves and there are variations between indi- External fixators are mainly of the unilateral-planar
viduals. None of the present-day nail designs are or circular types; there are also designs that combine
(a) (b) (c) (d)

12.10 Intramedullary nails These are excellent for stabilizing shaft fractures of the major long bones: (a) femur; (b) tibia.
Locked nails have the added benefit of controlling length and torsion. Flexible and elastic nails work by three-point fixation
316 and are suitable for paediatric fractures where damage to the physis can be avoided (c, d).
osteoprogenitor cells; (2) they provide linkage across 12
defects and a scaffold upon which new bone can
form. Osteogenesis is brought about partly by the
activity of cells surviving on the surface of the graft
but mainly by the action of osteoprogenitor cells in
the host bed.
Three basic requirements for osteogenesis are the
presence of osteoprogenitor cells, a bone matrix and
growth factors.
(a)
AUTOGRAFTS (AUTOGENOUS GRAFTS)
Bone is transferred from one site to another in the
same individual. These are the most commonly used
grafts and are satisfactory provided that sufficient
bone of the sort required is available and that, at the
recipient site, there is a clean vascular bed.
Cancellous autografts
Cancellous bone can be obtained from the thicker
(b)
portions of the ilium, greater trochanter, proximal
metaphysis of the tibia, lower radius, olecranon, or
from an excised femoral head. Cortical autografts can
be harvested from any convenient long bone or from
the iliac crest; they usually need to be fixed with
screws, sometimes reinforced by a plate and can be
placed on the host bone, or inlaid, or slid along the
long axis of the bone. Cancellous grafts are more rap-
idly incorporated into host bone than cortical grafts,
but sometimes the greater strength of cortical bone is
needed to provide structural integrity.
The autografts undergo necrosis, though a few sur-
face cells remain viable. The graft stimulates an
(c) inflammatory response with the formation of a
12.11 External fixators (a) These are useful for fibrovascular stroma; through this, blood vessels and
provisional fracture control, as in severe open fractures. osteoprogenitor cells can pass from the recipient bone
Fixators are also used for definitive fracture treatment into the graft. Apart from providing a stimulus for
(b) and for Ilizarov limb reconstruction surgery (c). bone growth (osteoinduction), the graft also provides
a passive scaffold for new bone growth (osteoconduc-
tion). Cancellous grafts become incorporated more
aspects of both types (hybrid). Each possesses specific quickly and more completely than cortical grafts (Fig.
biomechanical properties with regard to control of 12.12).
movement at the fracture or osteotomy site, especially
when the patient loads the limb on walking. The
choice of a fixator type will depend on many factors Vascularized grafts
including the intended purpose of its use and the sur- This is theoretically the ideal graft; bone is transferred
geon’s familiarity with the device. complete with its blood supply, which is anastomosed
to vessels at the recipient site. The technique is diffi-
cult and time consuming and requires microsurgical
skill. Available donor sites include the iliac crest (com-
BONE GRAFTS AND SUBSTITUTES plete with one of the circumflex arteries), the fibula
(with the peroneal artery) and the radial shaft. Vascu-
Bone grafts are both osteoinductive and osteoconduc- larized grafts remain completely viable and become
tive: (1) they are able to stimulate osteogenesis incorporated by a process analogous to fracture
through the differentiation of mesenchymal cells into healing. 317
12
(a)
(b) (c) (d)
12.12 Autogenous cancellous bone grafts (a) Here autogenous grafts are used to fill a defect of the ulna and they
unite with the host bone in 4 months (b). Free vascularized bone transfer (in this case a portion of fibula) is also helpful
when larger defects need to be filled (c,d).
Bone marrow aspirates be plentiful, are particularly useful when large defects
have to be filled. However, sterility must be ensured.
Bone marrow contains stem cells and osteoprogenitor The potential for transfer of infection is either from
cells, which are able to transform into osteoblasts in contamination at the time of harvesting or from dis-
the appropriate environment and with stimulation. eases present in the donor. The graft must be har-
The number of these mesenchymal cells in aspirates vested under sterile conditions and the donor must be
from the iliac crest decreases with age and more so in cleared for malignancy, syphilis, cytomegalovirus,
females (Muschler et al., 2001). In addition, the hepatitis and HIV; this requires prolonged (several
aspiration technique from the iliac crest can influence months) testing of the donor before the graft is used.
the number of osteoblast progenitors obtained; this Sterilization of the donor material can be done by
may account for the variable results reported in the exposure to ethylene oxide or by ionizing radiation,
small clinical series thus far published. The recom- but the physical properties and potential for osteoin-
mended procedure is to take multiple small-volume duction are considerably altered (De Long et al.,
aspirates (four 1 mL aspirates from separate site punc- 2007).
tures). Centrifugation of the aspirate, in order to con- Fresh allografts, though dead, are not immunolog-
centrate the cellular contents, has provided ically acceptable. They induce an inflammatory
encouraging results in animal experiments; early evi- response in the host and this may lead to rejection.
dence suggests this also may be the optimal method However, antigenicity can be reduced by freezing (at
for using bone marrow aspirates in humans –70°C), freeze-drying or by ionizing radiation.
(Hernigou et al., 2005). Demineralization is another way of reducing anti-
genicity and it may also enhance the osteoinductive
Platelet-derived activators properties of the graft. Acid extraction of allograft
bone yields demineralized bone matrix, which con-
‘Activators’ are now available through centrifugation tains collagen and growth factors. It is available in a
of venous blood. These factors activate repair of tis- variety of forms (putty, powder, granules) and is
sues (not just bone) and may augment healing sometimes combined with other types of bone substi-
processes in vivo. Further strong clinical evidence to tutes. The osteoinductive capability of demineralized
their efficacy is awaited. bone matrix is variable; most human studies have not
shown the impressive osteoinductive capacity found in
animal experiments. One way to supplement the
ALLOGRAFTS (HOMOGRAFTS) properties of demineralized bone matrix is to use it as
an autologous bone graft expander.
Allografts consist of bone transferred from one indi- Allografts are most often used in reconstructive sur-
vidual (alive or dead) to another of the same species. gery where pieces are inserted for structural support;
318 They can be stored in a bone bank and, as supplies can an example is revision hip arthroplasty where bone
loss from prosthesis loosening is replaced. The process 12
of incorporation of allografts (when it occurs) is simi-
lar to that with autografts but slower and less com-
plete.
BONE MORPHOGENETIC PROTEINS
(BMPS) (a)
These substances were originally extracted from allo-

graft bone but were too difficult to produce in com-
mercially suitable quantities. BMP-2 and BMP-7 are
now manufactured using recombinant techniques and
are available commercially.
BMPs are osteoinductive. There is evidence to sup-
port their use in the treatment of non-union and open
tibial fractures where the success rate is equivalent to (b) (c)
that of autogenous bone grafts. They are used with a
12.13 Synthetic bone substitutes These are used
carrier, which may be allograft, demineralized bone
primarily as osteoconductive agents or as a delivery
matrix, collagen or bioactive bone cement. Currently, medium for antibiotics. Several forms are available,
the cost of purchase is a barrier to widespread adop- including putties and injectable pastes (a,b). They are used
tion. to fill small defects or can act as antibiotic-eluting spacers
after bone resection in chronic osteomyelitis (c).
CALCIUM-BASED SYNTHETIC SUBSTITUTES

Calcium phosphate, hydroxyapatite (a crystalline cal- DISTRACTION OSTEOGENESIS AND
cium phosphate) and calcium sulphate are primarily LIMB RECONSTRUCTION – ILIZAROV
osteoconductive and need a pore size of around METHOD
400 μm for osteoprogenitor cells to lay down bone.
The calcium phosphate and hydroxyapatite varieties Distraction osteogenesis is a form of tissue engineer-
are usually used to fill metaphyseal defects in fracture ing founded on the principle of tension-stress, which is
surgery, e.g. tibial plateau, distal radius and calcaneal the generation of new bone in response to gradual
fractures; in this context, several studies have reported increases in tension. Discovered in the 1950s by
good results. Various forms of the material are avail- Gavril Ilizarov in Russia, the application of this prin-
able, including granules, chips and paste. Despite ciple to orthopaedic conditions represents a signifi-
claims by manufacturers to the contrary, these syn- cant advance; it has opened opportunities for
thetic substitutes do not possess sufficient compres- treatment in conditions that hitherto were poorly
sive strength to withstand high loads and should be treated or even untreatable. The term ‘Ilizarov
used in stabilized fractures and not as a means of con- method’ embraces the various applications of this prin-
tribution to stability. Calcium phosphate materials are ciple, emphasizing minimally invasive surgery (many
usually absorbed completely by 6–9 months, but of the techniques are performed percutaneously) and
hydroxyapatite substitutes are still visible on x-ray an early return of function.
after several years. This slow resorption has prompted
hydroxyapatite and calcium phosphate mixtures to be
made available, in the hope that the faster resorption
of the latter will enable more rapid bone replacement. DISTRACTION OSTEOGENESIS
Calcium phosphate has also been successfully mixed
with autologous bone marrow and bovine collagen to
Callotasis
produce results equivalent to those of autogenous Callus distraction, or callotasis, is perhaps the single
bone graft (Chapman et al., 1997). most important application of the tension-stress prin-
In contrast, calcium sulphate materials are usually ciple. It is used for limb lengthening or filling of large
resorbed within 6–9 weeks and are useful, in combi- segmental defects in bone, either through bone trans-
nation with gentamicin or tobramycin, as a means of port or other strategies. The basis of the technique is
local antibiotic delivery in the treatment of cavities or to produce a careful fracture of bone, followed by a
‘dead space’ after surgery in chronic osteomyelitis short wait before the young callus is gradually dis-
(McKee et al., 2002) (Fig. 12.13). tracted via a circular or unilateral external fixator. It is 319
12 worth noting that all tissue types are created during zone), which gradually disappears when the column of
the distraction process and the term distraction histo- bone completely ossifies. Regular x-rays allow the sur-
genesis is perhaps more appropriate. geon to check on the quality of regenerate (Fig.
The external fixator is applied using transfixing 12.14).When cortices of even thickness are seen in the
wires or screws proximal and distal to the proposed regenerate on x-ray, the fixator is ready to be
osteogenesis site. The surgical fracture to allow dis- removed. Throughout treatment, physiotherapy is
traction osteogenesis to commence is done by several important to preserve joint movement and avoid con-
methods. In a corticotomy, the bony cortex is partially tractures.
divided with a sharp osteotome through a small skin
incision and the break completed by osteoclasis, leav-
ing the medullary blood supply and endosteum CHONDRODIATASIS
largely intact. Alternatively, the periosteum can be
incised and elevated and the bone then drilled several Bone lengthening can also be achieved by distracting
times before using an osteotome to complete the divi- the growth plate (chondrodiatasis). No osteotomy is
sion; the periosteum is then repaired. Both techniques needed but the distraction rate is slower, usually
are exacting – simply dividing the bone with a power 0.25 mm twice daily. Although a wide, even column
saw results in nothing being formed in the gap. After of regenerate is usually seen, the fate of the physis is
an initial wait of 5–10 days, distraction is begun and sealed – the growth plate frequently closes after the
proceeds at 1 mm a day, with small (usually 0.25 mm) process. This technique is best reserved for children
increments spaced out evenly throughout the day. close to the end of growth.
The first callus is usually seen on x-ray after 3–4
weeks; in optimum conditions it appears on x-ray as
an even column of partially radio-opaque material in BONE TRANSPORT
the gap between the bone fragments (this is called the
regenerate). If the distraction rate is too fast, or the Distraction osteogenesis is used not only for limb
osteotomy performed poorly, the regenerate may be lengthening but also as a means of filling segmental
thin with an hourglass appearance; conversely if dis- defects in bone. In bone transport, the defect (or gap)
traction is too slow, it may appear bulbous or worse is filled gradually by creating a ‘floating’ segment of
still may consolidate prematurely, thereby preventing bone through a corticotomy either proximal or distal
any further lengthening. to the defect, and this segment is moved slowly across
When the desired length is reached, a second wait the defect. An external fixator provides stability and
follows, which allows the regenerate column to con- the ability to control this segment during the process.
solidate and harden. Weightbearing is permitted As the segment is transported from the corticotomy
throughout this period and it assists the consolidation site to the new docking site, new bone is created in its
process. The regenerate column is first seen on x-ray wake, which effectively fills the defect (Fig. 12.15).
to be divided by an irregular line (the fibrous inter- A variant of the bone transport technique is bifocal
(a) (b) (c) (d)
12.14 Distraction osteogenesis Early on there is little activity in the distracted gap (a). A little later, columns of bone are
seen reaching for the centre of the distracted zone, leaving a clear space in between – the fibrous interzone (b). When the
320 columns bridge the gap, the regenerate bone matures and, finally, a medullary cavity is re-established (c,d).
resistant club-foot deformity is dealt with by applying 12
gradual tension to the contracted soft tissue structures
through an external fixator and slowly altering the
position of the ankle, subtalar and midtarsal joints
until a normal position is achieved. The assembly of
the external fixator to accomplish this technique is
complex, but the results are often gratifying.
LEG LENGTH EQUALIZATION
(a) (b) Inequality of leg length may result from many causes,
including congenital anomalies, malunited fractures,
epiphyseal and physeal injuries, infections and paraly-
sis. Marked inequality leads to inefficient walking and
a noticeable limp. The longer leg has to be lifted
higher to clear the ground during swing-through and
the pelvis and shoulders dip noticeably during the
stance phase on the shorter side; both of these adjust-
ments increase energy consumption. Pelvic tilt and
the associated compensatory scoliosis tend to cause
backache, and there is a higher reported incidence of
osteoarthritis of the hip on the longer side – possibly
because of the ‘uncovering’ of the femoral head due
to pelvic obliquity.
(c) (d)
Inequality greater than 2.5 cm needs treatment,
12.15 Bone transport. A segment of bone ‘travels’ which may amount to no more than a shoe-raise, or it
across a defect. The limb length is, therefore, unchanged. may involve an operation to either the shorter or
(a,b,c) The segment is created by osteotomy and gradual
distraction produces new bone. The docking site (arrow)
longer leg.
often needs attending to in order to heal (d).
Techniques for correcting leg length
There are four choices:
compression-distraction. With this method, the defect • shortening the longer leg
is closed by instantly bringing the bone ends together; • slowing growth in the longer leg
a corticotomy is then performed at a different level • lengthening the shorter leg
and length is restored by callotasis. In this case the • speeding up growth in the shorter leg.
limb is shortened temporarily, whereas in bone trans-
The problem of leg length inequality often presents
port overall limb length remains unchanged.
in childhood. Several questions need to be answered
before a technique appropriate for the particular child
is determined:
CORRECTING BONE DEFORMITIES AND • What will the discrepancy be when the child is
JOINT CONTRACTURES mature?
• What is the expected adult height of the child?
Angular deformities are corrected by carefully planned
• When will the child reach skeletal maturity?
osteotomies. However, the amount of correction
• Is there a deformity associated with the leg length
needed may induce, if undertaken acutely, an unwanted
discrepancy?
sudden tension on soft tissues, particularly nerves. With
the Ilizarov method, it is now possible to undertake Leg length difference at maturity is estimated
large corrections with a much lower risk. The correc- through charts and tables and by plotting the rate of
tion is performed gradually with the aid of an external change in discrepancy over a period. Expected adult
fixator; length, rotation and translation deformities can height is calculated through formulae – the TW3
be dealt with simultaneously (Fig. 12.16). method is one (Tanner et al., 2001), and the time of
The principle of tension stress can also be applied to skeletal maturity is obtained by reading the bone age
correcting soft-tissue contractures. For example, a from an x-ray of the non-dominant hand. 321
12
(b) (c) (d)
(a)
12.16 Correction of deformity (a) Varus malunion in a fracture of

the proximal tibia was corrected by osteotomy and gradual
realignment in a circular external fixator (b–d). A cuneiform-shaped
(e) mass of bone formed after the axes were aligned (e).
OPERATIONS ON THE LONGER LEG tal femoral and proximal tibial physeal arrest per-
formed about 3 years before skeletal maturity.
Physeal arrest Epiphyseodesis produces approximate length
equalization, often to within 10 mm of estimated
In children, physeal arrest is an effective method of length, if performed in a timely fashion. Other meth-
slowing the rate of growth of the longer leg; it can be ods of predicting the timing of epiphyseodesis are
temporary, using removable staples fixed across the chart based (Moseley, 1977; Eastwood and Cole,
growth plate, or permanent, by drilling across the 1995) or use a multiplier method (Aguilar et al.,
physis and curetting out the growth plate. Another 2005).
method is to excise a rectangular block of bone across
the physis, rotate the block through 90 degrees and
then reinsert it into the original bed. When the physis
Bone shortening
fuses (epiphyseodesis), longitudinal growth at that
site ceases and the overall gain in length of the limb is Epiphyseodesis is feasible only in a growing child. In
retarded. In due course the difference in lengths adults, it is necessary to excise a segment of bone,
should be reduced. preferably from the femur, since tibial shortening is
The timing and technique of epiphyseodesis is more complicated and is cosmetically unattractive; up
important. If it is inaccurately timed, a difference in to 7.5 cm of femoral shortening can be achieved with-
leg lengths will remain, and if improperly done, defor- out permanent loss of function. The safest technique
mity may occur. Physeal arrest will create a loss of is to excise a segment from between the lesser
10 mm of length a year from the distal femur and trochanter and the femoral isthmus, to approximate
6 mm a year from the proximal tibia. As the physes the cut ends, and to fix them together with a locking
close naturally at 16 years of age in boys and 14 years intramedullary nail or plate. Open excision of bone
in girls, a predicted length discrepancy at maturity of segments from the long leg has several disadvantages,
322 45 mm can, for example, be addressed by both a dis- among which scarring and poor muscle tone are
important. The scarring results from a longitudinal associated with a substantial number of complications 12
incision being suddenly subjected to a concertina such as pin-site sepsis, deformity or fracture. More-
effect, which causes the wound to gape widely. over, gain in height is not the same as ‘normality’.
Shorter segments can be removed by ‘closed’ Nevertheless, successful treatment is so rewarding
intramedullary techniques, which rely on an (“People no longer look at me in the street; I can now
intramedullary saw and bone splitter, and thereby get things off a shelf without having to climb up”)
avoid the problem with scars. In general, shortening that it should not be withheld if the patient is other-
of the long leg is reserved for situations where the wise normal and is psychologically prepared. Referral
patient is too old for an epiphyseodesis or where to a specialized centre is wise.
lengthening the short leg is deemed too risky, e.g. in The techniques of lengthening are as described ear-
the presence of unstable joints or infection. lier and two bones can be dealt with simultaneously.
Shortening should, of course, be applied only if the It is more usual to lengthen both tibiae at one proce-
patient’s residual height will still be acceptable. It dure and both femora at another. Gains in height
should also be remembered that the longer leg is usu- averaging 20–25 cm have been achieved by combin-
ally the normal one and if a serious complication such ing the bone lengthening with soft-tissue releases
as non-union ensues, the patient may be worse off (McAndrew and Saleh, 2007).
than not having an operation in the first place.
LENGTHENING THE SHORTER LEG OPERATIONS ON JOINTS

Lengthening the short leg is most easily accomplished
by wearing a raised shoe, but this is often inadequate ARTHROTOMY
or unacceptable – a shoe raise of more than 5 cm can
risk injury to the ankle! Arthrotomy (opening a joint) may be indicated to: (1)
Stimulation of the growth plate can be achieved by inspect the interior or perform a synovial biopsy; (2)
the technique of periosteal division. A circumferential drain a haematoma or an abscess; (3) remove a loose
5 mm strip is excised from around the distal femoral body or damaged structure (e.g. a torn meniscus); (4)
or proximal tibial physis (Wilde and Baker, 1987). to excise inflamed synovium. The intra-articular tis-
The physis responds with an accelerated growth rate sues should be handled with great care, and if postop-
that may last for up to 2 years. However, like epiphy- erative bleeding is expected (e.g. after synovectomy) a
seodesis, poor technique may produce deformity; the drain should be inserted – postoperative haemarthro-
method is probably best reserved for young children sis predisposes to infection. Following the operation
(younger than 6 years) as the effects on older children the joint should be rested for a few days, but there-
are unpredictable. after movement must be encouraged.
Limb lengthening by the Ilizarov method is a suit-
able method for predicted length discrepancies of
greater than 5 cm. Distraction osteogenesis has
become much safer since it was appreciated that dis- ARTHRODESIS
traction has to be slow if neural or vascular damage is
to be avoided (see earlier). Major length corrections The most reliable operation for a painful or unstable
can be tackled by staging the treatment process over joint is arthrodesis; where stiffness does not seriously af-
several years, or by attempting to lengthen at two lev- fect function, this is often the treatment of choice. Ex-
els within the same bone (bifocal lengthening). The amples are the spine, tarsus, ankle, wrist and interpha-
latter method, although attractive, has a higher rate of langeal joints. Arthrodesis is also useful for a knee that
complications largely from the soft tissues being dis- is already fairly stiff (provided the other knee has good
tracted too quickly. movement) and for a flail shoulder. More controversial
is arthrodesis of the hip. Though it is a reasonable al-
ternative to arthroplasty or osteotomy for joint disease
in young patients, there is an understandable resistance
OPERATIONS TO INCREASE STATURE to sacrificing all movement in such an important joint.
It is difficult to convey to the patient that a fused hip
Bilateral leg lengthening is a feasible procedure for can still ‘move’ by virtue of pelvic tilting and rotation;
people with achondroplasia and other individuals of the best approach is to introduce the patient to some-
short stature, but detailed consultation is an essential one who has had a successful arthrodesis.
preliminary. The prospective patient must understand The principles of arthrodesis are straightforward
that treatment is painful, prolonged, and may be and involve four stages: (1) exposure – both joint 323
12
(a) (b) (c) (d) (e)
12.17 Arthrodesis (a) Compression arthrodesis; (b) screw plus bone graft; (c) similar technique using the acromion.
(d,e) Subtalar mid-tarsal fusion.
surfaces need to be well visualized and often this • Excision arthroplasty – Sufficient bone is excised
means an extensile incision, but some smaller joints from the articulating parts of the joint to create a
are now accessible by arthroscopic means; (2) prepa- gap at which movement can occur (e.g. Girdle-
ration – both articular surfaces are denuded of carti- stone’s hip arthroplasty). This movement is limited
lage and sometimes the subchondral bone is and occurs through intervening fibrous tissue,
‘feathered’ to increase the contact area; (3) coaptation which forms in the gap. In some situations, e.g.
– the prepared surfaces are apposed in the optimum after excising the trapezium, a shaped ‘spacer’ can
position, ensuring good contact; (4) fixation – the be inserted; this is often tendon harvested from
surfaces are held rigidly by internal or external fixa- nearby.
tion. Sometimes bone grafts are added in the larger • Partial replacement – One articulating part only is
joints to promote osseous bridging (Fig. 12.17). replaced (e.g. a femoral prosthesis for a fractured
The main complication is non-union with the for- femoral neck, without an acetabular component);
mation of a pseudoarthrosis. Rigid fixation lessens this or one compartment of a joint is replaced (e.g. the
risk; where feasible (e.g. the knee and ankle), the bony medial or lateral half of the tibiofemoral joint). The
parts are squeezed together by compression-fixation prosthesis is kept in position either by acrylic
devices. cement or by a press-fit between implant and bone.
• Replacement – Both the articulating parts are
replaced by prosthetic implants; for biomechanical
reasons, the convex component is usually metal and
ARTHROPLASTY the concave high-density polyethylene. Metal-on-
metal replacements are also becoming more com-
Arthroplasty, the surgical refashioning of a joint, aims mon. Irrespective of type, these components are
to relieve pain and to retain or restore movement. The fixed to the host bone, either with acrylic cement or
following are the main varieties (Fig. 12.18): by a cementless press-fit technique. Using hip
replacement as an example, the rationale, indica-
tions and complications of total joint replacement
are discussed in detail in Chapter 19.
MICROSURGERY AND LIMB

REPLANTATION
Microsurgical techniques are used in repairing nerves
(a) (b) (c)
and vessels, transplanting bone or soft tissue with a
vascular pedicle, transferring a less essential digit (e.g.
12.18 Arthroplasty The main varieties as applied to the a toe) to replace a lost essential one (e.g. a thumb)
hip joint: (a) excision arthroplasty (Girdlestone); (b) partial
and – occasionally – for reattaching a severed limb or
replacement – an Austin Moore prosthesis has been
inserted after removing the femoral head; (c) total digit. Essential prerequisites are an operating micro-
324 replacement – both articular surfaces are replaced. scope, special instruments, microsutures, a chair with
12.19 Microsurgery and limb
replantation (a) The problem –
12
a severed hand. (b) The solution –
replantation with microsurgical
techniques. (c) The bones of the
severed hand have been fixed with
K-wires as a preliminary to suturing
vessels and nerves. (d) The
appearance at the end of the
operation. (e,f) The limb 1 year
later; the fingers extend fully and
bend about halfway. But the hand
survived, has moderate sensation
and the patient was able to return
to work (as a guillotine operator in
(a) (b) (c) a paper works!)
(d) (e) (f)
arm supports and – not least – a surgeon well prac-

tised in microsurgical techniques. AMPUTATIONS
For replantation, the severed part should be kept
cool during transport. The more muscle in the ampu- INDICATIONS
tated part, the shorter the period it will last; warm
ischaemic periods of greater than 6 hours are likely to Alan Apley, in characteristic style, encapsulated the
result in permanent muscle damage and may even indications for amputation in the never-to-be-
produce severe systemic upset in the patient when forgotten ‘three Ds’: (1) Dead, (2) Dangerous and
reperfusion of the muscle occurs. Two teams dissect, (3) Damned nuisance:
identify and mark each artery, nerve and vein of the Dead (or dying) Peripheral vascular disease accounts for
stump and the limb. Following careful debridement almost 90 per cent of all amputations. Other causes of
the bones are shortened to reduce tension and are sta- limb death are severe trauma, burns and frostbite.
bilized internally. Next the vessels are sutured – veins
first and (if possible) two veins for each artery. Nerves Dangerous ‘Dangerous’ disorders are malignant
and tendons next need to be sutured. Only healthy tumours, potentially lethal sepsis and crush injury. In
ends of approximately equal diameter should be crush injury, releasing the compression may result in
joined; tension, kinking and torsion must be pre- renal failure (the crush syndrome).
vented. Decompression of skin and fascia, as well as Damned nuisance Retaining the limb may be worse
thrombectomy, may be needed in the postoperative than having no limb at all. This may be because of: (1)
period (Fig. 12.19). pain; (2) gross malformation; (3) recurrent sepsis or
Replantation surgery is time consuming, expensive (4) severe loss of function. The combination of
and often unsuccessful. It should be carried out only deformity and loss of sensation is particularly trying,
in centres specially equipped and by teams specially and in the lower limb is likely to result in pressure
trained for this work. ulceration. 325
12 VARIETIES demands of prosthetic design and local function. Too
short a stump may tend to slip out of the prosthesis.
A provisional amputation may be necessary because Too long a stump may have inadequate circulation and
primary healing is unlikely. The limb is amputated as can become painful, or ulcerate; moreover, it compli-
distal as the causal conditions will allow. Skin flaps suf- cates the incorporation of a joint in the prosthesis (Fig.
ficient to cover the deep tissues are cut and sutured 12.20). For all that, the skill of the modern prosthetist
loosely over a pack. Re-amputation is performed has made it possible to amputate at almost any site.
when the stump condition is favourable.
A definitive end-bearing amputation is performed
when pressure or weight is to be borne through the PRINCIPLES OF TECHNIQUE
end of a stump. Therefore the scar must not be ter-
minal, and the bone end must be solid, not hollow, A tourniquet is used unless there is arterial insuffi-
which means it must be cut through or near a joint. ciency. Skin flaps are cut so that their combined length
Examples are through-knee and Syme’s amputations. equals 1.5 times the width of the limb at the site of
A definitive non-end-bearing amputation is the amputation. As a rule anterior and posterior flaps of
commonest variety. All upper limb and most lower equal length are used for the upper limb and for trans-
limb amputations come into this category. Because femoral (above-knee) amputations; below the knee a
weight is not to be taken at the end of the stump, the long posterior flap is usual.
scar can be terminal. Muscles are divided distal to the proposed site of
bone section; subsequently, opposing groups are
sutured over the bone end to each other and to the
periosteum, thus providing better muscle control as
AMPUTATIONS AT SITES OF well as better circulation. It is also helpful to pass the
ELECTION sutures that anchor the opposing muscle groups
through drill-holes in the bone end, creating an
Most lower limb amputations are for ischaemic disease osteomyodesis. Nerves are divided proximal to the bone
and are performed through the site of election below cut to ensure a cut nerve end will not bear weight.
the most distal palpable pulse. The selection of ampu- The bone is sawn across at the proposed level. In
tation level can be aided by Doppler indices; if the an- trans-tibial amputations the front of the tibia is usually
kle/brachial index is greater than 0.5, or if the occlu- bevelled and filed to create a smoothly rounded con-
sion pressure at the calf and thigh are greater than tour; the fibula is cut 3 cm shorter.
65 mmHg and 50 mmHg respectively, then there is a The main vessels are tied, the tourniquet is
greater likelihood the below-knee amputation will suc- removed and every bleeding point meticulously lig-
ceed (Sarin et al., 1991). An alternative means is by us- ated. The skin is sutured carefully without tension.
ing transcutaneous oxygen tension as a guide, but the Suction drainage is advised and the stump covered
level that assures wound healing and avoids unnecessary without constricting passes of bandage; figure-of-
above-knee amputations has not been confidently de- eight passes are better suited and prevent the creation
termined. The knee joint should be preserved if clini- of a venous tourniquet proximal to the stump.
cal examination and investigations suggest this is at all
feasible – energy expenditure for a trans-tibial amputee
is 10–30 per cent greater as compared to a 40–67 per AFTERCARE
cent increase in trans-femoral cases (Czerniecki, 1996;
Esquenazi and Meier, 1996; Mattes et al., 2000). If a haematoma forms, it is evacuated as soon as
The sites of election are determined also by the possible. After satisfactory wound healing, gradual
12.20 Amputations The

traditional sites of election;
the scar is made terminal
because these are not end-
bearing stumps.
326
compression stump socks are used to help shrink the children because the lower femoral physis is pre- 12
stump and produce a conical limb-end. The muscles served, effectively permitting a stump length equiva-
must be exercised, the joints kept mobile and the lent to an above-knee amputation to be reached when
patient taught to use his prosthesis. the child is mature.
Transtibial (below-knee) amputations Healthy below-
knee stumps can be fitted with excellent prostheses
AMPUTATIONS OTHER THAN AT allowing good function and nearly normal gait. Even a
SITES OF ELECTION 5–6 cm stump may be fitted with a prosthesis in a thin
patient; greater length makes fitting easier, but there is
no advantage in prolonging the stump beyond the
Interscapulo-thoracic (forequarter) amputation) This
conventional 14 cm.
mutilating operation should be done only for traumatic
avulsion of the upper limb (a rare event), when it offers Above the ankle Syme’s amputation This is sometimes very
the hope of eradicating a malignant tumour, or as satisfactory, provided the circulation of the limb is good.
palliation for otherwise intractable sepsis or pain. It gives excellent function in children, and shares the
same advantage as a through-knee amputation in that
Disarticulation at the shoulder This is rarely indicated,
the distal physis is preserved. In adults it is well accepted
and if the head of the humerus can be left, the
by men, but women find it cosmetically undesirable. The
appearance is much better. If 2.5 cm of humerus can
indications are few and the operation is difficult to do
be left below the anterior axillary fold, it is possible to
well. Because the stump is designed to be end-bearing,
hold the stump in a prosthesis.
the scar is brought away from the end by cutting a long
Amputation in the forearm The shortest forearm stump posterior flap. The flap must contain not only the skin of
that will stay in a prosthesis is 2.5 cm, measured from the heel but the fibrofatty heel pad so as to provide a
the front of the flexed elbow. However, an even shorter good surface for weightbearing. The bones are divided
stump may be useful as a hook to hang things from. just above the malleoli to provide a broad area of
cancellous bone, to which the flap should stick firmly;
Amputations in the hand These are discussed in
otherwise the soft tissues tend to wobble about.
Chapter 16.
Pirogoff’s amputation Similar in principle to Syme’s but
Hemipelvectomy (hindquarter amputation) This operation
this is rarely performed. The back of the os calcis is fixed
is performed only for malignant disease.
onto the cut end of the tibia and fibula.
Disarticulation through the hip This is rarely indicated
Partial foot amputation The problem here is that the
and prosthetic fitting is difficult. If the femoral head,
tendo-Achillis tends to pull the foot into equinus; this
neck and trochanters can be left, it is possible to fit a
can be prevented by splintage, tenotomy or tendon
tilting-table prosthesis in which the upper femur sits
transfers. The foot may be amputated at any convenient
flexed; if, however, a good prosthetic service is available,
level; for example, through the mid-tarsal joints
a disarticulation and moulding of the torso is preferable.
(Chopart), through the tarsometatarsal joints (Lisfranc),
Transfemoral amputations A longer stump offers the through the metatarsal bones or through the
patient better control of the prosthesis and it is usual to metatarsophalangeal joints. It is best to disregard the
leave at least 12 cm below the stump for the knee classic descriptions and to leave as long a foot as possible
mechanism. However, recent gait studies suggest some provided it is plantigrade and that an adequate flap of
latitude is present as long as the amputated femur is at plantar skin can be obtained. The only prosthesis needed
least 57 per cent of the length of the contralateral is a specially moulded slipper worn inside a normal shoe.
femur (Baum et al., 2008).
In the foot Where feasible, it is better to amputate
Around the knee The Stokes–Gritti operation (in through the base of the proximal phalanx rather than
which the trimmed patella is apposed to the trimmed through the metatarsophalangeal joint. With diabetic
femoral condyle) is rarely performed because the bone gangrene, septic arthritis of the joint is not uncommon;
may not unite securely; the end-bearing stump is rarely the entire ray (toe plus metatarsal bone) should be
satisfactory and there is no room for a sophisticated amputated.
knee mechanism.
Amputation through the knee is used at times but
is often associated with poorer functional and psycho-
logical outcomes to above-knee amputees. Fitting a PROSTHESES
modern knee mechanism is troublesome and the sit-
ting position reveals the knees to be grossly unequal All prostheses must fit comfortably, should function
in level. The main indication for this procedure is in well and look presentable. The patient accepts and 327
12 uses a prosthesis much better if it is fitted soon after Nerve A cut nerve always forms a neuroma and
operation; delay is unjustifiable now that modular occasionally this is painful and tender. Excising 3 cm of
components are available and only the socket need be the nerve above the neuroma sometimes succeeds.
made individually. Alternatively, the epineural sleeve of the nerve stump is
In the upper limb, the distal portion of the pros- freed from nerve fascicles for 5 mm and then sealed
thesis is detachable and can be replaced by a ‘dress with a synthetic tissue adhesive or buried within muscle
hand’ or by a variety of useful terminal devices. Elec- or bone away from pressure points.
trically powered limbs are available for both children
‘Phantom limb’ This term is used to describe the
and adults.
feeling that the amputated limb is still present. In
In the lower limb, weight can be transmitted
contrast, residual limb pain exists in the area of the
through the ischial tuberosity, patellar tendon, upper
stump. Both features are prevalent in amputees to a
tibia or soft tissues. Combinations are permissible;
varying extent, and appear to have greater significance
recent developments in silicon and gel materials pro-
in those who also have features of depressive
vide improved comfort in total-contact self-suspend-
symptoms. The patient should be warned of the
ing sockets.
possibility; eventually the feeling recedes or disappears
but, in some, long-term medication may be needed. A
painful phantom limb is very difficult to treat.
COMPLICATIONS OF AMPUTATION Joint The joint above an amputation may be stiff or
STUMPS deformed. A common deformity is fixed flexion and
fixed abduction at the hip in above-knee stumps
(because the adductors and hamstring muscles have
EARLY COMPLICATIONS been divided). It should be prevented by exercises. If
it becomes established, subtrochanteric osteotomy may
In addition to the complications of any operation (es-
be necessary. Fixed flexion at the knee makes it difficult
pecially secondary haemorrhage), there are two special
to walk properly and should also be prevented.
hazards: breakdown of skin flaps and gas gangrene:
Bone A spur often forms at the end of the bone, but
Breakdown of skin flaps This may be due to ischaemia,
is usually painless. If there has been infection, however,
suturing under excess tension or (in below-knee
the spur may be large and painful and it may be
amputations) an unduly long tibia pressing against the
necessary to excise the end of the bone with the spur.
flap.
If the bone is transmitting little weight, it becomes
Gas gangrene Clostridia and spores from the perineum
osteoporotic and liable to fracture. Such fractures are
may infect a high above-knee amputation (or re-
best treated by internal fixation.
amputation), especially if performed through ischaemic
tissue.
LATE COMPLICATIONS IMPLANT MATERIALS

Skin Eczema is common, and tender purulent lumps METAL
may develop in the groin. A rest from the prosthesis is
indicated.
Metal used in implants (screws, plates, intramedullary
Ulceration is usually due to poor circulation, and nails and joint replacement prostheses) should be
re-amputation at a higher level is then necessary. If, tough, strong, non-corrosive, biologically inert and
however, the circulation is satisfactory and the skin easy to sterilize. Those commonly used are stainless
around an ulcer is healthy, it may be sufficient to steel, cobalt–chromium alloys and titanium alloys. No
excise 2.5 cm of bone and resuture. one material is ideal for all purposes.
Stainless steel, because of its relative plasticity, can be
Muscle If too much muscle is left at the end of the
cold worked. This is a process in which the metal is
stump, the resulting unstable ‘cushion’ induces a
reshaped or resized, usually at room temperature,
feeling of insecurity that may prevent proper use of a
which increases its hardness and strength. The form of
prosthesis; if so, the excess soft tissue must be excised.
stainless steel used in orthopaedic surgery is 316L; in
Blood supply Poor circulation gives a cold, blue stump addition to iron, it contains chromium (which forms
that is liable to ulcerate. This problem chiefly arises an oxide layer providing resistance to corrosion), car-
with below-knee amputations and often re-amputation bon (which adds strength but needs to be in low con-
328 is necessary. centrations – hence the L suffix – or else it offsets
corrosion resistance), nickel and molybdenum as the Corrosion 12
main elements used in the alloy. The tensile plasticity
(ductility) of stainless steel makes it possible to bend Corrosion is inevitable unless the implanted metal is
plates to required shapes during an operation without treated, e.g. by passivation, which creates a protective
seriously disturbing their strength. passive layer; this is usually an oxide layer formed from
Cobalt chromium-based alloys are widely used in chemical treatment. In stainless steel and cobalt
joint prosthesis manufacture. Chromium is added to chromium, it is the chromium component that helps in
cobalt for passivation; an adherent oxide layer formed creating an oxide layer but, in titanium, the element
by the chromium provides corrosion resistance, as it itself forms it. With passivated metal alloys used in or-
does in stainless steel. Other elements are sometimes thopaedic surgery, corrosion is rarely a problem except
added, e.g. tungsten and molybdenum, to improve when damage to the passive layer occurs; it may be ini-
strength and machining ability. These alloys have a tiated by abrasive damage or minute surface cracks due
long track record of biocompatibility in human tissue to fatigue failure. Even in the absence of these faults,
and have also, through forging and cold-working, failure can occur through crevice corrosion (where the
high strength. process is heightened by low oxygen concentrations in
Titanium alloys are used in fracture fixation devices crevices – e.g. beneath the heads of screws and plates)
and joint prostheses. They usually contain aluminium or stress corrosion (where repeated low stresses in a cor-
and vanadium in low concentrations for strength; pas- rosive environment cause failure before the fatigue life
sivation (and thus corrosion resistance) is obtained by of the implant is reached). The products of corrosion,
creating a titanium oxide layer. The elastic modulus of metal ions and debris, cause a local inflammatory
the metal is close to that of bone and this reduces the response that accelerates loosening.
stress concentrations that can occur when stainless
steel or cobalt chromium alloys are used. Additionally, Dissimilar metals
the corrosion resistance (which is superior to that of
the other two alloys) augments this metal’s biocom- Dissimilar metals immersed in solution in contact with
patibility. A disadvantage of titanium alloy is notch one another may set up galvanic corrosion with accel-
sensitivity; this is when a scratch or sharp angle cre- erated destruction of the more reactive (or ‘base’)
ated in the metal, either at manufacture or during metal. In the early days of implant surgery, when
insertion of the implant, can significantly reduce its highly corrodible metals were used, the same thing
fatigue life. happened in the body. However, the passive alloys
now used for implants do not exhibit this phenome-
non (titanium being particularly resistant to chemical
Implant failure attack), and the traditional fear of using dissimilar
Metal implants may fail for a variety of reasons: (1) metals in bone implants is probably exaggerated.
defects during manufacture; (2) incorrect implant
selection for intended purpose; (3) exposure to
repeated high stresses from incorrect seating of the
Friction and wear
implant or from exceeding the fatigue life as when These mechanical concepts are relevant to under-
there is delay in a fracture union (Fig. 12.21). standing joint function and prosthesis design. Friction
(a) (b) (c) (d) (e)
12.21 Fatigue failure of implants Fatigue failure can be due to (a,b) incorrect implant selection (too
small or too weak) or (c,d) incorrect positioning. Other factors are also involved: infection may delay union
and lead to eventual implant fracture (e). 329
12 between two sliding surfaces will not be affected by colonies of the bacteria and render them immune to
the area of contact or the speed of movement but will defence mechanisms and antibiotics; (3) the implant
depend on the applied load. Therefore, any two sur- impedes drainage.
faces can have a coefficient of friction derived to rep-
resent this interaction – it is the ratio of the force
Malignancy
needed to start a sliding movement to the normal
compression force between the surfaces. A few cases of malignancy at the site of metal implants
Normal human joints possess coefficients of friction have been reported, but the number is so small in
that are about ten times lower than those of various comparison with the number of implants that the risk
combinations of metal-on-metal. Metal-on-ultra high can probably be discounted.
molecular weight polyethylene produces a better
(lower) coefficient of friction and this is improved fur-
ther if the metal is replaced by a ceramic, e.g. alumina
or zirconium. ULTRA-HIGH MOLECULAR WEIGHT
An important modulator of friction characteristics POLYETHYLENE
in joints is lubrication. Synovial fluid reduces the coef-
ficient of friction either by forming a layer of fluid that Ultra-high molecular weight polyethylene
is greater in width than the surface irregularities on (UHMWPE) is an inert thermoplastic polymer. Its
normal articular cartilage (fluid film lubrication) or, in density is close to that of the low-density polyethyl-
the absence of this interposed fluid layer, a molecular- enes but the very high molecular weight provides
width coating that resists abrasion (boundary lubrica- increased strength and wear resistance over other
tion). Both methods may be involved under different types of polyethylene. The material is manufactured
joint loading conditions. for hip (acetabular cup) and knee (tibial tray) pros-
Friction and joint lubrication are related to wear – theses and sterilized by gamma irradiation. The latter
the loss of surface material due to sliding motion process was noted to cause oxidation of the material
under load. Wear is proportional to the load and dis- and detrimentally alter its physical and chemical prop-
tance of movement between the two surfaces. Wear erties to the extent that a ‘shelf life’ for the compo-
between surfaces can be the result of abrasion (a nent was created. Consequently, current techniques of
harder surface eroding the surface of the softer mate- sterilization involve gamma irradiation in an oxygen-
rial), adhesion (where the two surfaces bond more free environment, e.g. in nitrogen. Although ethylene
tightly than particles within one of the surfaces), or oxide sterilization is an alternative way, irradiation of
from debris that becomes trapped between articulating UHMWPE enables cross-linking of the polymer,
surfaces and causes abrasion (third-body wear). Metal which also improves wear rates.
wear particles may cause local inflammation and scar- In contact to polished metal UHMWPE has a low
ring, and occasionally a toxic or allergic reaction; most coefficient of friction and it therefore seemed ideal for
importantly, however, they may cause implant loosen- joint replacement. This has proved to be true in hip
ing following their uptake by macrophages and subse- reconstruction with a simple ball-and-socket articula-
quent activation of osteoclastic bone resorption. Metal tion. However, UHMWPE has disadvantages: (1) the
wear particles have also been demonstrated in lymph cross-linked form may have improved wear properties
nodes and other organs far distant from the implant; but poorer yield strength, which may influence crack
the significance of this finding is uncertain. Wear of development and propagation; (2) being a viscoelastic
articular cartilage is offset partly by an ability to repair, material, it is susceptible to deformity (stretching) and
although this capacity diminishes with age; this mech- creep; (3) UHMWPE is also easily abraded, a reflec-
anism is obviously not possessed by prostheses. tion of poor hardness, and chips of bone or acrylic
cement trapped on its surface cause it to disintegrate.
Infection
Metal does not cause infection. Titanium alloys have
been shown to be less susceptible to the development SILICON COMPOUNDS
of infection when exposed to the same inoculums of
bacteria (as compared to stainless steel), but the There is a wide variety of silicon polymers, of which
mechanism of this difference is uncertain. Once infec- silicone rubber (Silastic) is particularly useful. It is
tion is established, several mechanisms come into play firm, tough, flexible and inert, and was used to make
that encourage its persistence: (1) the metal implant hinges for replacing finger and toe joints. However,
acts as a foreign body that is devoid of blood supply long-term results are tainted by the material’s suscep-
and thereby inaccessible to immune processes; (2) it tibility to fracture if the implant surface is nicked or
330 promotes the formation of biofilms that encase micro- torn by a sharp instrument or piece of bone.
The presence of silicon particles in the body may When the partially polymerized cement is forced 12
induce a giant-cell synovitis; sometimes bone erosion into the bone there is often a drop in blood pressure;
and ‘cyst’ formation are seen at some distance from this is attributed to the uptake of residual monomer,
the actual implant. For these reasons the main use for which can cause peripheral vasodilatation, but there
Silastic is as temporary spacers to lie within tendon may also be fat embolization from the bone marrow.
pulleys prior to tendon transplants. This is seldom a problem in fit patients with
osteoarthritis, but in elderly people who are also
osteoporotic, monomer and marrow fat may enter the
circulation very rapidly when the cement is com-
CARBON pressed and the fall in blood pressure can be alarming
(and occasionally fatal).
This eminently biocompatible material is looking for a With good cementing technique osseointegration
purpose. As graphite it has wear and lubricant proper- can and does take place on the acrylic surface. How-
ties that might fit it for joint replacement. As carbon ever, if the initial cement application is not perfect, a fi-
fibre it is sometimes used to replace ligaments; it brous layer forms at the cement/bone interface, its
induces the formation of longitudinally aligned thickness depending on the degree of cement penetra-
fibrous tissue, which substitutes for the natural ligation into the bone crevices. In this flimsy membrane
ment. However, the carbon fibres tend to break up fine granulation tissue and foreign body giant cells can
and if particles find their way into the synovial cavity be seen. This relatively quiescent tissue remains un-
they induce a synovitis. Carbon composites are also changed under a wide range of biological and me-
used to manufacture plates and joint prostheses; these chanical conditions, but if there is excessive movement
have a lower modulus of elasticity than metal and may at the cement/bone interface, or if polyethylene or
therefore be more compatible with the bone to which metallic wear products track down into the cement/
they are attached. Carbon fibre is also extensively used bone interface, an aggressive reaction ensues that
for the manufacture of external fixation devices, e.g. produces bone resorption and disintegration of the
connecting rods and even circular rings, as the com- interlocking surface; occasionally this is severe enough
bination of lightweight, rigidity and x-ray lucency is to justify the term ‘aggressive granulomatosis’ or ‘ag-
attractive. gressive osteolysis’. Bone resorption and cement loos-
ening may also be associated with low-grade infection,
which can manifest for the first time many years after
the operation; whether the infection in these cases pre-
ACRYLIC CEMENT cedes the loosening or vice versa is still not known.
In joint replacement the prostheses are often fixed to

the bone with acrylic cement (polymethylmethacry-
late – PMMA), which acts as a grouting material. It is HYDROXYAPATITE
usually presented as a liquid (the PMMA monomer)
and powder (the PMMA polymer plus copolymers or The mineral phase of bone exists largely in the form of
other additives), which is mixed to set off an exother- crystalline hydroxyapatite (HA). It is not surprising,
mic reaction of polymerization. Before the mixture therefore, that this material has been used to reproduce
sets, it is applied to the bone in which the prosthesis the osteoinductive and osteoconductive properties of
is embedded. With sufficient pressure the pasty mate- bone grafts. Porous hydroxyapatite obtained from coral
rial is forced into the bony interstices and, when fully exoskeleton is rapidly incorporated in living bone and
polymerized, the hard compound prevents all move- synthetic implants consisting of hydroxyapatite and tri-
ment between prosthesis and bone. It can withstand calcium phosphate are commercially available as bone
large compressive loads but is easily broken by tensile graft substitutes (see earlier). HA can also be plasma
stress. sprayed onto implants; the HA coating is a highly ac-
Cement mixing and cement introduction tech- ceptable substrate for bone cells and promotes rapid os-
niques have been shown to influence the tensile seointegration. This technique has found a place in
strength. An almost 50 per cent increase in tensile the use of uncemented hip replacement prostheses and
strength can be obtained by vacuum mixing or cen- with external fixator pins.
trifugation of the mixture prior to application; this
reduces the number of voids within the mixture.
Additionally, pressurization of the cement within the REFERENCES AND FURTHER READING
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333
Section 2
Regional
Orthopaedics
13 The shoulder and pectoral girdle 337
14 The elbow and forearm 369
15 The wrist 383
16 The hand 413
17 The neck 439
18 The back 453
19 The hip 493
20 The knee 547
21 The ankle and foot 587
The shoulder and
pectoral girdle 13
Andrew Cole, Paul Pavlou
CLINICAL ASSESSMENT THE PAINFUL SHOULDER

Referred pain syndromes Rotator cuff disorders
SYMPTOMS Cervical spondylosis Tendinitis
Mediastinal pathology Rupture
Pain is the commonest symptom. However, ‘pain in
Cardiac ischaemia Frozen shoulder
the shoulder’ is not necessarily ‘shoulder pain’! If the
patient points to the top of the shoulder, think of the Joint disorders Instability
acromioclavicular joint, or referred pain from the Glenohumeral arthritis Dislocation
neck. Pain from the shoulder joint and the rotator Acromioclavicular arthritis Subluxation
cuff is felt, typically, over the front and outer aspect of
Bone lesions Nerve injury
the joint, often as far down as the middle of the arm.
Infection Suprascapular nerve
The relationship to posture may be significant:
Tumours entrapment
pain which appears when the arm is in the ‘window-
cleaning’ position is characteristic of rotator cuff
impingement; pain which comes on suddenly when
the arm is held high overhead suggests instability. SIGNS
Beware the trap of referred pain. Mediastinal disor-
ders, including cardiac ischaemia, can present with The patient should always be examined from in front
aching in either shoulder. and from behind. Both upper limbs, the neck, the out-
Weakness may appear as a true loss of power, sug- line of the scapula and the upper chest must be visible.
gesting a neurological disorder, or as a sudden and
surprising inability to abduct the shoulder – perhaps
Look
due to a tendon rupture. Between these extremes
there is weakness in performing only certain move- Skin Scars or sinuses are noted; do not forget the
ments and weakness associated with pain. axilla!
Instability symptoms may be gross and alarming
Shape The two sides should be compared. Asymme-
(‘my shoulder jumps out of its socket when I raise my
try of the shoulders, winging of the scapula, wasting
arm’); more often they are quite subtle: a click or jerk
of the deltoid, supraspinatus and infraspinatus muscles
when the arm is held overhead, or the ‘dead arm’ sen-
and acromioclavicular dislocation are best seen from
sation that overtakes the tennis player as he or she pre-
behind; swelling of the acromioclavicular or sterno-
pares to serve.
clavicular joint or wasting of the pectoral muscles is
Stiffness may be progressive and severe – so much
more obvious from the front. A joint effusion causes
so as to merit the term ‘frozen shoulder’.
swelling anteriorly and occasionally ‘points’ in the
Swelling may be in the joint, the muscle or the
axilla. Wasting of the deltoid suggests a nerve lesion
bone; the patient will not know the difference.
whereas wasting of the supraspinatus may be due to
Deformity may consist of muscle wasting, promi-
either a full-thickness tear or a suprascapular nerve
nence of the acromioclavicular joint, winging of the
lesion. The typical ‘Popeye’ bulge of a ruptured
scapula or an abnormal position of the arm.
biceps is more easily seen if the elbow is flexed.
Loss of function is usually expressed as difficulty with
dressing and grooming, or inability to lift objects or Position If the arm is held internally rotated, think of
work with the arm above shoulder height. posterior dislocation of the shoulder.
13
REGIONAL ORTHOPAEDICS
(a) (b) (c)
(d) (e) (f)
(g) (h) (i)
13.1 Examination Active movements are best examined from behind the patient, paying careful attention to symmetry
and the coordination between scapulo-thoracic and gleno-humeral movements. (a) Abduction; (b) limit of gleno-humeral
abduction; (c) full abduction and elevation, a combination of scapulo-thoracic and gleno-humeral movement. (d) The
range of true gleno-humeral movement can be assessed by blocking scapular movement with a hand placed firmly on the
top edge of the scapula. (e) External rotation. (f,g) Complex movements involving abduction, rotation and flexion or
extension of the shoulder. (h) Testing for serratus anterior weakness. (i) Feeling for supraspinatus tenderness.
Feel Move
Skin Because the joint is well covered, inflammation Active movements Movements are observed first from
rarely influences skin temperature. in front and then from behind, with the patient either
standing or sitting. Sideways elevation of the arms
Bony points and soft tissues The deeper structures are normally occurs in the plane of the scapula, i.e. about
carefully palpated, following a mental picture of the 20 degrees anterior to the coronal plane, with the arm
anatomy. Start with the sternoclavicular joint, then rising through an arc of 180 degrees. However, by
follow the clavicle laterally to the acromioclavicular convention, abduction is performed in the coronal
joint, and so onto the anterior edge of the acromion plane and flexion–extension in the sagittal plane.
and around the acromion. The anterior and posterior Abduction starts at 0 degrees; the early phase of
margins of the glenoid should be palpated. With the movement takes place almost entirely at the gleno-
shoulder held in extension, the supraspinatus tendon humeral joint, but as the arm rises the scapula begins
can be pinpointed just under the anterior edge of the to rotate on the thorax and in the last 60 degrees of
acromion; below this, the bony prominence bounding movement is almost entirely scapulo-thoracic (hence
the bicipital groove is easily felt, especially if the arm sideways movement beyond 90 degrees is sometimes
is gently rotated so that the hard ridge slips medially called ‘elevation’ rather than ‘abduction’). The rhyth-
and laterally under the palpating fingers. Crepitus mic transition from gleno-humeral to scapulo-
over the supraspinatus tendon during movement sug- thoracic movement is disturbed by disorders in the
338 gests tendinitis or a tear. joint or by dysfunction of the stabilizing tendons
13.2 Scapulo-humeral 13
rhythm (a–c) During the
early phase of abduction,
most of the movement takes
place at the gleno-humeral
joint. As the arm rises, the
scapula begins to rotate on
The shoulder and pectoral girdle

the thorax (c). In the last
phase of abduction,
movement is almost entirely
scapulo-thoracic (d).
(a) (b) (c) (d)
around the joint. Thus, abduction may be (1) difficult

to initiate, (2) diminished in range or (3) altered in
rhythm, the scapula moving too early and creating a
shrugging effect. If movement is painful, the arc of
pain must be noted; pain in the mid-range of abduc-
tion suggests a minor rotator cuff tear or supraspina-
tus tendinitis; pain at the end of abduction is often
due to acromioclavicular arthritis.
Flexion and extension are examined by asking the
patient to raise the arms forwards and then back-
wards. The normal range is 180 degrees of flexion and
40 degrees of extension.
(a) (b) (c)
Rotation is tested in two ways: The arms are held
close to the body with the elbows flexed to 90 13.3 Normal range of movement (a) Abduction is
degrees; the hands are then separated as widely as pos- from 0° to 160° (or even 180°), but only 90° of this takes
place at the gleno-humeral joint (in the plane of the
sible (external rotation) and brought together again
scapula, 20° anterior to the coronal place); the remainder is
across the body (internal rotation). This is a rather scapular movement. (b) External rotation is usually about
unnatural movement and one learns more by simply 80° but internal is rather less because the trunk gets in the
asking the patient to clasp his (or her) fingers behind way. (c) With the arm abducted to a right angle, internal
his neck (external rotation in abduction) and then to rotation can be assessed without the trunk getting in the
way.
reach up his back with his fingers (internal rotation in
adduction); the two sides are compared.
Passive movements To test the range of gleno- with his arms tucked into his side and the elbows
humeral movement (as distinct from combined gleno- flexed, then to externally rotate against resistance.
humeral and scapular movement) the scapula must Weakness may be associated with a rotator cuff lesion,
first be anchored; this is done by the examiner press- instability or a neurological disorder.
ing firmly down on the top of the shoulder with one
hand while the other hand moves the patient’s arm. Other systems Clinical assessment is completed by
Grasping the angle of the scapula as a method of examining the cervical spine (as a common source of
anchorage is less satisfactory. referred pain), testing for generalized joint laxity (a
frequent accompaniment of shoulder instability) and
Power The deltoid is examined for bulk and tautness performing a focussed neurological examination.
while the patient abducts against resistance. To test
serratus anterior (long thoracic nerve, C5, 6, 7) the
patient is asked to push forcefully against a wall with
Special clinical tests
both hands; if the muscle is weak, the scapula is not Special clinical tests have been developed for localiz-
stabilized on the thorax and stands out prominently ing more precisely the site of pain and tenderness, the
(winged scapula). Pectoralis major is tested by having source of muscle weakness and the presence of insta-
the patient thrust both hands firmly into the waist. bility. These are described in the relevant sections that
Rotator power is tested by asking the patient to stand follow. 339
13 Examination after local anaesthetic by an anteroposterior projection with the tube tilted
injection upwards 20 degrees (the cephalic tilt view). The sub-
acromial space is viewed by tilting the tube down-
It is sometimes possible to localize the source of wards 30 degrees (the caudal tilt view).
shoulder pain by injecting local anaesthetic into the
target site (for example the supraspinatus tendon or Arthrography This is useful for detecting rotator cuff
the acromioclavicular joint) and thus to see whether tears and some larger Bankart lesions found with ante-
there is a temporary reduction in pain on movement. rior instability. It is now usually combined with CT or
Injection into the subacromial space may help to dis- MRI.
tinguish loss of movement due to pain from that due Computed tomography Particularly when enhanced
to a rotator cuff tear. with intra-articular contrast, CT scans can identify
cuff tears and labral detachments.
Diagnostic focus Ultrasound In experienced hands, ultrasound pro-
Important as it is to adopt a systematic approach in vides a reliable and simple means of identifying rota-
the clinical examination, the practical exercise of tor cuff tears, calcific tendinitis and biceps problems.
working towards a diagnosis requires a sensible bal- It can also be useful to identify areas of hypervascu-
ance in the focus of attention. A young athletic person larity and perform ultrasound-guided injections and
who develops pain and weakness on abduction and barbotage (the practice of inserting a needle into a
external rotation of the shoulder is more likely to be calcific deposit and aspirating or fragmenting the
suffering from a rotator cuff disorder than an inflam- material).
matory arthritis of the shoulder and therefore the full
Magnetic resonance imaging The information which is
panoply of special tests for localization of pain and
provided by MRI depends on the quality of the equip-
weakness would be justified, whereas some of these
ment and the imaging sequences which are chosen.
tests would be quite inappropriate in an elderly person
For patients with suspected rotator cuff pathology,
with the longstanding pain and swelling of an arthritic
MRI gives information on the site and size of a tear,
condition.
as well as the anatomy of the coracoacromial arch and
acromioclavicular joint (Recht and Resnick, 1993).
For patients with symptoms and signs suggesting
IMAGING instability, it can demonstrate associated anomalies of
the capsule, labrum, glenoid and humeral head. MRI
X-rays At least two x-ray views should be obtained:
is also useful in detecting osteonecrosis of the head of
an anteroposterior in the plane of the shoulder and an
the humerus and in the diagnosis and staging of
axillary projection with the arm in abduction to show
tumours.
the relationship of the humeral head to the glenoid.
Look for evidence of subluxation or dislocation, joint Magnetic resonance arthrography Using MR arthrog-
space narrowing, bone erosion and calcification in the raphy, a sensitivity of 91 per cent and a specificity of
soft tissues. The acromioclavicular joint is best shown 93 per cent have been reported in the detection of
3
4
2 1
(a) (b) (c) (d)
13.4 Imaging (a) Anteroposterior x-ray. (b) Axillary view showing the humeral head opposite the shallow glenoid fossa,
and the coracoid process anteriorly. The acromion process shadow overlaps that of the humeral head. (c) Lateral view; the
head of the humerus should lie where the coracoid process, the spine of the scapula and the blade of the scapula meet.
(d) MRI. Note (1) the glenoid, (2) the head of the humerus, (3) the acromion process and (4) the supraspinatus (with
340 degeneration of the tendon).
pathological labral conditions (Palmer et al., 1994). exertion; the patient may know precisely which move- 13
For identifying rotator cuff partial undersurface tears, ments now reignite the pain and which to avoid, pro-
MRA has been shown to be more sensitive and spe- viding a valuable clue to its origin. ‘Rotator cuff’ pain
cific than MRI alone (Tirman et al., 1994) typically appears over the front and lateral aspect of
the shoulder during activities with the arm abducted
and medially rotated, but it may be present even with

ARTHROSCOPY the arm at rest. Tenderness is felt at the anterior edge
of the acromion.
Arthroscopy can be useful to diagnose (and treat) Pain and tenderness directly in front along the
intra-articular lesions, detachment of the labrum or delto-pectoral boundary could be associated with the
capsule and impingement or tears of the rotator cuff. biceps tendon. Localized pain over the top of the
Arthroscopy is said to be the best means by which shoulder is more likely to be due to acromioclavicular
superior labrum, anterior and posterior (SLAP) tears pathology, and pain at the back along the scapular
may be diagnosed. border may come from the cervical spine. All these
sites should be inspected for muscle wasting, carefully
palpated for local tenderness and constantly compared
with the opposite shoulder.
DISORDERS OF THE ROTATOR If there is weakness with some movements but not
with others, then one must rule out a partial or com-
CUFF plete tendon rupture; here again, as with pain, local-
ization to a specific site is the key to diagnosis. In both
The rotator cuff is made up of the lateral portions of cases clinical examination should include a number of
the infraspinatus, supraspinatus and subscapularis provocative tests to determine the source of the
muscles and their conjoint tendon which is inserted patient’s symptoms. These are described in the rele-
into the greater tuberosity of the humerus. The vant sections below.
musculotendinous cuff passes beneath the coraco-
acromial arch, from which it is separated by the sub-
acromial bursa; during abduction of the arm the cuff
slides outwards under the arch. The deep surface of IMPINGEMENT SYNDROME,
the cuff is intimately related to the joint capsule and SUPRASPINATUS TENDINITIS AND
the tendon of the long head of the biceps. CUFF DISRUPTION
Although contraction of the individual muscles that
make up the rotator cuff exerts a rotational pull on
the proximal end of the humerus, the main function
Pathology
of the conjoint structure is to draw the head of the Rotator cuff impingement syndrome is a painful disor-
humerus firmly into the glenoid socket and stabilize it der which is thought to arise from repetitive compres-
there when the deltoid muscle contracts and abducts sion or rubbing of the tendons (mainly supraspinatus)
the arm. Consequently, patients with rotator cuff ten- under the coracoacromial arch. Normally, when the
dinitis experience pain and weakness on active abduc- arm is abducted, the conjoint tendon slides under the
tion and those with a severe tear of the cuff are unable coracoacromial arch. As abduction approaches 90 de-
to initiate abduction but can hold the arm abducted grees, there is a natural tendency to externally rotate the
once it has been raised aloft by the examiner. arm, thus allowing the rotator cuff to occupy the widest
The commonest cause of pain around the shoulder part of the subacromial space. If the arm is held per-
is a disorder of the rotator cuff. This is sometimes sistently in abduction and then moved to and fro in
referred to rather loosely as ‘rotator cuff syndrome’, internal and external rotation (as in cleaning a window,
which comprises at least four conditions with distinct painting a wall or polishing a flat surface) the rotator
clinical features and natural history: cuff may be compressed and irritated as it comes in con-
tact with the anterior edge of the acromion process and
• supraspinatus impingement syndrome and tendini-
the taut coracoacromial ligament. This attitude (ab-
tis
duction, slight flexion and internal rotation) has been
• tears of the rotator cuff
called the ‘impingement position’. Perhaps significantly,
• acute calcific tendinitis
the site of impingement is also the ‘critical area’ of
• biceps tendinitis and/or rupture.
diminished vascularity in the supraspinatus tendon
In all these conditions the patient is likely to com- about 1 cm proximal to its insertion into the greater
plain of pain and/or weakness during certain move- tuberosity.
ments of the shoulder. Pain may have started recently, Although the concept of ‘impingement’ as a pri-
sometimes quite suddenly, after a particular type of mary pathogenetic factor has now become 341
13 2 3 4 Other factors which may predispose to repetitive
impingement are osteoarthritic thickening of the
acromioclavicular joint, the formation of bony ridges
or ‘osteophytes’ on the anterior edge of the acromion,
and swelling of the cuff or the subacromial bursa in
5 inflammatory disorders such as gout or rheumatoid
1
arthritis. In 1986, Bigliani and Morrison described
three variations of acromial morphology. Type I is flat,
type II curved and type III the hooked acromion.
They suggested that the type III variety was most fre-
quently associated with impingement and rotator cuff
6 tears.
The mildest injury is a type of friction, which may
give rise to localized oedema and swelling (‘tendini-
tis’). This is usually self-limiting, but with prolonged
or repetitive impingement – and especially in older
people – minute tears can develop and these may be
followed by scarring, fibrocartilaginous metaplasia or
calcification in the tendon. Healing is accompanied by
13.5 Anatomy The tough coracoacromial ligament
stretches from the coracoid to the underside of the anterior a vascular reaction and local congestion (in itself
third of the acromion process; the humeral head moves painful) which may contribute to further impinge-
beneath this arch during abduction and the rotator cuff ment in the constricted space under the coraco-
may be irritated or damaged as it glides in this confined acromial arch whenever the arm is elevated.
space. Key: 1 Rotator cuff; 2 acromion process;
Sometimes – perhaps where healing is slow or fol-
3 coracoacromial ligament; 4 coracoid process;
5 suscapularis; 6 long head of biceps. lowing a sudden strain – the microscopic disruption
extends, becoming a partial or full-thickness tear of
entrenched, it should be mentioned that there is still the cuff; shoulder function is then more seriously
some controversy about whether supraspinatus ten- compromised and active abduction may be impossi-
dinitis may also occur ab initio in response to severe ble. The tendon of the long head of biceps, lying adja-
repetitive stress, and the slightly swollen tendon then cent to the supraspinatus, also may be involved and is
start impinging on the acromioclavicular arch. often torn.
1 2 3 4 5
(a) (b)
13.6 Rotator cuff impingement Coronal sections through the shoulder to illustrate show how the subdeltoid bursa
and supraspinatus tendon can be irritated by repeated impingement under the coracoacromial arch or a rough
acromioclavicular joint during abduction. (a) Joint at rest. (b) In abduction. Key: 1 Supraspinatus muscle;
342 2 acromioclavicular joint; 3 subdeltoid bursa; 4 deltoid muscle; 5 supraspinatus tendon; 6 synovial joint.
Secondary arthropathy Large tears of the cuff eventually as the arm traverses an arc between 60 and 120 13
lead to serious disturbance of shoulder mechanics. The degrees. Repeating the movement with the arm in
humeral head migrates upwards, abutting against the full external rotation may be much easier for the
acromion process, and passive abduction is severely re- patient and relatively painless.
stricted. Abnormal movement predisposes to os- • Neer’s impingement sign: The scapula is stabilized
teoarthritis of the gleno-humeral joint. Occasionally with one hand while with the other hand the exam-

this progresses to a rapidly destructive arthropathy – the iner raises the affected arm to the full extent in pas-
so-called ‘Milwaukee shoulder’ (named after the city sive flexion, abduction and internal rotation, thus
where it was first described by McCarty). bringing the greater tuberosity directly under the
coracoacromial arch. The test is positive when pain,
located to the subacromial space or anterior edge of
CLINICAL FEATURES acromion, is elicited by this manoeuvre. The test is
over 80 per cent sensitive for subacromial impinge-
Early clinical features are typically those of a ‘rotator ment or a rotator cuff tear but it has poor specificity
cuff syndrome’, as described above. Subsequent and may be positive also in patients with acromio-
progress depends on the stage of the disorder, the age clavicular osteoarthritis, gleno-humeral instability
of the patient and the vigour of the healing response. and SLAP lesions.
Three patterns are encountered: • Neer’s test for impingement: If the previous
manoeuvre is positive, it may be repeated after
• Subacute tendinitis – the ‘painful arc syndrome’,
injecting 10 mL of 1 per cent lignocaine into the
due to vascular congestion, microscopic haemor-
subacromial space; if the pain is abolished (or sig-
rhage and oedema.
nificantly reduced), this will help to confirm the
• Chronic tendinitis – recurrent shoulder pain due to
diagnosis.
tendinitis and fibrosis.
• Hawkins–Kennedy test (Hawkins and Kennedy,
• Cuff disruption – recurrent pain, weakness and loss
1980): The patient’s arm is placed in 90 degrees
of movement due to tears in the rotator cuff.
forward flexion with the elbow also flexed to 90
degrees. The examiner then stabilizes the upper
Subacute tendinitis (painful arc arm with one hand while using the other hand to
syndrome) internally rotate the arm fully. Pain around the
anterolateral aspect of the shoulder is noted as a
The patient develops anterior shoulder pain after positive test. As with the Neer sign, this test is
vigorous or unaccustomed activity, e.g. competitive highly sensitive but weakly specific.
swimming or a weekend of house decorating. The
shoulder looks normal but is acutely tender along the Subacute tendinitis is often reversible, settling
anterior edge of the acromion. Point tenderness is down gradually once the initiating activity is avoided.
most easily elicited by palpating this spot with the arm
held in extension, thus placing the supraspinatus ten-
don in an exposed position anterior to the acromion
process; with the arm held in flexion the tenderness
Chronic tendinitis
disappears. The patient, usually aged between 40 and 50, gives a
history of recurrent attacks of subacute tendinitis, the
TESTS FOR CUFF IMPINGEMENT PAIN pain settling down with rest or anti-inflammatory
• The painful arc: On active abduction scapulo- treatment, only to recur when more demanding activ-
humeral rhythm is disturbed and pain is aggravated ities are resumed.
(a) (b) (c)
13.7 Supraspinatus tenderness (a) The tender spot is at the anterior edge of the acromion
process. When the shoulder is extended (b) tenderness is more marked; with the shoulder slightly
flexed (c) the painful tendon disappears under the acromion process and tenderness disappears. 343
13
(a) (b) (c) (d)

13.8 The painful arc (a,b) In abduction, scapulo-humeral rhythm is disturbed on the right and the patient starts to
experience pain at about 60°. (c,d) As the arm passes beyond 120° the pain eases and the patient is able to abduct and
elevate up to the full 180°.
Characteristically pain is worse at night; the patient sively rotated; this may signify a partial tear or marked
cannot lie on the affected side and often finds it more fibrosis of the cuff. Small, unsuspected tears are quite
comfortable to sit up out of bed. Pain and slight stiff- often found during arthroscopy or operation.
ness of the shoulder may restrict even simple activities
such as hair grooming or dressing. The physical signs
Cuff disruption
described above should be elicited. In addition there
may be signs of bicipital tendinitis: tenderness along The most advanced stage of the disorder is progres-
the bicipital groove and crepitus on moving the biceps sive fibrosis and disruption of the cuff, resulting in
tendon. either a partial or full thickness tear. The patient is
A disturbing feature is coarse crepitation or palpable usually aged over 45 and gives a history of refractory
snapping over the rotator cuff when the shoulder is pas- shoulder pain with increasing stiffness and weakness.
(a) (b) (c) (d)
(e) (f) (g)
13.9 Torn supraspinatus (a–d) Partial tear of left supraspinatus: the patient can abduct actively once pain has been
abolished with local anaesthetic. (e–g) Complete tear of right supraspinatus: active abduction is impossible even when pain
subsides (f), or has been abolished by injection; but once the arm is passively abducted, the patient can hold it up with her
344 deltoid muscle (g).
Partial tears may occur within the substance or on In longstanding cases of partial or complete rupture, 13
the deep surface of the cuff and are not easily secondary osteoarthritis of the shoulder may super-
detected, even on direct inspection of the cuff. They vene and movements are then severely restricted.
are deceptive also in that continuity of the remaining
cuff fibres permits active abduction with a painful arc, TESTS FOR ISOLATED WEAKNESS
making it difficult to tell whether chronic tendinitis is The ‘abduction paradox’ and ‘drop arm sign’ are

complicated by a partial tear. helpful in the diagnosis of a complete rupture of the
A full thickness tear may follow a long period of cuff. For partial tears of the cuff, more subtle tests are
chronic tendinitis, but occasionally it occurs sponta- used to identify weakness in isolated components of
neously after a sprain or jerking injury of the shoulder. the cuff.
There is sudden pain and the patient is unable to
abduct the arm. Passive abduction also may, in the • Supraspinatus – the ‘empty can’ test (Jobe and Jobe,
early stages, be limited or prevented by pain. If the 1983): Supraspinatus strength can be tested in iso-
diagnosis is in doubt, pain can be eliminated by inject- lation as follows. The patient (seated or standing) is
ing a local anaesthetic into the subacromial space. If asked to raise his or her arms to a position of 90
active abduction is now possible the tear must be only degrees abduction, 30 degrees of forward flexion
partial. If active abduction remains impossible, then a and internal rotation (thumbs pointing to the floor,
complete tear is likely. as if emptying an imaginary can). The examiner
If some weeks have elapsed since the injury the two stands behind the patient and applies downward
types are more easily differentiated. With a complete pressure on both arms, with the patient resisting
tear, pain has by then subsided and the clinical pic- this force. The result is positive when the affected
ture is unmistakable: active abduction is impossible side is weaker than the unaffected side, suggesting
and attempting it produces a characteristic shrug; but a tear of the supraspinatus tendon.
passive abduction is full and once the arm has been • Infraspinatus – resisted external rotation: The patient
lifted above a right angle the patient can keep it up by stands holding his or her arms close to the body and
using his deltoid (the ‘abduction paradox’); when he the elbows flexed to 90 degrees. He or she is
lowers it sideways it suddenly drops (the ‘drop arm instructed to externally rotate both arms while the
sign’). examiner applies resistance; lack of power on one side
With time there may be some recovery of active signifies weakness of infraspinatus. The test can be re-
abduction, though power in both abduction and peated, this time with the arm in 90 degrees of for-
external rotation is weaker than normal. There is usu- ward elevation in the plane of the scapula. The pa-
ally wasting of the supraspinatus and infraspinatus, tient is asked to laterally rotate the arm against
and on testing the biceps there may be an old tear of resistance; the ability to do so despite feeling pain can
the long head tendon (see Fig. 13.10). There is often indicate tendinitis whilst an inability to resist at all
tenderness of the acromioclavicular joint. suggests a tear of infraspinatus.
• Infraspinatus and posterior cuff – the ‘lag sign’ and
Degeneration the ‘drop sign’: For the external rotation lag sign the
patient’s arms are lifted slightly away from the body
and placed in maximum external rotation; a positive
Impingement test is signalled when the patient cannot maintain
that position on one side and allows the arm to drift
into a more neutral position. This suggests a tear of
infraspinatus or supraspinatus. The drop sign is sim-
Osteoarthritis ilar: here the examiner lifts and places the arm in 90
degrees of abduction, the elbow at a right angle and
the arm maximally externally rotated; when the
Swelling Trauma examiner lets go the patient would normally hold
that position, but if the arm ‘drops’ it signals a pos-
itive test (Hertel et al., 1996). This is seen in
Cuff patients with tears of the infraspinatus and posterior
disruption part of the rotator cuff.
• Subscapularis – ‘the lift-off’ test: The patient is asked
to stand and place one arm behind his or her back
with the dorsum of the hand resting against the mid-
Vascular lumbar spine. The examiner then lifts the patient’s
reaction hand off the back and the patient is told to hold it
13.10 The vicious spiral of rotator cuff lesions there. Inability to do this signifies subscapularis 345
13
(a) (b) (c) (d)
13.11 Tests for cuff weakness (a) Position for Jobes’ test for supraspinatus power. (b) Jobe’s test – right side weaker
than left. (c) Test for infraspinatus – right side weaker than left. (d) Normal ‘lift-off’ test for subscapularis.
weakness, possibly due to rupture (Gerber and valuable ancillary information (regarding lesions of
Krushell, 1991). A drawback is that the test calls for the glenoid labrum, joint capsule or surrounding
full passive internal rotation, so it cannot be used if in- muscle or bone). However, it should be remembered
ternal rotation is painful or restricted. that up to a third of asymptomatic individuals have
abnormalities of the rotator cuff on MRI (Sher et al.,
1995). Changes on MRI need to be correlated with
IMAGING FOR ROTATOR CUFF DISORDERS the clinical examination.
Ultrasonography Ultrasonography has comparable
X-ray examination X-rays are usually normal in the
accuracy with MRI for identifying and measuring the
early stages of the cuff dysfunction, but with chronic
size of full thickness and partial thickness rotator cuff
tendinitis there may be erosion, sclerosis or cyst for-
tears (Teefe et al., 2004). It has the disadvantage that
mation at the site of cuff insertion on the greater
it cannot identify the quality of the remaining muscle
tuberosity. In chronic cases the caudal tilt view may
as well as MRI and cannot always be accurate in pre-
show roughening or overgrowth of the anterior edge
dicting the reparability of the tendons.
of the acromion, thinning of the acromion process
and upward displacement of the humeral head.
Osteoarthritis of the acromioclavicular joint is com-
mon in older patients and in late cases the gleno- TREATMENT OF CUFF DISORDERS
humeral joint also may show features of osteoarthritis.
Sometimes there is calcification of the supraspinatus, Conservative treatment
but this is usually coincidental and not the cause of
Uncomplicated impingement syndrome (or tendini-
pain (see Fig. 13.12).
tis) is often self-limiting and symptoms settle down
Magnetic resonance imaging MRI effectively demon- once the aggravating activity is eliminated. Patients
strates the structures around the shoulder and gives should be taught ways of avoiding the ‘impingement
(a) (b) (c)
13.12 Supraspinatus tendinitis – x-rays (a) X-ray of the shoulder showing a typical thin band of
sclerosis at the insertion of supraspinatus and narrowing of the subacromial space. The rest of the joint
looks normal. (b) X-ray at a later stage showing upward displacement of the humeral head due to a large
cuff rupture. There is almost complete loss of the subacromial space, and osteoarthritis of the gleno-
humeral joint. (c) MRI showing thickening of the supraspinatus and erosion at its insertion; the
346 acromioclavicular joint is swollen and clearly abnormal.
13.13 Rotator conservative treatment, or if they recur persistently 13
cuff tear – after each period of treatment, an operation is advis-
MRI High able. Certainly this is preferable to prolonged and
signal on MRI,
indicating a repeated treatment with anti-inflammatory drugs and
full-thickness local corticosteroids. The indication is more pressing
tear of the if there are signs of a partial rotator cuff tear and in

rotator cuff. particular if there is good clinical evidence of a full
thickness tear in a younger patient. The object is to
decompress the rotator cuff by excising the coraco-
acromial ligament, undercutting the anterior part of
the acromion process and, if necessary, reducing any
bony excrescences at the acromioclavicular joint
(Rockwood and Lyons, 1993). This can be achieved
by open surgery or arthroscopically. The latter is tech-
nically more demanding but it can produce results
equivalent to those of open surgery (Sachs et al.,
1994; Nutton et al., 1997).
OPEN ACROMIOPLASTY
position’. Physiotherapy, including ultrasound and Through an anterior incision the deltoid muscle is
active exercises in the ‘position of freedom’, may tide split and the part arising from the anterior edge of the
the patient over the painful healing phase. A short acromion is dissected free, exposing the coracoacro-
course of non-steroidal anti-inflammatory tablets mial ligament, the acromion and the acromioclavicu-
sometimes brings relief. If all these methods fail, and lar joint. The coracoacromial ligament is excised and
before disability becomes marked, the patient should the anteroinferior portion of the acromion is removed
be given one or two injections of depot corticosteroid by an undercutting osteotomy. The cuff is then
into the subacromial space. In most cases this will inspected: if there is a defect, it is repaired. Excres-
relieve the pain, and it is then important to persevere cences on the undersurface of the acromioclavicular
with protective modifications of shoulder activity for joint are pared down. If the joint is hypertrophic, the
at least 6 months. Healing is slow, and a hasty return outer 1 cm of clavicle is removed; this last step
to full activity will often precipitate further attacks of exposes even more of the cuff and permits recon-
tendinitis. struction of larger defects. An important step is care-
ful reattachment of the deltoid to the acromion, if
Surgical treatment necessary by suturing through drill holes in the
acromion; failure to obtain secure attachment may
The indications for surgical treatment are essentially lead to postoperative pain and weakness. After the
clinical; the presence of a cuff tear does not necessar- operation, shoulder movements are commenced as
ily call for an operation. Provided the patient has a soon as pain subsides.
useful range of movement, adequate strength and
well-controlled pain, non-operative measures are ade- ARTHROSCOPIC ACROMIOPLASTY
quate. If symptoms do not subside after 3 months of Arthroscopic acromioplasty should achieve the same
basic objectives as open acromioplasty (Nutton et al.,
1997). The underside of the acromion (and, if neces-
13.14 Impingement sary, the acromioclavicular joint) must be trimmed
syndrome – surgical
and the coracoacromial ligament divided or removed.
treatment The coraco-
acromial ligament and If a cuff tear is encountered, then it may be possible
underside of the to repair it; otherwise the edges can be debrided or an
anterior third of the open repair undertaken (Gartsman, 1997).
acromion are removed This procedure has now become the gold standard
to enlarge the space for
and allows earlier rehabilitation than open acromio-
the rotator cuff. This
can be performed by plasty because detachment of the deltoid is not per-
open surgery or formed. Arthroscopy allows good visualization inside
arthroscopically. the gleno-humeral joint and therefore the detection
of other abnormalities which may cause pain (present
in up to 30 per cent of patients). It allows good
visualization of both sides of the rotator cuff and the
identification of partial and full thickness tears. 347
13 OPEN REPAIR OF THE ROTATOR CUFF the supraspinatus tendon slightly medial to its
The indications for open repair of the rotator cuff are insertion, occasionally elsewhere in the rotator cuff.
chronic pain, weakness of the shoulder and significant The condition is not unique to the shoulder, and sim-
loss of function. The younger and more active the ilar lesions are seen in tendons and ligaments around
patient, the greater is the justification for surgery. The the ankle, knee, hip and elbow.
operation always includes an acromioplasty as The cause is unknown but it is thought that local
described above. The cuff is mobilized, if necessary by ischaemia leads to fibrocartilaginous metaplasia and
releasing the coraco-humeral ligament and the gle- deposition of crystals by the chondrocytes. Calcifica-
noid attachment of the capsule; this dissection should tion alone is probably not painful; symptoms, when
not stray more than 2 cm medial to the glenoid rim they occur, are due to the florid vascular reaction
lest the suprascapular nerve is damaged. which produces swelling and tension in the tendon.
It may be possible to approximate the ends of the cuff Resorption of the calcific material is rapid and it may
defect. Larger tears can be dealt with by suturing the soften or disappear entirely within a few weeks.
cuff tendon directly to a roughened area on the greater
tuberosity using drill holes or soft-tissue anchors.
Clinical features
Postoperatively, movements are restricted for 6–8
weeks and then graded exercises are introduced. The condition affects 30–50 year-olds. Aching, some-
The results of open cuff repair are reasonably good, times following overuse, develops and increases in
with satisfactory pain relief in about 80 per cent of severity within hours, rising to an agonizing climax.
patients. This alone usually improves function, even if After a few days, pain subsides and the shoulder grad-
strength and range of movement are still restricted ually returns to normal. In some patients the process
(Ianotti, 1994). is less dramatic and recovery slower. During the acute
Massive full thickness tears that cannot be recon- stage the arm is held immobile; the joint is usually too
structed are treated by subacromial decompression and tender to permit palpation or movement.
debridement of degenerate cuff tissue; the relief of
pain may allow reasonable abduction of the shoulder by
the remaining muscles (Rockwood et al., 1995). Other
methods to reconstruct irreparable tears in the younger X-RAYS
patient include supraspinatus advancement, latissimus
dorsi transfer, rotator cuff transposition, fascia lata au- Calcification just above the greater tuberosity is
tograft and synthetic tendon graft. always present. An initially well-demarcated deposit
Acute rupture of the rotator cuff in patients over 70 becomes more ‘woolly’ and then disappears.
years usually becomes painless; although movement is
restricted, operation is contraindicated.
Treatment
ARTHROSCOPIC ROTATOR CUFF REPAIR NON-OPERATIVE TREATMENT
Since the 1990s the repair of full thickness tears has un- Conservative treatment is successful in up to 90 per
dergone a transition from open techniques to arthro- cent of patients. The main methods are non-steroidal
scopically assisted (mini open) repairs and now full anti-inflammatory drugs, subacromial injection of
arthroscopic techniques. The arthroscopic instruments, corticosteroids, physiotherapy, extracorporeal shock-
suture anchors and knot tying techniques have quickly wave therapy, needle aspiration and irrigation.
evolved to allow full arthroscopic repairs although most Non-steroidal anti-inflammatory drugs are the
authors describe a steep learning curve. Advantages of the mainstay of non-operative treatment. Although corti-
techniques include less soft-tissue damage, faster reha- costeroid injections are commonly used in the treat-
bilitation and a better cosmetic appearance. Double row ment of calcifying tendinitis, there is no conclusive
arthroscopic repair is now producing similar outcomes evidence that they promote resorption of the calcium
and results to open repairs (Huijsmans et al., 2007). deposit. The efficacy of physiotherapy in the form of
therapeutic ultrasound remains uncertain.
Extracorporeal shockwave therapy employs acoustic
waves to induce fragmentation of the mechanically
CALCIFICATION OF THE ROTATOR
hard crystals. Its use as an alternative treatment for
CUFF calcifying tendinitis has gained increasing popularity
in the last few years and its efficacy has been con-
ACUTE CALCIFIC TENDINITIS firmed in several prospective studies which show that
the deposit disappears in up to 86 per cent of cases
Acute shoulder pain may follow deposition of calcium with a significant reduction in pain. However, most of
348 hydroxyapatite crystals, usually in the ‘critical zone’ of these studies have only a short-term follow-up.
13

(a) (b) (c)
13.15 Acute calcification of

supraspinatus (a) Dense mass in the
tendon. (b) Following the ‘reaction’ some
calcium has escaped into the subdeltoid
bursa; (c) spontaneous dispersal. (d) An
attempt at treatment by aspiration; this
procedure is much more likely to succeed
if image-intensification and ultrasound
control are used.
(d)
Needle aspiration and irrigation (barbotage) aims

to drain a substantial portion of the calcium deposit,
LESIONS OF THE BICEPS TENDON
thereby stimulating cell-mediated progressive resorp-
tion. Needle aspiration can be readily done under Tendinitis
local anaesthesia in the outpatient setting with ultra- The long head of biceps is subject to tenosynovitis
sound guidance. A combination of local anaesthetic because of its anatomy; the tendon has a synovial sheath
and corticosteroid is used. The best results are and follows a constrained path in the bicipital groove.
obtained in patients with an acutely painful shoulder, Bicipital tendinitis usually occurs together with
typically during the resorption stage in which the cal- rotator cuff impingement; rarely, it presents as an iso-
cium is of toothpaste-like consistency. lated problem in young people after unaccustomed
shoulder strain. Tenderness is sharply localized to the
OPERATIVE TREATMENT bicipital groove. Two manoeuvres that often cause
While operative treatment is still a controversial issue, pain are: (1) resisted flexion with the elbow straight
there is wide agreement that surgery is indicated for and the forearm supinated (Speed’s test); and (2)
patients with severe disabling symptoms which have resisted supination of the forearm with the elbow bent
persisted for more than 6 months and are resistant to (Yergason’s test).
conservative treatment. The procedure involves a Rest, local heat and deep transverse friction usually
gleno-humeral arthroscopy with special attention to bring relief. If recovery is delayed, a corticosteroid
the ‘critical zone’ of the rotator cuff. Once the cal- injection will help. For refractory cases, a number of
cium deposit is identified, the capsule is carefully surgical solutions have been described including
incised from the bursal side with a knife in line with arthroscopic decompression, biceps tenotomy and
fibre orientation of the tendon; a curette is then used biceps tenodesis.
to milk out the toothpaste-like deposit. A subacromial
decompression is also usually performed.
Rupture
CHRONIC CALCIFICATION Rupture of the tendon of the long head of biceps usu-
ally accompanies rotator cuff disruption, but some-
Asymptomatic calcification of the rotator cuff is com- times the biceps lesion is paramount. The patient is
mon and often appears as an incidental finding in usually aged over 50. While lifting he or she feels
shoulder x-rays. When it is seen in association with the something snap in the shoulder and the upper arm
impingement syndrome, it is tempting to attribute the becomes painful and bruised. Ask the patient to flex
symptoms to the only obvious abnormality – the elbow: the detached belly of the biceps forms a
supraspinatus calcification. However, the connection prominent lump in the lower part of the arm.
is spurious and treatment should be directed at the Isolated tears in elderly patients need no treatment.
impingement lesion rather than the calcification. However, if the rupture is part of a rotator cuff lesion 349
13 hand) can damage the superior labrum anteriorly and
posteriorly (SLAP). The injury of the superior labrum
begins posteriorly and extends anteriorly, stopping
before or at the mid-glenoid notch and including the
‘anchor’ of the biceps tendon to the labrum. Four
main types are described:
1. non-traumatic superior labral degeneration,

usually in older people and often asymptomatic;
2. avulsion of the superior part of the labrum – the
commonest type (Fig. 3.17);
3. a ‘bucket handle’ tear of the superior labrum;
4. as for type 3 with an extension into the tendon of
long head of biceps.
13.16 Ruptured long head of biceps The lump in the
front of the arm becomes even more prominent when the Further subtypes that include associated lesions
patient contracts the biceps against resistance.
have also been described.
– and especially if the patient is young and active – this

is an indication for anterior acromioplasty; at the same
time the distal tendon stump can be sutured to the
Biceps tendon
bicipital groove (biceps tenodesis). Postoperatively
the arm is lightly splinted with the elbow flexed for 4
Coracoid
weeks. (Avulsion of the distal attachment of the biceps Acromion process
is discussed in Chapter 14.)
Hypertrophy and intra-articular

entrapment (the hourglass biceps)
The biceps tendon sometimes hypertrophies, e.g. in Spine of scapula
association with advanced disease of the rotator cuff,
Labrum Glenoid fossa
and is unable to slide into the bicipital groove. This
causes buckling of the tendon on elevation of the Capsule
shoulder with entrapment of the tendon between the
humeral head and glenoid, leading to pain and a block
to terminal elevation. (a)
Instability Labral
detachment
Both subluxations and dislocations of the long head
of biceps have been described. Subluxation is defined
as a partial and/or transient loss of contact between
the tendon and its groove. Dislocation is the complete
and permanent loss of contact between the tendon
and the groove; it is usually classified into intra-
articular, intra-tendinous and extra-articular subtypes.
Dislocation is nearly always associated with a tear of
subscapularis, except in the rare cases of extra-articular
dislocation in which the tendon is resting anterior to
subscapularis.
(b)
13.17 SLAP lesions (a) Diagram of the normal anatomy,

SLAP LESIONS looking into the glenoid fossa. Note that the biceps tendon
takes its origin from the superior part of the labrum.
(b) The labrum may tear or become detached from the
Compressive loading of the shoulder in the flexed glenoid. This illustration shows a partial tear. Other types
350 abducted position (e.g. in a fall on the outstretched are described in the text.
13.18 SLAP lesions Treatment 13
Arthroscopic appearance
of a type III SLAP lesion. Very few patients with SLAP lesion injuries return to
full capability without surgical intervention. Arthro-
scopic repair of an isolated superior labral lesion is suc-
cessful in the majority (91 per cent) of patients.

However, the results in patients who participate in
overhead sports are not as satisfactory as those in
patients who are not involved in overhead sports
(Seung-Ho Kim et al., 2002)
Clinical features
There is usually a history of a fall on the arm. As the ADHESIVE CAPSULITIS (FROZEN
initial acute symptoms settle, the patient continues to SHOULDER)
experience a painful ‘click’ on lifting the arm above
shoulder height, together with loss of power when
The term ‘frozen shoulder’ should be reserved for a
using the arm in that position. He or she may also
well-defined disorder characterized by progressive
complain of an inability to throw.
pain and stiffness of the shoulder which usually
O’Briens test The patient is instructed to flex the arm resolves spontaneously after about 18 months. The
to 90 degrees with the elbow fully extended and then cause remains unknown. The histological features are
to adduct the arm 10–15 degrees medial to the sagit- reminiscent of Dupuytren’s disease, with active
tal plane. The arm is then maximally internally rotated fibroblastic proliferation in the rotator interval, ante-
and the patient resists the examiner’s downward force. rior capsule and coraco-humeral ligament (Bunker,
The procedure is repeated in supination. Pain elicited 1997. The condition is particularly associated with
by the first manoeuvre which is reduced or eliminated diabetes, Dupuytren’s disease, hyperlipidaemia,
by the second signifies a positive test. hyperthyroidism, cardiac disease and hemiplegia. It
occasionally appears after recovery from neurosurgery.
Imaging
Clinical features
MRI is the modality of choice though the diagnosis is
best confirmed by arthroscopic examination and at The patient, aged 40–60, may give a history of
the same time the lesion is treated by debridement or trauma, often trivial, followed by aching in the arm
repair. and shoulder. Pain gradually increases in severity and
13.19 Frozen
shoulder
(a) Natural history of
frozen shoulder. The
face tells the story.
(b,c) This patient has
hardly any abduction
but manages to lift her
arm by moving the
(b) scapula. She cannot
reach her back with
her left hand.
(c)
(a) 351
13 often prevents sleeping on the affected side. After 13.20 Shoulder pain – the
scratch test ‘Shoulder’ pain
several months it begins to subside, but as it does so
stiffness becomes an increasing problem, continuing may be due to disorders of the
shoulder joint itself (e.g.
for another 6–12 months after pain has disappeared. gleno-humeral arthritis), the
Gradually movement is regained, but it may not acromioclavicular joint (injury
return to normal and some pain may persist. or arthritis) or structures
Apart from slight wasting, the shoulder looks quite around the joint (e.g. the
normal; tenderness is seldom marked. The cardinal rotator cuff syndromes). But it
could also be referred from
feature is a stubborn lack of active and passive move- more distant lesions (e.g.
ment in all directions. brachial neuralgia, cervical
X-rays are normal unless they show reduced bone spondylosis or cardiac
density from disuse. Their main value is to exclude (a) ischaemia). If the patient can
other causes of a painful, stiff shoulder. scratch the opposite scapula in
these three ways, the shoulder
joint and its tendons are
Diagnosis unlikely to be at fault.
Not every stiff or painful shoulder is a frozen shoul-

der, and indeed there is some controversy over the cri-
teria for diagnosing ‘frozen shoulder’ (Zuckerman et
al., 1994). Stiffness occurs in a variety of conditions –
arthritic, rheumatic, post-traumatic and postopera-
tive. The diagnosis of frozen shoulder is clinical, rest-
ing on two characteristic features: (1) painful
restriction of movement in the presence of normal x- (b)
rays; and (2) a natural progression through three suc-
cessive phases.
When the patient is first seen, a number of condi-
tions should be excluded:
Infection In patients with diabetes, it is particularly
important to exclude infection. During the first day or
two, signs of inflammation may be absent.
Post-traumatic stiffness After any severe shoulder
injury, stiffness may persist for some months. It is
maximal at the start and gradually lessens, unlike the
pattern of a frozen shoulder.
(c)
Diffuse stiffness If the arm is nursed over-cautiously
(e.g. following a forearm fracture) the shoulder may
stiffen. Again, the characteristic pattern of a frozen
Exercises are encouraged, the most valuable being
shoulder is absent.
‘pendulum’ exercises in which the patient leans for-
Reflex sympathetic dystrophy Shoulder pain and stiff- ward at the hips and moves his arm as if stirring a
ness may follow myocardial infarction or a stroke. The giant pudding (this is really a form of assisted active
features are similar to those of a frozen shoulder and movement, the assistance being supplied by gravity).
it has been suggested that the latter is a form of reflex However, the patient is warned that moderation and
sympathetic dystrophy. In severe cases the whole regularity will achieve more than sporadic masochism.
upper limb is involved, with trophic and vasomotor The role of physiotherapy is unproven and the bene-
changes in the hand (the ‘shoulder–hand syndrome’). fits of steroid injection are debatable.
Manipulation under general anaesthesia may
improve the range of movement. The shoulder is
Treatment
moved gently but firmly into external rotation, then
CONSERVATIVE TREATMENT abduction and flexion. Special care is needed in eld-
Conservative treatment aims to relieve pain and pre- erly, osteoporotic patients as there is a risk of fractur-
vent further stiffening while recovery is awaited. It is ing the neck of the humerus. At the end, the joint is
important not only to administer analgesics and anti- injected with methylprednisolone and lignocaine. An
inflammatory drugs but also to reassure the patient alternative method of treatment is to distend the joint
352 that recovery is certain. by injecting a large volume (50–200 mL) of sterile
Table 13.1 The painful shoulder physiological range and which is asymptomatic. Joint 13
instability is an abnormal symptomatic motion for
Referred pain syndromes Rotator cuff disorders that shoulder which results in pain, subluxation or dis-
Cervical spondylosis Tendinitis
Mediastinal pathology Rupture
location of the joint.
Cardiac ischaemia Frozen shoulder Dislocation is defined as complete separation of the
gleno-humeral surfaces, whereas subluxation implies a

Joint disorders Instability
Gleno-humeral arthritis Dislocation symptomatic separation of the surfaces without dislo-
Acromioclavicular arthritis Subluxation cation.
Bone lesions Nerve injury
Infection Suprascapular nerve entrapment Pathogenetic classification
Tumours
The aetiology and classification of shoulder instability
is complex, although the Stanmore Instability Classi-
fication system developed at the Royal National
saline under pressure. Arthroscopy has shown that Orthopaedic Hospital in London is now increasingly
both manipulation and distension achieve their effect used. It recognizes that there are two broad reasons
by rupturing the capsule. why shoulders become unstable: (1) structural
The results of conservative treatment are subjec- changes due to major trauma such as acute dislocation
tively good, most patients eventually regaining pain- or recurrent micro-trauma; and (2) unbalanced mus-
less and satisfactory function; however, examination is cle recruitment (as opposed to muscle weakness)
likely to show some residual restriction of movement resulting in the humeral head being displaced upon
(especially external rotation) in over 50 per cent of the glenoid.
cases (Shaffer et al., 1992). From a clinical and therapeutic point of view, three
Most studies on outcome are small. In the largest of polar types of disorder can be identified:
these, Hand et al. (2008) reported on patients who
Type I Traumatic structural instability.
were followed up for a mean of 4.4 years: 59 per cent
Type II Atraumatic (or minimally traumatic)
had normal or near-normal shoulders, and of the
structural instability.
remainder 94 per cent had only mild symptoms.
Type III Atraumatic non-structural instability
(muscular dyskinesia).
SURGICAL TREATMENT
Surgery does not have a well-defined role. The main The triangular relationship between these condi-
indication is prolonged and disabling restriction of tions allows for the fact there are intermediate types
movement which fails to respond to conservative that lie between the ‘poles’; the balance of abnormal-
treatment. ities can shift and patients may ‘move’ from one group
Arthroscopic capsular release is increasingly to another over time or present with a combination of
employed. New techniques enable the surgeon to pathologies: for example, a purely structural disorder
release intra-articular, subacromial and subdeltoid which, if allowed to persist, becomes associated with
adhesions without dividing the subscapularis. Active abnormal muscle patterning to the extent that both
range of motion can be started immediately (Harry-
man et al., 1997).
Polar Type I
Traumatic
Structural
INSTABILITY OF THE SHOULDER
The shoulder achieves its uniquely wide range of Less
movement at the cost of stability. The humeral head is Muscle
held in the shallow glenoid socket by the glenoid Patterning
labrum, the gleno-humeral ligaments, the coraco-
humeral ligament, the overhanging canopy of the
coracoacromial arch and the surrounding muscles.
Failure of any of these mechanisms may result in insta-
bility of the joint. Polar Type III Polar Type II
One must distinguish between joint laxity and joint Muscle Patterning Less Atraumatic
instability. Joint laxity implies a degree of translation Non-Structural Trauma Structural
in the gleno-humeral joint which falls within a 353
13 13.21 Shoulder instability – the
apprehension test (a) This is the
apprehension test for anterior
subluxation or dislocation. Abduct,
externally rotate and extend the patient’s
shoulder while pushing on the head of
the humerus. If the patient feels that the
joint is about to dislocate, she will

forcibly resist the manoeuvre.
(b) Posterior dislocation can be tested
for in the same way by drawing the arm
forward and across the patient’s body
(adduction and internal rotation).
(a) (b)
Table 13.2 Pathological changes in each of the polar absent, although the inferior gleno-humeral ligament
types will be stretched. In patients over the age of 50, dis-
Pathology Group I Group II Group III
location is often associated with tears of the rotator
cuff.
Trauma Yes No No
Articular Yes Yes No
surface Clinical features
damage
The patient is usually a young man or woman who
Capsular Bankart lesion Dysfunctional Dysfunctional gives a history of the shoulder ‘coming out’, perhaps
problem
during a sporting event. The first episode of acute dis-
Laxity Unilateral Uni/bilateral Often bilateral location is a landmark and he or she may be able to
Muscle Normal Normal Abnormal describe the mechanism precisely: an applied force
patterning with the shoulder in abduction, external rotation and
extension. The diagnosis may have been verified by x-
ray and the injury treated by closed reduction and
conditions need to be treated and the problems grow ‘immobilization’ in a bandage or sling for several
in complexity. The system also recognizes that there is weeks. This may be the first of many similar episodes:
a gradation in the opposite direction, from dyskinetic recurrent dislocation requiring treatment develops in
muscle patterning to structural abnormality (Lewis, about one-third of patients under the age of 30 and in
Kitamura and Bayley, 2004). about 20 per cent of older patients, with an overall
redislocation rate of 48 per cent (Hovelius et al.,
1996). Some studies have reported instability rates
following acute dislocation between 88 per cent and
TRAUMATIC ANTERIOR INSTABILITY 95 per cent in patients under the age of 20. A greater
– POLAR TYPE I proportion have instability without actual dislocation.
Recurrent subluxation Symptoms and signs of recur-
PATHOLOGY rent subluxation are less obvious. The patient may
This is far and away the commonest type of instability, describe a ‘catching’ sensation, followed by ‘numb-
accounting for over 95 per cent of cases. Traumatic ness’ or ‘weakness’ – the so-called ‘dead arm syn-
anterior instability usually follows an acute injury in drome’ – whenever the shoulder is used with the arm
which the arm is forced into abduction, external rota- in the overhead position (e.g. throwing a ball, serving
tion and extension. In recurrent dislocation the at tennis or swimming). Pain with the arm in abduc-
labrum and capsule are often detached from the ante- tion may suggest a rotator cuff syndrome; it is as well
rior rim of the glenoid (the classic Bankart lesion). In to remember that recurrent subluxation may actually
addition there may be an indentation on the postero- cause supraspinatus tendinitis.
lateral aspect of the humeral head (the Hill–Sachs On examination, between episodes of dislocation,
lesion), a compression fracture due to the humeral the shoulder looks normal and movements are full.
head being forced against the anterior glenoid rim Clinical diagnosis rests on provoking subluxation. In
each time it dislocates. In some cases recurrent sub- the apprehension test, with the patient seated or lying,
luxation may alternate with recurrent dislocation. In the examiner cautiously lifts the arm into abduction,
other cases the shoulder never dislocates completely external rotation and then extension; at the crucial
354 and in these the labral tear and bone defect may be moment the patient senses that the humeral head is
about to slip out anteriorly and his or her body taut- Treatment 13
ens in apprehension. The test should be repeated with
the examiner applying pressure to the front of the If dislocation recurs at long intervals, the patient may
shoulder; with this manoeuvre, the patient feels more choose to put up with the inconvenience and simply
secure and the apprehension sign is negative. try to avoid vulnerable positions of the shoulder.
The same effect can be demonstrated by the ful- There is some evidence that dislocation predisposes to

crum test. With the patient lying supine, arm abducted osteoarthritis, although it is probably the initial dislo-
to 90 degrees, the examiner places one hand behind cation rather than recurrence that causes this (Hov-
the patient’s shoulder to act as a fulcrum over which elius et al., 1996).
the humeral head is levered forward by extending and
laterally rotating the arm; the patient immediately OPERATIVE TREATMENT
becomes apprehensive. The indications for operation include frequent dislo-
If instability is marked the drawer test may be posi- cation, especially if this is painful, and recurrent sub-
tive (see Fig. 13.23). With the patient supine, the luxation or a fear of dislocation sufficient to prevent
scapula is stabilized with one hand while the upper participation in everyday activities, including sport.
arm is grasped firmly with the other so as to manipu- There is growing evidence to support primary surgery
late the head of the humerus forwards and backwards in young adults engaged in highly demanding physi-
(like a drawer). cal activities following first acute traumatic dislocation
(Handoll et al., 2004). Two types of operation are
employed:
Investigations Anatomical repairs These are operations that repair
the torn glenoid labrum and capsule, e.g. the Bankart
Most cases can be diagnosed from the history and
procedure (Bankart, 1939; Gill et al., 1997).
examination alone. The Hill–Sachs lesion (when it is
present) is best shown by an anteroposterior x-ray with Non-anatomical repairs These procedures are designed
the shoulder internally rotated, or in the axillary view. to counteract the pathological tendency to joint dis-
Subluxation is seen in the axillary view. placement: (a) operations that shorten the anterior
MRI or MR arthrography is useful for demonstrat- capsule and subscapularis by an overlapping repair
ing bone lesions and labral tears. (e.g. the Putti–Platt operation); (b) operations that re-
Arthroscopy is sometimes needed to define the labral inforce the anteroinferior capsule by redirecting other
tear. muscles across the front of the joint (e.g. the Bristow–
Examination under anaesthesia can help to deter- Laterjet operation); and (c) a bone operation to cor-
mine the direction of instability. This forms an essen- rect a reduced retroversion angle of the humeral head
tial part of assessing instability. Both shoulders need by osteotomy (Kronberg and Brostrum, 1995).
to be examined. Reports have demonstrated sensitivi- The Putti–Platt operation, in which the subscapu-
ties and specificities of 100 per cent and 93 per cent, laris is overlapped and shortened, prevents redisloca-
respectively. tion but at the cost of significant loss of external
(a) (b) (c)
13.22 Anterior instability – imaging (a) The plain x-ray shows a large depression in the posteriosuperior part of the
humeral head (the Hill–Sachs sign). (b,c) MRI shows both a Bankart lesion, with a flake of bone detached from the anterior
edge of the glenoid, and the Hill–Sachs lesion (arrows). 355
13 rotation. It is now not commonly used. The Bristow– They develop symptoms of instability due to overload
Laterjet operation, in which the coracoid process with and fatigue in the stabilizing muscles of the shoulder;
its attached muscles is transposed to the front of the dislocation may occur in several different directions. It
neck of the scapula, produces less restriction of exter- is doubly important in these cases to rule out the pres-
nal rotation (Singer et al., 1995). In general, non- ence of any pathological condition, such as a labral le-
anatomical operations are now thought to have a sion, and to assess whether there is any contributory el-
limited role in the management of shoulder instability. ement of abnormal muscle patterning.
They do not address the underlying pathological
changes and they are often associated with an unac-
Treatment
ceptable loss of function. Reports of recurrent insta-
bility of 20 per cent, loss of external rotation and REHABILITATIVE MEASURES
late-onset degenerative joint disease are common. Dedicated physiotherapy is focused on strengthening
If the labrum and anterior capsule are detached, the muscles normally involved in stabilizing the shoul-
and there is no marked joint laxity, the Bankart oper- der and restoring muscular coordination and control.
ation combined with anterior capsulorrhaphy is the Associated problems of muscle patterning are also
procedure of choice. The labrum is re-attached to the addressed and patients may need special instruction in
glenoid rim with suture anchors or drill holes and, if the kinematics of shoulder movements and control of
necessary, the capsule is tightened by an overlapping stability, as well as advice about modification of phys-
tuck without shortening the subscapularis. Bankart ical activities.
initially described this as an open operation through
the deltopectoral approach; however, arthroscopic SURGICAL TREATMENT
techniques have been developed with advanced If rehabilitative measures fail to reduce the problem
anchor materials and the development of specialized and the patient is genuinely incapacitated operative
arthroscopic instruments. With careful patient selec- treatment may be required – usually some type of cap-
tion clinical outcomes and recurrence rates of arthro- sular plication (which can be performed arthroscopi-
scopic and open stabilization are now comparable; cally) or a capsular shift (by open operation) (Neer
however, after either type of operation there is still a and Foster, 1980).
significant recurrence rate (about 20 per cent), usually
following another injury (Cole et al., 2000). If there
is bone loss on either the glenoid aspect or the
humeral head the outcome following arthroscopic
surgery is considerably worse (Boileau et al., 2006).
ATRAUMATIC OR MINIMALLY
TRAUMATIC INSTABILITY – POLAR
TYPES II AND III
The terminology of these groups is somewhat confus-

ing: ‘atraumatic instability’ can include entities such (a)
as the ‘loose shoulder’, multidirectional instability,
voluntary dislocation and habitual dislocation. In
these cases it is often difficult to decide whether the
problem is ‘structural’ or ‘non-structural’.
ATRAUMATIC STRUCTURAL INSTABILITY

This is an acquired multidirectional instability due
either to repetitive micro-trauma which has placed
undue stress upon the soft tissues or to rapid, forceful
movements that contribute to the development of (b)
overall laxity of the joint; occasionally a predisposing
13.23 Multidirectional instability (a) The anterior and
factor such as glenoid dysplasia is identified. (b) posterior drawer tests are best performed with the
Atraumatic structural instability is a recognized prob- patient lying supine. The amount of movement is
356 lem in athletes, particularly swimmers and throwers. compared with that on the unaffected side.
ATRAUMATIC NON-STRUCTURAL 13
INSTABILITY (ALTERED MUSCLE
PATTERNING)
The stability of the shoulder joint throughout its large

range of motion comes partly from precise synchro-
nized muscle contractions and relaxations during
movement. Each of the muscles moving and stabiliz-
ing the shoulder needs to be activated at a specific
time in coordination with other protagonistic and
antagonistic muscles. If this pattern is altered instabi-
lity can occur.
Muscle patterning instability usually occurs
in younger patients who can voluntarily slip the shoul- (a) (b)
der out of joint as a trick movement (habitual), but 13.25 Inferior subluxation (a) X-ray of a young woman
may then go on to dislocate repeatedly (uncontrolled who developed ‘clicking’ and instability in the right
or involuntary dislocation). shoulder after recovering from an injury to the neck and
right upper limb. Plain x-ray examination showed no
abnormality, but when the anteroposterior view was
Treatment repeated with the patient carrying 15 kg weights in both
hands, subluxation due to laxity of the anteroinferior
The aim is to regain normal neuromuscular control capsule was demonstrated to the right side (b).
and patterning. This can be difficult, time consuming
and require the participation of a full team comprising
a specialist shoulder physiotherapist, shoulder surgeon head of the humerus lying below the glenoid socket
and sometimes an occupational therapist and a psy- on the affected side (Fig. 13.25). The condition is due
chologist. Treatment follows much the same lines as to (temporary) weakness of the shoulder muscles,
for atraumatic structural instability but surgery should usually because of prolonged splintage of the arm and
be avoided if possible. lack of exercise.
The condition usually corrects itself after a period
of normal muscular activity, but physiotherapy will
INFERIOR SUBLUXATION help to speed up the process. In the occasional case,
tissue laxity is more persistent and capsular reefing
Some weeks after an injury to the shoulder girdle a may be advisable.
patient sometimes develops a feeling of instability in
the shoulder, as if it ‘slips out of joint’, particularly
when carrying something heavy with that arm. X-ray
examination of the shoulder may show that the head POSTERIOR INSTABILITY
of the humerus has subluxated inferiorly; if this is not
immediately apparent, further views with the patient
carrying a 10 kg weight in each hand will show the Pathology
This condition is usually due to a violent jerk in an
unusual position or following an epileptic fit or a
severe electric shock. Dislocation may be associated
with fractures of the proximal humerus, the posterior
capsule is stripped from the bone or stretched, and
there may be an indentation on the anterior aspect of
the humeral head. Recurrent instability is almost
always a posterior subluxation with the humeral head
riding back on the posterior lip of the glenoid.
Clinical features
Acute posterior dislocation is rare, and when it does
occur it is often missed. There may be a history of
13.24 Habitual subluxation The clue is in the fairly violent injury or an electric shock. On examina-
unconcerned expression. tion the arm is held in internal rotation and attempts 357
13 DISORDERS OF THE GLENO-
HUMERAL JOINT
TUBERCULOSIS (see also Chapter 2)

Tuberculosis of the shoulder is uncommon. It usually

starts as an osteitis but is rarely diagnosed until arthri-
tis has supervened. This may proceed to abscess and
sinus formation, but in some cases the tendency is to
fibrosis and ankylosis. If there is no exudate the term
‘caries sicca’ is used; however, one suspects that many
such cases, formerly diagnosed on the basis of coex-
(a) (b)
isting pulmonary tuberculosis rather than joint biopsy
13.26 Posterior dislocation (a) In the anteroposterior or bacteriological examination, are actually examples
view the humeral head looks globular – the so-called ‘light of frozen shoulder.
bulb’ appearance. (b) In the lateral view one can see the
humeral head is lying behind the glenoid fossa, with an
impaction fracture on the anterior surface of the head. Clinical features
Adults are mainly affected. They complain of a con-
at external rotation are resisted. The anteroposterior stant ache and stiffness lasting many months or years.
x-ray may show a typical ‘light bulb’ appearance of the The striking feature is wasting of the muscles around
proximal humerus (the humeral head looks symmetri- the shoulder, especially the deltoid. In neglected cases
cally bulbous because the shoulder is internally a sinus may be present over the shoulder or in the
rotated). If the arm can be abducted, an axillary view axilla. There is diffuse warmth and tenderness and all
will show the dislocation quite clearly. movements are limited and painful. Axillary lymph
Recurrent posterior instability usually takes the form nodes may be enlarged.
of subluxation when the arm is used in flexion and X-rays show generalized rarefaction, usually with
internal rotation. On examination, the posterior some erosion of the joint surfaces. There may be
drawer test (scapular spine and coracoid process in abscess cavities in the humerus or glenoid, with little
one hand, humeral head pushed backwards with the or no periosteal reaction.
other) and posterior apprehension test (forward flex-
ion and internal rotation of the shoulder with a pos- Treatment
terior force on the elbow) confirm the diagnosis.
In addition to systemic treatment with antitubercu-
TREATMENT lous drugs, the shoulder should be rested until acute
Recurrent posterior instability due to muscle pattern-
ing and proprioceptive problems should be treated
with physiotherapy. It is essential that this is under-
taken by a therapist trained and experienced in deal-
ing with shoulder instability, as the rehabilitation can
be long and arduous.
Surgery should be considered only if the primary
abnormality is found to be structural (e.g. a Bankart
lesion, bony lesion or capsular injury). The particular
operation depends on the injuries; it is therefore
essential to identify the pathology and treat accord-
ingly. No single operation applies to all patients with
posterior instability. Soft-tissue reconstructions are
the mainstay of treatment. Rarely there is a bone
problem, such as excessive glenoid retroversion
(shown on CT scan), in which case glenoid osteotomy
should be considered. In extreme cases a bony block
to posterior translation of the humeral head is
employed though failure rates are reported to be 13.27 Tuberculosis X-ray of the shoulder showing
358 high. tuberculous abscesses in the head of the humerus.
symptoms have settled. Thereafter movement is Pain and swelling are the usual presenting symp- 13
encouraged and, provided the articular cartilage is not toms; the patient (usually a woman) has increasing
destroyed, the prognosis for painless function is good. difficulty with simple tasks such as combing her hair
If there are repeated flares, or if the articular surfaces or washing her back. Although it may start on one
are extensively destroyed, the joint should be side, the condition usually becomes bilateral.
arthrodesed. Synovitis of the joint results in swelling and tender-

ness anteriorly, superiorly or in the axilla. Tenosynovi-
tis produces features similar to those of cuff lesions,
including tears of supraspinatus or biceps. Joint and
tendon lesions usually occur together and conspire to
RHEUMATOID ARTHRITIS cause the marked weakness and limitation of move-
(see also Chapter 3) ment that are features of the disease.
This is the most common arthropathy to affect the

X-rays
shoulder complex; 90 per cent of patients with
rheumatoid arthritis have involvement of the Neer described three radiological patterns: wet (peri-
acromioclavicular joint, the shoulder joint and the articular erosions, rapid progress, early cuff rupture),
various synovial pouches around the shoulder. dry (subchondral sclerosis, osteophytes, slow
The acromioclavicular joint develops an erosive progress, cuff intact) and resorptive (marked bone loss,
arthritis which may go on to capsular disruption and few erosions).
instability. This is sometimes the first site to be diag-
nosed from routine x-rays of the chest.
Treatment
The gleno-humeral joint, with its lax capsule and
folds of synovium, shows marked soft-tissue inflam- The general treatment of rheumatoid arthritis is dis-
mation. Often there is an accumulation of fluid and cussed in Chapter 3. In the early stages, local treat-
fibrinoid particles which may rupture the capsule and ment in the form of intra-articular injections of
extrude into the muscle planes. Cartilage destruction methylprednisolone may be needed.
and bone erosion are often severe. If synovitis persists, operative synovectomy is car-
The subacromial bursa and the synovial sheath of the ried out; at the same time, cuff tears may be repaired.
long head of biceps become inflamed and thickened; Excision of the lateral end of the clavicle may relieve
often this leads to rupture of the rotator cuff and the acromioclavicular pain.
biceps tendon. In advanced cases pain and stiffness can be very dis-
abling. Provided the rotator cuff is not completely
destroyed and there is still adequate bone stock, total
Clinical features joint replacement with an unconstrained prosthesis
The patient may be known to have generalized may be carried out. This operation provides good pain
rheumatoid arthritis; occasionally, however, acromio- relief, moderate shoulder function and reasonable
clavicular erosion discovered on an x-ray of the chest durability (Stewart and Kelly, 1997). Surface replace-
is the first clue to the diagnosis. ment arthroplasty has comparable outcomes to total
(a) (b) (c) (d)
13.28 Rheumatoid arthritis (a) Large synovial effusions cause easily visible swelling; small ones are likely to be missed,
especially if they present in the axilla (b). (c) X-rays show progressive erosion of the joint. (d) X-ray appearance after total
joint replacement. 359
13 joint replacement but is not suitable for severely dam- shoulders are involved then the disability can be
aged joints in which the humeral head is insufficient severe.
or too soft (Levy et al., 2004). In advanced cases, if pain becomes intolerable,
If the rotator cuff is destroyed, or bone erosion very shoulder arthroplasty is justified. Arthroplasty is dis-
advanced, arthrodesis may be preferable; despite its cussed in more detail later in this section. It may not
apparent limitations, it gives improved function always improve mobility much, but it does relieve
because scapulo-thoracic movement is usually undis- pain. The alternative is arthrodesis.

turbed.
RAPIDLY DESTRUCTIVE
OSTEOARTHRITIS ARTHROPATHY (MILWAUKEE
SHOULDER)
Osteoarthritis of the gleno-humeral joint is more
common than is generally recognized. It is usually Occasionally, in the presence of longstanding or mas-
secondary to local trauma, recurrent subluxation or sive cuff tears, patients develop a rapidly progressive
longstanding rotator cuff lesions. Often chondrocalci- and destructive form of osteoarthritis in which there
nosis is present as well but it is not known whether is severe erosion of the gleno-humeral joint, the
this predisposes to osteoarthritis or appears as a sequel acromion process and the acromioclavicular joint –
to joint degradation. what Neer and his colleagues (1983) called a cuff tear
arthropathy. The changes are now attributed to
hydroxyapatite crystal shedding from the torn rotator
Clinical features cuff and a synovial reaction involving the release of
The patient is usually aged 50–60 and may give a his- lysosomal enzymes (including collagenases) which
tory of injury, shoulder dislocation or a previous lead to cartilage breakdown (McCarty et al., 1981). A
painful arc syndrome. There is usually little to see similar condition is seen in other joints such as the hip
but shoulder movements are restricted in all direc- and knee. The shoulder disorder, however, has come
tions. to be known as Milwaukee shoulder, after the city from
X-rays show distortion of the joint, bone sclerosis whence McCarty hailed.
and osteophyte formation; the articular ‘space’ may
be narrowed or may show calcification.
Clinical features
The patient is usually aged over 60 and may have suf-
Treatment fered with shoulder pain for many years. Over a
Analgesics and anti-inflammatory drugs relieve pain, period of a few months the shoulder becomes swollen
and exercises may improve mobility. Most patients and increasingly unstable. On examination there is
manage to live with the restrictions imposed by stiff- marked crepitus in the joint and loss of active move-
ness, provided pain is not severe. However, if both ments.
(a) (b) (c)
13.29 Osteoarthritis of the shoulder (a) This woman has advanced osteoarthritis of both shoulders. Movements are so
restricted that she has difficulty dressing herself and combing her hair. (b,c) X-rays show the severe degree of articular
360 destruction.
13

13.30 Milwaukee shoulder X-ray showing a destructive
arthropathy with marked swelling and calcification in the
soft tissues around the shoulder.
X-rays show severe erosion of the articular surfaces,

subluxation of the joint and calcification in the soft
tissues. 13.31 Osteonecrosis A young woman with systemic
lupus erythematosus was treated with large doses of
prednisolone. She developed pain in one hip and one
Treatment shoulder. X-ray of the shoulder shows the classic features
of osteonecrosis, including a long subarticular fracture of
Resurfacing arthroplasty relieves pain and allows good the humeral head.
rotations at waist level but will not improve abduc-
tion, because the rotator cuff is disrupted and the
joint is unstable. It is quick and minimally invasive,
retaining bone stock and keeping options open for
future revision or arthrodesis.
Reverse shoulder arthroplasty in cuff tear arthro- DISORDERS OF THE SCAPULA
pathy allows good elevation in the presence of a well-
functioning deltoid as it depends less on the status of AND CLAVICLE
the cuff. Problems may occur in the long-term follow-
up regarding progressive glenoid loosening due to the CONGENITAL ELEVATION OF THE
so-called ‘inferior notching’, which is supposed to be SCAPULA
a result of an impingement at the inferior glenoid rim
followed by increased polyethylene wear and progres-
The scapulae normally complete their descent from
sive osteolysis. It is thus advisable to avoid reverse
the neck by the third month of fetal life; occasionally
shoulder arthroplasty in the younger patient.
one or both scapulae remain incompletely descended.
Associated abnormalities of the cervical spine are
common and sometimes there is a family history of
OSTEONECROSIS (see also Chapter 6) scapular deformity.
CLINICAL FEATURES
The shoulder is the second most common site of
Two similar, and possibly related, conditions are
steroid-induced osteonecrosis. The condition may
encountered.
also be seen in association with marrow storage dis-
orders, sickle-cell disease and caisson disease, or Sprengel’s deformity Deformity is the only symptom
following irradiation of the axilla. and it may be noticed at birth. The shoulder on the
The clinical features and diagnosis are discussed in affected side is elevated; the scapula looks and feels
Chapter 6. Articular collapse occurs more slowly than abnormally high, smaller than usual and somewhat
in weightbearing joints and operative treatment can prominent; occasionally both scapulae are affected.
usually be delayed for several years. If this should The neck appears shorter than usual and there may be
become necessary, joint replacement is the method of kyphosis or scoliosis of the upper thoracic spine.
choice. Shoulder movements are painless but abduction and 361
13 CLEIDOCRANIAL DYSOSTOSIS
This is a heritable disorder (autosomal dominant)

characterized by hypoplasia or aplasia of the clavicles
and flat bones (pelvis, scapulae and skull). Those
affected have a typical appearance, with drooping

shoulders, an usually narrow chest and the ability to
bring the shoulders together across the front of the
chest.
X-rays show hypoplasia or complete absence of the
clavicles, and sometimes also of the scapulae. Other
skeletal defects, which occur in varying degree, are
(a) (b) delayed closure of the fontanelles, brachycephaly,
13.32 Scapular disorders (a) Sprengel shoulder; underdevelopment of the pelvis, coxa vara and
(b) Klippel–Feil syndrome. scoliosis.
Treatment is usually unnecessary and, despite the
widespread defects, patients enjoy good function.
elevation may be limited by fixation of the scapula. CONGENITAL PSEUDARTHROSIS OF

X-rays will show the elevated scapula and any associ- THE CLAVICLE
ated vertebral anomalies; sometimes there is also a
bony bridge between the scapula and the cervical The typical clinical picture is that of a child with a
spine (the omo-vertebral bar). painless lump in the mid-shaft of the clavicle. This
Klippel–Feil syndrome This is usually a more wide- always occurs on the right side, except in the presence
spread disorder. There is bilateral failure of scapular of dextrocardia. X-ray shows the break in the clavicle,
descent associated with marked anomalies of the cer- which usually heals only after excision of the ‘non-
vical spine and failure of fusion of the occipital bones. union’ and bone grafting.
Patients look as if they have no neck; there is a low Treatment, if required, is by excision of the
hairline, bilateral neck webbing and gross limitation of pseudarthrosis and bone grafting across the gap.
neck movement. This condition should not be con-
fused with bilateral shortness of the sternomastoid mus-
cle in which the head is poked forward and the chin
thrust up; the absence of associated congenital lesions SCAPULAR INSTABILITY
is a further distinguishing feature.
Winging of the scapula is due to weakness of the ser-
TREATMENT ratus anterior muscle. It results in asymmetry of the
Mild cases are best left untreated. Surgical treatment shoulders but the deformity may not be obvious until
aims to decrease deformity and improve shoulder the patient tries to contract the serratus anterior
function. In children under 6 years of age, the scapula against resistance. The typical appearance is shown in
can be repositioned by releasing the muscles along the Figure 13.33.
vertebral and superior borders of the scapula, excising There are several causes of weakness or paralysis of
the supraspinous portion of the scapula and the omo- the serratus anterior muscle:
vertebral bar, pulling the scapula down, then reat-
taching the muscles to hold it firmly in its new • neuralgic amyotrophy (see page 259)
position. In older children this carries a risk of brachial • injury to the brachial plexus (a blow to the top of
nerve compression or traction between the clavicle the shoulder, severe traction on the arm or carrying
and first rib; here it is safer merely to excise the heavy loads on the shoulder
supraspinous portion of the scapula in order to • direct damage to the long thoracic nerve (e.g.
improve the appearance but without improving move- during radical mastectomy)
ment. Before undertaking any operation the cervical • fascioscapulohumeral muscular dystrophy.
spine should be carefully imaged in order to identify Disability is usually slight and is best accepted.
any abnormalities of the odontoid process or base of However, if function is noticeably impaired, it is pos-
362 skull. sible to stabilize the scapula by transferring the sternal
SEPTIC ARTHRITIS OF THE STERNO- 13
CLAVICULAR JOINT
This condition is rare except in drug abusers follow-

ing intravenous injections, and as a secondary compli-

cation of sterno-clavicular haemarthrosis following
trauma. Local signs may be misleadingly mild but per-
sistent pain, swelling and tenderness associated with
systemic signs of infection should arouse suspicion.
Imaging X-rays are usually normal until fairly late
when they may show erosion of the sterno-clavicular
joint and the adjacent bone. If infection is suspected
then further imaging of the joint will be required,
such as MRI or CT, which will allow the extent of any
13.33 Winged scapula This young woman’s right scapula spread of infection or bony destruction to be identi-
was somewhat prominent even at rest, but here the fied. Radioscintigraphy is able to identify multifocal
‘winging’ is enhanced by having her thrust her arms septic arthritis.
forcibly against the wall.
Investigations If infection is suspected then blood cul-
tures and aspiration of the joint will be required. A
wide range of organisms have been found to cause
infection at this site.
portion of pectoralis major and attaching it via a fas- Treatment If frank pus is present in the joint then an
cia lata graft to the lower pole of the scapula; or the arthrotomy with formal washout will be required. If
scapula can be fixed to the rib-cage to provide the del- there is delay in diagnosis or institution of the correct
toid and the rotator cuff muscles with a stable base treatment, rupture of the joint capsule may occur with
from which to control the shoulder. tracking of pus into the chest wall, retrosternum or
A less obvious, but sometimes more disabling, form superior mediastinum.
of scapular instability may follow injury to the spinal
accessory nerve (e.g. following operations in the poste-
rior triangle of the neck). The trapezius muscle is an
important stabilizer of the shoulder and loss of this
STERNO-CLAVICULAR
function results in weakness and pain on active abduc- HYPEROSTOSIS
tion against resistance. Early recognition may permit
nerve repair or grafting. Several individually uncommon disorders are associ-
ated with pain and swelling over the clavicle or the
sterno-clavicular joint. They are often confused,
though certain characteristic features permit appropri-
ate differentiation in the majority of cases.
GRATING SCAPULA
Asymptomatic scapulo-thoracic crepitus is found in

Condensing osteitis of the clavicle
about a third of healthy persons. People with symp- This is usually seen in women of 20–40 years who
toms complain of grating or clicking on moving the present with pain at the medial end of the clavicle,
arm; the condition is often painless but annoying, which is aggravated by abducting the arm. The clavi-
though it does sometimes become painful. Usually no cle may be thickened and tender. X-rays reveal sclero-
cause is found, though bony, muscular and bursal sis and radionuclide scanning shows increased activity
abnormalities have been blamed. in the affected bone (Cone et al., 1983).
Tangential x-ray views of the scapula should be The condition may be no more than a reaction to
obtained to exclude an osteochondroma on the the mechanical stress of excessive lifting activities, and
undersurface of the scapula. A CT scan with three- treatment consists simply of avoiding such activities.
dimensional reconstruction can be helpful; if an Of greater importance is the need to distinguish it
osteochondroma is present, the lesion can be excised. from the other hyperostotic disorders.
If a bony lesion is not identified conservative treat- Condensing osteitis shares both morphological and
ment is usually adopted. radiological features with osteitis of the ilium and 363
13 osteitis of the pubis. It has been noted that all of these
bones have a fibrocartilaginous covering which may
explain the predilection of the condition for those
sites.
Sterno-costo-clavicular hyperostosis
This condition in some ways resembles condensing

osteitis, but it is seen in slightly older people (both
men and women) and is usually bilateral. Patients
develop pain, swelling and tenderness over the sterno-
clavicular region and x-rays show hyperostosis of the
medial ends of the clavicles, the adjacent sternum, the
anterior ends of the upper ribs and the soft tissues in
between. Vertebrae also may be affected and the ESR
may be increased; little wonder that it has been sug-
gested that this is a type of seronegative spon- 13.34 Osteoarthritis of the acromioclavicular joint
darthropathy. Biopsy is of little help; the histological Osteophytic thickening of the acromioclavicular joint
changes are non-specific and micro-organisms have produced a small (but very tender) bump on top of the left
shoulder. Occasionally the joint capsule herniates,
not been identified. A peculiarity which links this con- producing a large ‘cyst’ over the acromioclavicular joint.
dition with the next is an association with pustular
lesions on the palms and soles (palmo-plantar pustu-
losis) and pustular psoriasis.
Subacute or chronic multifocal

osteomyelitis any suggestive history. The patient may complain of
‘shoulder pain’, but if you ask him or her to point, he
Multifocal osteomyelitis usually occurs in children and
or she will direct your attention to the prominent
adolescents; the clavicle and lower limb metaphyses
bump at the outer end of the clavicle; tenderness is
are sites of predilection. It may present as a painful,
sharply localized to this area. Shoulder movements are
fusiform swelling of the clavicle and x-rays show thick-
usually not restricted (unless the shoulder joint itself
ening and sclerosis of the medial third of the bone.
is involved) but there may be pain at the extremes of
Like sterno-costo-clavicular hyperostosis, it is some-
abduction and flexion.
times associated with palmo-plantar pustulosis. The
X-ray shows the characteristic features of osteo-
diagnosis is strongly suggested if pustulosis is present,
arthritis; the changes are often bilateral, even though
otherwise it usually emerges gradually as other sites
only one side may be hurting. In some cases the con-
become affected over the course of the next year or
dition is discovered while examining the patient for an
two and x-rays show the typical lytic areas in the meta-
impingement syndrome; indeed, acromioclavicular
physes and/or epiphyses close to the physis. The full-
OA may cause impingement.
blown picture is well described in the paper by Carr et
al. (1993). There is no effective treatment; the lesions
almost invariably heal spontaneously over a period of TREATMENT
months or years, the only trace of the condition being If analgesics or steroid injections are ineffectual, pain
the thickened bone ends. may be relieved by excision of the lateral end of the
clavicle. This procedure can now be performed
arthroscopically. Trimming of the bony roughness, or
excision of the outer end of the clavicle, may also be
needed during subacromial decompression for rotator
OSTEOARTHRITIS OF THE ACROMIO- cuff impingement.
CLAVICULAR JOINT
Osteoarthritis of the acromioclavicular joint is com-

mon in middle-aged and older people. Predisposing
factors are trauma (subluxation of the joint) and occu- OPERATIONS
pational stress (habitually carrying weights on the
shoulder or working with pneumatic hammers and Rotator cuff surgery and shoulder stabilization are
364 drills), but the condition also occurs in the absence of described in the relevant sections.
5. tumours of the proximal humerus 13
ARTHROSCOPY 6. severe arthritis with cuff arthropathy.
Arthroscopy is a useful technique for the diagnosis of The choice of procedure lies between total shoulder
peri-articular and intra-articular disorders, such as replacement, humeral head replacement (hemiarthro-
rotator cuff disruption and instability. At the same plasty) – which can be stemmed or resurfacing – and
more constrained shoulder replacements such as the

time a biopsy can be taken which may assist in the diag-
nosis of synovial disorders such as rheumatoid arthri- reverse polarity shoulder replacements. The relative
tis or pigmented villonoduar synovitis. merits of total shoulder arthroplasty and hemiarthro-
Arthroscopic surgery is now well established. There has plasty are not clear. Glenoid resurfacing is contraindi-
been a transition over the last 20 years from its usage in cated if inadequate bone stock or irreparable rotator
diagnosis to that of repair and reconstructive proce- cuff tears (or both) are present. Hemiarthroplasty
dures. It is the first-line surgical option for subacromial affords the benefits of decreased operation time,
decompression, acromioclavicular joint excisions, de- blood loss and technical difficulty which would other-
bridement of rotator cuff tears and release of frozen wise attend glenoid exposure and resurfacing. On the
shoulder. Arthroscopic repair of Bankart lesions produces other hand, individual studies have reported less con-
results comparable to those obtained by open surgery. sistent pain relief with isolated humeral head replace-
ment. With isolated humeral head replacement, the
glenoid can undergo progressive erosion over time,
often leading to deteriorating results.
ARTHROPLASTY OF THE SHOULDER Relative indications for TSR in patients with gleno-
humeral arthritis include loss of articular cartilage or
Shoulder replacement was initially introduced by incongruent osseous surfaces, with normal or repara-
Neer in the 1950s for the treatment of proximal ble rotator cuff tendons. TSR requires more operative
humeral fractures. Subsequent modifications and the time and is technically more challenging than hemi-
introduction of glenoid resurfacing broadened the arthroplasty, and the procedure introduces the con-
indications to include other disease processes, includ- cern of glenoid loosening, the most common
ing end-stage gleno-humeral osteoarthritis and complication. However, proponents of TSR suggest it
rheumatoid arthritis. If non-operative treatment fails, may yield more consistent pain relief and a better
the two surgical options commonly considered are range of motion.
humeral head replacement (HHR) and total shoulder In a systematic review of the literature there is a
replacement (TSR). The optimal treatment choice, suggestion that overall TSR may yield superior results;
however, remains controversial. however, it remains unclear if one procedure is sig-
nificantly better than the other (Radnay et al., 2007).
Indications
The indications for arthroplasty are: Complications
1. osteoarthritis causing pain and loss of movement The commonest, in order of frequency, are loosening
2. rheumatoid arthritis of the components, gleno-humeral instability, rotator
3. complex fractures of the proximal humerus cuff failure, peri-prosthetic fracture, infection and
4. avascular necrosis of the humeral head implant failure. Glenoid fixation remains a challenge;
(a) (b) (c) (d)
13.35 Shoulder replacements (a,b) Osteoarthritis and a resurfacing arthroplasty. (c) Early postoperative x-ray of a
reverse polarity shoulder replacement. (d) Total shoulder replacement with replacement of the glenoid. 365
13 lucent lines around the glenoid component are very tains the position of the arthrodesis and can decrease the
common, although not always symptomatic (Wirth length of time spent in plaster immobilization.
and Rockwood, 1996). The optimal position is 30 degrees of flexion, 30
degrees of abduction and 30 degrees of internal rota-
tion. A thermoplastic orthosis needs to be worn for 6
Outcome
weeks.
This depends largely on the indications for surgery.

Arthroplasty for fractures, avascular necrosis or proximal
humeral tumours gives good pain relief and shoulder
Outcome
movement, although power is always diminished. Despite the restriction of gleno-humeral movement,
Where there is more extensive joint destruction and dis- postoperative function is surprisingly good; and of
ruption of the soft tissues (e.g. in rheumatoid arthritis), course the joint is free of pain!
pain relief is still excellent but the range of movement is Complications include non-union, infection, malpo-
only moderately improved. The greater the integrity of sition often with too much internal rotation, promi-
the surrounding soft tissues (and especially the rotator nence of the internal fixation and fracture of the
cuff), the more stable will the new joint be, and thus the humerus.
better the outcome of the operation. In severe cuff fail-
ure, reverse geometry arthroplasty has been used with
reasonable success in the short term in elderly patients,
though further research is needed to assess longevity
and continued functional improvement. NOTES ON APPLIED ANATOMY
Joints
The anatomy of the shoulder is uniquely adapted to
ARTHRODESIS allow freedom of movement and maximum reach for
the hand.
Arthrodesis of the gleno-humeral joint is now seldom Five ‘articulations’ are involved:
performed, but it is still a useful operation for severe
• the gleno-humeral joint
shoulder dysfunction.
• the pseudojoint between the humerus and the
coracoacromial arch
Indications • the sternoclavicular joint
• the acromioclavicular joint
The indications for shoulder arthrodesis are:
• the scapulothoracic articulation.
1. paralysis of the scapulo-humeral muscles
2. infective disorders of the gleno-humeral joint
Stability
(including tuberculous arthritis)
3. advanced erosive arthritis with massive disruption The shallow gleno-humeral articulation has little
of the rotator cuff inherent stability because the glenoid surface area is
4. failed total shoulder arthroplasty only one-quarter that of the humeral articular surface.
5. uncontrolled instability. The extent to which the socket is deepened by the
labrum may seem trivial, but it must be significant
because labral tears are associated with dislocation.
The operation Stability depends mainly on the integrity of the liga-
A prerequisite is stable and powerful scapulo-thoracic ments and capsule. The muscles provide kinetic sta-
movement, because with a fused shoulder ‘move- bility: during abduction the rotator cuff muscles draw
ment’ is achieved entirely by rotation of the scapula the head of the humerus firmly into its socket while
on the thorax. A number of techniques have been the deltoid elevates the arm.
reported with extra-articular arthrodesis, intra-articu-
lar arthrodesis and a combination of both. Internal
fixation has been used more frequently in recent years,
Rotator cuff
Extra-articular arthrodesis is primarily a historic The rotator cuff is a sheet of conjoint tendons closely
procedure that was used before the antibiotic era to applied over the top of the shoulder capsule and
treat tuberculosis. inserting into the greater tuberosity of the humerus.
A variety of methods of internal fixation for intra-ar- It is made up of subscapularis in front, supraspinatus
ticular arthrodesis have been described. It is generally above and infraspinatus and teres minor behind. The
366 agreed that internal fixation is desirable because it main- ‘rotator’ muscles have an important function in
stabilizing the head of the humerus by pulling it Boileau P, Villalba M, Héry JY et al. Risk factors for recur- 13
firmly into the glenoid whenever the deltoid lifts the rence of shoulder instability after arthroscopic Bankart
arm forwards or sideways. The rotator interval lies repair. J Bone Joint Surg 2006; 88A: 1755–1763.
between the supraspinatus and infraspinatus tendons. Bunker TD. Frozen shoulder: unravelling the engima. Ann
Arching over the cuff is a fibro-osseous canopy – R Coll Surg Engl 1997; 79: 210–13.
the coracoacromial arch – formed by the acromion Carr AJ, Cole WG, Roberton DM, Chow CW. Chronic

process posterosuperiorly, the coracoid process ante- multifocal osteomyelitis. J Bone Joint Surg 1993; 75B:
riorly and the coracoacromial ligament joining them. 582–91.
Separating the tendons from the arch, and allowing Cole BJ, L’Insalata J, Irrgang J, Warner JJ. Comparison of
them to glide, is the subacromial bursa. Of the four arthroscopic and open anterior shoulder stabilization. A
cuff tendons, the supraspinatus is the most exposed; it two to six-year follow-up study. J Bone Joint Surg 2000;
runs over the top of the shoulder under the anterior 82A: 1108–14.
edge of the acromion and the adjacent acromio- Cone RD, Resnick D, Goergen TG et al. Condensing
clavicular joint, with the intra-articular portion of the osteitis of the clavicle. Am J Roentgenol 1983; 141:
biceps tendon closely applied to its deep surface. 387–8.
Gartsman GM. Arthroscopic treatment of rotator cuff dis-
ease. J Shoulder Elbow Surg 1995; 4: 228–41.
Movement Gartsman GM. Combined arthroscopic and open treat-
Abduction and flexion of the shoulder look simple; in ment of tears of the rotator cuff. J Bone Joint Surg 1997;
fact they are very complex movements involving all the 79A: 776–83.
joints of the shoulder girdle. Imagine what would hap- Gerber C, Krushell RJ. Isolated rupture of the tendon of
pen if the deltoid muscle acted alone in abducting the the subscapularis muscle. Clinical features in 16 cases.
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368
The elbow and
forearm 14
David Warwick
as the front. Often the neck, shoulders and hands also

CLINICAL ASSESSMENT need to be examined.
SYMPTOMS Look
Pain from the elbow is fairly diffuse and may extend Both upper limbs must be completely exposed. The
into the forearm. Localized pain over the lateral or patient holds his or her arms alongside the body,
medial epicondyle of the humerus is usually due to elbows fully extended, with palms forwards. In this
tendinitis. The patient may have noticed that it is trig- position the forearms are normally angled slightly
gered, or aggravated, by certain activities. So often is outwards – a valgus or carrying angle of 5–15
this the case that the symptom has acquired colloquial degrees. ‘Varus’ or ‘valgus’ deformity is determined by
definitions: ‘tennis elbow’ for lateral epicondylar pain angular deviations medialwards or lateralwards
and ‘golfer’s elbow’ for medial epicondylar pain. Pain beyond those limits or, in unilateral abnormalities, by
over the back of the elbow is often due to an olecra- comparison with the normal side.
non bursitis. Remember that ‘pain in the elbow’ is some- Varus and valgus deformities (cubitus varus and
times referred pain from the cervical spine! cubitus valgus) are usually the result of trauma around
Stiffness, if it is mild, may hardly be noticed. If it is the elbow. By far the best way to demonstrate a varus
severe, it can be very disabling; the patient may be deformity is to ask the patient to lift his or her arms
unable to reach up to the mouth (loss of flexion) or sideways to shoulder height; in this position the defor-
the perineum (loss of extension); limited supination mity becomes much more obvious, the arm taking on
makes it difficult to carry large objects. the appearance of a rifle butt (gunstock deformity,
Swelling may be due to injury or inflammation; a shown in Fig. 14.5).
soft lump on the back of the elbow suggests an ole-
cranon bursitis.
Deformity is uncommon except in rheumatoid arthri- Feel
tis and after trauma. Always ask about previous injuries. Start by identifying the most obvious bony landmarks:
Instability – the feeling that the elbow ‘moves out the olecranon process posteriorly, the medial and
of joint’ – is due either to previous trauma or to lateral epicondyles and the head of the radius just
destructive joint disease.
Ulnar nerve symptoms (tingling, numbness and
weakness of the hand) may occur in elbow disorders 14.1 Examination Feeling
because of the nerve’s proximity to the joint. begins with the skin. Is
there undue warmth? Next,
Loss of function is noticed mainly in grooming, car- feel the bony landmarks.
rying and placing activities. However good the hand, With the elbow flexed, the
if the elbow cannot put it out into the environment tips of the medial and
and bring it back to the individual, upper limb func- lateral epicondyles and the
tion is seriously degraded. olecranon process form an
isosceles triangle. With the
elbow extended, they lie
transversely in line with
each other. These
SIGNS relationships are disturbed
in post-traumatic
Both upper limbs should be completely exposed, and deformities of the elbow.
it is essential to look at the back of the elbow as well
14 14.2 (a,b) The best way to examine
active movements is to stand in front
of the patient and show her what to
do. (c,d) The normal range of flexion
is from 0° (full extension) to 140°
(full flexion). (e,f) To test pronation
and supination, ask the patient to
tuck her elbows tightly into her body

and then turn the hands fully palms
down and then palms up. The
normal range is 90° in each
direction.
(a) (b)
(c) (d)
(e) (f)
distal to the lateral epicondyle; pronating and supinat- to the zero position (absolutely straight); people with
ing the forearm makes it easier to find the mobile lax joints can extend even beyond that point. As a
radial head and the lateral joint line. The ulna can be rough guide, people are normally able to flex the
palpated throughout its length, the radius only at its elbow sufficiently to touch the top of the shoulder
proximal end and in the distal third of the forearm. with their fingers, but bear in mind that those with
The back of the elbow is palpated for warmth and bulky upper arm muscles may not be able to do so.
swelling (signs of an olecranon bursitis) and subcuta- Pronation and supination of the forearms are tested
neous nodules (a feature of rheumatoid arthritis). Feel with the patient holding the arms tucked into the
more widely for synovial thickening and fluid (fluctu- waist and flexed to a right angle; 80–90 degrees each
ation on each side of the olecranon). The ulnar nerve way is normal. Stability must also be tested carefully
is very superficial behind the medial condyle and here after trauma. The humerus is stabilized, the elbow is
it can be rolled under the fingers to feel if it is thick- flexed to about 25 degrees to unlock any contribution
ened or hypersensitive. to stability by the olecranon and the elbow is stressed
Last of all, feel for tenderness and try to determine in torsion and collateral stress.
which structure is affected.
General examination
Move
Clinical examination should include the neck and
Active and passive flexion and extension are compared shoulder (which are sources of referred pain to the el-
370 on the two sides. The elbow should be able to extend bow) and the hand (for signs of nerve dysfunction).
Function is usually surprisingly good and pain is 14
unusual. Surgery is therefore rarely required; how-
ever, if the lump limits elbow flexion it can be excised
(beware of the posterior interosseous nerve).
CONGENITAL SYNOSTOSIS
The elbow and forearm

(a) (b)
Congenital deficiencies of the forearm bones are occa-
14.3 Normal range of movement (a) The extended sionally associated with fusion of the humerus to the
position is recorded as 0° and any hyperextension as a
minus quantity; flexion is full when the arm and forearm
radius or ulna. This disabling condition is, fortunately,
make contact. (b) From the neutral position the radio-ulnar very rare. A more useful angle of forearm rotation can
joint rotates 90° into pronation and 90° into supination. be achieved by osteotomy.
Proximal radio-ulnar synostosis causes loss of rota-
tion, but elbow flexion is retained and the inconven-
ience is often only moderate. Surgery to regain
IMAGING rotation rarely succeeds. A rotational osteotomy can
Plain x-ray give a more suitable angle of pronation–supination
tailored to the individual patient’s needs.
The position of each bone is noted, then the joint line
and space. Next, the individual bones are inspected for
evidence of old injury or bone destruction. There may
be some calcification over the epicondyles in cases of ACQUIRED DEFORMITIES
tennis or golfer’s elbow. Finally, loose bodies are sought.
In children the epiphyses are largely cartilaginous
CUBITUS VALGUS
and the articular relations often have to be deduced The normal carrying angle of the elbow is 5–15
from the shape and position of the emerging second- degrees of valgus; anything more than this is regarded
ary ossific centres. The average ages at which they as a valgus deformity, which is usually quite obvious
appear are easily remembered by the mnemonic when the patient stands with arms to the sides and
CRITOE: Capitulum – 2 years; Radial head – 4 years; palms facing forwards.
Internal (medial) epicondyle – 6 years; Trochlea – 8 The commonest cause is longstanding non-union
years; Olecranon – 10 years; External (lateral) epi- of a fractured lateral condyle; the deformity may be
condyle – 12 years. associated with marked prominence of the medial
condylar outline. The importance of cubitus valgus is
Computed tomography the liability to delayed ulnar palsy; years after the
causal injury the patient notices weakness of the hand,
Arthrography with CT imaging is a useful method for with numbness and tingling of the ulnar fingers. The
defining loose bodies and detailed changes in deformity itself needs no treatment, but for delayed
osteoarthritis. ulnar palsy the nerve should be transposed to the
front of the elbow. Great care is needed in performing
Magnetic resonance imaging the operation. Excessive dissection of the nerve or
rough handling can impair nerve function.
MRI will be needed to reveal articular changes (such
as osteochondritis dissecans) and soft-tissue abnor-
malities (e.g. ligament injuries). CUBITUS VARUS (‘GUN-STOCK’ DEFORMITY)
The deformity is most obvious when the elbow is
extended and the arms are elevated. The most com-
CONGENITAL DISORDERS mon cause is malunion of a supracondylar fracture.
The deformity can be corrected by a wedge
CONGENITAL DISLOCATION OF THE osteotomy of the lower humerus but this is best left
until skeletal maturity.
RADIAL HEAD
This may be anterior or posterior and is usually bilat-
eral. The patient may notice the lump, which is easily
SUBLUXATION OF THE RADIAL HEAD
palpable and can be felt to move when the forearm is This is commonly associated with bone dysplasias in
rotated. X-rays show that the dislocated radial head is which the ulna is disproportionately shortened (e.g.
dome-shaped (due to abnormal modelling). hereditary multiple exostosis). It usually causes little 371
14
(a) (b)
14.6 Dislocated radial head (a) Anterior dislocation

from old Monteggia fracture; (b) posterior dislocation,
most likely congenital.
(a) (b) disability, but if it becomes too troublesome the radial

14.4 Cubitus valgus (a) This man has excessive valgus of head can be excised after all growth has ceased.
the right elbow. But his main complaint was of weakness
and deformity in the hand, which was caused by traction
on the ulnar nerve secondary to the elbow deformity. UNREDUCED DISLOCATION OF THE HEAD
(b) Valgus deformity from an un-united fracture of the OF RADIUS
lateral condyle.
An unreduced Montegia fracture-dislocation will
leave the radial head permanently dislocated. Open
reduction and realignment of the ulna, together with
soft-tissue reconstruction, may improve function.
‘PULLED ELBOW’
Downward dislocation of the head of the radius from
the annular ligament is a fairly common injury in chil-
dren under the age of 6 years. There may be a history
of the child being jerked by the arm and subsequently
complaining of pain and inability to use the arm. The
limb is held more or less immobile with the elbow
fully extended and the forearm pronated; any attempt
to supinate the forearm is resisted. The diagnosis is
essentially clinical, though x-rays are usually obtained
in order to exclude a fracture.
The radial head can be forcibly pulled out of the
noose of the annular ligament only when the forearm
is pronated; even then the distal attachment of the lig-
(a) (b) ament is sometimes torn.
If the history and clinical picture are suggestive, an
attempt should be made to reduce the subluxation or
dislocation. While the child’s attention is diverted, the
elbow is quickly supinated and then slightly flexed;
the radial head is relocated with a snap. (This some-
times happens ‘spontaneously’ while the radiographer
is positioning the arm!)
OSTEOCHONDRITIS DISSECANS
(see also Chapter 6)
(c)
The capitulum is one of the common sites of osteo-
14.5 Cubitus varus (a) Note that the elbows are chondritis dissecans. This may be due to repeated
normally held in 5–10° of valgus (the carrying angle). stress following prolonged or unaccustomed activity
(b) This young boy ended up with slight varus angulation
after a supracondylar fracture of the distal humerus. The but can occur spontaneously. The pathological
deformity is much more obvious (c) when he raises his changes are described in Chapter 6.
372 arms (gun-stock deformity). The patient – usually a young male adolescent –
14

(a) (b)
14.7 Osteochondritis dissecans (a) The capitulum is

fragmented and slightly flattened. (b) Sometimes the
fragment separates and lies in the joint.
complains of aching which is aggravated by activity

and relieved by rest. On examination there may be
swelling, signs of an effusion, tenderness over the 14.8 Loose body CT scan showing a loose body in the
capitulum and slight limitation of movement. If the back of the elbow joint.
fragment has separated, there may be intermittent
locking.
X-rays may show fragmentation or, at a much later TUBERCULOSIS (see also Chapter 2)
stage, flattening of the capitulum.
CT and MRI are more useful for defining the Clinical features
lesion.
Treatment is usually symptomatic. The lesion can The elbow is affected in about 10 per cent of patients
heal and symptoms resolve. Repeated CT or MRI with skeletal tuberculosis. Although the disease begins
scanning will monitor this. However, if the fragment as synovitis or osteomyelitis, patients are rarely seen
has separated and is lying free in the joint, it should be until arthritis supervenes. The onset is insidious with
removed. A large loose fragment which is often still a long history of aching and stiffness. The most strik-
partly attached can be pinned back. These procedures ing physical sign is the marked wasting. While the dis-
can be done arthroscopically. ease is active the joint is held flexed, looks swollen,
and feels warm and diffusely tender; movement is
considerably limited and accompanied by pain and
spasm. Always feel for the supratrochlear and axillary
LOOSE BODIES lymph nodes; they may be enlarged.
Loose bodies in the elbow may be due to: (1) acute X-rays
trauma (an osteocartilaginous fracture); (2) osteo-
chondritis dissecans; (3) synovial chondromatosis (a The typical features are peri-articular osteoporosis and
cluster of mainly cartilaginous ‘pebbles’); or (4) joint erosion. There may also be subchondral cystic
osteoarthritis (separation of osteophytes). lesions.
The patient may complain of sudden locking and
unlocking of the joint. Symptoms of osteoarthritis
may coexist.
A loose body is rarely palpable. When degenerative
changes have occurred, extremes of movement are
limited.
X-rays may reveal the loose body or bodies (see Fig.
14.8); in the special case of osteochondritis dissecans
there is a rarefied cystic area in the capitulum and
enlargement of the radial head. A CT arthrogram will
define the size and the number of loose bodies.
If loose bodies are troublesome, they should be
(a) (b)
removed by arthroscopic or open means, depending
on the size of the loose body and the experience of 14.9 Tuberculosis of the elbow Muscle wasting is
the surgeon. marked and bone destruction extensive. 373
14
(a) (b) (c)

14.10 Rheumatoid arthritis (a) This rheumatoid patient has nodules over the olecranon
and a bulge over the radiohumeral joint; (b) his x-rays show deformity of the radial head and
marked erosion of the rest of the elbow. (c) Excision of the radial head combined with
synovectomy relieved the pain and improved elbow movement.
Other investigations X-rays

Aspiration, synovial biopsy and microbiological inves- X-ray examination reveals bone erosion, with gradual
tigation will usually confirm the diagnosis. destruction of the radial head and widening of the
trochlear notch of the ulna. Sometimes large synovial
extensions penetrate the articular surface and appear
Treatment as ‘cysts’ in the proximal radius or ulna.
General antituberculous treatment is essential. The
elbow is rested until the acute symptoms subside – at
Treatment
first in a splint and positioned at 90 degrees of flexion
and mid-rotation, later simply by applying a collar and In addition to general treatment, the elbow should be
cuff. As soon as possible, however, movement is splinted during periods of active synovitis. Local injec-
encouraged. tions of corticosteroid preparations may reduce pain
Late residual effects – chronic pain, stiffness or and swelling – at least for a while.
deformity – may be troublesome enough to justify
excisional or replacement arthroplasty or (rarely) OPERATIVE TREATMENT
arthrodesis. If, despite adequate conservative treatment, synovitis
persists – and more particularly if this is associated
with erosion of the radial head – synovectomy is
worthwhile. This is usually performed through a lat-
RHEUMATOID ARTHRITIS eral approach, with excision of the radial head. There
are two reasons for this: first, the radio-capitellar sur-
The elbow is involved in more than 50 per cent of faces are almost invariably eroded, and second, radial
patients with polyarticular rheumatoid arthritis, and in head excision permits wider access to the hyper-
the majority of cases the condition is bilateral. trophic synovium. The operation relieves pain and
may slow the progress of the disease, but after 5–6
years erosion of the humeroulnar joint often causes
Clinical features increasing instability and recurrence of pain. A draw-
Ulnar bursitis and rheumatoid nodules are often back of radial head excision is that it may jeopardize
found on the back of the elbow even if the joint itself the result of joint replacement if this should later
is not affected. With true joint involvement, synovitis become necessary.
gives rise to pain and tenderness, especially over the Progressive bone destruction and instability may
lateral aspect of the radio-humeral joint. call for reconstructive surgery. Arthrodesis is very dis-
Later the entire elbow may be swollen. Movements abling and is unlikely to be accepted by the patient.
are restricted but, if bone destruction is marked, the Joint replacement is usually successful in relieving pain
joint becomes unstable. and maintaining a functional range of movement.
Synovial swelling occasionally causes ulnar nerve or Good 10-year results have been reported in about 80
posterior interosseous nerve compression, with symp- per cent of patients. However, the operation is diffi-
toms and signs in the wrist and hand. It is important cult to perform and prone to complications such as
to distinguish these features from those of local weak- infection, instability and dislocation, ulnar neuropathy
374 ness and tendon rupture due to generalized disease. and aseptic loosening of the implants.
14.11 Total elbow 14
replacement
(a) Severe rheumatoid
arthritis of the elbow.
(b) X-ray after joint
replacement. (c) The Souter
arthroplasty; a metal

humeral prosthesis and
polyethylene ulnar implant.
(a) (b) (c)
typical positively birefringent crystals in fluid aspirated

GOUT AND PSEUDOGOUT from the joint. Treatment is as for osteoarthritis (see
below).
The elbow – or more precisely the olecranon bursa –
is a favourite site for gout. In an acute attack the area
rapidly becomes painful, swollen and inflamed. The
swelling and redness may extend well down the fore- OSTEOARTHRITIS (see also Chapter 4)
arm and the condition is easily mistaken for cellulitis
or joint infection. The serum uric acid level may be Osteoarthritis of the elbow is uncommon and usually
raised and the bursal aspirate will contain urate crys- denotes some recognizable underlying pathology – a
tals. Treatment is with high dosage anti-inflammatory previous fracture or ligamentous injury, loose bodies
preparations. in the joint, longstanding occupational stress, inflam-
Similar attacks occur in pseudogout, due to the matory arthritis or gout. ‘Primary’ osteoarthritis –
deposition of CPPD crystals, which can be identified especially when it is part of a polyarticular disorder –
in the aspirate (see Chapter 4). suggests calcium pyrophosphate deposition disease
Chronic calcium pyrophosphate arthropathy This condi- (see above).
tion should always be suspected when ‘osteoarthritic’
changes appear spontaneously in an unusual site such Clinical features
as the elbow; x-rays may show, additional features such
as chondrocalcinosis and peri-articular calcification. The patient usually complains of pain and stiffness,
The diagnosis can be confirmed by demonstrating the especially following periods of inactivity. Examination
shows local tenderness, thickening of the joint, crepi-
tus and restriction of movement. Osteophytic hyper-
14.12 Pyrophosphate trophy may cause ulnar nerve palsy.
arthropathy
Osteoarthritis of the
elbow is unusual except X-rays
after trauma. These
x-rays show a X-ray examination shows narrowing of the joint space
destructive arthritis and with sclerosis and osteophytes. One or more loose
typical flared bodies may be seen; chondrocalcinosis and peri-artic-
osteophytes in a patient ular calcification are typical of pyrophosphate
with generalized
pyrophosphate
arthropathy.
arthropathy.
Treatment
Treatment is usually limited to pain control and the
use of non-steroidal anti-inflammatory preparations. 375
14 the anterior compartment (the so-called ‘OK proce-
dure’). This will improve movement and impinge-
ment pain, often for several years.
An alternative to joint replacement in the younger
patient is an interposition arthroplasty, in which a
layer of fascia, subcutis or tendon is placed into the
joint space. A hinged external fixator maintains some

distraction yet allows movement and protects the
reconstruction.
In advanced cases in older patients, joint replace-
ment can be considered; however, upper limb activi-
ties will have to be permanently restricted in order to
reduce the risk of implant loosening.
(a)
NEUROPATHIC ARTHRITIS
Neuropathic arthritis of the elbow is seen in
syringomyelia and diabetes mellitus. Sometimes neu-
rological features predominate and the diagnosis may
be known; occasionally the patient presents with pro-
gressive instability of the elbow. The joint may be
markedly swollen and hypermobile, with coarse crepi-
tation on passive movement, or it may be completely
flail.
The condition must be distinguished from other
causes of flail elbow, such as advanced rheumatoid
arthritis and unreduced (or ununited) fracture-
dislocations.
(b) (c) Treatment consists of splintage to maintain stability.
Arthrodesis usually fails and is functionally disabling.
14.13 Osteoarthritis
(a) Valgus elbow; (b,c) x-ray A semi-constrained arthroplasty is technically difficult
with new bone and loose and prone to early failure in this setting.
bodies; (d) transposition of
ulnar nerve anteriorly to
treat the associated ulnar
nerve symptoms. STIFFNESS OF THE ELBOW
Stiffness of the elbow may be due to congenital abnor-

malities (various types of synostosis, or arthrogrypo-
sis), infection, inflammatory arthritis, osteoarthritis or
the late effects of trauma. Most of these conditions
(d) are dealt with in other chapters. Here consideration
will be given to post-traumatic stiffness, which is an
important cause of disability.
Loose bodies, if they cause locking, should be
removed. If there are signs of ulnar neuropathy, the
nerve should be transposed. POST-TRAUMATIC STIFFNESS
Debridement of the joint may be helpful. This can
be done arthroscopically with debridement of syn- For reasons that are not entirely clear, the elbow is
ovium and loose cartilage, burring of osteophytes, particularly prone to post-traumatic stiffness. The
trimming of the olecranon and coronoid fossae and more obvious causes (as with other joints) are either
removal of loose cartilage. The debridement can also extrinsic (e.g. soft-tissue contracture or heterotopic
be performed by an open approach. Through a dorsal bone formation), intrinsic (e.g. intra-articular adhe-
incision the posterior compartment is cleared; the thin sions and articular incongruity), or a combination of
376 bone of the olecranon fossa is then removed to expose these. Clinical assessment should include examination
of all the joints of the upper limb as well as an evalu- 14
ation of the functional needs of the particular patient.
Most of the activities of daily living can be managed
with a restricted range of elbow motion: flexion from
30 to 130 degrees and pronation and supination of 50
degrees each. Any greater loss is likely to be disabling.

NON-OPERATIVE TREATMENT
The most effective treatment is prevention, by early
active movement through a functional range. If move-
ment is restricted and fails to improve with exercise,
serial splintage may help; aggressive passive manipula-
tion may aggravate more than help.
OPERATIVE TREATMENT
The indication for operative treatment is failure to
regain a functional range of movement at 12 months
(a)
after injury. There are a few caveats: the limb as whole
should be useful; there should be no over-riding neu-
rological impairment; and the patient should be coop-
erative and motivated. If there is heterotopic
ossification, it is important to wait until the bone is
‘mature’, i.e. showing clear cortical margins and tra-
becular markings on x-ray. There is no point in a soft-
tissue release if the x-ray or CT shows that bone
incongruity is blocking movement.
The objectives are determined by the type of
pathology. Heterotopic bone can be excised. Capsular
release or capsulectomy (open or arthroscopic) may
restore a satisfactory range of movement. Intra-artic-
ular procedures include fixing of ununited fractures or
correction of malunited fractures.
Post-traumatic radio-ulnar synostosis sometimes fol-
lows internal fixation of fractures of the radius and
(b)
ulna. It is treated by resection when the synostosis has
matured (this takes about one year) followed by dili-
gent physiotherapy.
RECURRENT ELBOW INSTABILITY
Following a dislocation or severe sprain, the lateral

collateral ligament can be stretched or ruptured. The
patient may present with painful clunking and lock-
ing. On examination, an apprehension response can
be elicited by supinating the forearm while applying a
valgus force to the elbow during flexion.
The lateral collateral ligament can be directly
repaired or reconstructed with a tendon autograft
(e.g. palmaris longus).
Medial instability is less frequent after trauma; a
chronic instability can develop in javelin throwers and
(c) baseball players. Ligament reconstruction with a ten-
14.14 Elbow stiffness (a) Osteochondrosis; (b) radio- don graft and careful graduated rehabilitation can give
ulnar synostosis; (c) osteoarthritis. very good results. 377
14
(a) (b) (c) (d)

14.15 Flail elbow (a,b) Following gunshot wound; (c,d) neuropathic arthritis.
common extensor tendon) is a common complaint

EPICONDALGIA among tennis players – but even more common in
non-players who perform similar activities involving
The elbow is prone to painful disorders of the tendon forceful repetitive wrist extension. It is the extensor
attachment. Sometimes this occurs spontaneously, carpi radialis tendon (which automatically extends the
sometimes after sudden unaccustomed use. These wrist when gripping) which is pathological in tennis
conditions have acquired names derived from the elbow (Fig. 14.16). Like supraspinatus tendinitis, it
activities in which they were encountered when they may result in small tears, fibrocartilaginous metaplasia,
were first described. microscopic calcification and a painful vascular reac-
tion in the tendon fibres close to the lateral epicondyle.
TENNIS ELBOW (LATERAL EPICONDALGIA) Clinical features

Pain and tenderness over the lateral epicondyle of the The patient is usually an active individual of 30 or 40
elbow (or, more accurately, the bony insertion of the years. Pain comes on gradually, often after a period of
(b)
(a)
14.16 Tennis elbow (a) Tenderness over the anterior

aspect of the lateral epicondyle; (b) pain provoked by
resisted wrist extension; (c) tennis elbow surgery – the
378 abnormal extensor carpi radialis brevis origin is excised. (c)
unaccustomed activity involving forceful gripping and neuropathy. A medial collateral ligament injury 14
wrist extension. It is usually localized to the lateral should be excluded.
epicondyle, but in severe cases it may radiate widely. It Treatment is the same as for lateral epicondylitis but
is aggravated by movements such as pouring out tea, the outcome of surgery seems less predictable. The
turning a stiff doorhandle, shaking hands or lifting abnormal tissue at the flexor–pronator origin is
with the forearm pronated. Among tennis players it is excised, great care being taken to preserve the medial

usually blamed on faulty technique. collateral ligament. The medial antebrachial cuta-
The elbow looks normal, and flexion and extension neous nerve must be respected during the skin inci-
are full and painless. sion to avoid a troublesome postoperative neuroma.
Characteristically there is localized tenderness at or
just below the lateral epicondyle; pain can be repro-
duced by passively stretching the wrist extensors (by BASEBALL PITCHER’S ELBOW
the examiner acutely flexing the patient’s wrist with
the forearm pronated) or actively by having the Repetitive, vigorous throwing activities can cause
patient extend the wrist with the elbow straight. damage to the bones or soft-tissue attachments
X-ray is usually normal, but occasionally shows cal- around the elbow. Professional baseball players may
cification at the tendon origin. develop hypertrophy of the lower humerus and incon-
gruity of the joint, or loose-body formation and
osteoarthritis. The junior equivalent (‘little leaguer’s
Diagnosis elbow’) is due to partial avulsion of the medial epi-
In patients with longstanding symptoms which do not condyle. The only remedy – however grudgingly
respond to treatment, the possibility of a painful radial accepted – is to stay off baseball until the condition
nerve entrapment (‘radial tunnel syndrome’) should clears up completely.
be considered (see Chapter 11).
Treatment JAVELIN THROWER’S ELBOW

Many methods of treatment are available but the ben- The over-arm action employed by javelin throwers
efits of most are unclear; it is well to remember that may avulse or cause impingement upon the tip of the
90 per cent of ‘tennis elbows’ will resolve sponta- olecranon process. However, this sport (like other
neously within 6–12 months. throwing sports) places huge strain on the medial col-
The first step is to identify, and then restrict, those lateral ligament which can become either acutely
activities which cause pain. Modification of sporting injured or chronically attenuated. There may also be
style may solve the problem. A tennis elbow clasp is symptoms of ulnar nerve impairment. The pain usu-
helpful. The role of physiotherapy and manipulation is ally settles down after a period of rest and modifica-
uncertain. Injection of the tender area with corticos- tion of activities. However, an attenuated medial
teroid and local anaesthetic relieves pain but is not collateral ligament may need reconstruction with a
curative. tendon graft.
OPERATIVE TREATMENT
Some cases are sufficiently persistent or recurrent for
operation to be indicated. The origin of the common
AVULSION OF THE DISTAL TENDON
extensor muscle is detached from the lateral epi-
condyle. Additional procedures such as division of the OF BICEPS
orbicular ligament or removal of a ‘synovial fringe’ are
sometimes advocated; they probably make very little The typical patient is a man of about 45 years who
difference to the outcome. Surgery is successful in feels sudden pain and weakness at the front of the
about 85 per cent of cases. elbow after strenuous effort. Feel for the distal biceps
tendon while the patient flexes the elbow against
resistance (ask him to grip the desk or table as if to lift
it; normally the biceps tendon stands out as a taut
GOLFER’S ELBOW (MEDIAL cord across the elbow crease). Loss of supination
EPICONDYLITIS) power with the elbow flexed (negating supinator
muscle) is a good physical sign. The tendon may be
This is similar to tennis elbow but about three times partially or completely avulsed from its insertion into
less common. In this case it is the pronator origin that the bicipital tuberosity of the radius.
is affected. Often there is an associated ulnar nerve The diagnosis is often missed because elbow flexion 379
14 and supination, although weaker than normal, are A chronically enlarged bursa may prove a severe
preserved by brachialis and supinator action. MRI nuisance and need to be excised. However, wound
helps to confirm the diagnosis but must not delay healing can be a problem.
surgical treatment. Clinical diagnosis should usually
suffice.
Treatment OPERATIONS
Operative repair is not always necessary; some patients
are content to manage with slightly reduced elbow ARTHROSCOPY
flexion: in time, the other elbow flexors will compen-
sate (brachioradialis, brachialis). However, there will Arthroscopy of the elbow is technically demanding; its
be a very obvious cosmetic defect and greatly reduced role for diagnosis and treatment continues to evolve.
power of supination. For these reasons, many patients
will choose repair. The best results are achieved by
operation within 2 weeks, before the tendon retracts
Indications
and the interosseous tunnel becomes occluded. A An arthroscopic approach may be employed for intra-
two-incision technique is recommended to avoid articular procedures such as removal of loose bodies,
nerve damage and heterotopic ossification; tissue irrigation for infection or trimming of osteophytes.
anchors or sutures-through-drillholes can be used to More advanced indications include synovectomy, cap-
attach the tendon to its insertion point. The results of sular release, removal of coronoid or olecranon osteo-
early surgery and careful rehabilitation are usually very phytes and radial head excision.
good.
Technique
The risk of this operation is a devastating injury to the
BURSITIS median nerve, ulnar nerve or posterior interosseous
nerve, each of which lies less than a centimetre from
The olecranon bursa sometimes becomes enlarged as the joint and very close to the portals used for access.
a result of continual pressure or friction; this used to The operation therefore requires special training and
be called ‘student’s elbow’. If the enlargement is a a very clear appreciation of the anatomy. Pre-disten-
nuisance the fluid may be aspirated. sion of the joint with fluid and the use of blunt
The commonest non-traumatic cause is gout; there trochars help to reduce the risk. Capsulectomy carries
may be a sizeable lump with calcification on x-ray. In a particularly high risk.
rheumatoid arthritis, also, the bursa may become en-
larged, and sometimes nodules can be felt in the lump
or just distal to it over the proximal ulna. In both con- ARTHROPLASTY
ditions other joints are likely to be affected as well.
A complex anatomy and relatively fragile bone struc-
ture make it more challenging to repeat the success
stories of hip and knee replacement. Nevertheless, in
specific circumstances it is better than the alternative
of a painful, stiff or unstable joint.
Indications
The most common indication for arthroplasty is
rheumatoid arthritis; it is also occasionally suitable for
the treatment of osteoarthritis. It has a valuable role
in the treatment of comminuted distal humerus frac-
tures in osteopaenic bone for those individuals with
lower demands. Elbows which are ankylosed (e.g. due
to previous infection) can be successfully salvaged
with elbow replacement.
14.17 Olecranon bursitis The enormous red lumps over
the points of the elbows are enlarged olecranon bursae; One should think carefully before advocating this
the ruddy complexion completes the typical picture of operation to patients who intend to return to heavy
380 gout. work or leisure activities or to those with single-joint
disease, i.e. without the protective effect against over- 130 degrees and 90 degrees of both pronation and 14
use of other involved joints in the same limb. supination, the functional range of movement is 30–
130 degrees of flexion and 50 degrees both pronation
and supination.
Design
The forearm is normally in slight valgus relative to
Earlier constrained (single-axis hinge) implants had a the upper arm, the average carrying angle being about

high failure rate due to loosening. Unconstrained 15 degrees. The complex geometry of the joint allows
designs are associated with instability and dislocation. for the fact that when the elbow is flexed the forearm
However, 90 per cent good results can be achieved in comes to lie directly upon the upper arm. The carry-
carefully selected patients (those with good bone stock ing angle may be altered by malunion of a fracture or
and competent ligaments). Semi-constrained implants by damage to the physis, resulting in cubitus valgus or
allow some of the forces to be absorbed by the soft tis- cubitus varus.
sues whilst maintaining some intrinsic stability. The joint acts as a ‘sloppy hinge’, permitting a few
degrees of valgus/varus movement and some rota-
tional laxity. Stability is provided by: (1) the relative
Outcome
conformity of the humeral trochlea with the olecranon;
The majority of patients with an elbow replacement (2) the medial collateral ligament (particularly the an-
can expect relief of pain and a functional range of terior band); and (3) the lateral collateral ligament
movement. Ten-year survival rates as high as 80 per (particularly the ulnar part). The radial head is a sec-
cent have been achieved in patients with rheumatoid ondary constraint to valgus instability; it can be excised
arthritis (perhaps the joint is protected because of with relative impunity as long as the medial collateral
poor function in the rest of the limb) whereas the sur- ligament, humero-ulnar articulation and interosseous
vival rate for those with osteoarthritis or distal membrane are intact. The elbow is not really a ‘non-
humeral non-union is less certain. A good outcome weightbearing’ joint – forces of up to three times body
can also be achieved in selected trauma patients (older weight pass across it with normal use.
individuals with low demand). Pronation and supination take place mainly at the
radio-ulnar joints with a small amount of abduction
and adduction between the olecranon and the
Complications
trochlea. The movement is often supplemented by
The operation has a relatively high complication rate, rotation at the shoulder. The humeroradial joint is
particularly ulnar nerve palsy, wound failure and col- held in position by the strong annular (orbicular) and
lateral ligament instability. This is particularly likely in collateral ligament which embraces the head and neck
patients with inadequate bone stock due to rheumatoid of the radius but is not attached to it. The capsule of
disease, previous infection or previous operations. the elbow is attached to the annular ligament but is
not attached to the radius. The circular and slightly
concave upper surface of the radius ensures that in all
ARTHRODESIS positions of rotation it retains adequate contact with
the hemispheric capitulum.
Arthrodesis is rarely indicated. It is a technically diffi-
cult and very disabling procedure. Even with normal
Nerves
wrist and shoulder function it is not possible to fuse the
elbow in a position which would facilitate both feeding The ulnar nerve passes behind the medial condyle of
(i.e. 100 degrees of flexion) and perineal hygiene the humerus; it may be stretched if there is marked
(about 45 degrees of flexion). Compression plating is cubitus valgus. Distal to the condyle the nerve is
the most straightforward and stable technique. closely applied to the elbow capsule, and there also it
may be compromised if the joint is osteoarthritic.
On the lateral side of the elbow the radial nerve passes
between brachialis and brachioradialis. It then splits to
NOTES ON APPLIED ANATOMY become the superficial radial nerve and the posterior in-
terosseous nerve. The latter passes beneath extensor
The elbow needs to be able to convey the hand carpi radialis brevis and then between two parts of the
upwards to the head and mouth, downwards to the supinator muscle; it is vulnerable to injury during surgi-
perineum and legs, and also to a wide variety of work- cal approaches to the proximal part of the radius.
ing positions at bench, desk, wall or table. A varied In front of the elbow lie the brachialis muscle and
combination of flexion and extension with pronation also the median nerve in company with the great ves-
and supination is clearly needed. Although the normal sels; these relationships make an anterior approach to
elbow is capable of full extension, flexion to about the elbow somewhat challenging. 381
The wrist
15
The wrist and hand function together, for all practical SIGNS
purposes, as a single articulated unit. The hand would
be unable to perform its range of complicated move- Examination of the wrist is not complete without also
ments without the reciprocal movement, positioning examining the elbow, forearm and hand. Both upper
and stabilizing action of the wrist. Loss of movement limbs should be completely exposed.
at the wrist limits the manipulative skill of the fingers
and thumb; and pain in the wrist makes it impossible
to grip or pinch with full strength. Disorders of the
Look
wrist and hand are often interrelated and therefore, in The skin is inspected for scars. Both wrists and fore-
the clinical setting, these two units should be exam- arms are compared to see if there is any deformity. If
ined and analysed together. However, for the sake of there is swelling, note whether it is diffuse or localized
emphasis, they are treated here in two separate chap- to one of the tendon sheaths. Look also at the hands
ters. and fingers to see if there are any related abnormali-
ties.
The posture of the wrist at rest and during move-
ment varies with different positions of the hand and
CLINICAL ASSESSMENT fingers. This is discussed in the opening sections of
Chapter 16.
SYMPTOMS
Feel
Pain may be localized to the radial side (especially in
Palpation of the wrist will yield valuable information
de Quervain’s disease and thumb base arthritis), to
only if the surface anatomy is thoroughly understood
the ulnar side (e.g. in distal radio-ulnar joint arthritis
and piso-triquetral arthritis) or to the dorsum (in
radio-carpal arthritis, Kienböck’s disease and occult (f)
dorsal wrist ganglion). (e)
Stiffness is often not noticed until it is severe in the
flexion–extension plane; loss of rotation is noticed ear-
lier and can be very disrupting.
Swelling may signify involvement of either the joint
or the tendon sheaths or a ganglion.
(a)
Deformity is a late symptom except after trauma or
radial nerve palsy. Ask if it is localized to a particular site (b)
(e.g. an overly-prominent head of ulna, suggesting (c)
subluxation of the distal radio-ulnar joint) or involving
(d)
the posture of the wrist as a whole [progressive radial
deviation in advanced rheumatoid arthritis (RA)].
Loss of function refers mainly to the hand, though 15.1 Tender points at the wrist (a) Tip of the radial
the patient may be aware that the problem lies in the styloid process; (b) anatomical snuffbox, bounded on the
radial side by (c) the extensor pollicis brevis and on the
wrist. ulnar side by (d) the extensor pollicis longus; (e) the
Clicks are common and usually of no relevance; extensor tendons of the fingers; and (f) the head of the
clunks with pain or weakness may signify instability. ulna.
15
(a) (b) (c)
15.2 (a) Tenderness at the tip of the radial styloid suggests de Quervain’s disease (tenovaginitis of the combined sheath for
extensor pollicis brevis and abductor pollicis longus). This diagnosis can be confirmed by Finkelstein’s test. Hold the patient’s
hand with his thumb tucked firmly unto the palm; then turn the wrist into full ulnar deviation; in a positive test, this will
elicit sharp pain in the affected sheath. (b) Tenderness in the anatomical snuffbox is typical of a scaphoid injury
(c) Tenderness just distal to the head of the ulna is found in extensor carpi ulnaris tendinitis.
(a) (b) (c) (d)
15.3 (a-f) Testing for wrist

flexion, extension, ulnar
deviation, radial deviation,
pronation and supination.
When testing pronation and
supination, the patient must
keep his elbows flexed.
(g,h) This is a good way to test
flexion and extension of the
wrists; you can compare the
two sides.
(e) (f)
384 (g) (h)

(see Figure 15.1). Tender areas must be accurately Provocative tests 15
localized and the various landmarks compared with
those of the normal wrist. The site of tenderness may Special tests are needed to assess stability of the carpal
be diagnostic, for example in de Quervain’s disease articulations. The luno-triquetral joint is tested by
(tip of radial styloid), scaphoid fracture (anatomical gripping or pinching the lunate with one hand, the
snuffbox), carpo-metacarpal osteoarthritis (base of triquetral-pisiform with the other, and then applying a
The wrist
first metacarpal), Kienböck’s disease (over the lunate), sheer stress: pain or clicking suggests an incompetent
triangular fibrocartilage complex (just distal to the luno-triquetral ligament. The piso-triquetral joint is
head of the ulna) and localized tenosynovitis of any of tested by pushing the pisiform radialwards against the
the wrist tendons. At the same time note if the skin triquetrum. Stability of the scapho-lunate joint is
feels unduly warm. tested by pressing hard on the palmar aspect of the
If the head of the ulna seems abnormally prominent scaphoid tubercle while moving the wrist alternately
on the dorsum of the wrist, try to jar the distal radio- in abduction and adduction: pain or clicking on
ulnar joint by pressing down sharply on the ulnar abduction (radial deviation) is abnormal. The trian-
prominence; if it moves up and down the joint is gular fibrocartilage is tested by pushing the wrist
unstable (this is aptly named the ‘piano-key sign’). medially then flexing and extending it. The distal
radio-ulnar joint is tested for stability by holding the
radius and then ballotting the ulnar head up and
Move down. These tests are mentioned again in the section
Passive movements To compare passive dorsiflexion of on carpal instability.
the wrists the patient places his palms together in the
position of prayer, then elevates his elbows. Palmar
flexion is examined in a similar way. Radial and ulnar
deviation are measured in either the palms-up or the
palms-down position. With the elbows at right angles IMAGING
and tucked in to the sides, pronation and supination
are assessed. X-rays
While testing passive movements, the presence of Anteroposterior and lateral views are obtained rou-
abnormal ‘clunks’ should be noted; they may signify tinely. Note the position and shape of the individual
one or other form of carpal instability. carpal bones and whether there are any abnormal
Active movements Ask the patient to pull the hand spaces between them. Then look for evidence of joint
backwards to its limit (extension), then forwards as far space narrowing, especially at the radio-carpal joint
as possible (flexion), and then sideways to right and and the carpo-metacarpal joint of the thumb. The
left (radial and ulnar deviation). Active pronation wrist x-ray should be taken in a standard position of
and supination should be performed with the mid-pronation with the elbow at 90 degrees; often
patient’s elbows tucked tightly into the waist. These both wrists must be x-rayed for comparison. Special
movements are then repeated but carried out against views may be necessary to show a scaphoid fracture or
resistance, to test for muscle power. Finally, grip carpal instability. Moving the wrist under image inten-
strength is measured, preferably using a mechanical sification is useful to investigate some cases of carpal
dynamometer. Loss of power may be due to pain, ten- instability.
don rupture or muscle weakness.
Arthrography
The wrist contains three separate compartments – the
radio-carpal joint, the distal radio-ulnar joint and the
midcarpal joint. Defects in the triangular fibrocarti-
lage, scapho-lunate ligaments or luno-triquetral liga-
ments can be identified by arthrography.
Computed tomography
CT is the ideal method for assessing congruity of the
15.4 Normal range of movement From the neutral
position dorsiflexion is slightly less than palmarflexion. distal radio-ulnar joint, fractures of the hook of
Most hand functions are performed with the wrist in ulnar hamate, and alignment of scaphoid fractures prior to
deviation; normal radial deviation is only about 15°. surgery for non-union or malunion. 385
15 CONGENITAL ANOMALIES OF THE
WRIST AND HAND
Abnormalities occurring in the upper limb anlage dur-

ing the first three months of embryonic life are likely
to affect more than one segment (or indeed the

e g whole) of the developing limb; not surprisingly, there-
d fore, congenital anomalies often occur together in the
f forearm, wrist and hand. For this reason, we have
c
a dealt with the subject under a single heading.
b The embryonic arm buds appear about 4 weeks
after fertilization and from then on the limbs develop
progressively from proximal to distal. By 6 weeks the
digital rays begin to appear and then develop in con-
cert with the general mesenchymal differentiation that
gives rise to the primitive skeleton and muscles.
Growth goes hand in hand with genetically pro-
grammed cell death that results in modelling of the
15.5 X-ray Note the shape and position of the bones limbs and the formation of joints and separate digits.
which make up the normal carpus: (a), scaphoid,
The process is more or less complete by the end of the
(b), lunate, (c), triquetrum overlain by pisiform,
(d), trapezium, (e), trapezoid, (f), capitate, (g), hamate. eighth week after fertilization, at which time primary
ossification centres begin to appear in the long bones.
Ossification centres in the epiphyses and carpal bones
do not emerge until after birth, so x-rays in the
Magnetic resonance imaging neonatal period must be interpreted with this in mind.
Malformations may occur during embryonic devel-
MRI is particularly useful for detecting changes asso- opment because of either defective formation or
ciated with scaphoid fractures, avascular necrosis of incomplete separation of mesenchymal components,
the lunate (Kienböck’s disease), occult dorsal ganglia the former accounting for partial or complete absence
and intra-osseous ganglia. The thickness of the cuts of a part and the latter for coalitions between adjacent
may be too large to detect injury to thin structures elements. It must also be remembered that other
such as the luno-triquetral ligament, scapho-lunate organs developing during the same period may also be
ligament or triangular fibrocartilage. MRI arthrogra- affected; thus musculoskeletal malformations are
phy increases the sensitivity. often associated with other abnormalities.
The overall incidence of congenital upper limb
Radionuclide scan anomalies is estimated to be about 1 in 600 live
births, but in only a fraction of those affected are the
A localized area of increased activity may reveal an defects severe enough to require operative treatment.
osteoid osteoma, an occult scaphoid fracture or early Some of the malformations are caused by heritable
osteoarthritis. genetic mutations or by intrauterine damage from
drugs, infection or ionizing radiation; in the majority
Fluoroscopy of cases the cause is unknown.
Fluoroscopic examination may be needed to demon-

strate some patterns of carpal instability. CLASSIFICATION
The classification of congenital limb malformation
ARTHROSCOPY adopted by the International Federation of Societies
for Surgery of the Hand (IFSSH) lists seven major
The wrist is suspended by finger traps, inflated with categories: (1) failure of formation of parts; (2) failure
saline and inspected through specific portals into the of differentiation of parts; (3) duplication; (4) over-
radio-carpal joint, the ulno-carpal joint and the mid- growth; (5) undergrowth; (6) constriction bands; and
carpal joint. Ligament tears, articular cartilage dam- (7) generalized skeletal abnormalities. Some condi-
age, osteoarthritis, occult ganglia, synovitis and tions do not fit readily into a single category (e.g.
triangular fibrocartilage lesions can be recognized and thumb hypoplasia, which sits equally well in ‘failure of
386 in some cases treated. formation’ and ‘undergrowth’).
GENERAL CONSIDERATIONS • Appearance: The hand is second only to the face in 15
self-consciousness of appearance. If it looks normal,
Initial consultation a child is more likely to use it normally. If it looks
abnormal, the child will hide it away. This concept
The parents and child are likely to be anxious, and is known as ‘dynamic cosmesis’.
may have been given conflicting information by non- • Pain: Although malformations are usually not
The wrist
specialist physicians. There may be issues of maternal painful, some may come to need treatment for this
guilt, parental anger and resentment, and unrealistic reason. An example is a tender fingertip in constric-
expectations about the outcome and possibilities of tion ring syndrome when there is poor soft-tissue
surgery. It is important to gain the confidence of the cover over the bone.
family at the initial consultation; remember that the
children are likely to be long-term patients.
They must be given a diagnosis, an indication of
prognosis, reassurance about the future and a long-term FAILURE OF FORMATION
plan of treatment, including a schedule of surgery,
which may have to be carried out in several stages. Transverse arrest
Many children manage well into adulthood with
untreated congenital anomalies, and the requirement This can exist anywhere between the shoulder and the
for surgery is not always clear. phalanges. The most common levels of absence are at
the proximal forearm and mid-carpus, then at the
metacarpals and humerus. Associated anomalies are
Clinical examination unusual.
The clinic should be held in a child-friendly setting. Proximal forearm Prosthetic fittings in young children
Toys should be available to allow children to play in may be desirable for cosmetic reasons. For older chil-
an unrestrained manner, which permits close observa- dren and adolescents, myoelectric prostheses may be
tion of hand function. It may be easier to examine a considered and can improve function, though many
child while he or she is sitting on the parent’s lap. The youngsters manage surprisingly well without them.
diagnosis is not always obvious, though the absence of Transverse arrest of fingers The child with vestigial
skin creases suggests some congenital abnormality fingers (symbrachydactyly) can be treated by microvas-
such as absent joints or joints which do not move. cular transfer of a toe if there are proximal enabling
Remember that many congenital wrist and hand structures available (skin, tendons and nerves), or by
anomalies are part of a larger syndrome. Radial dys- non-vascularized transfer of a toe phalanx into the
plasia, for example, may be associated with vertebral existing skin envelope.
anomalies, anal atresia, cardiovascular anomalies, tra-
cheo-oesophageal fistula, renal anomalies and other
limb defects (embodied in the acronym VACTERL). Longitudinal arrest
The child should always be investigated fully and, if Longitudinal arrest may involve radial (pre-axial),
necessary, referred to other specialists. Genetic coun- ulnar (post-axial), central (cleft hand) or intersegmen-
selling should be made available for inherited or tal (intercalated) structures.
unusual conditions, and indeed may be helpful in
reaching a diagnosis. RADIAL DYSPLASIA
This rare condition (incidence 1:50 000 to 1:100 000
live births) may involve any (or all) of the structures
Indications for operative treatment from the elbow to the thumb. It usually occurs as an
Whenever the need for operative treatment is consid- isolated abnormality but is occasionally associated
ered, four general precepts should be borne in mind: with other skeletal, cardiac, haematological, renal or
craniofacial anomalies, which should be sought.
• Function: Consider how important is the affected
The infant is born with the wrist in marked radial
part to everyday activity, for example when deciding
deviation – hence the use of the term ‘radial club hand’;
whether to use a normal index finger to reconstruct
half the patients are affected bilaterally. There is absence
an absent or defective thumb.
of the whole or part of the radius; often the thumb,
• Progression of deformity: Decide whether further
scaphoid and trapezium fail to develop normally.
growth is likely to increase the deformity or give
rise to other deformities. For example, syndactyly Treatment Mild radial dysplasia is treated from birth
involving digits of unequal length – say the ring and by gentle stretching and splintage, best done by the
little fingers – may cause progressive deviation of parents. More serious cases can be treated by distrac-
the fingers. tion prior to a tension-free soft-tissue correction 387
15 15.6 Radial dysplasia
(a) Bilateral. (b) X-ray
showing that the entire
radius is absent.
(a) (b)
which has less effect on growth of the carpus and dis-

tal ulna than the older technique of ‘centralizing’ the
carpus over the remaining forearm structures. Pro-
longed splintage is still required to avoid recurrence
of the deformity. Attention must be paid to the elbow;
if the joint is stiff, the radially deviated wrist can actu-
ally be advantageous, as the child can then get the
hand to his or her mouth (for eating) and the per-
ineum (for toilet care). Surgical correction of the wrist
in these cases can result in a functional disaster.
If the thumb is affected this can present serious
problems in treatment. Hypoplasia may be associated
with a tight first web space and instability of the
metacarpophalangeal (MCP) joint, requiring
advanced reconstructive surgery, tendon transfers and
joint stabilization. If the thumb is absent, pollicization
of the index finger or microvascular toe transfer may
be required. (See also below under Undergrowth.)
ULNAR DYSPLASIA
This is even less common than radial dysplasia. Most
cases are sporadic, but the condition may be part of a
larger syndrome, together with anomalies in other
limbs.
Here the new-born infant presents with ulnar devi- 15.7 Distal ulnar deformity The x-ray characteristically
ation of the wrist (or both wrists), due to partial or shows a tapering, carrot-shaped distal end of ulna. This
complete absence of the ulna; in addition some of the bilateral case was due to hereditary multiple exostoses;
carpal bones may be absent and the ulnar rays of there is bilateral bowing of the radius and on the right side
the hand may be missing. With growth the radius the radial head has subluxated.
elongates disproportionately and becomes bowed;
ultimately the radial head may dislocate.
or dyschondroplasia. If the distal ulna is affected in
Treatment During the first few months stretching and
these conditions, growth at the distal physis may be re-
splinting may be helpful. If wrist deformity and radial
tarded; the distal ulna tapers to a carrot shape and is
bowing are progressive and severe, surgery may be
short. If the radius remains unaffected and goes on
advisable and consists of excision of any tethering
growing normally, it becomes bowed and the radial
ulnar anlage and osteotomy of the radius. If the radial
head tends to subluxate or dislocate (see page 161). In
head has dislocated and elbow movement is restricted,
most cases the elbow and forearm are completely sta-
the radial head can be excised; if the forearm is unsta-
ble and no treatment is needed (except, possibly, for
ble, the distal radius can be fused to the proximal ulna
cosmetic reasons).
(the Straub procedure).
Secondary ulnar dysplasia A similar but milder deformity CENTRAL DYSPLASIA (CLEFT HAND)
sometimes occurs in children over the age of about 10 True cleft hand presents with a V-shaped cleft in the
388 years who were born with hereditary multiple exostoses centre of the hand which may be associated with the
absence of one or more digits, transverse bones, syn- If there is no significant loss of function then oper- 15
dactyly of digits bordering the cleft, and a tight first ative treatment is unnecessary. If important move-
web space. It is often familial (dominant inheritance), ments are affected (e.g. uncompensated loss of
may be unilateral or bilateral, and can be associated forearm rotation in proximal radio-ulnar synostosis,
with ‘cleft feet’. Other anomalies, such as cleft lip, or fusion at the elbow joint), osteotomy and re-posi-
cleft palate and congenital heart disease may also be tioning of the limb in a more favourable position may
The wrist
present. The condition differs from so-called ‘atypical be considered. Carpal fusions usually need no treat-
cleft hand’ (symbrachydactyly), which is not heritable ment.
and not associated with other anomalies.
Surgery is complex, having to deal with closure of
Camptodactyly
the cleft, reconstruction of the first web space and – in
some cases – correction of other anomalies in the ‘Bent finger’ is a flexion deformity of the proximal
adjacent digits. Redundant soft tissue from closing the interphalangeal joint, usually of the little finger. It
cleft can be used to augment the tight first web space. may be an isolated condition or part of a syndrome. It
may be inherited or sporadic, and two-thirds of cases
INTERCALARY SEGMENTAL DYSPLASIA are bilateral.
Very rarely an intercalary segment in the upper limb The condition presents as two groups: those occur-
fails to develop and the forearm or hand may be ring in infancy and affecting males and females
attached directly to the trunk, or the hand is attached equally, and those presenting in adolescence, mainly
to the humerus. This condition, also known as pho- affecting females. There is often an abnormal muscle
comelia, may affect more than one limb and is some- insertion (usually one of the lumbricals), and there
times associated with craniofacial deformities. may be a characteristic abnormal radiographic appear-
For the upper limb, there is no satisfactory treat- ance of the head of the proximal phalanx.
ment apart from designing and fitting a cosmetically The mainstay of treatment is splinting. Surgery may
preferable prosthesis. be indicated if the deformity is marked or is a severe
nuisance. Soft-tissue releases and/or muscle transfers
are advocated by some surgeons but the results are
disappointing. If there is a bony block to interpha-
FAILURE OF DIFFERENTIATION langeal extension, a corrective osteotomy will improve
the situation.
Syndactyly
Clinodactyly In this condition a digit is bent sideways
Conjoined digits is the commonest congenital malfor-
(radially or ulnarwards), usually due to an abnormally
mation of the hand (incidence about 1:2000 live
shaped middle phalanx – a so-called ‘delta’ deformity
births). The anomaly may be simple (soft tissue only)
in which the epiphysis is curved. It usually affects the
or complex (skin and bone), complete (affecting the
little finger and is often inherited and bilateral. As it is
entire web) or incomplete (only part of the web).
often part of a more widespread syndrome, the child
Mild, incomplete syndactyly of central digits may
should be examined for other defects. Severe cases can
need no treatment. Treatment of complete syndactyly
be treated by corrective osteotomy and bone grafting.
involves separation of the conjoined structures and
The condition must be distinguished from Kirner’s
skin grafting. When multiple digits are involved
syndrome, in which the distal phalanx of the little fin-
(achrosyndactyly), this should be tackled one web space
ger is similarly bent. This usually presents in adoles-
at a time, at separate operations, so as to avoid poten-
cence and treatment is the same as for clinodactyly.
tial compromise of both digital arteries. If the border
digits (thumb and index, ring and little fingers) are
affected this can cause progressive deformity with
growth and requires early surgical reconstruction. DUPLICATION
Polydactyly (‘extra digits’) may occur on the radial
Synostosis (pre-axial), the ulnar (post-axial) or the central part of
Failure of embryological separation of skeletal compo- the hand.
nents can result in conjoined normal-looking bones or Duplication of the little finger is one of the most
fused (unseparated) joints. This may occur at any level common congenital anomalies of the hand. It is often
from the fingers to the humerus and can be longitudi- inherited and is much commoner in black people than
nal (e.g. humero-ulnar synostosis) or transverse (e.g. in whites. The extra digit is often attached only by
proximal radio-ulnar synostosis or carpal coalitions). skin and a neurovascular bundle, and may be removed
The condition may appear in isolation or as part of a under local anaesthesia; this is easiest when the child
wider syndrome. is less than 4 months old. If a phalanx or entire digit 389
15 is duplicated, removal and soft-tissue reconstruction of the first CMC joint, it is usual to pollicize the index
should be performed a little later under formal oper- finger to reconstruct the thumb (as long as the index
ating theatre conditions. finger is not hypoplastic).
Duplications of the thumb or central digits are
extremely rare and require complex reconstructive
surgery of the digit, its tendons and the overlying CONSTRICTION RING SYNDROME
skin. In the thumb, even small ‘tags’ should be

approached with care so as to avoid the risk of dam- The aetiology of this condition is thought to be early,
aging tendons that need reconstruction. in utero, rupture of the amniotic membrane and the
formation of constricting amniotic membrane strands.
Associated deformities (e.g. club feet) are common.
OVERGROWTH The condition presents as a localized ‘strangulation’,
most commonly of the ring finger; the distal part of the
Macrodactyly must be distinguished from other forms finger may be painful, swollen and cyanotic, or some-
of enlarged digits (neurofibromatosis, multiple en- times threatened with amputation. Even if the disorder
chondromatosis, vascular malformations). There are is not that severe, it may compromise growth.
two forms: static (present at birth and growing pro- Treatment consists of excision of the constricting
portionately to other digits) and progressive (enlarge- band and soft-tissue reconstruction using multiple
ment of a digit in early childhood, growing faster than Z-plasties.
other digits with deviation of the digit). The condition
is rare, and the majority of cases are unilateral, affect-
ing the index, middle, thumb, ring or little finger, in or- MISCELLANEOUS CONDITIONS
der of frequency. The median or ulnar nerve is often en-
larged, and may become compressed. Madelung’s deformity In this deformity, which may be
Surgical correction is extremely difficult and gener- either congenital or post-traumatic, the lower radius
ally unrewarding. It includes debulking, epiphyseal curves forwards (ventrally), carrying with it the carpus
arrest (when the digit has reached adult size) and and hand but leaving the lower ulna sticking out as a
nerve excision and grafting. Amputation may be the lump on the back of the wrist. The congenital disor-
best option but beware, an adjacent digit may start der may appear as an isolated entity or as part of a
‘overgrowing’! generalized dysplasia; although the abnormality is
present at birth, the deformity is rarely seen before the
age of 10 years, after which it increases until growth is
UNDERGROWTH complete. Function is usually excellent.
If deformity is severe, the lower end of the ulna may
Undergrowth (brachydactyly) is common and may be be shortened; this is sometimes combined with
part of a wider syndrome (e.g. Turner’s syndrome). It osteotomy of the radius. Excision of the physeal tether
can affect a single bone, a digit or an entire limb. and replacement with a free fat graft is an alternative
Thumb hypoplasia ranges from mild (requiring no in certain cases.
treatment) to severe phalangeal or metacarpal
hypoplasia with joint instability, or even complete
absence of the digit. When the first carpo-metacarpal
(CMC) joint is present, reconstruction of the first
web space and ulnar collateral ligament as well as an
opposition transfer may be necessary. In the absence
(a) (b) (c)

(a) (b)
15.9 Madelung’s deformity (a) Note prominent ulnar
15.8 Congenital variations (a) Transverse failure of head and radial tilt; (b) characteristic x-ray showing
390 formation; (b) constriction rings. increased slope of radius and (c) subluxation of ulma.
Congenital clasped thumb Infants with this condition 15
clasp their thumbs persistently under the fingers. The
disorder appears to be due to weakness or absence of
the extensor tendons, in severe cases aggravated by
flexion contractures of the MCP and CMC joints. It
may present as an isolated problem or as part of a syn-
The wrist
drome. However, the diagnosis should not be made
before the third month as it is normal for infants to
hold their thumbs in the palm before then.
Treatment is by splintage, but if this fails tendon
transfers may be required later.
Congenital trigger thumb Care should be taken to dis-

tinguish this condition from the clasped thumb syn-
drome described above. It is unlikely that it is a truly
congenital disorder but it may occur within a few
months after birth. It appears to be a form of stenos-
ing tenovaginitis of flexor pollicis longus. Thickening
of the tendon, or a small nodule (Notta’s node), may (a) (b)
be palpable at the base of the thumb. 15.10 Growth plate arrest (a) Impacted physeal
Triggering often resolves spontaneously, but if the fracture; (b) later arrest of radius, relative overgrowth of
condition is still present at one year it can be treated ulna.
successfully by surgical division of the A1 pulley of the
flexor tendon sheath.
Symphalangism This term describes congenital stiffness bridge crossing the physis, if it is small, may be excised
of the proximal interphalangeal joints of the fingers. and replaced by a fat graft.
These joints are abnormal and the fingers are underde- Once growth slows down the deformity can be cor-
veloped. Surgical intervention is usually unrewarding. rected by a suitable osteotomy, if necessary combined
with soft-tissue release; the circular frame apparatus
Arthrogryposis multiplex congenita (AMC) Arthrogryposis is
can be used for this.
described in Chapter 10. Part or the whole of the upper
limb may be affected, giving rise to muscle weakness and
joint contractures. The shoulders are usually adducted,
the elbows stiff, the wrists and fingers flexed and the FOREARM FRACTURES
thumbs clasped in adduction and flexion. The overlying
After a Colles’ fracture radial deviation, posterior
skin is smooth and devoid of the normal creases.
angulation and prominence of the radial head are
Treatment is by early stretching and splinting; later
common. These deformities may be unsightly but
joint releases and tendon transfers may be called for.
cause little disability.
Other generalized syndromes Many generalized disor- Subluxation of the distal radio-ulnar joint may
ders involve the upper limbs. Examples include Down’s result in prominence of the ulnar head, painful rota-
syndrome (short little fingers), Marfan’s syndrome tion and loss of pronation or supination. This should
(long fingers, camptodactyly), neurofibromatosis be treated by reconstructing the distal radius; the
(macrodactyly) and cerebral palsy. The hand problems ulnar head should never be excised. Abnormal angula-
will require specialized treatment in their own right, in tion of the radius may lead to midcarpal malalignment
addition to management of the general disorder. with pain and loss of grip strength. A radial osteotomy
is then necessary; the bone fragments are fixed with a
locking plate and bone grafts are added.
ACQUIRED DEFORMITIES OF THE

WRIST RHEUMATOID DEFORMITIES
The typical rheumatoid deformity is radial deviation
PHYSEAL INJURY of the wrist, swelling of the extensor tendons, dorsal
prominence of the ulnar head and sometimes tendon
Fracture-separation of the distal radial epiphysis may rupture. The carpus falls into flexion and supination as
result in partial fusion of the physis, with pain and the ulnar side sags forwards away from the prominent
asymmetrical growth deformity of the wrist. The bony ulnar head. 391
15
(a)
15.12 The carpal joints This schematic section through

the wrist shows the radio-carpal joint between the radius
and the proximal row of carpal bones and the mid-carpal
joint between the proximal and distal rows of carpal bones.
The proximal row is an intercalcated segment.
(b)
15.11 Malunion of radius (a) Malunion of Colles’

fracture with dorsal tilt of distal radius. (b) Position Scaphoid
following corrective osteotomy. Triquetrum
Lunate
Ulno-lunate and
ulno-triquetral ligaments
‘DROP-WRIST’ Palmar radio-ulnar
ligament
Radial nerve palsy causes the wrist to drop into flex-
ion and active extension is lost. With a posterior Dorsal radio-ulnar
interosseous nerve palsy, the wrist will extend radial- ligament
wards because extensor carpi radialis longus function Extensor carpi ulnaris
is preserved.
If the nerve does not recover, tendon transfers will
greatly improve function (see Chapter 11).
15.13 The distal radio-ulnar joint The joint incorporates

the triangular fibrocartilage complex. The fibrocartilaginous
CHRONIC INSTABILITY OF THE plate is connected as its apex to the base of the ulnar
WRIST styloid process and laterally to the inferomedial ridge of the
radius. Its outer fibres blend with those of the ligaments
around the ulnar aspect of the wrist.
Movements of the wrist and hand are interdependent,
the wrist providing appropriate mobility and stability
to position and steady the hand for the remarkable THE DISTAL RADIO-ULNAR JOINT (DRUJ)
range of actions and tactile sensibility employed in our The distal radius and ulna are linked to each other by
daily activities. Abnormalities of wrist mechanics are a the interosseous membrane, the capsule of the DRUJ
common source of functional disability; this is seen and the triangular fibrocartilage complex (TFCC).
most often in rheumatoid arthritis, in association with The head of the ulna articulates congruently with the
congenital laxity and after local trauma. sigmoid notch of the distal radius; movement at the
joint occurs by the radius both rotating and sliding in
Articulations of the wrist an arc around the head of the ulna during pronation
and supination of the forearm. Interposed between
The wrist comprises three movable joints: the distal the head of the ulna and the carpus is a fibrocartilagi-
radio-ulnar joint, the radio-carpal joint (between the nous disc, a fan-shaped structure spreading from an
radius and the proximal row of carpal bones) and the apical attachment at the base of the ulnar styloid
midcarpal joint (between the proximal and distal rows process to the rim of the radial sigmoid notch. Its dor-
392 of carpal bones). sal and volar edges are coextensive with the dorsal and
palmar radio-ulnar ligaments; further attachments to 15
the joint capsule, the ulno-triquetral and ulno-lunate
ligaments, the ulnar collateral ligament and the sheath
of the extensor carpi ulnaris tendon complete the
fibrocartilage complex. The peripheral attachments of
the TFCC have a good vascular supply and can heal
The wrist
after injury; the central area of the triangular plate is
avascular and tears do not heal.
THE RADIO-CARPAL AND MIDCARPAL JOINTS

15.14 Ulnar head instability The ulnar head has
Movements in the sagittal plane (flexion and exten- subluxated dorsally.
sion) occur at both the radio-carpal and midcarpal
joints. Movements in the frontal plane (adduction or
ulnar deviation and abduction or radial deviation)
occur mainly at the radio-carpal joint, but they and slides radially. As the wrist abducts and adducts,
inevitably involve also the scaphoid which has to flex the helical surface of the hamate also causes the tri-
forwards as the trapezium moves towards the radial quetrum to move.
styloid during abduction.
The bones of the distal carpal row (hamate, capi-
tate, trapezium and trapezoid) are joined by ligaments
to each other and to the bases of the metacarpals. INSTABILITY OF THE DISTAL RADIO-
Although there is some movement of the fifth carpo- ULNAR JOINT
metacarpal joint, there is very little movement in the
remaining carpo-metacarpal articulations. Chronic instability of the distal radio-ulnar joint may
The distal row articulates through the midcarpal result from trauma, rheumatoid arthritis or excision of
joint with the bones of the proximal row (triquetrum, the distal end of the ulna. The previous history is
lunate and scaphoid), which are likewise held together therefore important. Fracture of the radial shaft is
by stout interosseous ligaments. Because these bones associated with dislocation of the distal radio-ulnar
have no muscles attaching to them, their position is joint (Galeazzi fracture-dislocation); after reduction
determined by the way they all fit together and by the of the radius, one must be certain that the radio-ulnar
constraints of the interosseous ligaments. The proxi- joint also is reduced.
mal row is, in a sense, ‘interposed’ between the fore- The patient complains of painful restriction of
arm bones and the hand bones and is called an pronation and supination, clunking and undue promi-
intercalated segment. nence of the ulnar head. There may be tenderness
The articular surface of the radius slopes obliquely directly over the radio-ulnar joint and grip strength is
forwards at 11 degrees and ulnarwards at 22 degrees; sometimes reduced. The unstable ulna can be ‘ballot-
the radial styloid is about 11 mm distal to the ulnar ted’ by holding the patient’s forearm pronated and
styloid (the ‘rule of elevens’).With the wrist in the pushing sharply upon the prominent head of the ulna
neutral position, tightening of the long muscles will (the piano-key sign).
tend to drag the carpus down the slope, and when the
wrist is pulled into abduction this tendency is Imaging
increased. By contrast, when the wrist is adducted
about 30 degrees, muscle pull draws the carpus most X-ray examination may show evidence of previous
securely into the radial ‘socket’. This is, in fact, the injury, previous surgery or rheumatoid arthritis. How-
‘position of function’ (or maximum stability) and there ever, the most effective way of demonstrating radio-
is a natural inclination to adopt this position during ulnar incongruity or subluxation is by CT.
power grip. This action is mediated by flexor carpi
ulnaris (which is why it is unwise to choose that mus- Treatment
cle for a tendon transfer).
The scaphoid is potentially the most unstable of all This depends on the cause. If the ulnar head is intact,
the carpal bones. As the wrist flexes and extends, so then the TFCC can be reattached by arthroscopic or
does the scaphoid bone; the lunate and triquetrum open methods. Ulnar shortening osteotomy can
follow passively, guided by the interosseous ligaments. tighten the ulnar corner and improve stability; a ten-
With abduction, the space between the trapezium and don weave to reproduce the volar and dorsal radio-
radial styloid closes down so the scaphoid moves out ulnar ligaments is the most reliable but difficult
of the way by flexing palmarwards and sliding ulnar- reconstruction. The ulnar head must never be excised
wards. During adduction, the scaphoid tilts dorsally to treat instability; that will only worsen the problem. 393
15 If the ulnar head has previously (and usually unwisely) wrist in adduction and compressing the ulnar head
been removed, ulnar head replacement will usually be against the carpus. The distal ulna should be tested for
needed to restore stability. Special implants have been instability. The diagnosis is confirmed by contrast
developed for the failed Sauve–Kapandji procedure arthrography, MRI or, most sensitively, arthroscopy.
(fusion of the distal ulna to the radius).
Treatment Peripheral tears can be re-attached by
either open or arthroscopic techniques with a reason-

able expectation that they will heal. If this fails then a
LONGITUDINAL INSTABILITY OF THE tendon reconstruction is needed. Central tears, in the
RADIUS AND ULNA absence of ulno-carpal impaction (see below), are best
managed by arthroscopic debridement.
Fracture of the radial head is sometimes accompanied
by disruption of the interosseous membrane and dis-
location of the distal radio-ulnar joint (the Essex- Ulno-carpal impaction and TFCC
Lopresti lesion). Excision of the radial head can lead degeneration
to proximal migration of the radius and ulno-carpal The TFCC tends to degenerate with age; usually this
impaction (see below); whenever possible the radial is asymptomatic. However, progressive degenerative
head should be preserved or replaced by a metal change may be associated with a relatively long ulna,
implant. Chronic longitudinal instability causes ulnar- impaction of the ulnar head against the ulnar side of
sided wrist pain and loss of grip strength. the lunate and ulno-carpal arthritis (the ulno-carpal
Treatment of the distal radio-ulnar joint symptoms impaction syndrome). X-ray examination (standard
is generally unsatisfactory. A combination of radial views with the shoulder abducted 90 degrees, the
head replacement and an ulnar shortening osteotomy elbow flexed 90 degrees and the forearm in mid-
sometimes improves symptoms. Radio-ulnar fusion is pronation-supination) may show a relatively long ulna
sometimes employed as the only salvage procedure. (‘positive ulnar variance’) and in late cases there may
be arthritic changes in the ulno-lunate articulation.
Treatment Initial treatment is with simple analgesics,
DISORDERS OF THE TRIANGULAR splintage and steroid injections. If this is not success-
FIBROCARTILAGE COMPLEX ful then the long ulna is shortened using a special jig
and compression plate. A better alternative for just 2
Clinically significant disorders of the TFCC can be or 3 mm of positive variance is an arthroscopic exci-
divided into traumatic and degenerative conditions. sion of the distal dome of the ulnar head. The ulnar
head itself should never be excised for this condition.
Traumatic disruption
There may be a history of a fall on the outstretched
hand or a twisting injury of the forearm. The patient CHRONIC INSTABILITY OF THE RADIO-
complains of pain, and sometimes clicking or even CARPAL AND INTERCARPAL JOINTS
instability in the distal radio-ulnar joint, particularly
on twisting the wrist. There is tenderness over the Abnormal movement between the carpus and the
ulno-carpal joint and pain on rotation of the forearm. forearm bones, or between individual carpal bones,
Symptoms can also be reproduced by holding the results from loss of the bony relationships and/or
(a) (b) (c)
15.15 Ulno-carpal impaction (a) X-ray; (b) MRI; (c) intra-operative x-ray during arthroscopic removal of the distal dome
394 of the ulna.
lunate, unrestrained by the triquetrum, but still con- 15
trolled by the scaphoid, tends to flex whilst the capi-
tate tends to extend.
Midcarpal instability This usually emerges as a chronic
problem, associated with generalized ligamentous lax-
ity. The proximal and distal rows become unstable
The wrist
through the midcarpal joint.
(a) Adaptive midcarpal instability If a distal radius fracture

heals with the radial articular surface tilted dorsally,
then the proximal carpal row tends also to tilt dorsally
and the midcarpal joint flexes to maintain the palm in
line with the forearm. This is painful and grip is
reduced.
Radio-carpal translocation Chronic synovitis and artic-
ular erosion (as in RA) gradually leads to attenuation
of the wrist ligaments and subluxation of the entire
radio-carpal joint. In advanced RA the carpus usually
(b)
shifts ulnarwards and simultaneously deviates into
abduction and supination.
Clinical features of carpal instability

The patient with scapho-lunate or luno-triquetral
incompetence presents with pain and weakness of the
(c) wrist, and sometimes also clunking during movement
or gripping actions. It is important to enquire about
15.16 Carpal instability The relationships of the carpal any previous injury, however trivial it may have
bones in (a) the normal wrist, (b) DISI and (c) VISI.
seemed at the time.
On examination, there may be generalized tender-
ness over the carpus from synovitis or more localized
ligamentous constraints which normally stabilize the tenderness, for example at the scapho-lunate junction
wrist. The initiating cause is usually some type of or over the scaphoid itself. Grip strength is reduced.
injury – a wrist sprain with ligament damage, sublux- Provocative tests are useful.
ation or dislocation at one of the radio-carpal or inter-
Watson’s test for scapho-lunate incompetence Thumb
carpal joints or a fracture of one of the wrist bones –
pressure is applied to the volar aspect of the wrist over
but chronic instability may also arise insidiously in
the distal pole of the scaphoid (this restores the align-
erosive joint disorders such a rheumatoid arthritis.
ment of the volar-tilted scaphoid). While maintaining
this position, the wrist is moved alternately into
PATTERNS OF CARPAL INSTABILITY adduction and abduction. A painful ‘clunk’ occurs as
Acute carpal injuries are dealt with in Chapter 25. the proximal pole of the scaphoid subluxes dorsally.
Here we shall consider the problems associated with
chronic carpal instability. The disorder affects mainly Luno-triquetral ballottement With one hand the exam-
the intercalated segment (proximal carpal row) of the iner grasps and stabilizes the lunate between index fin-
wrist. The common patterns are: ger and thumb. With the other thumb he presses on
the pisiform/triquetrum to produce a shearing
Dorsal intercalated segment instability (DISI) Following a motion between lunate and triquetrum. If there is
fracture of the scaphoid or rupture of the scapho- pain and excessive movement, this suggests incompe-
lunate ligament (scapho-lunate dissociation), the tence of the luno-triquetral ligament.
lunate no longer passively follows the scaphoid. The
The examiner grasps the patient’s fore-
Pivot shift test
scaphoid tends to flex and the lunate assumes its
arm with one hand and the patient’s hand with the
default position of extension (dorsal tilt).
other; he then compresses the wrist axially while mov-
Volar intercalated segment instability (VISI) Less coming it from abduction to adduction. A painful ‘clunk’
monly, the luno-triquetral ligament is ruptured. The suggests mid-carpal instability. 395
15
(c)
(a) (b) (d)

15.17 Carpal instability (a) A year after ‘straining’ his wrist this patient was still complaining of pain; the x-ray shows a
gap between the scaphoid and lunate (the Terry-Thomas sign) and rotation of the scaphoid. (b) The actor Terry Thomas
with the trademark gap between his front teeth (reproduced by permission; © United Artists Inc.). (c) In the lateral view the
lunate is tilted dorsally and the scaphoid ventrally (DISI); compare this with (d), an example of VISI, showing volar tilt of the
lunate.
X-rays Arthroscopy of the radio-carpal and midcarpal joints

is the best method for demonstrating carpal instabil-
An anteroposterior x-ray may show an old or new ity. Ligament tears, certain patterns of instability, syn-
scaphoid fracture. There may be widening of the ovitis and damaged articular cartilage can be detected.
scapho-lunate interval (the Terry-Thomas sign); if the
scaphoid is flexed, it will look foreshortened and the
tubercle may appear as a dense ‘ring’ in the bone.
Treatment
A true lateral view is examined to assess the relative
alignment of the distal radius, the lunate, capitate and Scapho-lunate and luno-triquetral dissociation The best
scaphoid. In a normal wrist, the articular surfaces of results are obtained if the ligaments heal in an
the radius, lunate and capitate are parallel. In the DISI anatomical position. The diagnosis should, therefore,
deformity, the capitate axis is shifted dorsally but it be made as soon as possible after injury; this requires
flexes relative to the lunate, the lunate tilts backwards a high index of suspicion. The surgeon should be
and the scaphoid flexes; the scapho-lunate angle is alerted by a history of wrist pain following a fall on
greater than 70 degrees. In a VISI deformity, the lu- the outstretched hand and a finding of midcarpal ten-
nate is flexed forwards and the scapho-lunate angle is derness. Arthrography or arthroscopy may be needed
less than 30 degrees; the capitate tilts dorsally. to secure the diagnosis. The ligaments are repaired,
In an anteroposterior ‘clenched fist view’ the the bones stabilized with K-wires and the wrist held in
scaphoid is seen to flex and a scapho-lunate gap a cast for at least 2 months.
becomes more apparent. Patients seen more than 3 months after injury will
Anteroposterior views with the wrist adducted and require a more extensive type of carpal reduction and
abducted emphasize scapho-lunate gaps and abnor- ligament reconstruction. For scapho-lunate incompe-
mal scaphoid flexion (the ring sign), particularly when tence various reconstructions have been described.
compared with x-rays of the other side. Perhaps the Brunelli is the most reliable. Half of flexor
carpi radialis tendon is passed through a drill hole in
the scaphoid and then secured across the back of the
Further investigations carpus. This pulls up the flexed scaphoid from its
Image intensification helps to define the site of insta- flexed position and tightens the carpus transversely.
bility in difficult cases. If the displacement cannot be reduced, or if soft-tis-
Arthrography shows leakage of contrast through sue repair fails or if osteoarthritis supervenes, then a
incompetent scapho-lunate or luno-triquetral spaces. salvage operation is needed. The options include a
MRI will reveal any associated injuries, such as a proximal row carpectomy (if the lunate-capitate junc-
scaphoid fracture. The scapho-lunate and luno-trique- tion is preserved) or a scaphoid excision with four-
tral interosseous ligaments are so slim that the resolu- corner fusion (if the lunate-capitate joint is arthritic or
tion of MRI scanning may be inadequate to detect if the patient needs the strongest wrist especially in
396 significant injuries. torsion).
For luno-triquetral instability, tendon reconstruc- stress or injury, though one cannot be certain about 15
tion is not reliable enough. A luno-triquetral fusion is this. It has been suggested that relative shortening of
the most suitable treatment. the ulna (‘negative ulnar variance’) predisposes to
stress overload of the lunate between the distal edge
Symptomatic midcarpal instability Treatment includes
of the radius and the carpus, but this has not been
proprioceptive training (a gyroscopic device can help).
proven convincingly.
Intractable symptoms may respond to arthroscopic
The wrist
shrinkage of the capsule with a diathermy probe. The
alternative of a ligament reconstruction is unreliable, Pathology
and midcarpal fusion causes very significant loss of
As in other forms of ischaemic necrosis, the patholog-
movement (about 50 per cent).
ical changes proceed in four stages: stage 1, ischaemia
Dorsal malunions of the distal radius A dorsal tilt defor- without naked-eye or radiographic abnormality; stage
mity that is symptomatic may be treated by a correc- 2, trabecular necrosis with reactive new bone forma-
tive osteotomy of the distal radius; normal carpal tion and increased radiographic density, but little or
alignment should be restored. no distortion of shape; stage 3, collapse of the bone;
and stage 4, disruption of radio-carpal congruence and
secondary osteoarthritis.
KIENBÖCK’S DISEASE
Clinical features
Robert Kienböck, in 1910, described what he called The patient, usually a young adult, complains of ache
‘traumatic softening’ of the lunate bone. This is a and stiffness; only occasionally is there a history of
form of ischaemic necrosis, probably due to chronic acute trauma. Tenderness is localized over the lunate
15.18 Kienböck’s disease

(a) In stage 2 the bone
shows mottled increase of
density, but is still normal in
shape. (b) In stage 3
density is more marked and
the lunate looks slightly
squashed. (c) In stage 4 the
bone has collapsed and
there is radio-carpal
osteoarthritis. In all three
the ulna looks
disproportionately short.
(a) (b) (c)
(a) (b) (c)
15.19 Kienböck’s disease grade (a) Not seen on x-ray; (b) seen on MRI scan; (c) treated by vascular bundle
implantation. 397
15
(b)
15.20 Kienböck’s disease-treatment

(a) Radial shortening; (b) scapho-capitate
fusion.
(a)
and grip strength is diminished. In the later stages lunate collapses, the relative length of the capitate from
wrist movements are limited and painful. third metacarpal bone to distal radius increases.
MRI is the most reliable way of detecting the early
changes. A gadolinium-enhanced MRI scan will demon-
Imaging
strate the condition even if plain x-rays are normal.
X-rays at first show no abnormality, but radioscintigra-
phy may reveal increased activity. Later, x-rays may show
Treatment
either mottled or diffuse density of the bone, and later
still the bone looks intensely sclerotic and irregular in NON-OPERATIVE TREATMENT
shape or squashed. The capitate migrates proximally In early cases, splintage of the wrist for 6–12 weeks
into the space left by the collapsing lunate and the relieves pain and possibly reduces mechanical stress. If
scaphoid flexes forward. Eventually, there are os- bone healing catches up with ischaemia, the lunate
teoarthritic changes in the wrist. Ulnar variance should may remain virtually undistorted; this is more likely in
be assessed by standardized x-ray examination with the very young patients. However, if pain persists, and
shoulder abducted to 90 degrees, the forearm in neutral even more so if the bone begins to flatten, operative
rotation and the wrist in neutral flexion-extension. As the treatment is indicated.
Table 15.1 Stages of Kienböck’s Disease
Stage X-ray, MRI Treatment

1 Normal x-ray, changes on MRI Cast for 3 months
Vascularized bone graft
2 Lunate sclerosis on plain x-ray, fracture lines sometimes present Vascularized bone graft
If negative ulnar variance: radial shortening
If positive ulnar variance: radial dome osteotomy
3a Fragmentation of lunate; height preserved Proximal row carpectomy
3b Collapse of lunate, proximal migration of capitate, fixed scaphoid rotation Scapho-capitate fusion
Scapho-trapezium-trapezoid fusion
Proximal row carpectomy
4 Midcarpal or radio-carpal arthritis Proximal row carpectomy
Total wrist fusion
Wrist replacement
398
OPERATIVE TREATMENT gradually and the hand feels weak. The forearm looks 15
In its earliest stages, before collapse, the bone can be wasted; the wrist is swollen and feels warm. Involve-
revascularized with a pedicled bone graft or vascular ment of the flexor tendon compartment may give rise
bundle implantation. to a large fluctuant swelling that crosses the wrist into
While the wrist architecture is only minimally dis- the palm (compound palmar ganglion). In a neg-
turbed (i.e. up to early stage 3), it seems rational to lected case there may be a sinus. Movements are
The wrist
aim for a reduction of carpal stress by shortening the restricted and painful.
radius. The same effect can be achieved by lengthen- X-rays show localized osteoporosis and irregularity
ing the ulna, but a bone graft is needed and union is of the radio-carpal and intercarpal joints; there may
less predictable. also be bone erosion.
Once the bone has collapsed, the options are lim-
ited. A wrist neurectomy is worth trying; this will pre-
Diagnosis
serve movement yet reduce pain. Lunate replacement
by a silicone prosthesis, once popular, gives poor The condition must be differentiated from rheuma-
long-term results and particle shedding is liable to toid arthritis. Bilateral arthritis of the wrist is nearly
cause synovitis. Other procedures, such as intercarpal always rheumatoid in origin, but when only one wrist
fusion or excision of the proximal row of the carpus, is affected the signs resemble those of tuberculosis.
may improve function but in the long term may not X-rays and serological tests may establish the diagno-
prevent the occurrence of osteoarthritis. sis, but often a biopsy is necessary.
If pain and restriction of movement become intoler-
able, radio-carpal arthrodesis is the one reliable way of
Treatment
providing a stable, pain-free wrist. Wrist replacement is
an alternative in individuals with lesser demands. Antituberculous drugs are given and the wrist is
splinted. If an abscess forms, it must be drained. If the
wrist is destroyed, systemic treatment should be con-
tinued until the disease is quiescent and the wrist is
PREISER’S DISEASE then arthrodesed.
This is a very rare condition in which the scaphoid

undergoes spontaneous avascular necrosis. Early dis-
ease, diagnosed on MRI, can respond to vascularized RHEUMATOID ARTHRITIS
bone grafting. Later symptomatic disease with (see also Chapter 3)
advanced destruction of the scaphoid would need
joint-preserving surgery (proximal row carpectomy or
After the metacarpo-phalangeal (MCP) joints, the wrists
scaphoidectomy–four-corner fusion).
and distal radio-ulnar joints are the most common sites
of rheumatoid arthritis. Wrist and hand should always be
considered together when dealing with this condition.
TUBERCULOSIS (see also Chapter 2)
Pathology
At the wrist, tuberculosis is rarely seen until it has pro- In the early stages, the characteristic features are syn-
gressed to a true arthritis. Pain and stiffness come on ovitis of the joints and tendon sheaths. If the disease
15.21
Tuberculosis
of the wrist
(a) Pointing
abscess;
(b) x-ray
showing
diffuse
osteoporosis.
(a) (b) 399

15 15.22 Rheumatoid arthritis
(a) Typical zig-zag deformity in
established rheumatoid arthritis.
The wrist is deviated radialwards
and the fingers ulnarwards.
(b) X-ray of the same patient.
(c) Enlarged x-ray view – note
the characteristic erosions at the

distal ends of the radius and
ulna (arrows).
(a) (b) (c)
persists, the distal radio-ulnar joint (DRUJ), radio-

carpal joint and intercarpal joints become eroded;
this, together with attenuation of the ligaments and
tendons, leads to instability and progressive deformity
of the wrist and hand.
The ulnar side of the carpus gradually shifts towards
flexion and volar subluxation, causing the head of the
ulna to jut out prominently on the dorsum of the
wrist. The proximal carpal row slides ulnarwards and
the metacarpal bones deviate radialwards, which
mechanically predisposes to a reciprocal ulnar devia-
tion of the fingers – a cardinal feature of the ‘rheuma-
toid hand’. At the same time, the scaphoid falls into
marked flexion because of erosion of the interosseous
ligaments and loss of carpal height. The combination
15.23 Rheumatoid arthritis Synovitis around the ulnar
of instability and erosive tenosynovitis eventually leads head with rupture of extensor digiti minimi.
to tendon rupture – typically one or more of the long
extensor tendons.
An unstable wrist means a weak hand; deformities rupture, either within the digital sheaths or in the
of the MCP joints are almost invariably associated cramped confines of the carpal tunnel.
with complementary deformities of the wrist. The proximal joints in both upper limbs should be
examined as well. It is important to know whether the
arm is able to place the wrist and hand in functional
positions.
Clinical features
Early symptoms are pain, swelling and stiffness of the
X-rays
wrists. At first the swelling is usually localized to the
common extensor tendon sheath or the extensor carpi Typical signs are peri-articular osteoporosis and ero-
ulnaris, but as time progresses the joints become sion of the ulnar styloid and the radio-carpal and
thickened and tender. Swelling of the synovium in the intercarpal joints. In most cases the hands also will be
carpal tunnel may cause median nerve compression. affected, but there is a well-recognized group of
Gradually the wrist becomes unstable as the articu- patients (mostly elderly men) in whom the wrists
lar surfaces erode and ligaments become attenuated. carry the brunt of the disease.
Early infiltration of tendons may lead to weakness of
wrist extension and flexion. Instability of the DRUJ
aggravates the dorsal protrusion of the ulnar head,
Treatment
which can often be jogged up and down by pressing EARLY STAGE DISEASE
upon it with your thumb (the piano-key sign). During the early stages of rheumatoid arthritis the
Tendon lesions are common in the late stage. The objectives of treatment are to relieve pain and coun-
first to rupture is usually the extensor digiti minimi, teract synovitis. In addition to systemic treatment,
followed by the extensor communis tendons of the lit- synovitis of the wrist and/or tendons will be helped
tle and ring fingers. Extensor pollicis longus tendon is by intermittent splintage and intrasynovial injections
400 also vulnerable. The flexor tendons also sometimes of corticosteroid preparations.
15.24 Rheumatoid 15
arthritis (a) At first the
x-rays show only soft-tissue
swelling. (b) Two years later,
this patient shows early bone
changes – peri-articular
osteoporosis and diminution
The wrist
of the joint space. (c) Five
years later still, bony erosions
and joint destruction are
marked.
(a) (b) (c)
ESTABLISHED DISEASE Ruptured extensor tendons can seldom be repaired;

As joint erosion makes its appearance, the focus turns side-to-side suture of a distal tendon stump to an
increasingly to the safeguarding of joint stability and adjacent tendon, tendon grafting or tendon transfer
the prevention of deformity. gives a satisfactory if not perfect result.
Extensor tenosynovectomy and soft-tissue stabilization Rupture of the flexor pollicis longus tendon in the
of the wrist may forestall further deterioration. Through carpal tunnel may be caused by scuffing of the tendon
a dorsal longitudinal incision the extensor retinaculum against the distal pole of the scaphoid or the edge of
is exposed and carefully dissected but left attached at the the trapezium – the so-called ‘Mannerfelt lesion’.
radial side. The thickened synovium around the exten- Repair or grafting gives disappointing results; the sim-
sor tendons, as well as any bony protrusions on the back plest way of dealing with this problem is to fuse the
of the wrist, are removed. The preserved extensor reti- thumb interphalangeal joint and rely on the other
naculum is then placed beneath the tendons to further motors to manipulate this important digit.
reduce the risk of later tendon rupture. Painful joint destruction, instability and deformity
If the radio-ulnar joint is involved, synovectomy can can be dealt with by either joint replacement or
be combined with excision of the ulnar head and trans- arthrodesis. Arthroplasty using a silicone ‘spacer’ has
position of the extensor carpi radialis longus to the a high failure rate; silicone synovitis and the difficulty
ulnar side of the wrist (to counteract the tendency to of revision have led to it being abandoned. Total wrist
radial deviation). Fusion of the lunate to the radius replacement with a metal–polyethylene device is
(Chamay procedure) prevents ulnar slide of the carpus. becoming more reliable, but is only suitable for those
Flexor tenosynovitis is not as obvious as extensor with well-preserved bone stock.
tendon involvement. It may present as carpal tunnel Arthrodesis is widely considered to be the best
syndrome – median nerve compression by swollen ten- option for dealing with painful instability in the radio-
dons in the carpal tunnel – which requires open carpal joint. If the wrist is already ‘fusing’ itself spon-
release of the flexor retinaculum and tenosynovec- taneously, simple stabilization with a Steinman pin
tomy. Obvious bony protrusions in the floor of the passed between the second and third metacarpals,
carpal tunnel (due to carpal collapse) should be across the carpus and into the distal radius is all that is
removed and the raw area covered with a soft-tissue needed. Bone grafts are not necessarily added but can
flap. Bear in mind that median nerve symptoms in be taken from the ulnar head if it is excised. For
patients with rheumatoid arthritis may be caused by patients with better bone stock, pin fixation is inade-
pathology in the proximal part of the limb or the cer- quate; formal arthrodesis with a wrist fusion plate is
vical spine, so these patients should always undergo preferable. In this group, ulnar head replacement
nerve conduction studies and electromyography rather than ulnar head excision should be considered.
before the carpal tunnel decompression. As a general rule, wrist deformities should be cor-
rected before hand deformities. Furthermore the dom-
LATE DISEASE inant wrist should, if possible, be fused in slight
In the late stage tendon ruptures at the wrist, joint extension to provide reliable power grip, while the non-
destruction, instability and deformity may require dominant wrist is fused in some flexion (or replaced) so
reconstructive surgery. as to provide the posture needed for perineal care. 401
15 X-rays
Typical features are narrowing of the radio-carpal
joint, subchondral sclerosis and osteophyte formation
at the margins of the joint. A predisposing cause, such
as an old fracture or Kienböck’s disease, may be
apparent.
Treatment
CONSERVATIVE MEASURES
Analgesic medication and rest, in a polythene splint,
are often sufficient treatment. However, if pain is
intolerable or if function is seriously disturbed (e.g. if
the patient is unable to grip firmly or lift moderately
heavy objects), surgical options have to be considered.
SURGICAL TREATMENT
Partial excision of the radial styloid Osteoarthritis fol-
lowing a scaphoid fracture may be limited to that part
of the joint. In that case excision of the tip of the
(a) (b) radial styloid process is helpful, but no more than
15.25 Rheumatoid arthritis wrist fusion Surgical fusion
7 mm must be removed to avoid destabilizing the
using a long intramedullary pin. The ulnar head has been carpus. This can be done by open or arthroscopic
excised. means and at the same time a partial wrist denervation
may be performed.
OSTEOARTHRITIS OF THE WRIST OPERATIONS ON CARPAL BONES

For advanced changes, it may be necessary to operate
on the carpus, but wrist movement should be pre-
Osteoarthritis of the wrist appears at three main sites:
served if possible. The entire proximal row of carpal
the radio-carpal joint, the distal radio-ulnar joint and
bones can be removed (proximal row carpectomy); the
the first carpo-metacarpal joint. Since these usually
head of the capitate then articulates on the lunate
present as distinct syndromes, they are considered
fossa of the radius. In some cases scaphoidectomy and
separately.
four-corner fusion may be more appropriate: the
lunate–capitate–hamate–triquetrum are fused with
wires, a circular plate or buried screws.
RADIO-CARPAL OSTEOARTHRITIS
Osteoarthritis of the radio-carpal joint is uncommon
and when it does occur it is sometimes a late sequel to
TYPICAL SITES OF X-RAY CHANGES
an injury such as an intra-articular fracture of the Distal radius: radius-scaphoid degeneration
distal radius, an ununited or malunited fracture of the
scaphoid, scapholunate ligament rupture or Kien- Scaphoid fracture (SNAC wrist = scaphoid
böck’s disease; yet it should be borne in mind that, non-union advanced collapse)
while trauma of all kinds is common, only a fraction Stage 1: radial styloid and distal scaphoid
of all such injuries lead to arthritis in later life. Stage 2: scapho-capitate joint (proximal scaphoid
and reciprocal radius spared, cf. scapho-lunate
arthritis)
Clinical features Stage 3: peri-scaphoid arthritis, lunate-capitate
The patient may have forgotten the original injury. arthritis. Lunate-radius preserved
Years later he or she complains of pain and stiffness. Scapho-lunate ligament failure (SLAC wrist =
At first these symptoms occur intermittently after use; scapho-lunate advanced collapse)
later they become more constant, with frequent exac- Stage 1: radial styloid and distal scaphoid
erbations or recurrent ‘wrist sprains’. Stage 2: entire radioscaphoid joint
The appearance may be normal but there is often Stage 3: capitate-lunate joint. Lunate-radius
swelling over the back of the wrist and movements are preserved
402 limited and painful.
DISTAL RADIO-ULNAR ARTHRITIS 15
Progressive destruction of the distal radio-ulnar joint
is a characteristic feature of severe rheumatoid arthri-
tis. Lesser degenerative changes are seen in secondary
osteoarthritis, possibly following marked and long-
The wrist
standing instability of the joint.
If pain and loss of function cannot be controlled by
conservative measures, the patient may benefit from
ulnar head replacement. Older operations that involve
excision of the ulnar head have been abandoned
because of the high risk of causing severe and
intractable instability.
15.26 Radio-carpal arthritis Early stage treated by
arthroscopic radial styloidectomy.
CARPO-METACARPAL OSTEOARTHRITIS
The outcome of these procedures is similar (about Osteoarthritis of the trapezio-metacarpal joint is com-
60 per cent grip strength, 60 per cent movement). mon in postmenopausal women. It is often accompa-
Proximal row carpectomy is easier to perform and nied by Heberden’s nodes of the finger joints, in
risks fewer complications; four-corner fusion gives a which case it is usually bilateral and part of a general-
more stable grip in torsion. ized osteoarthritis.
Total arthrodesis of the wrist This is occasionally neces-
sary. The radio-carpal and intercarpal joints are decor- Clinical features
ticated, bone graft is impacted and a compression
The patient, usually a middle-aged or older woman,
plate is fixed to the third metacarpal and the distal
complains of diffuse pain around the base of her
radius. Contouring the plate to 15 degrees of dorsi-
thumb. Pinch and grip are weakened. On examina-
flexion improves grip strength.
tion, the joint is swollen and in advanced cases is held
Arthroplasty Wrist replacement with metal or poly- in an adducted position, with prominence of the sub-
thene implants is becoming more reliable, although at luxed metacarpal base. With more established fixed
present this operation is reserved for those with low adduction of the thumb base, the metacarpo-
functional demands. Long-term survivorship studies phalangeal joint hyperextends to provide a competent
have yet to show whether replacement arthroplasty thumb–index span. The carpo-metacarpal joint is
will hold up in patients with higher demands. tender and the ‘grind test’ (compressing and rotating
15.27 Radiocarpal arthritis

(a,b) The so-called ‘SLAC
wrist’ – scapho-lunate
advanced collapse; (c) treated
by scaphoid excision and four-
corner fusion.
(a) (b) (c) 403

15
(a)
(a) (b)
15.29 Distal radio-ulnar joint arthritis – operative

treatment (a) Excising too much of the distal ulna may
cause painful radio-ulnar impingement. (b) One alternative
is an ulnar head replacement.
(b)
Excisional arthroplasty Excision of the trapezium gives

pain relief and return of function, though thumb pinch
is always weak. The bone can be removed through
either the palmar approach or the anatomical snuffbox,
taking care not to damage the superficial radial nerve,
the radial artery or the flexor carpi radialis tendon.
Attempts have been made to prevent postoperative
collapse of the joint and proximal migration of the
(c) metacarpal by re-routing a slip of flexor carpi radialis
or abductor pollicis longus tendon and attaching it to
15.28 Wrist arthritis (a) Total wrist fusion; (b,c) total
a drill hole in the metacarpal base; the benefit of this
wrist replacement.
extra intervention has not been established.
Replacement arthroplasty Replacement arthroplasty us-
the metacarpal longitudinally against the trapezium) is ing a silicone spacer has a high complication rate and
painful. the results are unpredictable. Metal-on-polyethene
X-rays show narrowing and then lateral subluxation of implants and pyrocarbon implants are also available
the trapezio-metacarpal joint. Radioscintigraphy is useful but longer-term outcome is uncertain.
in early cases when the diagnosis is in doubt; increased
Arthrodesis Arthrodesis of the trapezio-metacarpal
activity precedes the more obvious x-ray changes.
joint relieves pain, but the restriction of movement
and high failure rate are distinct drawbacks. The
Treatment scapho-trapezial joint should be normal.
If the metacarpo-phalangeal joint has been second-
Most patients can be treated by anti-inflammatory
arily damaged by hyperextension, then either a
preparations, local corticosteroid injections and tem-
sesamoid arthrodesis (which restores flexion but pre-
porary splintage. There may be a role for intra-articu-
serves movement) or fusion (at 25 degrees for stable
lar hyaluronidase. If these measures fail to control
pinch) is indicated.
pain, or if instability becomes marked, operative treat-
ment is considered.
SURGICAL TREATMENT SCAPHOID-TRAPEZIUM-TRAPEZOID (STT)

Joint-preserving operations In early cases, joint-pre- ARTHRITIS
serving operations are helpful in about 70 per cent.
The options are either extension osteotomy or ligament The joint between the distal end of the scaphoid and
reconstruction. These procedures alter the joint forces the underside of the trapezium and trapezoid (‘the
404 and thus improve pain and function. triscaphe joint’) can develop arthritis either in isola-
15
The wrist
(a) (b) (c) (d)
15.30 1st Carpo-metacarpal
osteoarthritis (a) Deformity of the thumb,
with fixed carpo-metacarpal flexion and
metacarpo-phalangeal hyperextension.
(b) X-ray showing articular destruction.
Treatment may be by (c) excision of
trapezium, (d) arthrodesis, (e,f) silastic
replacement or (g) total replacement
(e) (f) (g)
tion or in association with arthritis of the carpo- Treatment

metacarpal joint.
Late middle-aged females are most commonly Treatment is initially along standard lines – adaptive
affected. The patient points to the front of the measures, anti-inflammatory medication, cortisone
scaphoid tubercle as the source of the pain (whereas injection and splintage.
in carpo-metacarpal arthritis the patient points to the Patients with severe symptoms may benefit from
back of the thumb base). surgery. However, there is as yet no completely satis-
(a) (b) (c) (d)
15.31 Scaphoid-trapezium-trapezoid arthritis (a) Changes on x-ray; (b) steroid injection; (c) distal pole of scaphoid
excision (do not remove too much!); (d) STT fusion.
15.32 Piso-triquetral arthritis

(a) Shown on 30° supinated view.
(b) Pisiform excision gives good
results – but beware the ulnar
nerve!
(a) (b) 405

15 factory operation. STT fusion removes the painful Clinical features The patient is usually a woman aged
joint but is technically difficult. Excision of the distal 40–50, who complains of pain on the radial side of the
pole of the scaphoid is easier but can cause midcarpal wrist. There may be a history of unaccustomed activ-
collapse. Trapeziectomy with under-cutting of the ity such as pruning roses or wringing out clothes.
trapezoid is probably the most reliable and straight- Sometimes there is a visible swelling over the radial
forward, especially if there is concomitant trapezio- styloid and the tendon sheath feels thick and hard.
metacarpal arthritis. Pyrocarbon interposition Tenderness is most acute at the very tip of the radial
arthroplasty has also been employed but long-term styloid.
follow-up data are lacking. The pathognomonic sign is elicited by Finkelstein’s
test. The examiner places the patient’s thumb across
the palm in full flexion and then, holding the patient’s
hand firmly, turns the wrist sharply into adduction. In
TENOSYNOVITIS AND a positive test this is acutely painful; repeating the
TENOVAGINITIS movement with the thumb left free is relatively pain-
less. Resisted thumb extension (hitch-hiker’s sign) is
The extensor retinaculum has six compartments which also painful.
transmit tendons lined with synovium. Tenosynovitis The differential diagnosis includes arthritis at the
can be caused by unaccustomed overuse but some- base of the thumb, scaphoid non-union and the inter-
times it occurs spontaneously. The resulting synovial section syndrome (see below).
inflammation causes secondary thickening of the
sheath and stenosis of the compartment, which further Treatment
compromises the tendon. Early treatment, including
rest, anti-inflammatory medication and injection of The early case can be relieved by a corticosteroid
corticosteroids, may break this vicious circle. injection into the tendon sheath, sometimes com-
The first dorsal compartment (abductor pollicis bined with hand therapy (ultrasound, frictions, splin-
longus and extensor pollicis brevis) and the second tage). Resistant cases need an operation, which
dorsal compartment (extensor carpi radialis brevis) are consists of slitting the thickened tendon sheath.
most commonly affected. Sometimes there is duplication of tendons and even of
The flexor tendons are affected far less frequently. the sheath, in which case both sheaths need to be
divided. Care should be taken to prevent injury to the
dorsal sensory branches of the radial nerve, which may
DE QUERVAIN’S DISEASE cause intractable dysaesthesia.
Pathology
INTERSECTION SYNDROME
This condition, first described in 1895, is caused by
reactive thickening of the sheath around the extensor This condition, otherwise known as crossover syndrome
pollicis brevis and abductor pollicis longus tendons or peri-tendinitis crepitans, is characterized by pain,
within the first extensor compartment. It may be ini- swelling and crepitus over the tendons of extensor
tiated by overuse but it also occurs spontaneously, pollicis brevis and abductor pollicis longus 4–6 cm
particularly in middle-aged women, and sometimes proximal to the extensor retinaculum. It is found in
during pregnancy. weight-lifters, canoeists and rowers. It should be dis-
(a) (b) (c) (d)
15.33 De Quervain’s disease (a) There is point tenderness at the tip of the radial styloid process. (b,c) Finkelstein’s test:
Ulnar deviation with the thumb left free is relatively painless (b), but if the movement is repeated with the thumb held close
406 to the palm (c), the pull on the thumb tendons causes intense pain. (d) Injecting the tendon sheath.
tinguished clinically from de Quervain’s disease. The Flexor carpi ulnaris can become inflamed near its 15
condition is generally attributed to friction between insertion into the pisiform. Occasionally x-rays show
these tendons (the so-called ‘outcropping tendons’) calcific deposits around the sheath.
and the underlying longitudinally-aligned extensor Treatment of these conditions is the same as for the
tendons, leading to an adventitious bursa or a other types of tenosynovitis.
tenosynovitis. There is usually an associated tenosyn-
The wrist
ovitis within the second extensor compartment con-
taining extensors carpi radialis longus and brevis. OCCUPATIONAL PAIN DISORDERS
Treatment involves rest, splintage, steroid injection
and, in resistant cases, surgical widening of the second Terms such as repetitive stress injury and cumulative
compartment and exploration of the intersection. trauma disorder have been used for a controversial
syndrome comprising ill-defined and unusually dis-
abling pain around the wrist and forearm (and some-
OTHER SITES OF EXTENSOR TENOSYNOVITIS times the entire limb) which is usually ascribed to a
particular work practice. In some cases there is clinical
Overuse tenosynovitis of extensor carpi radialis brevis evidence of tenosynovitis, which could have been
(the most powerful extensor of the wrist) or extensor caused by unaccustomed or prolonged activity of a
carpi ulnaris may cause pain and point tenderness just particular kind. Other defined and treatable condi-
medial to the anatomical snuffbox or immediately dis- tions such as carpal tunnel syndrome, thumb base
tal to the head of the ulna, respectively (see Figure arthritis and de Quervain’s should be excluded. Epi-
15.2). Splintage and corticosteroid injections are usu- demiological studies suggest that these conditions are
ally effective. no more common amongst keyboard operators than
The common extensor compartment is occasionally in the general population. What has fuelled the con-
irritated by direct trauma. Patients present with pain troversy surrounding the ‘occupational’ disorders is
and crepitus on the dorsum of the wrist; flexing and their apparent severity and intractability compared
extending the fingers produces a fine, palpable crepi- with other types of overuse syndrome and the poten-
tus over the common extensor compartment. Treat- tial rewards for successful litigation. There are often
ment is by rest and splintage of the wrist. social and psychological aspects which confound the
Extensor tenosynovitis is also a common feature of picture. The term ‘work relevant upper limb disorder’
rheumatoid disease. is preferred as it acknowledges that the symptoms are
noticed at work but does not imply causation.
FLEXOR TENDINITIS
Except in specific inflammatory disorders such as
rheumatoid arthritis, the flexor tendons are rarely SWELLINGS AROUND THE WRIST
affected.
Flexor carpi radialis tendinitis causes pain on the
front of the wrist alongside the scaphoid tubercle;
GANGLION CYSTS
symptoms are reproduced by resisted wrist flexion.
Pathology
Tenderness is sharply localized and should be distin-
guished from that of de Quervain’s disease or The ganglion cyst is the most common swelling in the
osteoarthritis of the basal joint of the thumb. wrist. It arises from leakage of synovial fluid from a
15.34 Other types of

tendinitis
(a) Rheumatoid;
(b) calcific tendinitis of
flexor carpi ulnaris.
(a) (b) 407

15 EXTENSOR TENOSYNOVITIS
Localized swelling of a tendon sheath on the dorsum
of the wrist sometimes occurs in rheumatoid disease
and can be mistaken for a ‘cyst’.
CARPO-METACARPAL BOSS
A firm round swelling over the back of the second and
15.35 Volar wrist ganglion third carpo-metacarpal joint is sometimes seen in a
young adult. It is not always tender. It is thought that
it may be caused by some instability at the joint.
joint or tendon sheath and contains a glairy, viscous Treatment involves reassurance; the lump can be
fluid. Although it can appear anywhere around the excised but if it recurs, the underlying joint should be
carpus, it usually develops on the dorsal surface of the fused.
scapho-lunate ligament. Palmar wrist ganglia usually
arise from the scapho-lunate or scapho-trapezio-
trapezoid joint. ‘COMPOUND PALMAR GANGLION’
This lesion is neither a ganglion nor compound.
Clinical features Chronic inflammation distends the common sheath of
The patient, often a young adult, presents with a pain- the flexor tendons both above and below the flexor
less lump, though occasionally there is slight ache and retinaculum. Rheumatoid arthritis and tuberculosis
weakness. The lump is well defined, cystic and not are the commonest causes. The synovial membrane
tender; it can sometimes be transilluminated. It does
not move with the tendons. The back of the wrist is
the commonest site; less frequently a ganglion
emerges alongside the radial artery on the volar
aspect. Occasionally a small, hidden ganglion is found
to be the cause of compression of the deep (muscular)
branch of the ulnar nerve.
Treatment
Treatment is usually unnecessary. The lump can safely
be left alone; it often disappears spontaneously. How-
ever, it can be aspirated to reassure the patient. If it
becomes troublesome – and certainly if there is any
pressure on a nerve – operative removal is justified. (a)
Even then it may recur with embarrassing persistence;
it is not easy to ensure that every shred of abnormal
tissue is removed.
Occult ganglion or dorsal synovial

impingement
Sometimes patients complain of pain in the back of
the wrist, provoked by extending the wrist. On exam-
ination there is a discrete tender point over the back
of the mid-carpus and the pain is reproduced by full
passive wrist extension. Ultrasound, or preferably
MRI, will show either a small ganglion or thickening
of the synovium at the radio-carpal or midcarpal joint.
Treatment should be initially with a steroid injec- (b)
tion; if that fails, then arthroscopic excision may 15.36 Carpo-metacarpal boss (a) X-ray; (b) three-
408 succeed. dimensional CT.
becomes thick and villous. The amount of fluid is 15
increased and it may contain fibrin particles moulded
by repeated movement to the shape of melon seeds.
The tendons may eventually fray and rupture.
Clinical features
The wrist
23°
Pain is unusual but paraesthesia due to median nerve 12mm
compression may occur. The swelling is hourglass in 1mm
shape, bulging above and below the flexor retinacu-
lum; it is not warm or tender; fluid can be pushed
from one part to the other (cross-fluctuation).
Treatment
If the condition is tuberculous, general treatment is
begun. The contents of the sac are evacuated, strep- (a)
(a)
tomycin is instilled and the wrist rested in a splint. If
these measures fail, the entire flexor sheath is dissected
out. Complete excision is also the best treatment
when the cause is rheumatoid disease.
CARPAL TUNNEL SYNDROME
The carpal tunnel syndrome, due to median nerve

compression under the flexor retinaculum of the
wrist, is described together with other nerve compres-
sion disorders in Chapter 11.
(b)
(b) 11°
15.37 Angle of the distal radius

NOTES ON APPLIED ANATOMY
In most positions of the forearm the styloid process of

the radius is more distal than that of the ulna, but with Carpal height: the ratio between the distal edge of
the forearm supinated the two processes are at the capitate and proximal edge of the lunate/third
approximately the same level. This relationship, metacarpal is usually 0.54±0.03. The ratio is reduced
known as ulnar variance, may be altered as a result of in carpal collapse (e.g. with Kienböck’s disease and
growth abnormalities or injury. scapho-lunate ligament failure).
Relative shortness of the ulna appears as an anatom- Just distal to the radial styloid is the scaphoid,
ical variant in association with Kienböck’s disease. immediately beneath the anatomical snuffbox, which
Relative overlength is associated with ulna-carpal is one of the key areas for localizing tenderness. Ten-
impaction syndrome (central TFCC perforations and derness at the distal end of the snuffbox may incrimi-
late ulno-carpal arthritis). nate the carpo-metacarpal joint of the thumb. More
proximal tenderness, at the tip of the radial styloid, is
characteristic of de Quervain’s disease. Dorsal to the
Gilula’s arcs snuffbox the oblique course of extensor pollicis
These lines are helpful radiographic indicators. They longus exposes it to damage by a careless incision.
are illustrated in Figure 15.37. The congruent lines The carpal bones are arranged in two rows, with the
between the distal radius/ulna and proximal carpal pisiform as the odd man out. The scaphoid crosses
row, and between the proximal and distal carpal row, both rows. The scaphoid, trapezium and thumb com-
are disturbed in midcarpal instability, Kienböck’s dis- bine to function almost as a separate entity, a ‘jointed
ease and dislocation of the wrist. strut’, with independent movement; degenerative
Normal angles: radial tilt 22 degrees, palmar tilt 11 arthritis of the wrist occurs most commonly in the
degrees; scapho-lunate angle 30 to 65 degrees. joints of this strut. 409
15 Kinematics The ligaments of the wrist
Wrist flexion: the proximal row and distal row flex and The extrinsic ligaments are discrete consolidations of
ulnar deviate. the capsule. The palmar ligaments are stronger than
Wrist extension: the proximal row and distal row the dorsal (Fig. 15.38).
extend and radially deviate.
Radial deviation: there are two synchronized

movements. First, the proximal row flexes (to prevent
scaphoid blocking flexion between radial styloid and
trapezium); distal row slightly extends. Second, the
scaphoid slides ulnarwards, pushing lunate and tri-
quetrum. There is variable flexion and sliding between
individuals known as ‘column wrist’ and ‘row wrists’.
Ulnar deviation: the proximal row extends and dis-
tal row slightly flexes.
Radial–ulnar deviation is provided 60 per cent by
the midcarpal and 40 per cent by the radio-
carpal/ulno-carpal joints.
Flexion-extension: this is about 50 per cent mid-
carpal and 50 per cent radio-carpal.
Range of movement (a)

(a)
Normally the arc of flexion-extension is 110 to 150

degrees; radial tilt is 30 degrees and ulnar tilt 45
degrees. The functional range is about 10 degrees
flexion to 30 degrees extension.
Surgical anatomy of the nerves

On their volar aspect the carpal bones form a concav-
ity roofed over by the carpal ligament; in the tunnel
lie the flexor tendons and the median nerve. The
thenar branch of the nerve (supplying the all-impor-
tant thenar muscles) is in danger if, during a decom-
pression operation, the carpal ligament is divided too
far radially. On the dorso-radial side of the wrist,
branches of the superficial radial nerve are vulnerable (b)
(b)
(beware during operations for ganglia, de Quervain’s,
thumb carpo-metacarpal joint). On the ulnar side, the
close relationship of the ulnar nerve to the pisiform
and hamate hook must be borne in mind. Operations
at the distal end of the ulna threaten the dorsal branch
of the ulnar nerve which runs anteriorly about 3 cm DRC
proximal to the ulnar styloid. DIC
Ossification of the wrist bones

The ossific centre for the distal radius epiphysis appears
at age 2 and fuses at age 16–18. The other bones develop
ossification centres in clockwise order (looking at the
right hand from behind, fully pronated, i.e. face down).
Capitate (1 month), hamate (1 year); triquetrum (2–3
(c)
(c)
years); lunate (4 years); scaphoid (4–6 years); trapezium
(4–6 years); trapezoid (4–6 years); pisiform (8–10 years). 15.38 (a) Gilula’s lines; (b) palmar ligaments of the wrist;
N.B. In an adolescent, the incompletely ossified (c) dorsal ligaments of the wrist. DRC = Dorsal radiocarpal
410 scaphoid can be mistaken for a scapho-lunate dissociation. ligament. DIC = Dorsal intercarpal ligament.
EXTRINSIC CARPAL LIGAMENTS (DORSAL) ulno-triquetral. Blend into volar radio-lunate liga- 15
• Dorsal radio-carpal ligaments (radio-scaphoid, ment (i.e. anterior limb of TFCC). Ulno-triquetral
radio-triquetral). Rupture contributes to VISI. ligament blends into sub-sheath of extensor carpi
• Dorsal intercarpal ligament (triquetrum to ulnaris (also part of TFCC).
scaphoid and trapezoid). Available as a donor for
tenodesis against palmar rotation of scaphoid. SPACE OF POIRIER
The wrist
This is the gap between lunate and midcarpal joint
EXTRINSIC CARPAL LIGAMENTS (PALMAR) through which lunate can dislocate anteriorly.
• Radio-scapho-capitate. Attaches to palmar edge of
radial styloid. Fulcrum for scaphoid flexion.
INTRINSIC (INTEROSSEOUS LIGAMENTS)
Divided then carefully repaired during palmar
• Scapho-lunate interosseous ligament: C shaped,
approach to scaphoid. Readily seen in arthroscopy.
thickest dorsally.
Beware removing attachment by enthusiastic radial
• Luno-triquetral: C shaped, thickest palmarwards.
styloidectomy.
• Capitate-hamate, trapezium-capitate; trapezium-
• Long radio-lunate ligament. Restrains lunate from
trapezoid.
palmar dislocation.
• Ligament of Testut (radio-scapho-lunate). Synovial
fold, no stabilizing function. Landmark for scapho-
Blood supply of the wrist
lunate ligament in wrist arthroscopy.
• Short radio-lunate ligament. From ulnar edge of There are dorsal and palmar arches, supplied by the
distal radius to lunate, blends ulnarwards with the radial artery, ulnar artery and anterior interosseous
ulno-lunate ligament. artery. These can be used as flaps to vascularize the
• Ulno-carpal ligament. Ulno-capitate, ulno-lunate, scaphoid and lunate.
411
The hand
16
The hand is (in more senses than one) the medium may be referred from the neck, shoulder or medi-
of introduction to the outside world. Its unique astinum.
repertoire of prehensile movements, grasp, pinch, Deformity may appear suddenly (e.g. due to tendon
hook-action and tactile acuity sets us apart from all rupture) or slowly (suggesting bone or joint pathol-
other species. We can think of the hand as a sophis- ogy, a soft-tissue contracture or a postural defect due
ticated tool, but it is also an organ of communica- to a nerve lesion).
tion, used for gesturing and expressing a range of Swelling may be localized (and, if associated with
emotions from anxiety and fear to submission and throbbing pain, is almost certainly due to infection)
helplessness, scorn and hatred, determination and or it may be evident in many joints simultaneously.
control, or tenderness and love. We are more aware Ask whether the swelling is constant or intermittent,
of our hands than of any other part of the body; and how long it has been present.
when they go wrong we know about it from a very Sensory symptoms and motor weakness provide well-
early stage. defined clues to neurological disorders. A precise
description of the affected area tells us a great deal
about the level of the lesion.
CLINICAL ASSESSMENT Loss of function takes various forms. The patient
may have difficulty handling eating utensils, holding a
SYMPTOMS cup or glass, grasping a doorknob (or a crutch), dress-
ing or (most trying of all) attending to personal
Pain may be felt in the palm, the thumb or the finger hygiene. Equally important is loss of function due to
joints. Remember, though, that a poorly defined pain sensory change in the fingers.
(a) (b) (c)

16.1 Hand function (a) Pinch,
(b) key, (c) tripod, (d) grasp and
(e) power grip.
(d) (e)
16 If multiple joints are involved, take careful note of
their distribution. Characteristically, rheumatoid
arthritis causes swelling of the proximal joints –
metacarpo-phalangeal (MCP) and proximal interpha-
langeal (PIP) – while osteoarthritis affects mainly the
distal interphalangeal (DIP) joints.
Compare the thenar eminences of the two hands

(a) (b) and look for wasting on one or other side (a sign of
median nerve dysfunction).
16.2 Passive tenodesis Note the resting position of the
fingers with the wrist (a) flexed, (b) extended. Posture in different resting positions While looking at
the patient’s hands, observe their resting posture in
different positions. Normally, with the palm upwards,
SIGNS the fingers fall into a gentle cascade with the MCP
joints slightly flexed – about 30 degrees in the index,
Both upper limbs should be bared for comparison. ranging to 70 degrees in the little. The interpha-
Before focussing on the hands take a quick look at the langeal (IP) joints similarly lie in increasing flexion
shoulders and elbows and their range of movement. from index to little. When the hand is turned palm
Also ask which is the dominant hand. A rapid assess- downwards, the fingers straighten out, again in a gen-
ment can be carried out in a few minutes. A full exam- tle cascade with greater extension on the index finger
ination needs patience and meticulous attention to than the little finger. If the regular cascade is inter-
detail. rupted, then a tendon is probably either divided or
stuck. If the cascade is normal but active movements
are not possible, then a nerve injury should be sus-
Look
pected.
Note how the patient holds the hand and uses it dur- Note also that there is a reciprocal relationship
ing the interview; the resting posture may be sugges- between the position of the wrist and the resting posi-
tive of nerve or tendon damage. Ask the patient to tion of the fingers (Fig 16.2). Normally as the wrist
place both hands on the table in front of you, with the drops into flexion the fingers automatically tend to
palms first upwards and then downwards. The skin straighten, and when the wrist is pulled into extension
may be scarred, altered in colour, dry or moist, and the fingers flex slightly; contractures of the long flex-
hairy or smooth. Puckering and ridging of the skin in ors will cause the fingers to curl tightly in flexion
the palm, sometimes extending into one of the fin- when the wrist is extended.
gers, are cardinal signs of Dupuytren’s contracture.
Deformity of the fingers and the presence of any
lumps should be noted. Swelling may arise in the sub-
Feel
cutaneous tissues, in a tendon sheath or in a joint. Do The temperature and texture of the skin are noted and
not forget to look at the nails; they may show signs of the pulse is felt. Swelling or thickening may be in the
atrophy or disease: e.g. psoriasis, which is sometimes subcutaneous tissue, a tendon sheath, a joint or one of
associated with a typical arthropathy, or a ‘grooved’ the bones. If a nodule is felt, the underlying tendon
nail which is a tell-tale feature of a ganglion cyst at the should be moved (by flexing and extending the rele-
nail bed. vant finger) to discover if the nodule is attached to the
(a) (b) (c)

16.3 Gross active movement (a) Full extension. (b) Full flexion. (c) A good test for abductor power is to have the patient
spread his or her fingers as strongly as possible; slowly push the hands together until the tips of the little fingers are
414 forcefully opposing one another; the weaker one will collapse.
16.4 Joint laxity 16
(a) in the fingers,
(b,c) in the thumb.
The hand
(a) (b) (c)
tendon or its sheath. This will also reveal whether the don or loss of motor power. Active movements at
tendon glides smoothly or whether it gets stuck each of the MCP, PIP and DIP joints will have to be
momentarily with finger in flexion and then snaps free examined.
as the finger is extended (the ‘trigger finger’ effect).
Abduction and adduction When the MCP joints are
Any point of tenderness should, if possible, be accu-
held in extension, they are able to move sideways in
rately localized to a particular structure.
the plane of the flattened hand; this is because, in the
extended position, the collateral ligaments of the
MCP joints are somewhat lax. Spreading the fingers
Move apart is denoted as abduction and bringing them back
Passive movements There is a good argument for to the neutral position (all the fingers side by side) is
starting with passive movements, so that you can see adduction. Active power can be roughly gauged by
whether all the little finger joints are capable of mov- having the patient abduct the fingers forcibly and the
ing before testing the patient’s ability to move them. examiner then pressing against the spread-out index
The thumb and each finger are examined in turn and and little fingers, trying to force them back to the
the range of movement recorded. Note whether the neutral position. A better way is to ask the patient to
movement causes pain. spread the fingers of both hands to the maximum; the
Some degree of passive hyperextension at the MCP examiner then grasps the patient’s hands, pushes them
joints (tested by gently pushing each finger dorsal- towards each other and forces the two little fingers
wards to its limit) is normal but anything more than against each other. The weaker (non-dominant) side
90 degrees of (hyper)extension is suggestive of gener- will normally give way first, but if the difference in
alized joint laxity; the diagnosis can be confirmed by one or other hand is very marked it signifies true
testing the range of extension in other joints such as abductor weakness, a sign of ulnar nerve or T1 root
the thumbs, elbows and knees. dysfunction.
Active movements Ask the patient to place both hands Thumb movements Movements of the thumb and
with the palms facing upwards, to extend the fingers their nomenclature are unusual, comprising (as they
and thumbs fully and then to curl them into full flex- do) the combined mobility of both the first carpo-
ion as if making a gentle fist. A ‘lagging finger’ is metacarpal (CMC) and the first MCP joint. With the
immediately obvious, though it still remains to estab- hand lying flat, palm upwards, six types of movement
lish whether this is due to a stiff joint, a defective ten- are observed:
16.5 Thumb movements You should

have no difficulty defining the planes
of movement if you follow this routine:
(a) hold the patient’s hand flat on the
table and instruct him or her to ‘stretch
to the side’ (extension), (b) ‘point to
the ceiling’ (abduction), (c) ‘pinch my
finger’ (adduction) and (d) ‘touch your
little finger’ (opposition).
(a) (b) (c) (d) 415

16
(a) (b) (c) (d)

16.6 Testing for (a) FDP lesser fingers, (b) FDS lesser fingers, (c) FDP index, (d) FDS index.
• extension (sideways movement in the plane of the sometimes has no independent flexor digitorum
palm) superficialis. Second, the index finger often has an
• abduction (upward movement at right angles to the entirely separate flexor profundus, which cannot be
palm) inactivated by the usual mass action manoeuvre;
• adduction (pressing against the palm) instead, flexor superficialis is tested by asking the
• flexion (sideways movement across the palm) patient to pinch hard with the DIP joint in full exten-
• opposition (touching the tips of the fingers) sion and the PIP joint in full flexion; this position can
• retroposition (lifting the thumbs backwards behind be maintained only if the superficialis tendon is active
the plane of the hand). and intact.
Since the thumb has only a single IP joint, the flexor
Weakness of abduction (tested simply by pressing
pollicis longus is tested by immobilizing the thumb
against the abducted thumb of each hand) is a cardi-
MCP joint and then asking the patient to flex the IP
nal feature of median nerve dysfunction. In advanced
joint.
cases there will also be obvious wasting of the thenar
The long extensors are tested by asking the patient to
eminence.
extend the MCP joints. Inability to do this usually sig-
Pain, deformity and loss of motion at the base of
nifies either paralysis or tendon rupture; occasionally,
the thumb (the first CMC joint) are common symp-
a long extensor tendon may simply have slipped off
toms of osteoarthritis.
the knuckle into the interdigital gutter (a common
occurrence in rheumatoid arthritis).
The intrinsic muscles (lumbricals and interossei) can
Testing the muscles and tendons
act uniquely to flex the MCP joints with the IP joints
Flexion of the fingers is motivated mainly by flexor held simultaneously in extension (i.e. preventing the
digitorum profundus (FDP) and flexor digitorum long flexors from acting). Ask the patient to extend
superficialis (FDS); these muscles also assist in flexion the fingers with the MCP joints flexed (the ‘duckbill’
of the MCP joints but the main MCP flexors are the position). The interossei also motivate finger abduc-
intrinsic muscles. Active mass flexion can be tested by tion and adduction.
asking the patient to curl his or her fingers into flex-
ion so as to engage them in the examiner’s fingers in
a tug of strength. However, the patient’s flexors can
Grip strength
also be tested independently, as follows. Grip strength is an important indicator of hand and
To test for flexor digitorum profundus in an individ- wrist function. A painful wrist will result in a weak
ual finger, the PIP joint is held and immobilized in hand. Loss of finger function due to pain, stiffness,
extension and the patient is then asked to bend the tip instability or weakness will also reduce grip. Grip
of the finger. strength should be measured with a mechanical
To test flexor digitorum superficialis, the flexor pro- dynamometer; if this is not available, an indication can
fundus must first be inactivated, otherwise one cannot be derived from having the patient squeeze a partially
tell which tendon is flexing the PIP joint. This is done inflated sphygmomanometer cuff (normally a pressure
by grasping all the fingers, except the one being exam- of 150 mmHg can be achieved easily). Pinch grip also
ined, and holding them firmly in full extension; should be measured using a specific pinch gauge.
because the profundus tendons share a common mus-
cle belly, this manoeuvre automatically prevents all
the profundus tendons from participating in finger
Neurological assessment
flexion. The patient is then asked to flex the isolated If symptoms such as numbness, tingling or weakness
finger which is being examined; this movement must exist – and in all cases of trauma – a full neurological
be activated by flexor digitorum superficialis. There examination of the upper limbs should be carried
416 are two exceptions to this rule: First, the little finger out, testing power, reflexes and sensation. Further
16.7 Neurological assessment 16
(a) Light touch, (b) pinprick.
The hand
(a) (b)
refinement is achieved by testing monofilament important sensory organ; slight loss of movement
detection, two-point discrimination, vibration sensi- matters little, but if sensation is abnormal the patient
bility, proprioception and stereognosis (tactile dis- probably will not use the finger at all. The middle fin-
crimination). ger controls the position of objects in the palm. The
ring and little fingers are used for power grip; any loss
of movement here will affect function markedly.
Functional tests Stiffness is poorly tolerated in the little finger
Ultimately it is function that counts; patients learn to whereas instability is less worrisome; the opposite is
overcome their defects by ingenious modifications true for the thumb and index finger.
and trick movements. Function can be measured sub- Dexterity is lost in severe carpal tunnel syndrome
jectively using patient-completed scales, but objective (median nerve compression) because of the combina-
tests are more reliable. There are several types of grip, tion of thenar weakness, reduced sensation and dimin-
which can be tested by giving the patient a variety of ished stereognosis and proprioception.
tasks to perform: picking up a pin (precision grip),
holding a sheet of paper (pinch), holding a key (side-
ways pinch), holding a pen (chuck grip), holding a
bag handle (hook grip), holding a glass (span) and
gripping a hammer handle (power grip). Stereognosis CONGENITAL HAND ANOMALIES
is evaluated using Moberg’s pick-up test (1958). The
patient is instructed to pick up a number of small The incidence of congenital upper limb abnormalities
objects and place them in a box; the procedure is is estimated to be about 1 in 600 live births. Some are
timed and efficiency of the affected hand is compared confined to the hand but in most cases the wrist and
with that of the ‘good’ hand. forearm are involved as well. We have therefore cov-
Each finger has its special task: the thumb and index ered congenital anomalies of the wrist and hand as a
finger are used for pinch. The index finger is also an single subject in Chapter 15.
(a) (b) (c)
16.8 Congenital variations

(a) Transverse failure,
(b) radial club hand and
absent thumb,
(c) constriction rings,
(d) camptodactyly,
(e) clinodactyly.
(d) (e) 417

16 ACQUIRED DEFORMITIES If disability is marked, some improvement may be
obtained by lengthening the shortened tendons, or
else by excising the fibrosed muscles and restoring fin-
Deformity of the hand may result from acquired ger movement with tendon transfers.
disorders of the skin, subcutaneous tissues, muscles,
tendons, joints, bones or neuromuscular function. SHORTENING OF THE INTRINSIC MUSCLES
Often there is a history of trauma or infection or con- Shortening of the intrinsic muscles in the hand pro-
comitant disease; at other times the patient is unaware duces a characteristic deformity: flexion at the MCP
of any cause. joints with extension of the IP joints and adduction of
Problems arise for three main reasons: (1) the the thumb (the so-called ‘intrinsic-plus’ hand). Slight
defect may be unacceptable simply because of its degrees of deformity may not be obvious, but can be
unsightly appearance; (2) function is impaired; and diagnosed by Bunnell’s ‘intrinsic-plus’ test: with the
(3) the deformed part becomes a nuisance during MCP joints pushed passively into hyperextension
daily activities. (thus putting the intrinsics on stretch), it is difficult or
Assessment and management of hand deformities impossible to flex the IP joints passively; if the MCP
demands a detailed knowledge of functional anatomy joints are then placed in flexion, the IP joints can be
and, in particular, of the normal mechanisms of bal- passively flexed.
anced movement in the wrist and fingers. The causes of intrinsic shortening or contracture
are: (1) spasticity (e.g. in cerebral palsy); (2) volar
subluxation of the MCP joints (e.g. in rheumatoid
SKIN CONTRACTURE arthritis); (3) scarring after trauma or infection; and
(4) shrinkage due to ischaemia. Moderate contracture
Cuts and burns of the palmar skin are liable to heal can be treated by resecting a triangular segment of the
with contracture. Surgical incisions should never cross intrinsic ‘aponeurosis’ at the base of the proximal pha-
skin creases perpendicularly; they should lie more or lanx (Littler’s operation).
less parallel or oblique to them, or in the mid-axial
line of the fingers. A useful alternative is a zig-zag
incision with the middle part of the Z in the skin
crease. Longitudinal wounds can also be closed as TENDON LESIONS
Z-plasties. MALLET FINGER
Established contractures may require excision of This results from injury to the extensor tendon of the
the scar, Z-plasty of the remaining skin, skin grafts, a terminal phalanx. It may be due to direct trauma but
pedicled flap and occasionally a free flap. more often painlessly follows an innocent event when
the finger tip is forcibly bent during active extension,
perhaps while tucking the blankets under a mattress
SUPERFICIAL PALMAR FASCIA or trying to catch a ball. The terminal joint is held
(DUPUYTREN’S) CONTRACTURE flexed and the patient cannot straighten it, but passive
movement is normal. With the extensor mechanism
The superficial palmar fascia (palmar aponeurosis) fans unbalanced, the PIP joint may become hyperextended
out from the wrist towards the fingers, sending (‘swan-neck’).
extensions across the MCP joints to the fingers. X-rays are taken to show or exclude a fracture. If
Hypertrophy and contracture of the palmar fascia may there is a fracture but minimal subluxation of the
lead to puckering of the palmar skin and fixed flexion joint, it is treated by splintage with the DIP joint in
of the fingers. The condition is dealt with on page extension for 6 weeks. Operative treatment is consid-
421. ered only if there is a large fragment (>50 per cent)
and subluxation of the DIP joint. Otherwise surgery
is ill advised, as the complication rate is high and it is
MUSCLE CONTRACTURE unlikely to improve the outcome.
A mallet finger without bone injury is treated with
VOLKMANN’S ISCHAEMIC CONTRACTURE a plastic splint with the DIP joint in extension for 8
Contracture of the forearm muscles may follow circu- weeks, followed by 4 weeks of night splintage. This
latory insufficiency due to injuries at or below the treatment may still work if presentation is delayed for
elbow. Shortening of the long flexors causes the fin- a few weeks. The great majority do very well. Old
gers to be held in flexion; they can be straightened lesions need treatment only if the deformity is marked
only when the wrist is flexed so as to relax the long and hand function seriously impaired. The options
flexors. Sometimes the picture is complicated by asso- include fusion for painful arthritic joints or tendon
418 ciated damage to the ulnar or median nerve (or both). reconstruction.
RUPTURED EXTENSOR POLLICIS LONGUS therefore important; an impending deformity should 16
The long thumb extensor may rupture after fraying or be suspected in anyone with tenderness or a cut over
ischaemia where it crosses the wrist (e.g. after a Colles’ the dorsum of the PIP joint, especially if they cannot
fracture, or in rheumatoid arthritis). The distal phalanx actively extend the IP joint with the MCP joints and
drops into flexion; it can be passively extended, and wrist flexed.
there may still be weak active extension because of In the early post-traumatic case, splinting the PIP
The hand
thenar muscle insertion into the extensor expansion; joint in full extension for 6 weeks usually leads to
however, the thumb cannot be actively elevated back- healing; the DIP joint must be moved passively to
wards above the plane of the hand (retroposition). prevent the lateral bands from sticking. Open injuries
Direct repair is unsatisfactory and a tendon transfer, of the central slip should be repaired, with the joint
using the extensor indicis, is needed. The results are, in protected by a K-wire for 3 weeks.
over 90 per cent of cases, satisfactory. For later cases where the joint is still passively cor-
rectible, several operations have been invented (sug-
DROPPED FINGER gesting that none is too reliable). The easiest and
Sudden loss of finger extension at the MCP joint is usu- probably most successful procedure is to divide the
ally due to tendon rupture at the wrist (e.g. in rheuma- extensor tendon just proximal to its insertion into the
toid arthritis). Because direct repair is not usually pos- distal phalanx. This allows the extensor mechanism to
sible, the distal portion can be attached to an adjacent move proximally, thus enhancing PIP extension and
finger extensor or a tendon transfer performed. diminishing DIP extension.
Occasionally the deformity is due to catching of the Longstanding fixed deformities are extremely diffi-
collateral ligament on a metacarpal osteophyte or rup- cult to correct and may be better left alone.
ture of the sagittal band which centralizes the tendon
over the back of the knuckle. SWAN-NECK DEFORMITY
This is the reverse of the boutonnière deformity; the
BOUTONNIÈRE DEFORMITY PIP joint is hyperextended and the DIP joint flexed.
This lesion presents as a flexion deformity of the PIP The deformity can be reproduced voluntarily by lax-
joint and extension of the DIP joint. It is due to inter- jointed individuals. The clinical disorder has many
ruption or stretching of the central slip of the exten- causes, with two things in common: imbalance of
sor tendon where it inserts into the base of the middle extensor versus flexor action at the PIP joint and lax-
phalanx. The lateral slips separate and the head of the ity of the palmar plate. Thus it may occur: (1) if the
proximal phalanx thrusts through the gap like a but- PIP extensors overact (e.g. due to intrinsic muscle
ton through a buttonhole. Ironically while English spasm or contracture, after mallet finger, or following
speakers call it a ‘boutonniere’ deformity, the French volar subluxation of the MCP joint); (2) if the PIP
refer to it as ‘le buttonhole’.The usual causes are direct flexors are inadequate (inhibition or division of the
trauma or rheumatoid disease. Initially the deformity flexor superficialis); or (3) if the palmar plate of the
is slight and passively correctable; later the soft tissues PIP joint fails (in rheumatoid arthritis, lax-jointed
contract, resulting in fixed flexion of the proximal and individuals or trauma). If the deformity is allowed to
hyperextension of the DIP joint. Early diagnosis is persist, secondary contracture of the intrinsic muscles,
and eventually of the PIP joint itself, makes correction
increasingly difficult and ultimately impossible.
Treatment depends on the cause and whether or
not the deformity has become fixed. If the deformity
corrects passively, then a simple figure-of-eight ring
splint to maintain the PIP joint in a few degrees of
(a) flexion may be all that is required; if this works but
cannot be tolerated, then tenodesis of the PIP joint
works well. The options are either to attach one slip
of flexor digitorum superficialis to the proximal pha-
lanx, which prevents hyperextension, or to re-route a
lateral band anteriorly so it becomes a flexor rather
than an extensor of the PIP joint. If the intrinsics are
(b) (c)
tight they are released.
16.9 Boutonnière deformity (a) When the middle slip If the deformity is fixed, then it may respond to
of the extensor tendon first ruptures there is no more than
gentle manipulation supplemented by temporary K-
an inability to extend the PIP joint. (b) Gradually the lateral
slips slide volarwards, the knuckle pops through the wire fixation in a few degrees of flexion; if not, then
‘buttonhole’ and the DIP joint is pulled into lateral band release from the central slip may be
hyperextension. (c) Clinical appearance. needed. The dorsal skin may not close directly after 419
16 deformities are rare). The hands are small because of
premature fusion of the physis.
The mainstay of treatment is medical. Long-term
splintage of the hand is helpful and synovectomy is
sometimes needed. Later, wrist fusion, MCP joint
replacement and IP joint fusion also have a role, usu-
ally after skeletal maturity.
(a) (b) PSORIATIC ARTHRITIS

Erosive arthropathy of the IP joints leads to profound
weakness and instability; the PIP joints may develop
fixed flexion deformities. If the disease progresses,
psoriatic arthritis can devastate the small joints of the
hand (‘arthritis mutilans’) resulting in severe, and
sometimes bizarre, deformities of the IP and MCP
joints. The nails are often pitted (onychodystrophy)
and skin lesions (a guttate or pustular rash) may be
evident. Occasionally joint fusion is needed to relieve
(c) (d)
pain and to provide stability in a functional position.
SYSTEMIC LUPUS ERYTHEMATOSUS

This autoimmune disease, affecting women five times
more frequently than men, causes soft-tissue slacken-
ing with extensor tendon dislocation, ulnar deviation
at the MCP joints and swan-neck deformities of the
fingers. Soft-tissue corrections tend to fail with time
(e) (f) and eventually fusions may be needed to maintain
16.10 Deformities due to tendon lesions (a) Mallet function.
finger. (b) Dropped fingers due to extensor tendon
ruptures at the wrist. (c) Swan-neck deformities. SCLERODERMA
(d) Boutonnière deformities. (e) Rupture of extensor Typically the fingers are smooth-skinned and stiff
pollicis brevis. (f) Rupture of extensor pollicis longus. (sclerodactyly), with flexion deformities of the IP
joints. Raynaud’s phenomenon and painful ulcers may
develop. Early on, physiotherapy and splinting help;
in the later stages, joint fusion in a functional position
correction. If the swan-neck deformity is secondary to and digital sympathectomy to relieve ulcers may be
a mallet finger, then the latter should be addressed as needed. Painful calcific deposits can be excised but
described above. wound breakdown is a risk.
If function is severely impaired and does not
respond to one of the above measures, the joint is OSTEOARTHRITIS
arthrodesed in a more acceptable position. Osteoarthritis, by contrast, affects mainly the DIP
joints. It is common in postmenopausal women and
may cause deformity. The thumb CMC joint is
JOINT DISORDERS another common site, and this may result in adduc-
tion of the first metacarpal and flexion of the first
RHEUMATOID ARTHRITIS CMC joint. Treatment is discussed on page 403.
Rheumatoid arthritis causes multiple, symmetrical
deformities of both hands, typically ulnar deviation of GOUT
the MCP joints and boutonnière or swan-neck defor- Gouty swellings (tophi) and finger deformities are
mities of the proximal finger joints (see page 424). sometimes mistaken for rheumatoid disease. How-
ever, the lesions tend to be asymmetrical and the x-ray
JUVENILE IDIOPATHIC ARTHRITIS appearances are distinctive. The diagnosis can be con-
The pattern of involvement is different from that of firmed by identifying urate crystals in the tophaceous
adult disease. The wrists tend to develop ulnar (rather material. Curiously, gout and rheumatoid arthritis
than radial) deviation, the MCP joints develop flexion hardly ever occur in the same patient. In addition to
contractures (rather than ulnar drift), and the IP systemic treatment, evacuation of a tophus (or tophi)
420 joints also become fixed in flexion (swan-neck is sometimes advisable.
16
The hand
(a) (b) (c) (d)
16.11 Spastic contracture – hand deformities (a,b,c) cerebral palsy, and (d) head injury with brain damage.
TRAUMA shows wasting of the small muscles and moderate

Fractures may go on to malunion and joints may clawing, with extension of the MCP and partial flex-
become stiff and swollen. This subject is dealt with in ion of the IP joints. If all the intrinsics are affected
Chapter 26. (e.g. after poliomyelitis or a combined low median
and ulnar nerve injury) the thumb lies flat at the side
of the hand and cannot be opposed. In ulnar nerve
BONE LESIONS palsy only the ring and little fingers are clawed,
because the index and middle lumbricals are supplied
A variety of bone lesions (acute infection, tuberculo- by the median nerve; these muscles continue to flex
sis, malunited fractures, infantile rickets, tumours) the MCP joints and extend the IP joints. Thumb
may cause metacarpal or phalangeal deformity. X-rays opposition is retained but thumb pinch is unstable
usually show the abnormality. In addition to treating because index-finger abduction (first dorsal
the pathological lesion, deformity may need correc- interosseous) is weak, and loss of thumb adduction is
tion by osteotomy with internal fixation. compensated for by exaggerated IP flexion during
strong pinch (Froment’s sign).
The objectives of treatment are: (1) stabilization of
NEUROMUSCULAR DISORDERS the MCP joints in flexion – this can be achieved
dynamically by a tendon transfer (e.g. flexor superfi-
SPASTIC PARESIS
cialis into the intrinsic tendon) or statically by looping
Cerebral palsy, head injury and stroke may result in
a slip of flexor digitorum superficialis around the
typical deformities of the hand. The ‘intrinsic-plus’
flexor pulley (Zancolli’s operation); (2) restoration of
posture is easily recognized. Another common dis-
index abduction to provide stable pinch (e.g. by
ability is ‘thumb-in-palm’; the tendency to adduct and
extensor carpi radialis brevis tendon transfer to the
flex the thumb into the palm is increased by activity,
first dorsal interosseous); (3) restoration of thumb
especially finger flexion. Releasing the adductor polli-
opposition (if it is lost) by a tendon transfer looped
cis from the third metacarpal may improve the appear-
around a fascial or tendon pulley and attached to the
ance, but normal thumb pinch is rarely restored.
radial edge of the proximal phalanx of the thumb.
Before any of these operations, stiff finger joints must be
OTHER NEUROLOGICAL DISORDERS
made mobile.
Poliomyelitis, leprosy, syringomyelia and Charcot–
Marie–Tooth disease may cause hand deformities. If
there is only partial involvement, tendon transfer may
be feasible. DUPUYTREN’S CONTRACTURE
PERIPHERAL NERVE LESIONS This is a nodular hypertrophy and contracture of the

The postural deformities are so characteristic that the superficial palmar fascia (palmar aponeurosis). The
diagnosis should seldom be in doubt (see Chapter condition is inherited as an autosomal dominant trait
11). The most common are drop-wrist and drop-fin- and is most common in people of European (espe-
gers (radial nerve palsy), a simian thumb and pointing cially Anglo-Saxon) descent. It is more common in
index finger (median nerve palsy) and partial claw males than females; the prevalence increases with age,
hand (ulnar nerve palsy). The distribution of sensory but onset at an early stage usually means aggressive
loss helps to establish the site of the lesion. disease. There is a high incidence in epileptics receiv-
ing phenytoin therapy; associations with diabetes,
THE ‘INTRINSIC MINUS’ HAND smoking, alcoholic cirrhosis, AIDS and pulmonary
Among the late neurological defects, intrinsic paraly- tuberculosis have also been described. There is a con-
sis is particularly disabling. The ‘intrinsic minus’ hand tentious and weak association with injury to the hand. 421
16
(a) (b)
(a)
(c) (d)
16.12 Dupuytren’s disease Contractures may occur at

(a) palmar crease, (b) proximal interphalangeal joint,
(c) thumb web, (d) little finger.
(b)
16.14 Dupuytren’s disease – surgery (a) Z-plasty in the

(a) (b) hand shortly after operation and two weeks later when
16.13 Dupuytren’s disease – other manifestations (a) healing is almost complete; (b) skin graft in theatre.
Garrod’s pads, (b) Ledderhose’s nodules.
PATHOLOGY other. The palm is puckered, nodular and thick. If the

The essential problem in Dupuytren’s disease is pro- subcutaneous cords extend into the fingers they may
liferation of myofibroblasts; where they come from produce flexion deformities at the MCP and PIP
and why they proliferate remains unclear. After an joints. Sometimes the dorsal knuckle pads are thick-
initial proliferative phase, fibrous tissue within the pal- ened (Garrod’s pads). About 60 per cent of patients
mar fascia and fascial bands within the fingers con- give a family history.
tracts, causing flexion deformities of the MCP and Similar nodules may be seen on the soles of the feet
PIP joints. Fibrous attachments to the skin lead to (Ledderhose’s disease). There is a rare, curious associ-
puckering. The digital nerve is displaced or envel- ation with fibrosis of the corpus cavernosum (Pey-
oped, but not invaded, by fibrous tissue. Occasionally ronie’s disease).
the plantar aponeurosis also is affected.
Diagnosis
Clinical features Dupuytren’s contracture must be distinguished from
The patient – usually a middle-aged man – complains skin contracture (where the previous laceration is usu-
of a nodular thickening in the palm. Gradually this ally obvious), tendon contracture (in which the finger
extends distally to involve the ring or little finger. Pain deformity changes with wrist position) and PIP joint
may occur early on but is seldom a marked feature. contracture (in which there may be a history of clin-
422 Often both hands are involved, one more than the odactyly or joint injury).
Treatment secondary nodule can develop on the tendon. The 16
underlying cause is unknown but the condition is cer-
Operation is indicated if the deformity is a nuisance or tainly more common in patients with diabetes. People
rapidly progressing. In particular, PIP joint contrac- with rheumatoid disease may develop synovial thick-
tures can become irreversible. The aim is reasonable, ening or intratendinous nodules which can also cause
not complete, correction. Surgery does not cure the triggering. Occupational factors, though sometimes
The hand
disease, it only partially corrects the deformity, and blamed, are unlikely to be causative.
recurrence or extension is common. Correction of the
MCP joint is more predictable than the PIP joint.
Only the thickened part of the fascia is excised Clinical features
(complete fasciectomy is usually unnecessary). An iso- Any digit may be affected, but the thumb, ring and
lated cord across the front of the MCP joint can be middle fingers most commonly; sometimes several fin-
managed by dividing the contracture under local gers are affected. The patient notices a click as the fin-
anaesthetic with a bevelled needle (‘needle fas- ger is flexed; when the hand is unclenched, the
ciotomy’). If the disease is more extensive, the affected finger initially remains bent at the PIP joint
affected area is approached through a longitudinal or but with further effort it suddenly straightens with a
a Z-shaped incision and, after carefully freeing the snap. A tender nodule can be felt in front of the MCP
nerves and blood vessels, the cords are excised. Skin joint and the click may be reproduced at this site by
closure may be facilitated by multiple Z-plasties. This alternately flexing and extending the finger.
has the dual effect of improving the deformity and, if
recurrence occurs, preventing a longitudinal wound INFANTILE TRIGGER THUMB
contracture. The palmar section of the wound can be Parents sometimes notice that their baby or infant
left open; it will soon heal with dressings. This makes cannot extend the thumb tip. The diagnosis is often
skin closure easier and allows any haematoma (which missed, or the condition is wrongly taken for a ‘dislo-
may predispose to recurrence) to escape. After opera- cation’. Very occasionally the child grows up with the
tive correction a splint is applied, and removed after a thumb permanently bent. This condition must be dis-
few days for active motion exercises. Night splinting tinguished from the rare congenitally clasped thumb in
for a few months may reduce recurrence. which both the IP joint and the MCP joint are flexed
If there is severe skin involvement (particularly in because of congenital insufficiency of the extensor
surgery for recurrent disease), if there is a strong fam- mechanism (see Chapter 15).
ily history, or if the patient is particularly young, then
skin grafting should be considered. Amputation or
joint fusion is occasionally advisable for severe, recur- Treatment
rent disease in the little finger. In adults, early cases may be cured by an injection of
corticosteroid carefully placed at the mouth of the
tendon sheath. Recurrent triggering up to 6 months
later occurs in over 30 per cent of patients – particu-
TRIGGER FINGER (DIGITAL larly younger patients and those with diabetes, who
TENOVAGINOSIS) may then need a second injection. Refractory cases
need operation, through an incision over the distal
A flexor tendon may become trapped by thickening at palmar crease, or in the MCP crease of the thumb –
the entrance to its sheath; on forced extension it the A1 section of the fibrous sheath is incised until the
passes the constriction with a snap (‘triggering’). A tendon moves freely.
(a) (b) (c)
16.15 Trigger finger (a) Injection of steroid, (b,c) operative treatment. 423
16 In babies it is worth waiting until the child is about hands are affected, more or less symmetrically.
3 years old, as spontaneous recovery often occurs. If Swelling of tendon sheaths is usually seen on the dor-
not, then the pulley is released. sum of the wrist and along the ulnar border (extensor
Care should be taken to avoid injury to the digital carpi ulnaris); thickened flexor tendons may also be
neurovascular bundles during surgery. The risk is felt on the volar aspect of the proximal phalanges. The
greatest in the thumb (where the nerves are close to joints are tender and crepitus may be felt on moving
the midline) and the index finger (where the radical the tendons. Joint mobility and grip strength are
digital nerve crosses the tendon). diminished.
In patients with rheumatoid arthritis the fibrous As the disease progresses, early deformities make
pulley must be carefully preserved; damage to this their appearance: slight radial deviation of the wrist
structure will predispose to ulnar deviation of the fin- and ulnar deviation of the fingers, correctable swan-
gers. Flexor synovectomy with excision of one slip of neck deformities of some fingers, an isolated bouton-
flexor digitorum superficialis is preferred. nière or the sudden appearance of a drop-finger or
mallet thumb (from extensor tendon rupture).
In the late stage, long after inflammation may have
subsided, established deformities are the rule: the car-
RHEUMATOID ARTHRITIS (see also pus settles into radial tilt and volar subluxation; there
Chapter 3) is marked ulnar drift of the fingers and volar disloca-
tion of the MCP joints, often associated with multiple
swan-neck and boutonnière deformities. These
The hand, more than any other region, is where
‘rheumatoid deformities’ are so characteristic that
rheumatoid arthritis carves its story. The early stage is
they allow the diagnosis to be made at first glance.
characterized by synovitis of the joints and tendon
When the abnormalities become fixed, functional loss
sheaths. If the disease progresses, joint and tendon
may be so severe that patients can no longer dress or
erosions prepare the ground for mechanical derange-
feed themselves.
ment. In the late stage, joint destruction, attenuation
of the ligaments and tendon ruptures lead to instabil-
ity and progressive deformity.
With the advent of biological treatment such as
General features
anti-TNF agents, the need for surgical treatment has The hand should not be considered in isolation. Its
diminished considerably. functional interaction with the wrist and elbow is cru-
cial and, in a generalized disorder such as rheumatoid
disease, the condition of all the upper limb joints and
Clinical features the cervical spine should be carefully assessed.
Stiffness and swelling of the fingers are early symp-
Weakness Rheumatoid hands are weak because of a
toms; the patient may mention that the wrist also is
combination of generalized muscular weakness, pain
swollen. Sometimes the first symptoms are typical of
inhibition, tendon malalignment or rupture, joint
carpal tunnel compression, caused by flexor tenosyn-
stiffness and nerve compression.
ovitis at the wrist.
Examination may reveal swelling of the MCP and Rheumatoid nodules These are associated with aggres-
PIP joints, giving the fingers a spindle shape; both sive disease in seropositive patients. They tend to
(a) (b) (c)

16.16 Rheumatoid arthritis – clinical features (a) Early case with typical features: radial deviation of the wrist;
subluxation of the radio-ulnar joint; swollen MCP joints and ulnar deviation of the fingers. (b) More advanced changes,
424 including subluxation of the MCP joints. (c) Dropped fingers due to rupture of extensor tendons at the wrist.
16
The hand
(a) (b) (c)
16.17 Rheumatoid arthritis – x-ray changes (a) Early on, the x-rays may show no more than soft-tissue swelling and
juxta-articular osteoporosis. (b) A later stage showing characteristic punched-out juxta-articular erosions at the second and
third metacarpo-phalangeal joints. The wrist is now also involved. (c) In the most advanced stage, the metacarpo-
phalangeal joints are dislocated and the hand is severely deformed.
occur at pressure areas (e.g. the pulps of the fingers Treatment

and the radial side of the index finger).
EARLY STAGE DISEASE
Z-collapse If one of two adjacent joints changes direc- Treatment is directed essentially at controlling the sys-
tion, then the overlying long tendons will pull the temic disease and the local synovitis. In addition to
other joint into the opposite direction. In rheumatoid general measures, static splints may reduce pain and
arthritis, this is typified by radial tilt of the wrist with swelling. These splints are not corrective but are
ulnar drift of the MCP joints, the boutonnière defor- designed to rest inflamed joints and tendons; in mild
mity and the swan-neck deformity. cases they are worn only at night, in more active cases
during the day as well. Persistent synovitis of a few
X-rays joints or tendon sheaths may benefit from local
injections of corticosteroid with local anaesthetic.
During the early stage x-rays show only soft-tissue Only small quantities are injected (e.g. 0.5 mL for an
swelling and osteoporosis around the joints. Later one MCP joint or flexor tendon sheath and 1 mL for the
can usually discern joint ‘space’ narrowing and small wrist). This should not be repeated more than two or
peri-articular erosions; these are commonest at the three times. A boggy flexor tenosynovitis may not
MCP joints and in the styloid process of the ulna. In respond to this limited therapeutic assault; operative
advanced cases, articular destruction may be marked, synovectomy may be needed. If carpal tunnel symp-
affecting the MCP, PIP and wrist joints almost toms are present, the transverse carpal ligament is
equally. Joint deformity and dislocation are common. divided and, if necessary, a flexor synovectomy per-
formed.
Established disease As the disease progresses it becomes
increasingly important to prevent deformity. Uncon-
trolled synovitis of joints or tendons requires operative
synovectomy followed by physiotherapy. Excision of the
distal end of the ulna, synovectomy of the common ex-
tensor sheath and the wrist, and reconstruction of the
(a) (b) soft tissues on the ulnar side of the wrist may arrest joint
16.18 Rheumatoid arthritis – treatment (a) Swan-neck destruction and progressive deformity. Early instability
deformity; (b) swan-neck ‘figure of eight’ splint. and ulnar drift at the MCP joints can be corrected by 425
16 16.19 Rheumatoid
arthritis – synovectomy
Synovitis of the common
extensor sheath will
eventually damage the
tendons. (a) Here, after
synovectomy, one can see
nodules on several tendons.

(b) The sheath itself is
preserved intact and laid
beneath the tendons to
cover the back of the joint
and provide a bed upon
which the tendons can
move.
(a) (b)
excising the inflamed synovium, tightening the capsu- not suffice. For the MCP and IP joints of the thumb,
lar structures and releasing the ulnar pull of the intrin- arthrodesis gives predictable pain relief, stability and
sic tendons. Mobile boutonnière and swan-neck de- functional improvement. The MCP joints of the fin-
formities can be treated with splints; if they progress or gers can be excised and replaced with Silastic ‘spacers’,
are fixed, then surgery may be needed. Isolated tendon which improve stability and correct deformity.
ruptures are repaired or bypassed by appropriate ten- Replacement of IP joints gives less predictable results;
don transfers. These procedures are followed by splin- if deformity is very disabling (e.g. a fixed swan-neck)
tage and hand therapy. it may be better to settle for arthrodesis in a more
Destruction of the MCP joints without ulnar drift functional position. At the wrist, painless stability can
can be treated with surface replacement (chrome– be regained by fusion of the radio-carpal, midcarpal
polyethylene or pyrocarbon). and CMC joints. Wrist replacement with Silastic or
metal–plastic implants, whilst providing some move-
Late disease In late cases deformity is combined with ment, may well fail; the loss of bone stock that accom-
articular destruction; soft-tissue correction alone will panies failure means that salvage can be very difficult.
MANAGEMENT OF THUMB DEFORMITIES IN RHEUMATOID ARTHRITIS

Ruptured FPL • If deformity severe: trapeziectomy with soft-
• If painless: leave alone tissue reconstruction or fusion of MCP joint
• If painful: tendon graft, flexor digitorum
sublimus transfer or IP fusion Failure of ulnar collateral ligament (like
‘gamekeeper’s thumb’)
Simple boutonnière deformity
• Synovitis attenuates ulnar collateral ligament.
• If passively correctible: cortisone injection to MCP Pinch grip causes increasing deformity
joint and splintage
• Ligament reconstruction (if bone and soft-tissue
• MCP joint synovectomy and extensor realignment quality allow) or MCP joint fusion
unreliable
• If MCP joint fixed but IP joint passively correctible Swan-neck with MCP joint and CMC joint
and CMC joint mobile: fuse MCP joint preserved
• If MCP joint and IP joint fixed: fuse IP joint and • Synovitis of MCP joint causes hyperextension with
either fuse or replace MCP joint secondary passive flexion of IP joint
Boutonnière with CMC joint failure • Treat by palmar plate advancement or, if soft
tissues tenuous, MCP fusion
• Trapeziectomy and CMC joint stabilization, with
MCP joint and IP joint treated as above
Arthritis mutilans
Swan-neck deformity • Arthrodesis with interposition bone graft
• CMC joint failure causes adduction contracture of
thumb base and MCP joint hyperextension
426
The thumb in rheumatoid arthritis 16
TYPES OF SWAN-NECK DEFORMITY IN
The combination of soft-tissue failure and joint ero- RHEUMATOID ARTHRITIS
sion leads to characteristic deformities of the thumb:
rupture of flexor pollicis longus tendon, a bouton-
Type I PIP joint flexible, independent of MCP
nière lesion at the MCP joint, CMC instability, swan-
position (i.e. Bunnell’s test negative). Due
The hand
neck deformity and ulnar collateral ligament
to palmar plate failure at PIP joint ± fail-
instability.
ure of flexor digitorum superficialis
Depending on the deformity, the patient’s demands
and the condition of the rest of the hand, treatment Type II PIP joint flexibility dependent on MCP
may involve various combinations of splintage, ten- position. Intrinsic muscle tightness. Bun-
don repair, joint fusion, excision arthroplasty and nell’s test: with MCP joint passively
joint replacement. extended, passive PIP joint flexion limited
Treatment options are summarized in the accompa-
Type III PIP joint stiff regardless of MCP position.
nying box.
Due to contracture of joint
Type IV Destruction of PIP joint

Metacarpo-phalangeal deformities
Chronic synovitis of the MCP joints results in failure
of the palmar plate and the collateral ligaments. The
powerful flexor tendons drag the proximal phalanx has started, splintage may maintain function and
palmarwards, causing subluxation of the joint. The retard progression. With marked deformity but little
deformity may be aggravated by primary or secondary joint damage, a soft-tissue reconstruction (reefing of
intrinsic muscle tightness. the radial sagittal bands, tightening of the radial col-
The most obvious deformity of the rheumatoid lateral ligament with intrinsic muscle release and
hand is ulnar deviation of the MCP joints. There are transfer) can give a satisfactory and fairly durable cor-
several reasons for this: palmar grip and thumb pres- rection. Once there is marked damage to the joint sur-
sure naturally tend to push the index finger ulnar- face, replacement with a Silastic spacer, along with the
wards; weakening of the collateral ligaments and the soft-tissue reconstruction, is recommended. There is
first dorsal interosseous muscle reduces the normal no point in correcting the MCP joints unless any wrist
resistance to this force; the wrist is usually involved deformity is also corrected; the tendency to zig-zag
and, as it collapses into radial deviation, the MCP deformity will otherwise lead to recurrence of the
joints automatically veer in the opposite direction (the ulnar drift.
so-called ‘zig-zag mechanism’); once ulnar drift
begins, it becomes self-perpetuating due to tightening
Finger deformities
of the ulnar intrinsic muscles and stretching of the
radial intrinsics and the adjacent capsular structures. Boutonnière Synovitis in the proximal IP joint causes
As the sagittal bands fail, the extensor tendon slips elongation or rupture of the central slip which passes
ulnarwards and palmarwards, accentuating the defor- over the back of the joint before inserting into the
mity even further. base of the middle phalanx. The lateral bands slip
At an early stage, before joint destruction and soft- away from the central slip and pass in front of the axis
tissue instability, synovectomy may relieve pain but of rotation of the proximal joint but remain behind
the joint usually stiffens somewhat. When ulnar drift the axis in the distal joint, to form the characteristic
(a) (b) (c) (d)
16.20 Rheumatoid arthritis – joint replacement (a) Before operation. Subluxation and deformity of all the finger MCP
joints. (b, c) The eroded metacarpal heads are excised and flexible spacers inserted. (d) Postoperative result.
427
16 deformity. Early, correctable deformity responds to Rupture of flexor digitorum profundus is best treated
splinting and synovectomy; later, central slip recon- by distal IP joint fusion. Rupture of flexor pollicis
struction (an unpredictable procedure) may be longus (due to attrition against the underside of the
required; simple division of the distal insertion is a distal radius or flexor synovitis) can be treated either
simpler, and often effective, alternative. In fixed defor- by tendon grafting or by fusion of the thumb IP joint.
mities, or those with joint damage, fusion or replace-
ment is considered.
Swan-neck Chronic synovitis may lead to swan-neck OSTEOARTHRITIS
deformity by one or more of the following mecha-
nisms: failure of the palmar plate of the PIP joint; rup-
Eighty per cent of people over the age of 65 have radi-
ture of the flexor digitorum superficialis; dislocation
ological signs of osteoarthritis in one or more joints of
or subluxation of the MCP joint and consequent
the hand; fortunately, most of them are asympto-
tightening of the intrinsic muscles.
matic.
Treatment depends on a careful analysis of the
cause and will include figure-of-eight splintage, ten-
DISTAL INTERPHALANGEAL JOINTS
don transfer, intrinsic release and occasionally fusion.
Osteoarthritis of the DIP joints is very common in
postmenopausal women. It often starts with pain in
Tenosynovitis and tendon rupture one or two fingers; the distal joints become swollen
and tender, the condition usually spreading to all the
Extensor tendons Extensor tendon rupture is a com-
fingers of both hands. On examination there is bony
mon complication of chronic synovitis. Extensor dig-
thickening around the joints (Heberden’s nodes) and
iti minimi is usually the first to go and predicts
some restriction of movement.
rupture of the other tendons. Treatment consists of
Treatment is usually symptomatic. However, if pain
either suturing the distal tendon stump to an adjacent
and instability are severe, a cortisone injection will
tendon, inserting a bridge graft (e.g. palmaris longus)
give temporary relief. Joint fusion is a good solution.
or performing a tendon transfer (e.g. extensor indicis
The angle of fusion is debatable. Intramedullary dou-
proprius). Synovectomy and excision of the distal ulna
ble-pitched screws are effective and avoid the prob-
may also be necessary.
lems of percutaneous wires. However, the final
Flexor tendons Flexor tenosynovitis is one of the ear- position is one of extension which slightly reduces
liest and most troublesome features of rheumatoid grip in the little and ring fingers.
disease. The restriction of finger movement is easily Mucous cysts sometimes protrude between the
mistaken for arthritis; however, careful palpation of extensor tendon and collateral ligament of an
the palm and the nearby joints will quickly show osteoarthritic DIP joint. They press on the germinal
where the swelling and tenderness are located. Sec- matrix of the nail, causing an unsightly groove. They
ondary problems include carpal tunnel syndrome, occasionally ulcerate and septic arthritis can develop.
triggering of one or more fingers and tendon rupture. If the cyst is too bothersome, excision of the cyst with
Synovitis of the flexor digitorum superficialis also con- the underlying osteophyte is effective. With luck, the
tributes to the swan-neck deformity. nail will recover as well.
If carpal tunnel release is needed, the operation
should include a flexor tenosynovectomy. If the flexor PROXIMAL INTERPHALANGEAL JOINTS
tendons are bulky (best felt over the proximal pha- Not infrequently some of the PIP joints are involved
langes) and joint movement is limited, then flexor (Bouchard’s nodes). These are strongly associated
tenosynovectomy should improve movement and, with osteoarthritis elsewhere in the body (polyarticu-
just as important, should prevent tendon rupture. lar OA). The joints are swollen and tend to deviate
Triggering, likewise, should be treated by tenosyn- ulnarwards due to mechanical pressure in daily activi-
ovectomy rather than simple splitting of the sheath. ties.
16.21 Osteoarthritis
(a,b) The common picture is
one of ‘knobbly finger-tips’
due to involvement of the
DIP joints (Heberden’s
nodes). (c) In some cases the
PIP joints are affected as well
(Bouchard’s nodes).
428 (a) (b) (c)
16
The hand
(a) (b) (c)
16.22 Osteoarthritis – operative treatment (a) Pyrocarbon MCP joint replacement. (b) PIP joint replacement.
(c) Arthrodesis of the DIP joint.
Treatment is usually non-operative. If the joint is Carpo-metacarpal joint of the ring and little fingers These
very painful or unstable then surgery is considered. joints can become arthritic, particularly after a fracture-
Fusion restores reliable, pain-free pinch in the index dislocation. Because the fourth and fifth CMC joints
and middle finger PIP joints; fusion of the ring and normally flex forwards during power grip, pain can be
little fingers compromises grip and so joint replace- disabling, particularly in patients engaged in heavy
ment is usually preferable. Implants made from pyro- manual work. If a steroid injection fails to give im-
carbon, Silastic or metal–polyethylene are available. provement, then surgery (usually fusion) is indicated.
However, the results are unpredictable: some patients
do very well; others have problems with deformity,
instability or stiffness.
ACUTE INFECTIONS OF THE HAND
Metacarpo-phalangeal joints This is an uncommon site
for osteoarthritis. When it does occur, a specific cause Infection of the hand is frequently limited to one of
can usually be identified: previous trauma, infection, several well-defined compartments: under the nail-fold
gout or haemochromatosis. (paronychia); the pulp space (felon) and in the subcu-
Treatment is initially non-operative with the use of taneous tissues elsewhere; the deep fascial spaces;
analgesics, splints or local injections. Fusion of the tendon sheaths; and joints. Usually the cause is a
thumb MCP gives excellent results; however in the fin- staphylococcus which has been implanted during fairly
gers this operation has serious functional consequences trivial injury. However, cuts contaminated with un-
and is to be avoided. The MCP joints can be replaced usual organisms account for about 10 per cent of cases.
with pyrocarbon or metal–polyethylene implants, with
encouraging early and mid-term results. Pathology
Carpo-metacarpal joint of the thumb This is discussed Here, as elsewhere, the response to infection is an
on page 403. acute inflammatory reaction with oedema, suppura-
(a) (b) (c)
16.23 Swollen fingers Always be on the alert for ‘lookalikes’. The clues (in most cases) are: (a) Proximal joints =
rheumatoid arthritis; (b) distal joints = osteoarthritis; asymmetrical joints = gout. 429
16
(a) (b) (c) (d)
16.24 Acute infections (1) (a) Acute nail-fold infection (paronychia); and (b) chronic paronychia. (c) Pulp-space infection
(felon or whitlow) of the thumb due to a prick-injury on the patient’s own denture. (d) Septic granuloma. (Courtesy of
Professor S. Biddulph.)
tion and increased tissue tension. In closed tissue injection. Also, do not forget to enquire about pre-
compartments (e.g. the pulp space or tendon sheath) disposing conditions such as diabetes mellitus, intra-
pressures may rise to levels where the local blood venous drug abuse and immunosuppression.
supply is threatened, with the risk of tissue necrosis. On examination the finger or hand is red and
In neglected cases infection can spread from one com- swollen, and usually exquisitely tender over the site of
partment to another and the end result may be a per- tension. However, in immune-compromised patients,
manently stiff and useless hand. There is also a danger in the very elderly and in babies, local signs may be
of lymphatic and haematogenous spread; even appar- mild. With superficial infection the patient can usually
ently trivial infections may give rise to lymphangitis be persuaded to flex an affected finger; with deep in-
and septicaemia. fections active flexion is not possible. The arm should
be examined for lymphangitis and swollen glands, and
Clinical features the patient more generally for signs of septicaemia.
X-ray examination may disclose a foreign body but
Usually there is a history of trauma (a superficial abra- is otherwise unhelpful in the early stages of infection.
sion, laceration or penetrating wound), but this may However, a few weeks later there may be features of
have been so trivial as to pass unnoticed. A few hours osteomyelitis or septic arthritis, and later still of bone
or days later the finger or hand becomes painful and necrosis.
swollen. There may be throbbing and sometimes the If pus becomes available, this should be sent for
patient feels ill and feverish. Ask if he or she can recall bacteriological examination.
any causative incident: a small cut or superficial abra-
sion, a prick injury (including plant thorns) or a local
Diagnosis
In making the diagnosis, several conditions must be
excluded: an insect bite or sting (which can closely mimic
a subcutaneous infection), a thorn prick (which, itself,
can become secondarily infected), acute tendon rupture
(which may resemble a septic tenosynovitis) and acute
gout (which is easily mistaken for septic arthritis).
(a)
Plant-thorn injuries are extremely common and the
distinction between secondary infection and a non-
septic reaction to a retained fragment can be difficult.
Rose thorn and blackthorn are the usual suspects in
the UK, but any plant spine (including cactus needles)
can be implicated. The local inflammatory response
(b) (c) sometimes leads to recurrent arthritis or tenosynovi-
tis, which is arrested only by removing the retained
16.25 Acute infections (2) (a) Septic arthritis of the fragment. If the condition is suspected, the fragment
terminal interphalangeal joint following a cortisone
injection. (b) Infected insect ‘bite’. (c) Septic human bite may be revealed by ultrasound scanning or MRI. Sec-
resulting in acute infection of the fourth metacarpo- ondary infection with unusual soil or plant organisms
430 phalangeal joint. (Courtesy of Professor S. Biddulph.) may occur.
Principles of treatment 16
Superficial hand infections are common; if their treat-
ment is delayed or inadequate, infection may rapidly
extend, with serious consequences. The essentials of
treatment are:
The hand
• antibiotics
• rest, splintage and elevation
• drainage
• rehabilitation.
Antibiotics As soon as the clinical diagnosis is made,
and preferably after a specimen has been taken for
Gram stain and culture, antibiotic treatment is started
– usually with flucloxacillin or a cephalosporin. If
bone infection is suspected, fusidic acid may be added.
For bites (which should always be assumed to be 16.27 Infections The incisions for surgical drainage are
infected) a broad-spectrum penicillin is advisable. shown here: a, pulp space (directly over the abscess);
Agricultural injuries risk infection by anaerobic organ- b, nail-fold (it may also be necessary to excise the edge of
isms and it is therefore prudent to add metronidazole. the nail); c, tendon sheath; d, web space; e, thenar space;
The interim antibiotic may later be changed when the f, mid-palmar space.
bacterial sensitivity is known.
bandage can spread the sepsis. The incision should be
Rest, splintage and elevation In a mild case the hand is
planned to give access to the abscess without causing
rested in a sling. In a severe case the patient is admit-
injury to other structures but never at right angles
ted to hospital; the arm is held elevated in an overhead
across a skin crease. When pus is encountered it must
sling while the patient is kept under observation.
be carefully wiped away and a search made for deeper
Analgesics are given for pain. The hand must be
pockets of infection. Necrotic tissue should be excised.
splinted in the position of safe immobilization with the
The area is thoroughly washed out and, in some cases,
wrist slightly extended, the MCP joints in full flexion,
a catheter may be left in place for further, postopera-
the IP joints extended and the thumb in abduction.
tive, irrigation (e.g. in cases of flexor tenosynovitis).
Drainage If treated within the first 24–48 hours, The wound is either left open or lightly sutured, and is
many hand infections will respond to antibiotics, rest, then covered with a non-stick dressing and gauze. The
elevation and splintage. pus obtained is sent for culture.
If there are signs of an abscess – throbbing pain, At the end of the operation the hand is splinted in
marked tenderness and toxaemia – the pus should be the position of safe immobilization. A removable
drained. A tourniquet and either general or regional splint will permit repeated wound dressings and exer-
block anaesthesia are essential. The hand should be cises. A sling is used to keep the arm elevated.
exsanguinated by elevation only; an exsanguinating The hand should be re-examined within the next
(a) (b)
16.26 The position of safe immobilization The knuckle joints are 90º flexed, the finger joints extended and the thumb
abducted. This is the position in which the ligaments are at their longest and splintage is least likely to result in stiffness. 431
16 24 hours to ensure that drainage is effective; if it is
not, further operative drainage may be needed. Inad-
equate drainage of acute infection may lead to chronic
infection.
Postoperative rehabilitation As soon as the signs of
acute inflammation have settled, movements must be

started under the guidance of a hand therapist, other-
wise the joints are liable to become stiff. For the first
few days the resting splint is re-applied between exer-
cise sessions.
NAIL-FOLD INFECTION (PARONYCHIA) (a) (b)
Infection under the nail-fold is the commonest hand

infection; it is seen most often in children, or in older
people after rough nail-trimming. The edge of the
nail-fold becomes red and swollen and increasingly
tender. A tiny abscess may form in the nail-fold; if this
is left untreated, pus can spread under the nail.
At the first sign of infection, treatment with antibi-
otics alone may be effective. However, if pus is pres-
ent it must be released by an incision at the corner of
the nail-fold in line with the edge of the nail; a pled-
(c)
get of paraffin gauze is used to keep the nail-fold
open. If pus has spread under the nail, part or all of 16.28 Acute infections (a) Flexor tenosynovitis of the
the nail may need to be removed. middle finger following a cortisone injection.
(b) Tuberculous synovitis of flexor pollicis longus.
Chronic paronychia Chronic nail-fold infection may be (c) Diffuse septic extensor tendinitis. (Courtesy of Professor
due to (1) inadequate drainage of an acute infection, S. Biddulph.)
or (2) a fungal infection, which requires specific treat-
ment. Topical or oral antifungal agents are used to
to the bone, the joint or the flexor tendon sheath.
eradicate fungal infection, but failing this, or for
Postoperatively the finger is dressed with a loose
chronic bacterial infection, the nail bed may have to
packing of gauze; antibiotic treatment is modified if
be laid open (‘marsupialized’); care should be taken to
the results of culture and sensitivity so dictate, and is
avoid damaging the germinal nail matrix.
continued until all signs of infection have cleared. The
wound will gradually heal by secondary intention.
PULP INFECTION (FELON) Herpetic whitlow The herpes simplex virus may enter
the finger-tip, possibly by auto-inoculation from the
The distal finger pad is essentially a closed fascial com- patient’s own mouth or genitalia, or by cross infection
partment filled with compact fat and subdivided by during dental surgery. Small vesicles form on the fin-
radiating fibrous septa. A rise in pressure within the ger-tip, then coalesce and ulcerate. The condition is
pulp space causes intense pain and, if unrelieved, may self-limiting and usually subsides after about 10 days,
threaten the terminal branches of the digital artery but may recur from time to time. Herpes whitlow
which supply most of the terminal phalanx. should not be confused with a staphylococcal felon.
Pulp-space infection is usually caused by a prick Surgery is unhelpful and may be harmful, exposing
injury; blackthorn injuries are particularly likely to the finger to secondary infection. Aciclovir may be
become infected. The most common organism is effective in the early stages.
Staphylococcus aureus. The patient complains of throb-
bing pain in the finger-tip, which becomes tensely
swollen, red and acutely tender. OTHER SUBCUTANEOUS INFECTIONS
If the condition is recognized very early, antibiotic
treatment and elevation of the hand may suffice. Once Anywhere in the hand a blister, a superficial cut or an
an abscess has formed, the pus must be released insect ‘bite’ may become infected, causing redness,
through a small incision over the site of maximum ten- swelling and tenderness. A local collection of pus
432 derness. If treatment is delayed, infection may spread should be drained through a small incision over the
site of maximal tenderness (but never crossing a skin then irrigated (always from proximal to distal) with 16
crease or the web edge); in the finger, a mid-lateral Ringer’s lactate solution. Additional, proximal, inci-
incision is suitable. It is important to exclude a deeper sions may be needed if the synovial bursae are infected.
pocket of pus in a nearby tendon sheath or in one of Postoperatively the hand is swathed in absorbent
the deep fascial spaces. dressings and splinted in the position of safe immobi-
lization. The dressings should not be too bulky, as this
The hand
will make it difficult to ensure correct positioning of
TENDON SHEATH INFECTION the joints. The flexor sheath catheter is left in place;
using a syringe, the sheath is irrigated with 20 mL of
(SUPPURATIVE TENOSYNOVITIS) saline three or four times a day for the next 2 days.
The tendon sheath is a closed compartment extending The catheter and dressings are then removed and fin-
from the distal palmar crease to the DIP joint. In the ger movements are started.
thumb and fifth finger, the sheaths are co-extensive Stiffness is a very real risk and so early supervised
with the radial and ulnar bursae, which envelop the hand therapy must be arranged.
flexor tendons in the proximal part of the palm and
across the wrist; these bursae also communicate with
Parona’s space in the lower forearm. DEEP FASCIAL SPACE INFECTION
Pyogenic tenosynovitis is uncommon but danger-
ous. It usually follows a penetrating injury, the com- The large thenar and mid-palmar fascial spaces may be
monest organism being Staphylococcus aureus; infected directly by penetrating injuries or by second-
however, streptococcus and Gram-negative organisms ary spread from a web space or an infected tendon
are also encountered. sheath.
The affected digit is painful and swollen; it is usu- Clinical signs can be misleading; the hand is painful
ally held in slight flexion, is very tender, and the but, because of the tight deep fascia, there may be lit-
patient will not move it or permit it to be moved. tle or no swelling in the palm while the dorsum bulges
Early diagnosis is based on clinical findings; x-rays are like an inflated glove. There is extensive tenderness
unhelpful but ultrasound scanning may be useful. and the patient holds the hand as still as possible.
Delayed diagnosis results in a progressive rise in
Treatment As with other infections, splintage and
pressure within the sheath and a consequent risk of
intravenous antibiotics are commenced as soon as the
vascular occlusion and tendon necrosis. In neglected
diagnosis is made. For drainage, an incision is made
cases infection may spread proximally within the radial
directly over the abscess (being careful not to cross the
or ulnar bursa, or from one to the other (a ‘horse-shoe’
flexor creases) and sinus forceps inserted; if the web
abscess); it can also spread proximally to the flexor
space is infected it, too, should be incised. A thenar
compartment at the wrist and into Parona’s space in
space abscess can be approached through the first web
the forearm. Occasionally this results in median nerve
space (but do not incise in the line of the skin-fold) or
compression.
through separate dorsal and palmar incisions around
the thenar eminence. Great care must be taken to avoid
damage to the tendons, nerves and blood vessels. A
KANAVEL’S SIGNS OF FLEXOR SHEATH
thorough knowledge of anatomy is essential. The deep
INFECTION mid-palmar space (which lies between the flexor ten-
dons and the metacarpals) can be drained through an
Flexed posture of digit
incision in the web space between the middle and ring
Tenderness along the course of the tendon fingers, but wider exposure through a transverse or
oblique palmar incision is preferable, taking care not to
Pain on passive finger extension
cross the flexor creases directly. Above all, do not be
Pain on active flexion misled by the swelling on the back of the hand into
attempting drainage through the dorsal aspect.
Occasionally, deep infection extends proximally
Treatment Treatment must be started as soon as the across the wrist, causing symptoms of median nerve
diagnosis is suspected. The hand is elevated and compression. Pus can be drained by anteromedial or
splinted and antibiotics are administered intravenously anterolateral approaches; incisions directly over the
– ideally a broad-spectrum penicillin or a systemic flexor tendons and median nerve are avoided.
cephalosporin. If there is no improvement after 24 Operation wounds are either loosely stitched or left
hours, surgical drainage is essential. Two incisions are open. Bulky dressings and saline irrigation are
needed, one at the proximal end of the sheath and one employed, more or less as described for tendon sheath
at the distal end; using a fine catheter, the sheath is infections. 433
16 SEPTIC ARTHRITIS
Any of the MCP or finger joints may be infected,
either directly by a penetrating injury or intra-articu-
lar injection, or indirectly from adjacent structures
(and occasionally by haematogenous spread from a
distant site). Staphylococcus and Streptococcus are the

usual organisms; Haemophilus influenzae is a common
pathogen in children. A ‘fight-bite’ is a common cause
of infection of the MCP joints (see Fig. 16.25).
Pain, swelling and redness are localized to a single
joint, and all movement is resisted. The presence of
lymphangitis and/or systemic features may help to 16.29 Mycobacterium marinum Infection in an
clinch the diagnosis; in their absence, the early symp- aquarium keeper.
toms and signs are indistinguishable from those of
acute gout. Joint aspiration may give the answer.
apparatus and entered the MCP joint; this will not be
Treatment Intravenous antibiotics are administered
apparent if the wound is examined with the fingers in
and the hand is splinted. If the inflammation does not
extension because the extensor hood and capsule will
subside within 24 hours, or if there are overt signs of
have retracted proximally.
pus, open drainage is needed. Dorso-ulnar or dorso-
X-rays should be obtained (to exclude a fracture,
radial incisons between the collateral ligaments and
tooth fragment or foreign body) and swabs taken for
extensors are recommended for the finger IP joints;
bacterial culture and sensitivity.
for the MCP joints, mid-dorsal incision is needed.
The capsule is closed with a soluble suture but the
skin wounds are left open, to heal by secondary inten- Treatment
tion. Copious dressings are applied and the hand is
Fresh wounds should be carefully examined in the
splinted in the ‘position of safety’ for 48 hours; there-
operating theatre and, if necessary, extended and
after, movement is encouraged.
debrided. Search for a fragment of tooth or – with a
Intravenous antibiotics are continued until all signs
knuckle bite – for a divot of articular cartilage from
of sepsis have disappeared; it is prudent to follow this
the joint. The hand is splinted and elevated and
with another 2-week course of oral antibiotics.
antibiotics are given prophylactically until the labora-
tory results are obtained.
Infected bites will need debridement, wash-outs
BITES and intravenous antibiotic treatment. The common
infecting organisms are all sensitive to broad-spec-
ANIMAL BITES trum penicillins (e.g. amoxicillin with clavulanic acid)
Animal bites are usually inflicted by cats, dogs, farm and cephalosporins. With animal bites one should also
animals or rodents. Many become infected and, consider the possibility of rabies.
although the common pathogens are staphylococci Postoperative treatment consists, as usual, of copi-
and streptococci, unusual organisms like Pasteurella ous wound dressings, splintage in the ‘safe’ position
multocida are often reported. and encouragement of movement once the infection
has resolved. Tendon lacerations can be dealt with
HUMAN BITES when the tissues are completely healed.
Human bites are generally thought to be even more
prone to infection. A wide variety of organisms
(including anaerobes) are encountered, the common- MYCOBACTERIAL INFECTIONS
est being Staphylococcus aureus, Streptococcus Group A
and Eikenella corrodens. Tuberculous tenosynovitis is uncommon even in coun-
Bites can involve any part of the hand, fingers or tries where tuberculosis is still rife. The diagnosis
thumb; tell-tale signs of a human bite are lacerations should be considered in patients with chronic synovi-
on both volar and dorsal surfaces of the finger. Often, tis once the alternatives such as rheumatoid disease
though, the ‘bite’ consists only of a dorsal wound have been excluded; it can be confirmed by synovial
over one of the MCP knuckles, sustained during a fist- biopsy. Treatment is by synovectomy and then pro-
fight. All such wounds should be assumed to be infected. longed chemotherapy.
Moreover, it should be remembered that a laceration ‘Fishmonger’s infection’ is a chronic infection of
434 of the clenched fist may have penetrated the extensor the hand caused by Mycobacterium marinum. The
organism is introduced by prick-injuries from fish RAYNAUD’S DISEASE 16
spines or hard fins in people working with fish or
around fishing boats. It may appear as no more than Raynaud’s syndrome is produced by a vasospastic dis-
a superficial granuloma, but deep infection can give order which affects mainly the hands and fingers.
rise to an intractable synovitis of tendon or joint. Attacks are usually precipitated by cold; the fingers go
Other causes of chronic synovitis must be excluded; pale and icy, then dusky blue (or cyanotic) and finally
The hand
definitive diagnosis usually requires biopsy for histo- red. Between attacks the hands look normal. The con-
logical examination and special culture. dition is most commonly seen in young women who
Superficial lesions often heal on their own; if not, have no underlying or predisposing disease.
they can be excised. Deep lesions usually require sur- Raynaud’s phenomenon is the term applied when
gical synovectomy. Prolonged antibiotic treatment these changes are associated with an underlying dis-
is needed to avoid recurrence; the recommended ease such as scleroderma or arteriosclerosis. Similar,
drug is a broad-spectrum tetracycline such as minocy- though milder, changes are also seen in thoracic out-
cline, or else chemotherapy with ethambutol and let syndrome. The hands must be kept warm. Calcium
rifampicin. channel blockade, iloprost infusions or digital sympa-
thectomy (surgical removal of the sympathetic plexus
around the digital arteries) may be needed.
FUNGAL INFECTIONS
Superficial tinea infection of the palm and interdigital HAND–ARM VIBRATION SYNDROME
clefts (similar to ‘athlete’s foot’) is fairly common and
can be controlled by topical preparations. Tinea of the Excessive and prolonged use of vibrating tools can
nails can be more difficult to eradicate and may damage the nerves and vessels in the fingers. The
require oral antifungal medication and complete damage is proportional to the duration of exposure
removal of the nail. and amount of vibration. There are two components:
Subcutaneous infection by Sporothrix schenckii vascular and neurological. The vascular component is
(sporotrichosis) is rarely seen in the UK but is not similar to Raynaud’s phenomenon, with the finger-
uncommon in North America, where it is usually tips turning white in cold weather, then changing
caused by a thorn prick. Chronic ulceration at the through blue and red as the circulation is restored.
prick site, unresponsive to antibiotic treatment, may The neurological component involves numbness and
suggest the diagnosis, which can be confirmed by tingling in the finger-tips. In advanced cases there can
microbiological culture. The recommended treatment be reduced dexterity. Some patients have clear carpal
is oral potassium iodide. tunnel syndrome as well.
Deep mycotic infection may involve tendons or Treatment is generally unsatisfactory, but includes
joints. The diagnosis should be confirmed by avoidance of cold weather and smoking as well as, of
microscopy and microbiological culture. Treatment is course, vibrating tools. Carpal tunnel syndrome asso-
by local excision and administration of an intravenous ciated with vibration, in the absence of a more diffuse
antifungal agent. Resistant cases occasionally require neuropathy, responds fairly well to standard decom-
limited amputation. pression.
Opportunistic fungal infections are more likely to
occur in debilitated and immunosuppressed patients. ULNAR ARTERY THROMBOSIS
Repeated blows to the hand, especially using the
hypothenar eminence as a hammer, can damage the
intima of the ulnar artery, leading to either thrombo-
VASCULAR DISORDERS OF THE sis or an aneurysm. The patient presents with cold
intolerance in the little finger. Microvascular recon-
HAND struction of the ulnar artery is needed.
EMBOLI
Arising from the heart or from aneurysms in the arter- OTHER GENERAL DISORDERS
ies of the upper limb, emboli can lodge in distal ves-
sels causing splinter haemorrhages, or in larger, more A number of generalized disorders should always be
proximal vessels, causing ischaemia of the arm. A large borne in mind when considering the diagnosis of any
embolus leads to the classic signs of pain, pulseless- unusual lesion that appears to be confined to the hand.
ness, paraesthesia, pallor and paralysis. Untreated, It is beyond the scope of this book to enlarge on these
gangrene or ischaemic contracture ensues. conditions. The few examples shown in Figure 16.30 435
16
(a) (b) (c) (d)
(e) (f) (g)
16.30 The hand in general disorders Some general conditions

that may manifest with lesions in the hand: (a) scleroderma;
(b,c) gouty tophi; (d) psoriasis; (e) implantation dermoid;
(f) dermatofibroma; (g) Maffucci’s syndrome; (h) Secretan’s
syndrome (hard odema due to repetitive trauma, often self-
inflicted).
(h)
serve merely as a reminder that a general history and painless, stable wrist. Spreading the fingers produces
examination are as important as focussed attention on abduction to either side of the middle finger; bringing
the hand. them together, adduction. Abduction and adduction
of the thumb occur in a plane at right angles to the
palm (i.e. with the hand lying palm upwards, abduc-
tion points the thumb to the ceiling). By a combina-
NOTES ON APPLIED ANATOMY tion of movements the thumb can also be opposed to
each of the other fingers. Functionally, the thumb is
FUNCTION 40 per cent of the hand.
The hand serves three basic functions: sensory percep-
tion, precise manipulation and power grip. The first SKIN
two involve the thumb, index and middle fingers; The palmar skin is relatively tight and inelastic; skin
without normal sensation and the ability to oppose loss can be ill-afforded and wounds sutured under
these three digits, manipulative precision will be lost. tension are liable to break down. The acute sensibility
The ring and little fingers provide power grip, for of the digital palmar skin cannot be achieved by any
which they need full flexion though sensation is less skin graft. Although the dorsal skin seems lax and
important. mobile with the fingers extended, flexion will show
With the wrist flexed the fingers and thumb fall nat- that there is very little spare skin. Loss of skin there-
urally into extension. With the wrist extended the fin- fore often requires a graft or flap.
gers curl into flexion and the tips of the thumb, index Just deep to the palmar skin is the palmar aponeu-
and middle fingers form a functional tripod; this is the rosis, the embryological remnant of a superficial layer
position of function, because it is best suited to the of finger flexors; attachment to the bases of the prox-
actions of prehension. imal phalanges explains part of the deformity of
Finger flexion is strongest when the wrist is power- Dupuytren’s contracture. Incisions on the palmar sur-
436 fully extended; normal grasp is possible only with a face are also liable to contracture unless they are
16.31 Three positions 16
of the hand (a) The
position of relaxation,
(b) the position of
function (ready for action),
(c) the position of safe
immobilization, with the
The hand
(a) (b) (c) ligaments taut.
placed in the line of the skin creases, along the mid- they traverse the fingers; starting at the MCP joints
lateral borders of the fingers or obliquely across the (level with the distal palmar crease) they extend to the
creases. DIP joints. They serve as runners and pulleys, so pre-
venting the tendons from bowstringing during flex-
JOINTS ion. Scarring within the fibro-osseous tunnel prevents
The carpo-metacarpal joints The second and third normal excursion.
metacarpals have very little independent movement; The long extensor tendons are prevented from bow-
the fourth and fifth have more, allowing greater clo- stringing at the wrist by the extensor retinaculum;
sure of the ulnar part of the hand during power grip. here they are liable to frictional trauma. Over the
The metacarpal of the thumb is the most mobile and MCP joints each extensor tendon widens into an
the first CMC joint is a frequent target for degenera- expansion which inserts into the proximal phalanx and
tive arthritis. then splits in three; a central slip inserts into the mid-
dle phalanx, the two lateral slips continue distally, join
The metacarpo-phalangeal joints These flex to about 90
and end in the distal phalanx. Division of the middle
degrees. The range of extension increases progres-
slip causes a flexion deformity of the PIP joint (bou-
sively from the index to the little finger. The collateral
tonnière); rupture of the distal conjoined slip causes
ligaments are lax in extension (permitting abduction)
flexion deformity of the DIP joint (mallet finger).
and tight in flexion (preventing abduction). If these
joints are immobilized they should always be in flexion,
NERVES
so that the ligaments are at full stretch and therefore less
The median nerve supplies the abductor pollicis brevis,
likely to shorten if they should fibrose.
opponens pollicis and lumbricals to the middle and
The interphalangeal joints The IP joints are simple index fingers; it also innervates the palmar skin of the
hinges, each flexing to about 90 degrees. Their collat- thumb, index and middle fingers and the radial half of
eral ligaments send attachments to the volar plate and the ring finger.
these fibres are tight in extension and lax in flexion; The ulnar nerve supplies the hypothenar muscles,
immobilization of the IP joints, therefore, should always all the interossei, lumbricals to the little and ring fin-
be in extension. gers, flexor pollicis brevis and adductor pollicis. Sen-
sory branches innervate the palmar and dorsal skin of
the little finger and the ulnar half of the ring finger.
MUSCLES AND TENDONS
The radial nerve supplies skin over the dorsoradial
Two sets of muscles control finger movements: the
aspect of the hand.
long extrinsic muscles (extensors, deep flexors and
superficial flexors), and the short intrinsic muscles
(interossei, lumbricals and the short thenar and
hypothenar muscles). The extrinsics extend the MCP REFERENCES AND FURTHER READING
joints (long extensors) and flex the IP joints (long
flexors). The intrinsics flex the MCP and extend the Warwick D, Dunn R, Melikyan E, Vadher J. Hand Sur-
IP joints; the dorsal interossei also abduct and the pal- gery 2009: Oxford University Press, Oxford.
mar interossei adduct the fingers from the axis of the Green DP, Hotchkiss RN, Pederson WC, Wolfe SW.
middle finger. Spasm or contracture of the intrinsics Green’s Operative Hand Surgery, 5th Edition. Elsevier,
causes the intrinsic-plus posture – flexion at the MCP London.
joints, extension at the IP joints and adduction of the Mobergh E. Objective methods for determining the func-
thumb. Paralysis of the intrinsics produces the intrin- tional value of sensitivity in the hand. Journal of Bone and
sic-minus posture – hyperextension of the MCP and Joint Surgery 1958; 40B: 454–76.
flexion of the IP joints (‘claw hand’). Smith P. The Hand, Diagnosis and Indications. 4th Edition.
Tough fibrous sheaths enclose the flexor tendons as Churchill Livingstone, Edinburgh. 437
The neck
17
Stephen Eisenstein, Louis Solomon
intracranial lesions and disorders of the eyes or semi-

CLINICAL ASSESSMENT circular canals. Neck stiffness is usually fairly obvious.
SYMPTOMS Feel
Pain is felt in the neck itself, but it may also be The front of the neck is most easily palpated with the
referred to the shoulders or arms. If it starts suddenly patient seated and the examiner standing behind him
after exertion, and is exaggerated by coughing or or her. The best way to feel the back of the neck is
straining, think of a disc prolapse. Pain spreading with the patient lying prone and resting his or her
down an arm and forearm with paraesthesiae in the head over a pillow; this way he or she can relax and
hand will strengthen the likelihood of a disc prolapse the bony structures are more easily palpated. Feel for
with cervical root compression. Chronic or recurrent tender spots or lumps and note if the paravertebral
pain in older people is usually due to chronic disc muscles are in spasm.
degeneration and spondylosis. Always enquire if any
posture or movement makes it worse; or better. Move
Stiffness may be either intermittent or continuous.
Sometimes it is so severe that the patient can scarcely Forward flexion, extension, lateral flexion and rota-
move the head. tion are tested, and then shoulder movements. Range
Deformity usually appears as a wry neck; occasion- of motion normally diminishes with age, but even
ally the neck is fixed in flexion. then movement should be smooth and pain-free.
Numbness, tingling and weakness in the upper limbs While testing for both active and passive movements,
may be due to pressure on a nerve root; weakness in ask whether any motion is painful; this could be sug-
the lower limbs may result from cord compression in gestive of cervical intervertebral disc degeneration.
the neck. Movement-induced pain or paraesthesia down the arm
Headache sometimes emanates from the neck, espe- is particularly noteworthy. In Spurling’s test the
cially occipital headache, but if this is the only symp- patient is instructed to rotate the neck to one side with
tom other causes should be suspected. the chin elevated: if ipsilateral upper limb pain and
‘Tension’ is often mentioned as a cause of neck pain paraesthesiae are reproduced, that would increase the
and occipital headache. The neck and back are com- suspicion of a disc prolapse with cervical root com-
mon ‘target zones’ for psychosomatic illness. pression. Pain may be relieved by having the patient
place the arm overhead (the abduction relief sign).
Tests for arterial compression

SIGNS
If the thoracic outlet is tight, the radial pulse may dis-
No examination of the neck is complete without appear if, when the patient holds a deep breath, the
examination of the upper trunk, both upper limbs, neck is turned towards the affected side and extended
and shoulder joints. (Adson’s test), or if the shoulder is elevated and exter-
nally rotated (Wright’s test).
Look
Any deformity is noted. Wry neck, due to muscle
Neurological examination
spasm, may suggest a disc lesion, an inflammatory dis- Neurological examination of the upper limbs is
order or cervical spine injury, but it also occurs with mandatory in all cases; in some the lower limbs also
17 17.1 Examination
(a) Look for any
deformity or
superficial blemish
which might suggest a
disorder affecting the
cervical spine. (b) The
front of the neck is felt

with the patient
(a) (b) (c) seated and the
examiner standing
behind him. (c) The
back of the neck is
most easily and
reliably felt with the
patient lying prone
over a pillow; this way
muscle spasm is
reduced and the neck
(d) (e) (f) (g) is relaxed.
(d-g) Movement:
flexion (‘chin on
chest’); extension
(‘look up at the
ceiling’); lateral flexion
(‘tilt your ear towards
your shoulder’)’ and
rotation (‘look over
your shoulder’).
(h,i) Neurological
examination is
mandatory.
(h) (i)
IMAGING
X-RAYS
The standard radiographic series for the cervical spine
comprises anteroposterior, lateral and open-mouth
views. Additional lateral views with the neck in flexion
and extension should be obtained provided there is no
history of recent neck injury.
The anteroposterior view should show the regular,
undulating outline of the lateral masses; their symme-
try may be disturbed by destructive lesions or frac-
tures. A projection through the mouth is required to
show the upper two vertebrae.
When looking at the lateral view, make sure that all
seven vertebrae can be seen; patients have been paral-
17.2 Normal range of movement Flexion and extension ysed, and some have lost their lives, because a frac-
of the neck are best gauged by observing the angle of the ture-dislocation at C6/7 or C7/T1 was missed. The
occipitomental line – an imaginary line joining the tip of
the chin and the occipital protuberance. In full flexion, the
normal cervical lordotic curve shows four parallel
chin normally touches the chest; in full extension, the lines: one along the anterior surfaces of the vertebral
occipitomental line forms an angle of at least 45° with the bodies, one along their posterior surfaces, one along
horizontal, and more than 60° in young people. Lateral the posterior borders of the lateral masses and one
flexion is usually achieved up to 45° and rotation to 80° along the bases of the spinous processes; any
each way.
malalignment suggests subluxation. The disc spaces
are inspected; loss of disc height and the presence of
should be examined. Muscle power, reflexes and sen- osteophytic spurs at the margins of adjacent vertebral
sation should be carefully tested; even small degrees of bodies suggest chronic intervertebral disc degenera-
440 abnormality may be significant. tion. The posterior interspinous spaces are compared;
17
The neck
(a) (b) (c)
17.3 Imaging – normal x-rays (a) Anteroposterior view – note the smooth, symmetrical outlines and the clear, wide
uncovertebral joints (arrows). (b) Open mouth view – to show the odontoid process and atlanto-axial joints. (c) Lateral view
– showing all seven cervical vertebrae.
if one is wider than the rest, this may signify chronic However, this is an invasive investigation and fairly
instability of that segment, possibly due to a previ- non-specific. Its usefulness is enhanced by performing
ously undiagnosed subluxation. Flexion and extension a post-contrast CT scan.
views may be needed to demonstrate instability,
though after an acute injury this is best avoided! MRI SCAN
Children’s x-rays present special problems. Because This is non-invasive, does not expose the patient to
the ligaments are relatively lax and the bones incom- radiation and provides excellent resolution of the
pletely ossified, flexion views may show unexpectedly intervertebral disc and neural structures. It is the
large shifts between adjacent vertebrae; this is some- most sensitive method of demonstrating tumours and
times mistaken for abnormal subluxation. Thus, dur- infection. It provides information on the size of the
ing flexion, the lateral x-ray may show an spinal canal and neural foramina. Its sensitivity can be
atlanto-dental interval of 4 or 5 mm (which in an adult a drawback: 20 per cent of asymptomatic patients
would suggest rupture of the transverse ligament), or show significant abnormalities and the scans must
anterior ‘subluxation’ at C2/3. Note also that the therefore be interpreted alongside the clinical assess-
retropharyngeal space between the cervical spine and ment.
pharynx at the level of C3 increases markedly on
forced expiration (e.g. when crying) and this can be
misinterpreted as a soft-tissue mass. Another error is to 17.4 Magnetic resonance
mistake the normal synchondrosis between the dens imaging MRI of the lower
and the body of C2 (which only fuses at about 6 years) cervical and upper thoracic
for an odontoid fracture. Finally, remember that nor- spine, showing metastatic
mal-looking radiographs in children do not exclude deposits (dark grey areas in
this TI-weighted image) in
the possibility of a spinal cord injury. several vertebral bodies. The
large tumour deposit at T2/3
CT SCAN is encroaching perilously on
In the cervical spine, CT is particularly helpful for the spinal canal.
demonstrating the shape and size of the spinal canal
and intervertebral foramina, as well as the integrity of
the bony structures.
MYELOGRAPHY
Changes in the contour of the contrast-filled thecal
sac suggest intradural and extradural compression. 441
17 DEFORMITIES OF THE NECK IN
sternomastoid on that side may feel tight and hard.
There may also be asymmetrical development of the
CHILDREN face (plagiocephaly). These features become increas-
ingly obvious as the child grows.
A variety of deformities are encountered, some Other causes of wry neck (bony anomalies, discitis,
reflecting postural adjustments to underlying disor- lymphadenitis) should be excluded. The history and
ders and others due to developmental anomalies. the typical facial appearance are helpful clues. Radi-
ographs must be taken to exclude a bone abnormality
or fracture.
TORTICOLLIS Treatment If the diagnosis is made during infancy,
This is a description rather than a diagnosis. The chin daily muscle stretching by the parents may prevent the
is twisted upwards and towards one side. There are incipient deformity. Non-operative treatment is suc-
many causes. The condition may be either congenital cessful in most cases. If the condition persists beyond
or acquired. one year, operative correction is required to avoid
progressive facial deformity. The contracted muscle is
divided (usually at its lower end but sometimes at the
Infantile (congenital) torticollis upper end or at both ends) and the head is manipu-
This condition is common. The sternomastoid muscle lated into the neutral position. After operation, cor-
on one side is fibrous and fails to elongate as the child rection must be maintained, with a temporary rigid
grows; consequently, progressive deformity develops. orthosis followed by stretching exercises.
The cause is unknown; the muscle may have suffered
ischaemia from a distorted position in utero (the asso- Secondary torticollis
ciation with breech presentation and hip dysplasia is
Childhood torticollis may be secondary to congenital
supporting evidence), or it may have been injured at
bone anomalies, atlanto-axial rotatory displacement,
birth.
infection (lymphadenitis, retropharyngeal abscess,
A history of difficult labour or breech delivery is
tonsillitis, discitis, tuberculosis), trauma, juvenile
common. A lump may be noticed in the first few
rheumatoid arthritis, posterior fossa tumours,
weeks of life; it is well defined and involves one or
intraspinal tumours, dystonia (benign paroxysmal tor-
both heads of the sternomastoid. At this stage there is
ticollis) or ocular dysfunction.
neither deformity nor obvious limitation of move-
ment and within a few months the lump has disap- Atlanto-axial rotatory displacement The aetiology of
peared. Deformity does not become apparent until this condition is unclear, but it is thought to be due
the child is 1–2 years old. The head is tilted to one to muscle spasm resulting from inflammation of the
side, so that the ear approaches the shoulder; the ligaments, capsule and synovium of the atlanto-axial
17.5 Torticollis Natural history:

(a) sternomastoid tumour in a young
baby; (b) early wry neck; (c) deformity
with facial hemiatrophy in the
adolescent. Surgical treatment: (d) two
sites at which the sternomastoid may
be divided; (e,f) before and a few
months after operation.
(a) (b) (c)
442 (d) (e) (f)

region. There may be a history of trauma or upper 17
respiratory tract infection. The child presents with a
painful wry neck. Plain x-rays are difficult to interpret;
a CT scan in both neutral and maximum lateral rota-
tion is the most helpful investigation.
Most cases are mild and can be managed expec-
The neck
tantly with a soft collar and analgesics. If there is no
resolution after a week, halter traction, bed rest and
analgesics should be prescribed. In more resistant
cases, halo traction may be required. Occasionally
there is anterior displacement of C1 on C2; the artic-
ulation may not stabilize following traction and a
C1/2 fusion is then indicated.
VERTEBRAL ANOMALIES
There are many vertebral anomalies and most are very
rare. Three are described here.
KLIPPEL–FEIL SYNDROME (CERVICAL-

VERTEBRAL SYNOSTOSIS)
This developmental disorder represents a failure of
segmentation of the cervical somites; it is often asso-
ciated with abnormalities in the genito-urinary, nerv-
ous or cardiovascular systems. Some children have a
hearing impediment. 17.6 Klippel–Feil syndrome The short neck and
Children with synostosis have a characteristic vertebral anomalies in a typical patient.
appearance: the neck is short or non-existent and
there may be webbing; the hairline is low; and neck
movements are limited. About 1 in 3 children with
Klippel–Feil syndrome also has Sprengel’s deformity bone abnormality (associated with other bone defects
of the scapula. Scoliosis is present in about 60 per cent such as odontoid abnormalities, Morquio syndrome
and rib anomalies in about 30 per cent. Hand defor- and Klippel–Feil syndrome) or secondary to softening
mities such as syndactyly, thumb hypoplasia and extra of the bones (osteomalacia, rickets, rheumatoid
digits are often present. X-rays reveal fusion of two or arthritis, neurofibromatosis, etc.). The relationship
more cervical vertebrae. between the odontoid process and the foramen mag-
Symptoms tend to arise in the second or third num can be ascertained on plain radiographs; further
decades, not from the fused segments but from the information is acquired with CT or MRI. Patients
adjacent mobile segments. There may be pain due to may present – often in the second or third decade –
joint hypermobility, or neurological symptoms from with symptoms of raised intracranial pressure (because
instability. the aqueduct of Sylvius becomes blocked), weakness
Children with symptoms may need cervical fusion. and paraesthesia of the limbs (because of direct com-
For asymptomatic patients, treatment is unnecessary pression or ischaemia of the cord). Treatment involves
but parents should be warned of the risks of contact surgical decompression and stabilization.
sports; sudden catastrophic neurological compromise
can occur after minor trauma.
ODONTOID ANOMALIES
BASILAR IMPRESSION The odontoid may be absent or hypoplastic, or there
may be a separate ossicle (the os odontoideum). The
In this condition the floor of the skull is indented by anomaly should be suspected (and looked for even if
the upper cervical spine. The odontoid can impinge the child does not complain) in skeletal dysplasias
upon the brain-stem. The cause is either a primary which involve the spine. This is especially important in 443
17 patients undergoing operation; the atlanto-axial joint below the sixth cervical vertebra. In many cases (per-
may subluxate under anaesthesia. Some patients pres- haps in all) there is a predisposing abnormality of the
ent with pain or torticollis, or neurological complica- disc with increased nuclear tension. Prolapsed mate-
tions such as transient paralysis, myelopathy with rial may press on the posterior longitudinal ligament
upper motor neuron signs or sphincter disturbances. or dura mater, causing neck pain and stiffness as well
In the majority of cases the anomaly is discovered by as pain referred to the upper limb. Pressure on the
chance in a routine cervical spine x-ray following nerve roots causes paraesthesia, and sometimes weak-
trauma. Open-mouth radiographs show the abnor- ness, in one or both arms – usually in the distribution
mality; lateral flexion–extension views may show insta- of C6 or C7.
bility of the C1–C2 articulation.
Patients with symptoms should have surgical stabi-
Clinical features
lization; the prophylactic treatment of asymptomatic
patients is controversial. The original attack can sometimes be related to a spe-
cific strain episode, e.g. acute flexion of the neck dur-
ing intense physical exertion, or (occasionally) a
‘whiplash’ injury. Subsequent attacks may be sudden
or gradual in onset, and with trivial cause. The patient
ACUTE INTERVERTEBRAL DISC complains of: (a) pain and stiffness of the neck, the
PROLAPSE pain often radiating to the scapular region and some-
times to the occiput; and (b) pain and paraesthesia in
Acute disc prolapse is not as common in the neck as one upper limb (rarely both), often radiating to the
in the lower back; both segments of the spine are outer elbow, back of the wrist and the index and mid-
mobile but the mechanical environment in the cervi- dle fingers. Weakness is rare. Between attacks the
cal region is more favourable than that in the lum- patient feels well, although the neck may feel a bit
bosacral region. The pathological features are similar; stiff.
these are described in some detail in Chapter 18. The neck may be held tilted forwards and sideways.
The acute prolapse may be precipitated by local The muscles are tender and movements are restricted.
strain or injury, especially sudden unguarded flexion The arms should be examined for neurological deficit.
and rotation, and usually occurs immediately above or The C6 root innervates the biceps reflex, the biceps
17.7 Acute disc prolapse

(a,b) Acute wry neck due to a
prolapsed disc. (c) The
intervertebral disc space at C5/6
is reduced. (d) MRI in another
case showing a large disc
prolapse at C6/7.
(a)
444 (b) (c) (d)

muscle and wrist dorsiflexion, and sensation of the lat- 17
eral forearm, thumb and index finger; C7 innervates
the triceps and radial reflexes, the triceps muscle, wrist
flexors and finger extensors, and sensation in the mid-
dle finger. Rotation and tilting of the neck to the
affected side, combined with a Valsalva manoeuvre,
The neck
may provoke radicular symptoms.
Imaging
X-rays may reveal straightening out of the normal cer-
vical lordosis (due to muscle spasm) and narrowing of
the disc space (although this is unlikely during a first
attack). The most useful form of imaging is MRI, (a) (b)
which will show the disc and its relationship to the 17.8 Cervical disc prolapse – treatment (a,b) Operative
nerve root in most cases. Even more accurate, but not treatment usually consists of anterior disc removal and
used routinely because it involves intrathecal injection bone grafting. In this case the intervertebral disc height at
of contrast medium, is CT myelography. C5/6 has been restored but now, some years later, there
are signs of disc degeneration above and below the fused
segment.
Acute soft-tissue strain Acute strains of the neck are
often associated with pain, stiffness and vague ‘tin- fitting under the chin and occiput. Traction is applied
gling’ in the upper limbs. It is important to bear in intermittently for no more than 30 minutes at a time.
mind that pain radiating into the arm is not necessar-
ily due to nerve root pressure. Remove If symptoms are refractory and severe
enough, if there is a progressive neurological deficit or
Neuralgic amyotrophy This condition can closely if there are signs of an acute myelopathy then surgery
resemble an acute disc prolapse and should always be is indicated. The disc may be removed through an
thought of if there is no definite history of a strain anterior approach; bone grafts are inserted to fuse the
episode. Pain is sudden and severe, and situated over affected segment and to restore the normal interver-
the shoulder rather than in the neck itself. Careful tebral height. If only one level is affected, and there is
examination will show that more than one neural level no bony encroachment on the intervertebral foramen,
is affected – an extremely rare event in disc prolapse. anterior decompression can be expected to give good
Pain is unrelenting and local
Cervical spine infections long-term relief from radicular symptoms.
spasm severe. X-rays show erosion of the vertebral
end-plates.
CERVICAL SPONDYLOSIS
Cervical tumours Neurological signs are progressive
and x-rays reveal bone destruction. This vague term is applied to a cluster of abnormali-
ties arising from chronic intervertebral disc degenera-
Rotator cuff lesions Although the distribution of pain
tion. Changes are most common in the lower two
may resemble that of a prolapsed cervical disc, ten-
segments of the cervical spine (C5/6 and C6/7), the
derness is localized to the rotator cuff and shoulder
area which is prone to intervertebral disc prolapse.
movements are abnormal.
The discs degenerate, flatten and become less elastic.
The facet joints and the uncovertebral joints are
Treatment slightly displaced and become arthritic, giving rise to
pain and stiffness in the neck. Bony spurs, ridges or
Heat and analgesics are soothing but, as with lumbar
bars appear at the anterior and posterior margins of
disc prolapse, there are only three satisfactory ways of
the vertebral bodies; those that develop posteriorly
treating the prolapse itself.
may encroach upon the spinal canal or the interverte-
Rest A collar will prevent unguarded movement; bral foramina, causing pressure on the dura (which is
However, it seldom needs to be worn for more than a pain sensitive) and the neural structures.
week or two.
Clinical features
Reduce Traction may enlarge the disc space, permit-
ting the prolapse to subside. The head of the couch is The patient, usually aged over 40, complains of neck
raised and weights (up to 8 kg) are tied to a harness pain and stiffness. The symptoms come on gradually 445
17 17.9 Cervical spondylosis –
x-rays (a) Degenerative features
at one level, C6/7. Note the
prominent ‘osteophytes’ at the
anterior and posterior borders of
these two vertebral bodies.
(b) Marked degenerative changes
at multiple levels.
(a) (b)
and are often worse on first getting up. The pain may middle-aged and elderly people who complain of
radiate widely: to the occiput, the back of the shoul- pain, and it is easy to persuade oneself that they are
ders and down one or both arms; it is sometimes the cause of the patient’s symptoms.
accompanied by paraesthesia, weakness and clumsi-
Nerve entrapment syndromes Median or ulnar nerve
ness in the arm and hand. Typically there are exacer-
entrapment may give rise to intermittent symptoms of
bations of more acute discomfort, and long periods of
pain and paraesthesia in the hand. Characteristically
relative quiescence.
the symptoms are worse at night or are posture
The appearance is normal, but the muscles at the
related. Careful examination will show that the
back of the neck and across the scapulae are tender.
changes follow a peripheral nerve rather than a root
Neck movements are limited and painful.
distribution. In doubtful cases, nerve conduction
Sometimes the clinical picture is dominated by fea-
studies and electromyography will help to establish
tures arising from narrowing of the intervertebral
the diagnosis. Remember, though, that the patient
foramina and compression of the nerve roots (radicu-
may have symptoms from both a peripheral and a cen-
lopathy): these include pain referred to the interscapu-
tral abnormality; indeed, there is some evidence to
lar area and upper limb, numbness and/or
suggest that longstanding cervical spondylosis may
paraesthesiae in the upper limb or the side of the face,
make the patient more vulnerable to the effects of
muscle weakness and depressed reflexes in the arm or
peripheral nerve entrapment.
hand. In advanced cases there may be narrowing of
the spinal canal and changes due to pressure on the Rotator cuff lesions Pain may resemble that of cervical
cord (myelopathy – see below). spondylosis, but shoulder movements are abnormal
and there may be x-ray and MRI features of rotator
Imaging X-rays show narrowing of one or more
cuff degeneration.
intervertebral spaces, with spur formation (or lipping)
at the anterior and posterior margins of the disc. Cervical tumours Metastatic deposits in the cervical
These bony ridges (often referred to as ‘osteophytes’) spine can cause misleading symptoms, but sooner or
may encroach upon the intervertebral foramina. MRI later bone destruction produces diagnostic x-ray
is more reliable for showing whether the nerve roots changes. With tumours of the spinal cord, nerve roots
are compressed. or lymph nodes, symptoms are usually continuous,
and the lesion may appear on imaging.
Thoracic outlet syndrome This condition is described
Diagnosis in Chapter 11. Symptoms resemble those of cervical
Other disorders associated with neck and/or arm pain spondylosis; pain and sensory abnormalities appear
and sensory symptoms must be excluded. Cervical mainly down the ulnar border of the forearm and may
spine ‘degenerative changes’ are so common after the be aggravated by upper limb traction or by elevation
446 age of 40 years that they are likely to be seen in most and external rotation of the shoulder. Importantly,
neck movements are neither painful nor restricted. (worse) the vertebral artery are unusual if sufficient 17
X-rays may reveal a cervical rib, although the mere care is exercised. Postoperative dysphagia and dyspho-
presence of this anomaly is not necessarily diagnostic. nia (particularly if a plate has been applied) have been
reported. Graft dislodgement and failed fusion (with
pseudarthrosis) are less likely with intervertebral plat-
Conservative treatment
ing. More worrying is the possibility that fusion at
The neck
Analgesics are prescribed when necessary. Heat and one level may predispose to degeneration at an adja-
massage are often soothing, but restricting neck cent level.
movements in a collar is the most effective treatment
during painful attacks. Physiotherapy is the mainstay FORAMINOTOMY
of treatment, patients usually being maintained in rel- If the main problems are referred pain in the upper
ative comfort by various measures including exercises, limb and/or neurological symptoms and signs (fea-
gentle passive manipulation and intermittent traction. tures of a radiculopathy) and the MRI shows forami-
Prolonged use of a cervical collar or brace may do nal narrowing and nerve root compression at one or
more harm than good. two levels, foraminotomy (through a posterior
approach) may be indicated. Only part of the facet
Operative treatment joint is removed so this segment should not become
unstable. However, patients should be warned that
If conservative measures fail to relieve the patient’s pre-existing neck pain may not be eliminated; and, of
symptoms, and particularly if there are neurological course, adjacent segments may go on to develop
symptoms and signs arising from nerve root compres- symptomatic disc degeneration in the future, which
sion at one or two identifiable levels, surgical treat- may then require further surgery.
ment may be preferable.
INTERVERTEBRAL DISC REPLACEMENT
ANTERIOR DISCECTOMY AND FUSION Disc replacement operations have recently been
This operation has a ‘track record’ of more than 25 approved in some countries. This has the (theoretical)
years and is particularly suitable if the problem is pri- advantage of removing the offending disc and preserv-
marily one of unrelieved neck pain and stiffness, ing movement at the affected site. As yet it is too early
though it is also successful in relieving radicular symp- to assess the long-term outcome of these procedures.
toms (Bohlman et al., 1993). Through the anterior
approach the intervertebral disc can be removed with-
out disturbing the posteriorly placed neurological
OSSIFICATION OF THE POSTERIOR
structures. After preparation of the intervertebral
space, a suitably shaped bone graft (usually autoge- LONGITUDINAL LIGAMENT
nous, taken from the iliac crest) is inserted firmly
between the adjacent vertebral bodies. An anterior Reports on ossification of the posterior longitudinal
plate is added if there is uncertainty about stability or ligament (OPLL) have appeared mainly from Japan
if several levels are being fused. Operative complica- (Ono et al., 1977; Tsuyama, 1984). However, it is
tions such as injury to the recurrent laryngeal nerve or now recognized that this condition is quite common
17.10 Ossification of the

posterior longitudinal
ligament (a) Lateral x-ray of the
cervical spine showing the thin
dense band running down the
backs of the vertebral bodies
(arrows); this appearance is
typical of posterior longitudinal
ligament ossification, which
resulted in cervical spinal stenosis.
(b) X-ray taken after posterior
spinal decompression
(laminoplasty); the spinous
processes have been removed,
the laminae split on one side of
the mid-line and the posterior
arch ‘jacked’ open. The sagittal
diameter of the spinal canal is
now considerably greater than
before. (Courtesy of Mr H. K.
(a) (b) Wong, Singapore.) 447
17 and widespread. It occurs mainly in the cervical spine and clumsiness in the arms and legs. Symptoms may
and may be associated with bone-forming conditions be precipitated by acutely hyperextending the neck,
such as diffuse idiopathic skeletal hyperostosis and some patients present for the first time after a
(DISH) and fluorosis. The cause is unknown. The sig- hyperextension injury. They may experience involun-
nificance of the disease is that it may give rise to spinal tary spasms in the legs and, occasionally, episodes of
stenosis and cervical myelopathy. spontaneous clonus. In severe cases there may be uri-
The patient, usually a man between 50 and 70 years nary and rectal dysfunction or incontinence.
of age, may present with any combination of pain in The ‘classical’ picture of weakness and spasticity in
the neck and upper limb(s), sensory symptoms and the legs and numbness in the hands is easy to recog-
muscle weakness in the arms and upper motor neuron nize, but the signs are not always as clear-cut as that.
(cord) symptoms and signs in the lower limbs. The However, careful examination should reveal upper
most disturbing features are motor abnormalities such motor neuron signs in the lower limbs (increased
as weakness, incoordination, clumsiness, muscle wast- muscle tone, brisk reflexes and clonus), while sensory
ing and incontinence. signs depend on which part of the cord is compressed:
X-rays show dense ossification along the back of the there may be decreased sensibility to pain and tem-
vertebral bodies (and sometimes also the ligamentum perature (spinothalamic tracts) or diminished vibra-
flavum) in the mid-cervical spine. tion and position sense (posterior columns).
The condition is usually slowly progressive, but occa-
Treatment Treatment is not always necessary; indeed
sionally a patient with longstanding symptoms starts
people with typical x-ray features may be completely
deteriorating rapidly and treatment becomes urgent.
asymptomatic. If the symptoms and signs are disturb-
ing or progressive, operative decompression will be Imaging A plain lateral radiograph which shows an
needed. ‘Decompression’ is performed posteriorly anteroposterior diameter of the spinal canal of less
because of the multilevel nature of the condition, and than 11 mm strongly supports the diagnosis of cervi-
takes the form of one or other type of laminoplasty, cal spinal stenosis. A better measure is the Pavlov ratio
leaving the ossified ligament in place. (the anteroposterior diameter of the canal divided by
the diameter of the vertebral body at the same level)
because this is not affected by magnification error. A
SPINAL STENOSIS AND CERVICAL ratio of less than 0.8 is abnormal.
MYELOPATHY MRI demonstrates the spinal cord and soft-tissue
structures, and helps to exclude other causes of simi-
lar neurological dysfunction. CT myelography is supe-
The sagittal diameter of the mid-cervical spinal canal rior to MRI in demonstrating osseous detail.
(the distance, on plain x-rays, from the posterior sur-
face of the vertebral body to the base of the spinous DIFFERENTIAL DIAGNOSIS
process) varies considerably from one individual to Full neurological investigation is required to eliminate
another; anything less than 11 mm is suggestive of other diagnoses such as multiple sclerosis (episodic
stenosis. Abnormally small canals are seen in rare dys- symptoms), amyotrophic lateral sclerosis (purely
plasias, such as achondroplasia, and may give rise to motor dysfunction), syringomyelia and spinal cord
cord compression. Many asymptomatic, and appar- tumours.
ently normal, people also have small canals and they
are at risk of developing the clinical symptoms of
spinal stenosis if there is any further encroachment Treatment
due to intervertebral disc degeneration, posterior Most patients can be treated conservatively with anal-
‘osteophytosis’, osteoarthritis of the facet joints, gesics, a collar, isometric exercises and gait training.
thickening of the ligamentum flavum, ossification of Manipulation and traction should be avoided.
the posterior longitudinal ligament or vertebral dis- Patients with progressive myelopathy or rapid dete-
placement. If the changes are severe enough, the rioration should be considered for surgery. Acute,
patient may develop neurological symptoms and signs severe myelopathy is a surgical emergency, requiring
(cervical myelopathy), which are thought to be due to immediate decompression.
both direct compression and ischaemia of the cord
and nerve roots arising from impaired venous
drainage and reduced arterial flow.
PYOGENIC INFECTION
Clinical features
Pyogenic infection of the cervical spine is uncommon,
Patients usually have neck pain and brachialgia but and therefore often misdiagnosed in the early stages
448 also complain of paraesthesia, numbness, weakness when antibiotic treatment is most effective.
TUBERCULOSIS 17
Cervical spine tuberculosis is rare. As with other types

of infection, the organism is blood-borne and the in-
fection localizes in the intervertebral disc and the
The neck
anterior parts of the adjacent vertebral bodies. As the
bone crumbles, the cervical spine collapses into
kyphosis. A retropharyngeal abscess forms and points
behind the sternomastoid muscle at the side of the
neck. In late cases cord damage may cause neurologi-
cal signs varying from mild weakness to tetraplegia.
Clinical features
The patient – usually a child – complains of neck pain
and stiffness. In neglected cases a retropharyngeal
abscess may cause difficulty in swallowing or swelling
(a) (b) at the side of the neck. On examination the neck is
extremely tender and all movements are restricted. In
17.11 Pyogenic infection (a) The first x-ray, taken soon late cases there may be obvious kyphosis, a fluctuant
after the onset of symptoms, shows narrowing of the C5/6
disc space but no other abnormality. (b) Three weeks later abscess in the neck or a retropharyngeal swelling. The
there is dramatic destruction and collapse; the speed at limbs should be examined for neurological defects.
which these have occurred distinguishes pyogenic from X-rays show narrowing of the disc space and ero-
tuberculous infection. sion of the adjacent vertebral bodies.
Treatment
The organism – usually a staphylococcus – reaches Treatment is initially by antituberculous drugs and
the spine via the blood stream. Initially, destructive ‘immobilization’ of the neck in a cervical brace or
changes are limited to the intervertebral disc space plaster cast for 6–18 months.
and the adjacent parts of the vertebral bodies. Later,
abscess formation occurs and pus may extend into the
spinal canal or into the soft-tissue planes of the neck.
Clinical features
Vertebral infection may occur at any age. The patient
complains of pain in the neck, often severe and asso-
ciated with muscle spasm and marked stiffness. How-
ever, systemic symptoms are often mild. On
examination, neck movements are severely restricted.
Blood tests may show a leucocytosis and an increased
ESR.
X-rays at first show either no abnormality or only
slight narrowing of the disc space; later there may be
more obvious signs of bone destruction.
Treatment
Treatment is by antibiotics and rest. The cervical spine
is ‘immobilized’ by traction; once the acute phase
subsides, a collar may suffice. Operation is seldom
necessary; as the infection subsides the intervertebral
17.12 Tuberculosis This child had been complaining of
space is obliterated and the adjacent vertebrae fuse. If neck pain and stiffness for several months. Eventually she
there is frank abscess formation, this will require was brought to the clinic with a lump at the side of her
drainage. neck – a typical tuberculous abscess. 449
17 Operative treatment Debridement of necrotic bone ments are markedly restricted. Symptoms and signs of
and anterior cervical vertebral fusion with bone grafts root compression may be present in the upper limbs;
may be offered as an alternative to prolonged immo- less often there is lower limb weakness and upper
bilization in a brace or cast. More urgent indications motor neuron signs due to cord compression. There
for operation are (1) to drain a retropharyngeal may be symptoms of vertebro-basilar insufficiency,
abscess, (2) to decompress a threatened spinal cord, such as vertigo, tinnitus and visual disturbance. Some
or (3) to fuse an unstable spine. patients, though completely unaware of any neuro-
logical deficit, are found on careful examination to
have mild sensory disturbance or pyramidal tract signs
RHEUMATOID ARTHRITIS (e.g. abnormally brisk reflexes).
General debility and peripheral joint involvement
The cervical spine is severely affected in 30 per cent of can mask the signs of myelopathy. Lhermitte’s sign –
patients with rheumatoid arthritis. Three types of electric shock sensation down the spine on flexing the
lesion are common: (1) erosion of the atlanto-axial neck – may be present. Sudden death from cata-
joints and the transverse ligament, with resulting strophic neurological compression is rare.
instability; (2) erosion of the atlanto-occipital articu- X-rays X-rays show the features of an erosive arthri-
lations, allowing the odontoid peg to ride up into the tis, usually at several levels. Atlanto-axial instability is
foramen magnum (cranial sinkage); and (3) erosion of visible in lateral films taken in flexion and extension;
the facet joints in the mid-cervical region, sometimes in flexion the anterior arch of the atlas rides forwards,
ending in fusion but more often leading to subluxa- leaving a gap of 5 mm or more between the back of
tion. In addition, vertebral osteoporosis is common, the anterior arch and the odontoid process; on exten-
due either to the disease or to the effect of corticos- sion the subluxation is reduced. Atlanto-occipital ero-
teroid therapy, or both. sion is more difficult to see, but a lateral tomograph
Considering the amount of atlanto-axial displace- shows the relationship of the odontoid to the foramen
ment that occurs (often greater than 1 cm), neuro- magnum. Normally the odontoid tip is less than
logical complications are uncommon. However, they 5 mm above McGregor’s line (a line from the poste-
do occur – especially in longstanding cases – and are rior edge of the hard palate to the lowest point on the
produced by mechanical compression of the cord, by occiput); in erosive arthritis the odontoid tip may be
local granulation tissue formation or (very rarely) by 10–12 mm above this line. Flexion views may also
thrombosis of the vertebral arteries. show anterior subluxation in the mid-cervical region.
CT and MRI These methods are useful for imaging
Clinical features ‘difficult’ areas such as the atlanto-axial and atlanto-
The patient is usually a woman with advanced occipital articulations, and for viewing the soft-tissue
rheumatoid arthritis. She has neck pain, and move- structures (especially the cord).
(a) (b) (c) (d)
17.13 Rheumatoid arthritis (a) Movement is severely restricted; attempted rotation causes pain and muscle spasm.
(b) Atlanto-axial subluxation is common; erosion of the joints and the transverse ligament has allowed the atlas to slip
forward about 2 cm; (c) reduction and posterior fusion with wire fixation. (d) This patient has subluxation, not only at the
450 atlanto-axial joint but also at two levels in the mid-cervical region.
Treatment ten paces ahead, are indications for cervical spine 17
osteotomy.
Despite the startling x-ray appearances, serious neuro- The ankylosed spine is osteoporotic and prone to
logical complications are uncommon. Pain can usually fracture. A patient with ankylosing spondylitis and an
be relieved by wearing a collar. increase in neck pain must be assumed to have a frac-
The indications for operative stabilization of the ture until proven otherwise (by bone scan or MRI if
The neck
cervical spine are (1) severe and unremitting pain, and plain radiographs are normal). Neurological compro-
(2) neurological signs of root or cord compression. mise is common. A displaced fracture needs careful
Arthrodesis (usually posterior) is by bone grafting fol- closed reduction with halo traction then halo vest
lowed by a halo body cast, or by internal fixation (pos- immobilization. Surgery carries a high complication
terior wiring or a rectangular fixator) and bone rate.
grafting. Postoperatively a cervical brace is worn for 3
months; however, if instability is marked and opera-
tive fixation insecure, a halo jacket may be necessary. SPASMODIC TORTICOLLIS
In patients with very advanced disease and severe ero-
sive changes, postoperative morbidity and mortality This, the most common form of focal dystonia, is
are high. This is an argument for operating at an ear- characterized by involuntary twisting or clonic move-
lier stage for ‘impending neurological deficit’, as diag- ments of the neck. Spasms are sometimes triggered by
nosed from x-ray signs of severe atlanto-axial emotional disturbance or attempts at correction. Even
subluxation, upward migration of the odontoid or at rest the neck assumes an abnormal posture, the
subaxial vertebral subluxation together with CT, chin usually twisted to one side and upwards; the
myelographic or MR images of cord or brain-stem shoulder on that side may be elevated. In some cases
compression. involuntary muscle contractions spread to other areas
and the condition is revealed as a more generalized
form of dystonia.
ANKYLOSING SPONDYLITIS The exact cause is unknown, but some cases are
associated with lesions of the basal ganglia. Correc-
Ankylosing spondylitis is the most common seroneg- tion is extremely difficult; various drugs, including
ative spondyloarthropathy to affect the cervical spine. anticholinergics, have been used, though with little
Neck pain and stiffness tend to occur some years after success. Some patients respond to local injections of
the onset of backache. The neck becomes progres- botulinum toxin into the sternomastoid muscle.
sively stiff and kyphotic although some movement is
usually preserved at the atlanto-occipital and atlanto-
axial joints. NOTES ON APPLIED ANATOMY
An unacceptable ‘chin-on-chest’ deformity, or
inability to lift the head high enough to see more than In the upright posture the neck has a gentle anterior
convexity; this natural lordosis may straighten but is
never quite reversed, even in flexion, unless it is
abnormal.
Eight pairs of nerve roots from the cervical cord
pass through the relatively narrow intervertebral
foramina, the first between the occiput and C1, and
the eighth between C7 and the first thoracic (T1) ver-
tebra; thus each segmental root from the first to the
seventh lies above the vertebra of the same number.
Thus a lesion between C5 and C6 might compress the
sixth root.
The intervertebral discs lie close to the nerve roots
as they emerge through the foramina; even a small
herniation often causes root symptoms (shoulder gir-
dle and upper limb pain and paraesthesiae) rather than
neck pain. Moreover, disc degeneration is associated
with spur formation on both the posterior aspect of
the vertebral body and the associated facet joints; the
resulting encroachment on the intervertebral foramen
17.14 Spasmodic torticollis Attempted correction was traps the nerve root. It is important to remember,
forcibly resisted. The deformity can be very distressing. however, that ‘root pain’ alone (i.e. pain in the 451
17 shoulder and arm) does not necessarily signify nerve- Bohlman HH, Emery SE,Goodfellow DB, Jones PK.
root irritation; it may be referred from the facet joint Robinson anterior cervical discectomy and arthrodesis for
or the soft structures around it. Only paraesthesiae cervical radiculopathy: Long-term follow-up of one hun-
and sensory or motor loss are unequivocal evidence of dred and twenty patients. J Bone Joint Surg 1993; 75A:
nerve root compression. 1298–1307.
At the atlanto-occipital joint, the movements that Copley LA, Dormans JP. Cervical spine disorders in infants
occur are nodding and tilting (lateral flexion); there is and children. J Am Acad Orthop Surg 1998; 6: 204–14.
no rotation, and when this movement takes place (at Garfin SR, Herkowitz HN. (Guest Editors). The degener-
the atlanto-axial joint) the atlas and the skull move as ative neck. Orthop Clinics of North America 1992; 23(3).
one. In the rest of the cervical spine, movements that Hensinger RN. Congenital anomalies of the cervical spine.
occur are flexion, extension and tilting to either side; Clin Orthop Relat Res 1991; 264: 16–38.
the facets permit subluxation or dislocation to occur Law MD, Bernhardt M, White AA. Evaluation and man-
without fracture, a displacement that the strong pos- agement of cervical spondylotic myelopathy. J Bone Joint
terior ligaments normally prevent. Surg 1994; 76A: 1420–33.
Levine MJ, Albert TJ, Smith MD. Cervical radiculopathy. J
REFERENCES AND FURTHER READING Am Acad Orthop Surg 1996; 4: 305–316.
Ono K, Ota H, Tada K, et al. Ossified posterior longitudi-
Agarwal AK, Peppelman WC, Kraus DR, Eisenbeis CH. nal ligament. Spine 1977; 2: 126.
The cervical spine in rheumatoid arthritis. BMJ 1993; Tsuyama N. Ossification of the posterior longitudinal liga-
306: 79–80. ment of the spine. Clin Orthop 1984; 184: 71–84.
452
The back
18
Stephen Eisenstein, Surendar Tuli, Shunmugam Govender
Stiffness may be sudden in onset and almost com-

CLINICAL ASSESSMENT plete (in a ‘locked back’ attack, or after a disc prolapse)
or continuous and predictably worse in the mornings
SYMPTOMS (suggesting arthritis or ankylosing spondylitis).
The usual symptoms of back disorders are pain, stiff- Deformity is usually noticed by others, but the
ness and deformity in the back, and pain, paraesthesia patient may become aware of shoulder asymmetry or
or weakness in the lower limbs. The mode of onset is of clothes not fitting well.
very important: did it start suddenly, perhaps after a Numbness or paraesthesia is felt anywhere in the
lifting strain; or gradually without any antecedent lower limb, but can usually be mapped fairly accu-
event? Are the symptoms constant, or are there peri- rately over one of the dermatomes. It is important to
ods of remission? Are they related to any particular ask if it is aggravated by standing or walking and
posture? Has there been any associated illness or relieved by sitting down – the classic symptom of
malaise? spinal stenosis.
Pain, either sharp and localized or chronic and dif- Urinary retention or incontinence can be due to
fuse, is the commonest presenting symptom. Back- pressure on the cauda equina.
ache is usually felt low down and on either side of the Faecal incontinence or urgency, and impotence, may
midline, often extending into the upper part of the also occur.
buttock and even into the lower limbs. Back pain Other symptoms important in back disorders are: (1)
made worse by rest would suggest pain arising from urethral discharge; (2) diarrhoea; (3) sore eyes – clas-
the facet joints. Pain made worse by activity probably sical features of Reiter’s disease.
comes from any of the soft-tissue supports of the
spine (muscles and ligaments) including the annulus
of the intervertebral disc. SIGNS WITH THE PATIENT STANDING
Sciatica is the term originally used to describe Adequate exposure is essential; patients should strip to
intense pain radiating from the buttock into the thigh their underclothes.
and calf – more or less following the distribution of
the sciatic nerve and therefore suggestive of nerve
root compression or irritation. However Kellgren
Look
(1977), in a classic experiment, showed that almost Start by examining the skin. Scars (previous surgery or
any structure in a spinal segment can, if irritated suffi- injury), pigmentation (neurofibromatosis?) or abnor-
ciently, give rise to referred pain radiating into the mal tufts of hair (spina bifida?) are important clues to
lower limbs. Unfortunately, with the passage of time, underlying spinal disorders.
many clinicians have taken to describing all types of Look carefully at the patient’s shape and posture,
pain extending from the lumbar region into the lower both from the front and behind. Asymmetry of the
limb as ‘sciatica’. This is at best confusing and at worst chest, trunk or pelvis may be obvious, or may appear
a preparation for misdiagnosis! True sciatica, most only when the patient bends forward. Lateral devia-
commonly due to a prolapsed intervertebral disc tion of the spinal column is described as a list to one
pressing on a nerve root, is characteristically more or other side; lateral curvature is scoliosis.
intense than referred low back pain, is aggravated by Seen from the side, the back normally has a slight
coughing and straining and is often accompanied by forward curve, or kyphosis, in the thoracic region and
symptoms of root pressure such as numbness and a shorter backward curve, or lordosis, in the lumbar
paraesthesiae, especially in the foot. segment (the ‘hollow’ of the back). Excessive thoracic
18
(a) (b) (c) (d) (e)
18.1 Examination With the patient standing upright (a), look at his general posture and note particularly the presence of
any asymmetry or frank deformity of the spine. Then ask him to lean backwards (extension) (b), forwards to touch his toes
(flexion) (c) and then sideways as far as possible (d), comparing his level of reach on the two sides. Finally, hold the pelvis
stable and ask the patient to twist first to one side and then to the other (rotation). Note that rotation occurs almost
entirely in the thoracic spine (e) and not in the lumbar spine.
kyphosis is sometimes called hyperkyphosis, to distin- Feel

guish it from the normal; if the spine is sharply angu-
lated the prominence is called a kyphos or gibbus. The Feel for the spinous processes and the interspinous
lumbar spine may be excessively lordosed (hyperlor- ligaments, noting any unusual prominence or a ‘step’.
dosis) or unusually flat (effectively a lumbar kyphosis). Tenderness should be localized to: (1) bony struc-
Undue or asymmetrical prominence of the paraver- tures; (2) intervertebral tissues; (3) paravertebral mus-
tebral muscles may be due to spasm, an important cles and ligaments, especially where they insert into
sign in acute back disorders. the iliac crest.
If the patient consistently stands with one knee
bent (even though his legs are equal in length) this Move
suggests nerve root tension on that side; flexing the
knee relaxes the sciatic nerve and reduces the pull on Flexion is tested by asking the patient to try to touch
the nerve root. his toes. Even with a stiff back he may be able to do
(a) (b) (c)
18.2 Measuring the range of flexion Bending down and touching the toes may look like lumbar flexion but this is not
always the case. The patient in (a) has anklyosing spondylitis and a rigid lumbar spine, but he is able to reach his toes
because he has good flexibility at the hips. Compare his flat back with the rounded back of the model in Figure 18.1c. You
can measure the lumbar excursion. With the patient upright, select two bony points 10 cm apart and mark the skin (b); as
454 the patient bends forward, the two points should separate by at least a further 5 cm (c).
this by flexing the hips; so watch the lumbar spine to spasm, and examine the buttocks for gluteal wasting. 18
see if it really moves, or, better still, measure the spinal Feel the bony outlines (is there an unexpected ‘step’
excursion. The mode of flexion (whether it is smooth or or prominence?) and check for consistently localized
hesitant) and the way in which the patient comes back lumbosacral tenderness or soft-tissue ‘trigger’ points.
to the upright position are also important. In clinical The popliteal and posterior tibial pulses are felt,
lumbar instability the patient tends to regain the hamstring power is tested and sensation on the back of
The back
upright position by pushing on the front of his thighs. the limbs assessed.
To test extension, ask the patient to lean backwards, The femoral nerve stretch test (for lumbar 3rd or 4th
but see that he doesn’t cheat by bending his knees. A nerve root sensitivity) is carried out by gently flexing
patient with good forward bending but much pain on the patient’s knee or by lifting the hip into extension
extension probably has painful facet joints. (or both in one movement); pain may be felt in the
The ‘wall test’ will unmask a minor flexion defor- front of the thigh.
mity (kyphosis, as in ankylosing spondylitis or
Scheuermann’s osteochondrosis); standing with the
back flush against a wall, the heels, buttocks, shoul- SIGNS WITH THE PATIENT LYING SUPINE
ders and occiput should all make contact with the ver-
tical surface. The patient is observed as he turns – is there pain or
Lateral flexion is tested by asking the patient to stiffness? A rapid appraisal of the thyroid, chest (and
bend sideways, sliding his hand down the outer side of breasts), and abdomen (and scrotum) is advisable, and
his leg; the two sides are compared. Rotation is exam- essential if there is even a hint of generalized disease.
ined by asking him to twist the trunk to each side in Hip and knee mobility are assessed before testing for
turn while the pelvis is anchored by the examiner’s spinal cord or root involvement.
hands; this is essentially a thoracic movement and is The straight-leg raising test discloses lumbosacral
not limited in lumbosacral disease. root tension. Ask the patient to hold his or her knee
Rib-cage excursion is assessed by measuring the absolutely straight, then lift the patient’s leg slowly
chest circumference in full expiration and then in full until he or she experiences pain – not merely in the
inspiration; the normal difference is about 7 cm. A lower back (which is common and not significant) but
reduced excursion may be the earliest sign of ankylos- also in the buttock, thigh and calf (Lasègue’s test, but
ing spondylitis. attribution is controversial); the angle at which this
While the patient is standing, you can test muscle occurs is noted. Normally it should be possible to raise
power in the legs by asking him to stand up on his toes the limb to 80–90 degrees; people with lax ligaments
(plantarflexion) and then to rock back on his heels can go even further. In a full-blown disc prolapse with
(dorsiflexion); small differences between the two sides nerve root compression, straight-leg raising may be
are easily spotted. restricted to less than 30 degrees because of severe
pain in the sciatic distribution, not back pain. At the
point where the patient experiences discomfort, pas-
SIGNS WITH THE PATIENT LYING PRONE sive dorsiflexion of the foot may cause an additional
stab of sciatic pain. A gentler (and some would say
Make sure that the patient is lying comfortably on the more meaningful) way of testing straight-leg raising is
examination couch, and remove the pillow so that he to ask the patient to raise the leg with the knee straight
is not forced to arch his back (or smother himself). and rigid – and to stop when he or she feels pain.
Again, look for localized deformities and muscle The ‘bowstring’ sign is even more specific. Raise the
(a) (b) (c)
18.3 Examination with the patient prone (a) Feel for tenderness, watching the patient’s face for any reaction.
(b) Performing the femoral stretch test. You can test for lumbar root sensitivity either by hyperextending the hip or by
acutely flexing the knee with the patient lying prone. Note the point at which the patient feels pain and compare the two
sides. (c) While the patient is lying prone, take the opportunity to feel the pulses. The popliteal pulse is easily felt if the
tissues at the back of the knee are relaxed by slightly flexing the knee. 455
18 18.4 Sciatic stretch tests
(a) Straight-leg raising. The
knee is kept absolutely
straight while the leg is
slowly lifted (or raised by the
patient himself); note where
the patient complains of
tightness and pain in the

buttock – this normally
occurs around 80 or 90°.
(b) At that point a more
acute stretch can be applied
by passively dorsiflexing the
(a) (b) foot – this may cause an
added stab of pain. (c) The
‘bowstring sign’ is a
confirmatory test for sciatic
tension. At the point where
the patient experiences pain,
relax the tension by bending
the knee slightly; the pain
should disappear. Then apply
firm pressure behind the
lateral hamstrings to tighten
the common peroneal nerve
(d); the pain recurs with
renewed intensity.
(c) (d)
patient’s leg gently to the point where he or she expe- IMAGING

riences sciatic pain; now, without reducing the
amount of lift, bend the knee so as to relax the sciatic
Plain x-rays
nerve. Buttock pain is immediately relieved; pain may
then be re-induced without extending the knee by Begin with anteroposterior and lateral views of the
simply pressing on the lateral popliteal nerve behind spine; for the lumbar region, oblique views of the
the lateral tibial condyle, to tighten it like a bowstring. spine, an anteroposterior x-ray of the pelvis and a pos-
Sometimes straight-leg raising on the unaffected tero-anterior view of the sacroiliac joints may also be
side produces pain on the affected side. This crossed needed.
sciatic tension is indicative of severe root compression, In the anteroposterior view the spine should look
usually due to a large central disc prolapse, and warns perfectly straight and the soft-tissue shadows should
of the risk to the sacral nerve roots that control blad- outline the normal muscle planes. Curvature (scolio-
der function (the cauda equina syndrome – one of sis) is obvious, and best shown in erect views. Bulging
very few surgical emergencies in spinal disorders). of the psoas muscle or loss of the psoas shadow may
A full neurological examination of the lower limbs indicate a paravertebral abscess. Individual vertebrae
is then carried out. An absent ankle jerk on the side of may show alterations in structure, e.g. asymmetry or
sciatica, combined with paraesthesiae along the lateral collapse. Check the outlines of the pedicles, which
border of the foot, suggests compression of the S1 normally look like oval footprints near the lateral
nerve root; normal reflexes combined with paraesthe- edges of each rectangular vertebral body: a missing or
siae on the dorsum of the foot, suggests compression misshapen pedicle could be due to erosion by infec-
of the L5 nerve root. Check for clonus and a positive tion, a neurofibroma or metastatic disease.
Babinski sign; if present there should be some alarm In the lateral view the normal thoracic kyphosis (up
regarding possible spinal cord compression. to 40 degrees) and lumbar lordosis should be regular
Ankle clonus with a positive Babinski sign suggests and uninterrupted. Anterior shift of an upper segment
brain or spinal cord pathology until proved otherwise. upon a lower (spondylolisthesis) may be associated
The lower limbs should be carefully examined for with defects of the posterior arch, which show best in
length discrepancy and trophic changes; the pulses are felt oblique views. Vertebral bodies, which should be rec-
in the groin, the popliteal fossa and around the ankle. tangular, may be wedged or biconcave, deformities
Unless the signs point unequivocally to a spinal dis- typical of osteoporosis or old injury. Bone density and
order, rectal and vaginal examination may also be trabecular markings also are best seen in lateral films.
456 necessary. Lateral views in flexion and extension may reveal
18
Scalloping (erosion) of
vertebral bodies
The back
Vertebral body
Intervertebral disc Intervertebral disc
Facet joint
Pedicle
Facet joint
Spinous process
(a) (b)
18.5 Lumbar spine x-rays (a,b) The most important normal features are demonstrated in the lower lumbar spine. In this
particular case there are also signs of marked posterior vertebral body and facet joint erosions at L1 and L2, features that
are strongly suggestive of an expanding neurofibroma.
excessive intervertebral movement, a possible cause of patient’s symptoms. These are painful investigations,
back pain. no longer easily justified where MRI is available.
The intervertebral spaces may be edged by bony
spurs (suggesting longstanding disc degeneration) or
bridged by fine bony syndesmophytes (a cardinal fea-
Magnetic resonance imaging
ture of ankylosing spondylitis). MRI has virtually done away with the need for myel-
The sacroiliac joints may show erosion or ankylosis, ography, discography, facet arthrography, and much
as in tuberculosis (TB) or ankylosing spondylitis, and of CT scanning. The spinal canal and disc spaces are
the hip joints may show arthrosis, not to be missed in clearly outlined in various planes. Scans can reveal the
the older patient with backache. physiological state of the disc as regards dehydration,
as well as the effect of disc degeneration on bone mar-
row in adjacent vertebral bodies.
Radioisotope scanning
Isotope scans may pick up areas of increased activity,
suggesting a fracture, a local inflammatory lesion or a
‘silent’ metastasis. SPINAL DEFORMITIES
Computed tomography ‘Spinal deformity’ (as opposed to deformities of indi-
vidual vertebrae) affects the entire shape of the back
Computed tomography (CT) is helpful in the diagno- and manifests as abnormal curvature, in either the
sis of structural bone changes (e.g. vertebral fracture) coronal plane (scoliosis) or the sagittal plane (hyper-
and intervertebral disc prolapse. When combined with kyphosis and hyperlordosis).
myelography it gives valuable information about the Variations and abnormalities of segmentation are
contents of the spinal canal. common; they include anomalies such as lumbariza-
tion of the first sacral segment, ‘sacralization’ of one
Discography and facet joint or both transverse processes of the fifth lumbar verte-
bra and asymmetry of the apophyseal joints, all of
arthrography which are harmless, as well as such conditions as
These are sometimes performed in the investigation hemivertebra, which may give rise to severe spinal
of chronic back pain. Remember, though, that disc deformity (see later).
degeneration and facet joint arthritis are common in The most serious type of congenital defect is spina
older people and are not necessarily the cause of the bifida. 457
18
L2
L3
L4
L5
S1
(a) (b) (c)
18.6 MRI and discography (a) The lateral T2-weighted MRI shows a small posterior disc bulge at L4/5 and a larger
protrusion at L5/S1. (b) The axial MRI shows the disc prolapse encroaching on the intervertebral canal and the nerve root
on the left side. (c) Discography, showing normal appearance at the upper level and a degenerate disc with prolapse at the
level below.
spasm associated with a prolapsed lumbar disc may

SCOLIOSIS cause a skew back; although sometimes called ‘sciatic
scoliosis’ this, too, is a spurious deformity.
Scoliosis is an apparent lateral (sideways) curvature of
the spine. ‘Apparent’ because, although lateral curva-
ture does occur, the commonest form of scoliosis is
actually a triplanar deformity with lateral, anteropos-
Structural scoliosis
terior and rotational components (Dickson et al., In structural scoliosis there is a non-correctable defor-
1984). Two broad types of deformity are defined: pos- mity of the affected spinal segment, an essential com-
tural and structural. ponent of which is vertebral rotation. The spinous
processes swing round towards the concavity of the
curve and the transverse processes on the convexity
Postural Scoliosis
rotate posteriorly. In the thoracic region the ribs on
In postural scoliosis the deformity is secondary or the convex side stand out prominently, producing the
compensatory to some condition outside the spine, rib hump, which is a characteristic part of the overall
such as a short leg, or pelvic tilt due to contracture of deformity. Dickson and co-workers (1984) have
the hip. When the patient sits (thereby cancelling leg pointed out that this is really a lordoscoliosis associ-
length asymmetry) the curve disappears. Local muscle ated with rotational buckling of the spine. The initial
18.7 Postural
scoliosis (a) This
young girl presented
with thoracolumbar
‘curvature’. When she
bends forwards, the
deformity disappears;
this is typical of a
postural or mobile
scoliosis. (b) Short-leg
scoliosis disappears
when the patient sits.
(c) Sciatic scoliosis
disappears when the
prolapsed disc settles
down or is removed.
458 (a) (b) (c)
18
The back
(a) (b) (c) (d)
18.8 Structural scoliosis (a) Slight curves are often missed on casual inspection but the deformity becomes apparent
when the spine is flexed (b). The young girl in (c) has a much more obvious scoliosis and asymmetry of the hips but what
really worries her is the prominent rib hump, seen best when she bends over (d).
deformity is probably correctable, but once it exceeds for MRI. Scoliosis in children is a painless deformity.
a certain point of mechanical stability the spine buck- Scoliosis with pain suggests a spinal tumour until
les and rotates into a fixed deformity that does not proved otherwise.
disappear with changes in posture. Secondary (com- There may be a family history of scoliosis or a
pensatory) curves nearly always develop to counter- record of some abnormality during pregnancy or
balance the primary deformity; they are usually less childbirth; the early developmental milestones should
marked and more easily correctable, but with time be noted.
they, too, become fixed. The trunk should be completely exposed and the pa-
Once fully established, the deformity is liable to tient examined from in front, from the back and from
increase throughout the growth period. Thereafter, the side. Skin pigmentation and congenital anomalies
further deterioration is slight, though curves greater such as sacral dimples or hair tufts are sought.
than 50 degrees may go on increasing by 1 degree per The spine may be obviously deviated from the mid-
year. With very severe curves, chest deformity is line, or this may become apparent only when the
marked and cardiopulmonary function is usually patient bends forward (the Adams test). The level and
affected. direction of the major curve convexity are noted (e.g.
Most cases have no obvious cause (idiopathic scolio- ‘right thoracic’ means a curve in the thoracic spine
sis); other varieties are congenital or osteopathic (due and convex to the right). The hip (pelvis) sticks out
to bony anomalies), neuropathic, myopathic (associ- on the concave side and the scapula on the convex.
ated with some muscle dystrophies) and a miscella- The breasts and shoulders also may be asymmetrical.
neous group of connective-tissue disorders. With thoracic scoliosis, rotation causes the rib angles
to protrude, thus producing an asymmetrical rib
hump on the convex side of the curve. In balanced
Clinical features deformities the occiput is over the midline; in unbal-
anced (or decompensated) curves it is not. This can
Deformity is usually the presenting symptom: an obvi- be determined more accurately by dropping a plumb-
ous skew back or a rib hump in thoracic curves, and line from the prominent spinous process of C7 and
asymmetrical prominence of one hip in thoracolum- noting whether it falls along the gluteal cleft.
bar curves. Balanced curves sometimes pass unnoticed The diagnostic feature of fixed (as distinct from
until an adult presents with backache. Where school postural or mobile) scoliosis is that forward bending
screening programmes are conducted, children will be makes the curve more obvious. Spinal mobility should
referred with very minor deformities. be assessed and the effect of lateral bending on the
Pain is a rare complaint and should alert the clini- curve noted; is there some flexibility in the curve and
cian to the possibility of a neural tumour and the need can it be passively corrected? 459
18 18.9 Adolescent
idiopathic scoliosis
(a) Typical thoracic
deformity. (b) Serial
x-rays show how this
curve increased over
a period of 4 years.
(c) The angle of

curvature is measured
on the x-ray by
Cobb’s method: Lines
projected from the
top of the uppermost
and the bottom of
the lowermost
vertebral bodies in
the primary curve
define Cobb’s angle.
(a) (b) (c)
Side-on posture should also be observed. There fied as the levels where vertebrae start to angle away
may appear to be excessive kyphosis or lordosis. from the curve. The degree of curvature is measured
Neurological examination is important. Any abnor- by drawing lines on the x-ray at the upper border of
mality suggesting a spinal cord lesion calls for CT the uppermost vertebra and the lower border of the
and/or MRI. lowermost vertebra of the curve; the angle subtended
Leg length is measured. If one side is short, the by these lines is the angle of curvature (Cobb’s angle).
pelvis is levelled by standing the patient on wooden The site of the curve apex should be noted. Right
blocks and the spine is re-examined. thoracic curves are the commonest, the great majority
General examination includes a search for the pos- in girls in adolescent idiopathic scoliosis. Left thoracic
sible cause and an assessment of cardiopulmonary curves are so unusual that if seen they should be fur-
function (which is reduced in severe curves). ther investigated by MRI to exclude spinal tumours.
The primary structural curve is usually balanced by
Imaging compensatory curves above and below, or by a second
‘primary’ curve also with vertebral rotation (some-
PLAIN X-RAYS times there are multiple ‘primary’ curves).
Full-length posteroanterior (PA) and lateral x-rays of What is not readily appreciated from these films is
the spine and iliac crests must be taken with the the degree of lordosis in the primary curve(s) and
patient erect. Structural curves show vertebral rota- kyphosis in the compensatory curves (Archer and
tion: in the PA x-ray, vertebrae towards the apex of Dickson, 1989); indeed it is postulated that flattening
the curve appear to be asymmetrical and the spinous or reversal of the normal thoracic kyphosis superim-
processes are deviated towards the concavity. Remem- posed on coronal plane asymmetry leads, with
ber that PA in relation to the patient is not PA in rela- growth, to progressive idiopathic scoliosis. Lateral
tion to the rotated vertebrae! bending views are taken to assess the degree of curve
The upper and lower ends of the curve are identi- correctability.
(a) (b)
18.10 Risser’s sign The iliac apophyses normally appear progressively from lateral to medial (stages 1–4). When fusion is
460 complete, spinal maturity has been reached and further increase of curvature is negligible (stage 5).
18
60 per cent male
90 per cent convex to left.
Infantile thoracic Associated with ipsilateral
plagiocephaly
The back
May be resolving or progressive.
Progressive variety becomes severe.
90 per cent female

90 per cent convex to right.
Rib rotation exaggerates the
Adolescent thoracic deformity.
50 per cent develop curves of
greater than 70°.
Slightly more common in females.

Slightly more common to right.
Thoracolumbar Features mid-way between
adolescent thoracic and lumbar.
More common in females.

80 per cent convex to left.
Lumbar One hip prominent but no ribs to
accentuate deformity.
Therefore not noticed early, but
backache in adult life.
Two primary curves, one in each

direction.
Combined Even when radiologically severe,
clinical deformity relatively slight
because always well balanced.
18.11 Patterns of idiopathic scoliosis Bracing is used far less than previously because of serious doubts as to its
effectiveness beyond natural history.
SKELETAL MATURITY – RISSER’S SIGN ment usually coincides with fusion of the vertebral
This is assessed in several ways (this is important ring apophyses. ‘Skeletal age’ may also be estimated
because the curve often progresses most during the from x-rays of the wrist and hand.
period of rapid skeletal growth and maturation). The
iliac apophyses start ossifying shortly after puberty;
ossification extends medially and, once the iliac crests SPECIAL IMAGING
are completely ossified, further progression of the sco- CT and MRI may be necessary to define a vertebral
liosis is minimal (Risser’s sign). This stage of develop- abnormality or cord compression. 461
18
(a) (b) (c) (d)
18.12 Structural scoliosis – bracing (a,b) The Milwaukee brace fits snugly over the pelvis below; chin and head pads
promote active postural correction and a thoracic pad presses on the ribs at the apex of the curve. (c) The Boston brace is used
for low curves. All braces are cumbersome, but (d) if well made they need not interfere much with activity. Nowadays bracing
is used far less often than before because of doubts about its ability to alter the natural progress of structural scoliosis.
Special investigations LATE-ONSET (ADOLESCENT) IDIOPATHIC

Pulmonary function tests are performed in all cases of SCOLIOSIS (AGED 10 OR OVER)
severe chest deformity. A marked reduction in vital
capacity is associated with diminished life expectancy This is the commonest type, occurring in 90 per cent
and carries obvious risks for surgery. of cases, mostly in girls. Primary thoracic curves are
Patients with muscular dystrophies or connective usually convex to the right, lumbar curves to the left;
tissue disorders require full biochemical and neuro- intermediate (thoracolumbar) and combined (double
muscular investigation of the underlying condition. primary) curves also occur. Progression is not
inevitable; indeed, most curves less than 20 degrees
either resolve spontaneously or remain unchanged.
Prognosis and treatment However, once a curve starts to progress, it usually
Prognosis is the key to treatment: the aim is to pre- goes on doing so throughout the remaining growth
vent severe deformity. Generally speaking, the period (and, to a much lesser degree, beyond that).
younger the child and the higher the curve the worse Reliable predictors of progression are: (1) a very
is the prognosis. Management differs for the different young age; (2) marked curvature; (3) an incomplete
types of scoliosis, which are considered later. Risser sign at presentation (Lonstein and Carlson,
1984). In prepubertal children, rapid progression is
liable to occur during the growth spurt.
IDIOPATHIC SCOLIOSIS
This group constitutes about 80 per cent of all cases

Treatment
of scoliosis. The deformity is often familial and the The aims of treatment are: (1) to prevent a mild
population incidence of serious curves (over 30 deformity from becoming severe; (2) to correct an
degrees and therefore needing treatment) is three per existing deformity that is unacceptable to the patient.
1000; trivial curves are very much more common. A period of preliminary observation may be needed
The age at onset has been used to define three groups: before deciding between conservative and operative
adolescent, juvenile and infantile. A simpler division treatment. At 4–9-monthly intervals the patient is
now in general use is early-onset (before puberty) and examined, photographed and x-rayed so that curves
462 late-onset scoliosis (after puberty). can be measured and checked for progression.
18
The back
18.13 Scoliosis – posterior instrumentation Idiopathic scoliosis treated by posterior double-rod fixation.
NON-OPERATIVE TREATMENT getting worse. Many orthopaedic surgeons no longer

If the patient is approaching skeletal maturity and the employ this method of treatment, arguing that there
deformity is acceptable (which usually means it is less is insufficient evidence of its benefits. Their preference
than 30 degrees and well balanced), treatment is now is to wait for the curve to progress to the stage
probably unnecessary unless sequential x-rays show when corrective surgery would be justified.
definite progression.
Exercises are often prescribed; they have no effect
OPERATIVE TREATMENT
on the curve but they do maintain muscle tone and
Surgery is indicated: (1) for curves of more than 30
may inspire confidence in a favourable outcome.
degrees that are cosmetically unacceptable, especially
Bracing has been used for many years in the treat-
in pre-pubertal children who are liable to develop
ment of progressive scoliotic curves between 20 and
marked progression during the growth spurt; (2) for
30 degrees. The Milwaukee brace is principally a tho-
milder deformity that is deteriorating rapidly. Bal-
racic support consisting of a pelvic corset connected
anced, double primary curves require operation only
by adjustable steel supports to a cervical ring carrying
if they are greater than 40 degrees and progressing.
occipital and chin pads; its purpose is to reduce the
The objectives are: (1) to halt progression of the
lumbar lordosis and encourage active stretching and
deformity; (2) to straighten the curve (including the
straightening of the thoracic spine. The Boston brace is
rotational component) by some form of instrumenta-
a snug-fitting underarm brace that provides lumbar or
tion; (3) to arthrodese the entire primary curve by
low thoracolumbar support. Corrective pads may be
bone grafting. Surgical options include:
added to these devices to apply pressure at a particu-
lar site. A well-made brace can be worn 23 hours out Harrington system In the original system a rod was
of 24 and does not preclude full daily activities, applied posteriorly along the concave side of the curve;
including sport and exercises. attached to the rod were movable hooks that were
It has long been recognized that bracing will not engaged in the uppermost and lowermost vertebrae so
improve the curve – at best it will merely stop it from as to distract the curve. If the curve is flexible, it will 463
18 concave and compress on the convex side of the curve;
by appropriate manipulation of the implants one can
obtain correction also in the sagittal plane. It has been
claimed that this system can correct the rotational
deformity. It is also sufficiently rigid to make
postoperative bracing unnecessary.
Anterior instrumentation (Dwyer; Zielke; Kaneda) Rigid

curves and thoracolumbar curves associated with
lumbar lordosis can be corrected by approaching the
spine from the front, removing the discs throughout
the curve and then applying a compression device
(either a braided cable or a rod linking transverse
vertebral body screws) along the convex side of the
curve. Bone grafts are added to achieve fusion. In some
cases combined anterior and posterior instrumentation
is necessary
Advantages of this system are: (1) that it provides
strong fixation with fewer vertebral segments having
to be fused; (2) that overall shortening of the
deformed section (by disc excision and vertebral com-
pression) lessens the risk of cord injury due to spinal
distraction. In some centres, transthoracic scoliosis
surgery is now performed endoscopically through
several ports, in order to reduce the morbidity asso-
ciated with open thoracic surgery and rib resection.
(a) (b) Warning Whatever method is used, spinal cord
18.14 Scoliosis – anterior instrumentation (a) This function should be monitored during the operation.
14-year-old girl had a very stiff lumbar curve. It was Ideally this is done by measuring somatosensory and
planned to correct this by two-stage anterior and posterior motor evoked potentials during spinal correction. If
release and fusion. (b) X-ray taken after the Zielke anterior these facilities are not available, the ‘wake-up test’ is
instrumentation.
used: anaesthesia is reduced to bring the patient to a
semi-awake state and he or she is then instructed to
move their feet. If there are signs of cord compromise,
passively correct and bone grafts are then applied to the instrumentation is relaxed or removed and re-
obtain fusion over the length of the curve. A major applied with a lesser degree of correction. Patients have
drawback of the original distraction instrumentation is no memory of the wake-up procedure.
that it does not correct the rotational deformity at the
Rib hump The best of the instrumentation systems
apex of the curve and thus the rib prominence remains
cannot completely eliminate the rib hump – and it is
virtually unchanged.
often this that troubles the patient most of all. If the
Rod and sublaminar wiring (Luque) This is a modification deformity is marked, it can be reduced significantly by
of the Harrington system. Wires are passed under the performing a costoplasty, where short sections of rib
vertebral laminae at multiple levels and fixed to the rod are excised at multiple levels on the rib hump (convex)
on the concave side of the curve, thus providing a side, close to the vertebral articulation.
more controlled and secure fixation. By bending the
rod and arranging the mechanism so that the wires pull
backwards rather than merely sideways, the rotational
Complications of surgery
component of the deformity can also be substantially Neurological compromise With modern techniques the
improved. However, the sublaminar wires are incidence of permanent paralysis has been reduced to
dangerously close to the dura and the risk of less than 1 per cent. From the patient’s point of view
neurological damage is increased. this is small comfort. Every effort should be made to
provide adequate safeguards.
Cotrel-Dubousset system This mechanism combines a
pedicle screw ‘box’ foundation at the caudal end of the Spinal decompensation Overcorrection may produce
deformity, with multiple hooks which can be placed at an unbalanced spine. This should be avoided by
various levels to produce either distraction or careful preoperative planning and selection of the
464 compression. With double rods one can distract on the appropriate levels of fusion.
Pseudarthrosis – Incomplete fusion occurs in about 2 resolve spontaneously, progressive curves can become 18
per cent of cases and may require further operation very severe; those in which the rib-vertebra angle at
and grafting. the apex of the curve differs on the two sides by more
than 20 degrees are likely to deteriorate (Mehta,
Implant failure – Hooks may cut out and rods may break.
1972). Because this also influences the development
If this is associated with a symptomatic pseudarthrosis,
of the lungs, there is a high incidence of cardiopul-
revision fusion/fixation will be needed.
The back
monary dysfunction.
Curves assessed as being potentially progressive
should be treated by applying serial elongation-
EARLY-ONSET (JUVENILE) IDIOPATHIC derotation-flexion (EDF) plaster casts under general
SCOLIOSIS anaesthesia, until the deformity resolves or until the
child is big enough to manage in a brace. From about
Presenting in children aged 4–9, this type is uncom- the age of 4 years onwards curve progression slows
mon. The characteristics of this group are similar to down or ceases and the child may not need further
those of the adolescent group, but the prognosis is treatment. If the deformity continues to deteriorate,
worse and surgical correction may be necessary before surgical correction may be required. This takes the
puberty. However, if the child is very young, a brace form of anterior disc excision and fusion to control
may hold the curve stationary until the age of 10 the apex of the curve, combined with posterior fusion
years, when fusion is more likely to succeed. to prevent posterior overgrowth.
EARLY-ONSET (INFANTILE) IDIOPATHIC OSTEOPATHIC (CONGENITAL) SCOLIOSIS

SCOLIOSIS
Although fractures and bone softening (as in rickets
This variety, which presents in children aged 3 or or osteogenesis imperfecta) may lead to scoliosis, the
under, is rare in North America and is becoming commonest bony cause is some type of vertebral
uncommon elsewhere, perhaps because most babies anomaly – hemivertebra, wedged vertebra (failure of
nowadays are allowed to sleep prone. Boys predomi- formation), and fused vertebrae – sometimes com-
nate and most curves are thoracic with convexity to bined with absent or fused ribs (failure of segmenta-
the left. Although 90 per cent of infantile curves tion). Overlying tissues often show angiomas, naevi,
18.15 Early onset scoliosis ‘Idiopathic’ curves in young

children usually resolve, but some increase progressively
and become very severe. Measurement of the rib-vertebra 18.16 Congenital scoliosis Failure of segmentation and
angles at the curve apex in the early stages of the formation of the vertebrae at T10, 11 and 12 has resulted
deformity is a good prognostic indicator (Mehta, 1972). in a localized scoliosis. 465
18
(a) (b)
18.17 Other types of scoliosis (a) This patient has a short structural curve plus multiple skin lesions – features
suggesting neurofibromatosis. (b) By contrast, the typical post-poliomyelitis ‘paralytic’ scoliosis shown in this x-ray is
characterised by a long C-shaped curve.
excess hair, dimples or a pad of fat. Spina bifida may NEUROPATHIC AND MYOPATHIC
be associated and visceral anomalies are common,
especially in the heart and kidneys. These children SCOLIOSIS
require painstaking clinical investigation and imaging
Neuromuscular conditions associated with scoliosis
(1) in order to discover any other congenital anom-
include poliomyelitis, cerebral palsy, syringomyelia,
alies; (2) to assess the risk of spinal cord damage.
Friedreich’s ataxia and the rarer lower motor neuron
While congenital scoliosis is often mild, some cases
disorders and muscle dystrophies; the curve may take
progress to severe deformity, particularly those with
some years to develop. The typical paralytic curve is
unilateral fusion of vertebrae (unilateral unsegmented
long, convex towards the side with weaker muscles
bar). There must be a management assumption that
(spinal, abdominal or intercostal), and at first is
the deformity will get worse, until proved otherwise.
mobile. In severe cases the greatest problem is loss of
Before any operation is undertaken, advanced imag-
stability and balance, which may make even sitting dif-
ing is needed to exclude an associated dysraphism,
ficult or impossible. Additional problems are general-
particularly diastematomyelia and cord tethering,
ized muscle weakness and (in some cases) loss of
which must be dealt with prior to curve correction.
sensibility with the attendant risk of pressure ulcera-
tion.
Treatment X-ray with traction applied shows the extent to
Treatment is more difficult and specialized than that which the deformity is correctable.
of idiopathic infantile scoliosis. Progressive deformi-
ties (usually involving rigid curves) will not respond Treatment
to bracing alone, and surgical correction carries a sig-
Treatment depends upon the degree of functional dis-
nificant risk of cord injury. These children should be
ability. Mild curves may require no treatment at all.
treated in special units: the approach is to undertake
Moderate curves with spinal stability are managed as
staged resection of the curve apex, followed by instru-
for idiopathic scoliosis. Severe curves, associated with
mentation and spinal fusion. If multiple segments of
pelvic obliquity and loss of sitting balance, can often
the spine are involved, surgery may be too hazardous
be managed by fitting a suitable sitting support. If this
and should probably be withheld.
466 does not suffice, operative treatment may be indi-
cated. This involves stabilization of the entire para- 18
lyzed segment by combined anterior and posterior
instrumentation and fusion.
SCOLIOSIS AND NEUROFIBROMATOSIS
The back
About one-third of patients with neurofibromatosis
develop spinal deformity, the severity of which varies
from very mild (and not requiring any form of treat-
ment) to the most marked manifestations accompa-
nied by skin lesions, multiple neurofibromata and
bony dystrophy affecting the vertebrae and ribs. The
scoliotic curve is typically ‘short and sharp’. Other
clues to the diagnosis lie in the appearance of the skin
lesions and any associated skeletal abnormalities.
Mild cases are treated as for idiopathic scoliosis.
More severe deformities will usually need combined
anterior and posterior instrumentation and fusion. As
with other forms of skeletal neurofibromatosis, graft
18.18 Postural kyphosis This tall teenager has Marfans’
dissolution and pseudarthrosis are not uncommon. disease and ligamentous laxity. He has also developed a
postural thoracic hyperkyphosis and lumbar hyperlordosis.
KYPHOSIS In adolescence the commonest cause is Scheuer-

mann’s disease (see later). In adults kyphosis could be
Rather confusingly, the term ‘kyphosis’ is used to due to an old childhood disorder; tuberculous
describe both the normal (gentle rounding of the spondylitis, ankylosing spondylitis or spinal trauma. In
thoracic spine) and the abnormal (excessive thoracic elderly people, osteoporosis may result in vertebral
curvature or straightening out of the cervical or lum- compression and an increase in a previously mild,
bar lordotic curves). Excessive thoracic curvature asymptomatic deformity.
might be better described as ‘hyperkyphosis’. Kyphos,
or gibbus, is a sharp posterior angulation due to local-
ized collapse or wedging of one or more vertebrae.
This may be the result of a congenital defect, a frac- CONGENITAL KYPHOSIS
ture (sometimes pathological) or spinal tuberculosis
(see Fig. 18.24). Vertebral anomalies leading to kyphosis may be due to
failure of formation (type I), failure of segmentation
(type II) or a combination of these:
Postural Kyphosis
Type I (failure of formation) This is the commonest
Postural kyphosis is usually associated with other pos-
(and the worst) type. If the anterior part of the verte-
tural defects such as flat feet. It is voluntarily cor-
bral body fails to develop, progressive kyphosis and
rectable. If treatment is needed, this consists of
posterior displacement of the hemivertebra may lead
posture training and exercises.
to cord compression. In children younger than 6 years
Compensatory kyphosis is secondary to some other
with curves of less than 40 degrees, posterior spinal
deformity, usually increased lumbosacral lordosis.
fusion alone may prevent further progression. Older
This deformity, too, is correctable.
children or more severe curves may need combined
anterior and posterior fusion, and those with neuro-
Structural kyphosis logical complications will require cord decompression
as well as fusion.
Structural kyphosis is fixed and associated with
changes in the shape of the vertebrae. In children this Type II (failure of segmentation) Type II usually takes
may be due to congenital vertebral defects; it is also the form of an anterior intervertebral bar; as the poste-
seen in skeletal dysplasias such as achondroplasia and rior elements continue to grow, that segment of the
in osteogenesis imperfecta. Older children may spine gradually becomes kyphotic. The risk of neuro-
develop severe deformity secondary to tuberculous logical compression is much less, but if the curve is pro-
spondylitis. gressive a posterior fusion will be needed. 467
18 ADOLESCENT KYPHOSIS (JUVENILE
lordosis. The deformity cannot be corrected by
changes in posture. Movements are normal but tight
OSTEOCHONDROSIS; hamstrings often limit straight leg raising. A mild sco-
SCHEUERMANN’S DISEASE) liosis is not uncommon. Rare complications are spastic
paresis of the lower limbs and – with severe deformity
Scheuermann, in 1920, described a condition that he of the thorax – cardiopulmonary dysfunction.
called ‘juvenile dorsal kyphosis’, distinguishing it from In later life patients with thoracic kyphosis may
the more common postural (correctable) kyphosis. The develop lumbar backache. This has been attributed to
characteristic feature was a fixed round-back deformity chronic low back strain or facet joint dysfunction due
associated with wedging of several thoracic vertebrae. to compensatory hyperextension of the lumbar spine.
The term ‘vertebral osteochondritis’ was adopted be- In some cases, however, lumbar Scheuermann’s dis-
cause the primary defect appeared to be in the ossifi- ease itself may cause pain (see later).
cation of the ring epiphyses that define the peripheral
rims on the upper and lower surfaces of each vertebral X-ray
body. The true nature of the disorder is still not known;
the cartilaginous end-plates may be weaker than normal In lateral radiographs of the spine the vertebral end-
(perhaps due to a collagen defect) and are then dam- plates of several adjacent vertebrae (usually T6–10)
aged by pressure of the adjacent intervertebral discs appear irregular or fragmented. The changes are more
during strenuous activity. The normal curve of the marked anteriorly and one or more vertebral bodies
thoracic spine ensures that the anterior edges of the ver- may become wedge shaped. There may also be small
tebrae are subjected to the greatest stress and this is radiolucent defects in the subchondral bone
where the damage is greatest. Similar changes may oc- (Schmorl’s nodes), which are thought to be due to
cur in the lumbar spine, but here wedging is unusual. central (axial) disc protrusions.
The angle of deformity is measured in the same way
as for scoliosis, except that here the lateral x-ray is
Clinical features used and the lines mark the uppermost and lowermost
The condition starts at puberty and affects boys more affected vertebrae. Wedging of more than 5 degrees
often than girls. The parents notice that the child, an in three adjacent vertebrae and an overall kyphosis
otherwise fit teenager, is becoming increasingly angle of more than 40 degrees are abnormal. Mild
round-shouldered. The patient may complain of back- scoliosis is not uncommon.
ache and fatigue; this sometimes increases after the
end of growth and may become severe.
A smooth thoracic kyphosis is seen; it may produce
a marked hump. Below it is a compensatory lumbar
(a) (b)
(a) (b)
18.20 Scheuermann’s disease – operative treatment
A severe curve may need operation especially if, as in this
18.19 Scheuermanns disease (a) A young girl with girl (a), it is associated with chronic pain. (b) The same girl
marked exaggeration of the usual thoracic kyphosis. after operative correction and fixation with Wisconsin rods;
(b) X-ray examination showed the typical indentations in bone grafts were added and can be expected to produce
468 the vertebral end-plates and wedging of vertebral bodies. fusion after a year or two.
Differential diagnosis 18
Postural kyphosis Postural ‘round back’ is common in
adolescence. It is painless, and the patient can correct
the deformity voluntarily. The curve is a long one and
other postural defects are common. The x-ray appear-
The back
ance is normal.
Discitis, osteomyelitis and tuberculous spondylitis If the
changes are restricted to one intervertebral level, they
can be mistaken for an infective lesion. However,
infection causes more severe pain, may be associated
with systemic symptoms and signs and produces more
marked x-ray changes, including signs of bone erosion
and paravertebral soft-tissue swelling.
Spondyloepiphyseal dysplasia In mild cases this can
produce changes at multiple levels resembling those
of Scheuermann’s disease. Look for the characteristic
defects in other joints.
Outcome (a) (b)
The condition is often quite painful during adoles- 18.21 Lumbar Scheuermann’s disease (a) The x-ray
cence, but (except in the most severe cases) symptoms appearances of lumbar Scheuermann’s disease are often
mistaken for a fracture (or worse). The ‘fragmentation’
subside after a few years. There may be a recurrence anteriorly is due to abnormal ossification of the ring
of backache in later life, though overall disability is sel- epiphysis. (b) Schmorl’s nodes (arrows) may also be seen.
dom marked (Murray et al, 1993).
inability to undertake sustained bending, lifting and

Treatment carrying activities. There is nothing striking to see on
Curves of 40 degrees or less require only back-strength- clinical examination and it may be difficult to deter-
ening exercises and postural training. More severe cur- mine whether the backache is due to the Scheuer-
vature in a child who still has some years of growth mann disorder or to some other condition such as
ahead responds well to a period of 12–24 months in a spondylolysis or facet joint dysfunction.
brace that holds the lumbar spine flat and the thoracic
spine in ‘extension’ (decreased kyphosis). Check the TREATMENT
position by x-ray to ensure that the brace is effective. Treatment consists of muscle strengthening exercises
The older adolescent or young adult with a rigid and avoidance of excessive bending and lifting.
curve of more than 60 degrees may need operative
correction and fusion using a hook-rod system (mod-
ified Harrington or Cotrel-Dubousset). In severe KYPHOSIS IN THE ELDERLY
cases (kyphosis of greater than 75 degrees), an ante-
rior release operation and fusion should precede the Degeneration of intervertebral discs probably pro-
posterior fusion. Even then, the deformity is usually duces the gradually increasing stoop characteristic of
only partially corrected. the aged. The disc spaces become narrowed and the
vertebrae slightly wedged. There is little pain unless
osteoarthritis of the facet joints is also present.
THORACO-LUMBAR SCHEUERMANN’S
DISEASE
OSTEOPOROTIC KYPHOSIS
Vertebral end-plate defects are sometimes limited to
the lower thoracic and/or the lumbar spine. In mild Postmenopausal osteoporosis may result in one or more
cases the condition is usually asymptomatic and dis- compression fractures of the thoracic spine. Patients
covered only incidentally when x-rays are obtained for are usually in their 60s or 70s and may complain of
other reasons (see Fig. 18.21). In some cases, how- pain. Kyphosis is seldom marked. Often the main
ever, the patient (usually a teenager at the end of complaint is of lumbosacral pain, which results from
growth or a young adult) complains of back pain and the compensatory lumbar lordosis in an ageing, 469
18 TREATMENT
Treatment is symptomatic. Bed rest and spinal bracing
merely aggravate the osteoporosis. More recently,
fresh compression fractures are being treated by the
transpedicular injection of methacrylate or bone graft
substitute paste in order to stop further deformity and
control pain (‘vertebroplasty’) or to correct the wedge

deformity and maintain correction (‘kyphoplasty’).
The authors believe it is too early to recommend this
treatment as the long-term outcome and potential
complications have yet to be fully assessed.
SPINAL INFECTION
The axial skeleton accounts for 2–7 per cent of all
cases of osteomyelitis. Predisposing factors include
(a) (b) diabetes mellitus, malnutrition, substance abuse,
18.22 Osteoporotic kyphosis (a,b) Postmenopausal human immunodeficiency virus (HIV) infection,
osteoporosis often results in compressive wedging of the malignancy, long-term use of steroids, renal failure
thoracic vertebral bodies and a gradual increase in the and septicaemia.
natural thoracic kyphosis.
PYOGENIC OSTEOMYELITIS
osteoarthritic spine. Treatment is directed at the
underlying condition and may include hormone and Acute pyogenic infection of the spine is uncommon
bone mineral replacement therapy. and diagnosis and treatment are often unnecessarily
Senile osteoporosis affects both men and women. delayed. The elderly, chronically ill and immunodefi-
Patients are usually over 75 years of age, often cient patients are at greatest risk.
incapacitated by some other illness, and lacking exer-
cise. They complain of back pain, and spinal deformity
Pathology
may be marked. X-rays reveal multiple vertebral frac-
tures. It is important to exclude other conditions such Staphylococcus aureus is responsible in 50–60 per cent
as metastatic disease or myelomatosis. of all cases, but in immunosuppressed patients Gram-
18.23 Pyogenic osteomyelitis

and discitis Typical x-ray
features are loss of disc height,
irregularity of the disc ‘space’,
end-plate erosion and reactive
sclerosis. Progressive changes
are shown in (a) and (b).
Reactive bone changes, shown
in (c), may end with fusion at
the affected level. In many cases
it is impossible to tell whether
the infection began in the disc
or in the adjacent bone.
470 (a) (b) (c)

negative organisms such as Escherichia coli and may help with diagnosis, but often no organism is 18
Pseudomonas are the most common. The usual sources found.
of infection are: (1) haematogenous spread from a dis-
tant focus of infection or (2) inoculation during inva-
Other investigations
sive procedures (spinal injections and disc operations).
The infection usually begins in the vertebral end- The white cell count, C-reactive protein (CRP) level
The back
plates with secondary spread to the disc and adjacent and erythrocyte sedimentation rate (ESR) are usually
vertebra. It may also spread along the anterior longi- elevated, and antistaphylococcal antibodies may be
tudinal ligament to an adjacent vertebra, or out- present in high titres. Agglutination tests for Salmo-
wards into the paravertebral soft tissues: from the nella and Brucella should be performed, especially in
thoracic spine along the psoas to the groin; from the endemic regions and in patients who have recently vis-
lumbar region to the buttock, the sacroiliac joint or ited these areas. Blood culture is essential in patients
the hip. who are febrile though it is often negative in the early
The spinal canal is rarely involved but when it is, in stages of infection.
the form of an epidural abscess, that is a surgical emer-
gency! Despite rapid surgical decompression, the
patient is often left with some degree of permanent
Treatment
paralysis. If the blood culture is negative a closed needle biopsy is
performed for bacteriological culture and tests for
antibiotic sensitivity. Treatment is started on the basis
Clinical features of a clinical diagnosis of infection and includes bed
Localized pain – the cardinal symptom – is often rest, pain relief and intravenous antibiotic administra-
intense, unremitting and associated with muscle tion using a ‘best guess’ preparation that can be
spasm and restricted movement. There may also be changed once the laboratory results and sensitivities
point tenderness over the affected vertebra. Inter- are known. As methicillin-resistant Staphylococcus
costal neuralgia is a frequent symptom with thoracic aureus (MRSA) has become a common infecting
spine involvement. agent, vancomycin or linezolid may be required.
The patient may give a history of some invasive Intravenous antibiotics are continued for 4–6
spinal procedure or a distant infection during the pre- weeks; if there is a good response (clinical improve-
ceding few weeks. A careful history and general exam- ment, a falling CRP and ESR. and a normal white cell
ination are essential to exclude a focus of infection count), oral antibiotics are then used for a further 6–
(skin, ENT, chest, pelvis). 8 weeks and the patient is mobilized in a spinal brace.
Systemic signs such as pyrexia and tachycardia are The duration of antibiotic treatment depends on the
often present but not particularly marked. In children clinical, haematological and radiological findings.
the diagnosis can be particularly difficult; often they During this period nutritional support and manage-
have an awkward gait with a stiff spine, or if the lum- ment of co-morbidities are essential in ensuring a suc-
bar spine is involved they can present with abdominal cessful outcome.
symptoms and signs. Operative treatment is seldom needed. The indica-
tions for an open biopsy and decompression are: (1)
failure to obtain a positive yield from a closed needle
Imaging biopsy and a poor response to conservative treatment;
X-rays may show no change for several weeks; if the (2) the presence of neurological signs; (3) the need to
diagnosis is delayed, the examination should be drain a soft-tissue abscess. An anterior approach is
repeated. Early signs are loss of disc height, irregular- preferred; necrotic and infected material is removed
ity of the disc space, erosion of the vertebral end-plate and, if necessary, the cord is decompressed. The ante-
and reactive new bone formation. Soft-tissue swelling rior column defect is reconstructed with rib or iliac
may be visible. The early loss of disc height distin- grafts. If the spine is unstable, posterior fixation may
guishes vertebral osteomyelitis from metastatic dis- be necessary. Postoperatively the spine is supported
ease, where the disc can remain intact despite in a brace until healing occurs. In the elderly and
advanced bony destruction. in immunocompromised patients a posterolateral
Radionuclide scanning will show increased activity extraplueral/retroperitoneal decompression and
at the site but this is non-specific. instrumentation is effective. For a primary epidural
MRI may show characteristic changes in the verte- abscess, laminectomy is indicated.
bral end-plates, intervertebral disc and paravertebral The outcome (with prompt and effective treatment)
tissues; this investigation is highly sensitive but not is usually favourable. Spontaneous fusion of infected
specific. vertebrae is a common radiological feature of healed
Similar features may be seen in discitis. Needle biopsy staphylococcal osteomyelitis. 471
18 DISCITIS TUBERCULOSIS
Infection limited to the intervertebral disc is rare and The spine is the most common site of skeletal tuber-
when it does occur it is usually due to direct inocula- culosis (TB), and accounts for 50 per cent of all
tion following discography, chemonucleolysis or dis- musculoskeletal TB. It is thought that there are
cectomy. The vertebral end-plates are rapidly attacked approximately 2 million people with spinal tuber-
and the infection then spreads into the vertebral body. culosis worldwide.
Clinical features and investigations Pathology

With direct infection there is always a history of some Blood-borne infection usually settles in a vertebral
invasive procedure. Acute back pain and muscle spasm body adjacent to the intervertebral disc. Bone de-
following an injection into the disc should never be struction and caseation follow, with infection spreading
attributed merely to the irritant effect of the injection. to the disc space and the adjacent vertebrae. A par-
Systemic features are usually mild, but the ESR is ele- avertebral abscess may form, and then track along mus-
vated. cle planes to involve the sacro-iliac or hip joint, or
In children the infection is assumed to be blood- along the psoas muscle to the thigh. As the vertebral
borne. There may be a history of a flu-like illness fol- bodies collapse into each other, a sharp angulation
lowed by back pain, muscle spasm and severe (gibbus or kyphos) develops. There is a major risk of
limitation of movement. X-rays, radioscintigraphy and cord damage due to pressure by the abscess, granula-
MRI show the same features as in pyogenic spondyli- tion tissue, sequestra or displaced bone, or (occasion-
tis. ally) ischaemia from spinal artery thrombosis.
With healing, the vertebrae recalcify and bony fusion
may occur between them. Nevertheless, if there has
Treatment been much angulation, the spine is usually ‘unsound’,
Prevention is always better than cure. Following an and flares are common, resulting in further illness and
injection into the disc, a broad-spectrum antibiotic further vertebral collapse. With progressive kyphosis
should be administered intravenously. Non-iatrogenic there is again a risk of cord compression.
discitis is usually self-limiting. During the acute stage
bed rest and analgesics are essential. If symptoms do
not resolve rapidly, a needle biopsy is advisable. Only
Clinical features
if there are signs of abscess formation or cord or nerve There is usually a long history of ill-health and back-
root pressure is surgical evacuation or decompression ache; in late cases a gibbus deformity is the dominant
indicated. This is rarely necessary. feature. Concurrent pulmonary TB is a feature in
(a) (b) (c) (d)
18.24 Tuberculosis of the spine (a) Early x-ray changes with loss of disc space. (b) Young patient with advanced
tuberculous deformity. (c) X-ray showing vertebral collapse and severe kyphosis. (d) X-ray appearance of a psoas abscess in
472 the paravertebral tissues.
most children under 10 years with thoracic spine shadows may be due either to oedema, swelling or a 18
involvement. Occasionally the patient may present paravertebral abscess. The radiological picture may
with a cold abscess pointing in the groin, or with mimic those of other infections including fungal infec-
paraesthesiae and weakness of the legs. There is local tions and parasitic infestations. A chest x-ray is essential.
tenderness in the back and spinal movements are With healing, bone density increases, the ragged
restricted. appearance disappears and paravertebral abscesses may
The back
In cervical spine disease dyspnoea and dysphagia are undergo resolution or fibrosis or calcification.
features of advanced infection, especially in children; MRI and CT scans are invaluable in the investiga-
these patients present with a stiff painful neck. Chil- tion of hidden lesions, involvement of posterior verte-
dren under 10 years of age with thoracic spine TB bral elements, paravertebral abscesses, an epidural
usually develop a pectus carinatum (‘pigeon chest’) abscess and cord compression. Myelography is appro-
deformity. priate when these facilities are not available.
Neurological examination may show motor and/or
sensory changes in the lower limbs. As spinal tuber-
culosis is found mostly in the thoracic spine, spastic
paraparesis is a common presentation in adults. The Mantoux test may be positive and in the acute
stage the ESR is raised. In patients with no neurolog-
ATYPICAL FEATURES ical signs a needle biopsy is recommended to confirm
Even in areas where tuberculosis is no longer as com- the diagnosis by histological and microbiological
mon as it was in the past, it is important to be alert to investigations. If this does not provide a firm diagno-
the possibility of this diagnosis. The task is made sis, tissue should be obtained by open operation. If
harder when the patient presents with atypical features: there are signs of neurological involvement, operative
debridement and decompression of the spinal cord
• Lack of deformity, e.g. a patient with a primary
will be required.
epidural abscess
Patients with HIV infection (usually showing gen-
• Involvement of only the posterior vertebral ele-
eralized lymphadenopathy, skin and mucocutaneous
ments
lesions and marked weight loss) should be referred for
• Infection confined to a single vertebral body
voluntary counselling and testing (VCT). If positive,
• Involvement of multiple vertebral bodies and pos-
the CD4/CD8 count should be monitored with TB
terior elements (especially in HIV-positive patients)
and antiretroviral therapy.
resulting in a kyphoscoliosis.
POTT’S PARAPLEGIA Differential diagnosis

Paraplegia is the most feared complication of spinal
Spinal tuberculosis must be distinguished from other
tuberculosis. Early-onset paresis (usually within 2 years
causes of vertebral pathology, particularly pyogenic
of disease onset) is due to pressure by inflammatory
and fungal infections, malignant disease and parasitic
oedema, an abscess, caseous material, granulation tis-
infestations such as hydatid disease. Disc space col-
sue or sequestra. The patient presents with lower limb
lapse is typical of infection; disc preservation is typical
weakness, upper motor neuron signs, sensory dys-
of metastatic disease. Metastases may cause vertebral
function and incontinence. CT and MRI may reveal
body collapse similar to that seen in TB but, in con-
cord compression. In these cases the prognosis for
trast to tuberculous spondylitis, the disc space is usu-
neurological recovery following surgery is good. Late-
ally preserved.
onset paresis is due to direct cord compression from
increasing deformity, or (occasionally) vascular insuf-
ficiency of the cord; recovery following decompres- Treatment
sion is poor.
The objectives are to: (1) eradicate or at least arrest
the disease; (2) prevent or correct deformity; (3) pre-
vent or treat the major complication – paraplegia.
Imaging Antituberculous chemotherapy (rifampicin 600 mg
The entire spine should be x-rayed, because vertebrae daily plus isoniazid 300 mg daily plus pyrazinamide
distant from the obvious site may also be affected with- 2 g daily) is as effective as any other method (includ-
out any obvious deformity. The earliest signs of infec- ing surgical debridement) in stemming the disease.
tion are local osteoporosis of two adjacent vertebrae and These drugs must be given in combination for
narrowing of the intervertebral disc space, with fuzzi- 6 months, dropping the pyrazinamide after the first
ness of the end-plates. Progressive disease is associated 2 months. The dosages listed are for adults of average
with signs of bone destruction and collapse of adjacent weight. Because so much of current tuberculosis is a
vertebral bodies into each other. Paraspinal soft-tissue complication of acquired immune deficiency syn- 473
18
(a)
(b) (c) (d)
18.25 Spinal tuberculosis – MRI features Scanning in several planes shows details that cannot be seen in plain x-rays.
(a) Sagittal MR images of advanced tuberculous infection with abscess formation beneath the anterior longitudinal ligament.
(b,c) Axial images showing psoas abscesses communicating across the front of the spine. (d) In countries where TB is
endemic, additional active lesions can be detected by MRI in almost 40 per cent of patients presenting with ‘local’ lesions.
drome (AIDS), resistant mycobacteria are an increas- not available, or where the technical problems are
ing problem. Ethionamide and streptomycin may too daunting (e.g. in lumbosacral tuberculosis) –
have to be substituted for isoniazid. provided there is no abscess that needs to be
However, conservative treatment alone carries the drained.
risk of progressive kyphosis if the infection is not • Operative treatment – is indicated: (1) when there
quickly eradicated. Anterior resection of diseased tis- is an abscess that can readily be drained; (2) for
sue and anterior spinal fusion with a strut graft offers advanced disease with marked bone destruction and
the double advantage of early and complete eradica- threatened or actual severe kyphosis; (3) neurolog-
tion of the infection and prevention of spinal defor- ical deficit including paraparesis that has not
mity (Figure 18.26). After weighing up the pros and responded to drug therapy.
cons, the following approach is advocated:
Through an anterior approach, all infected and
• Ambulant chemotherapy alone – is suitable for early necrotic material is evacuated or excised and the gap
or limited disease with no abscess formation or neu- is filled with iliac crest or rib grafts that act as a strut.
rological deficit. Treatment is continued for 6–12 If several levels are involved, anterior or posterior fix-
months, or until the x-ray shows resolution of the ation and fusion may be needed for additional stabil-
bone changes. Therapeutic compliance is some- ity. Children who are growing and are seen to be at
times a problem. risk of developing severe kyphosis may need fusion of
• Continuous bed rest and chemotherapy – may be used the posterior elements to minimize the expected
for more advanced disease when the necessary skills deformity. Antituberculous chemotherapy is still nec-
474 and facilities for radical anterior spinal surgery are essary, of course.
they may also affect a normal host. Aspergillosis and 18
Cryptococcus are airborne fungi that initially affect the
lungs; the spine is involved by haematogenous spread.
In children with chronic granulomatous disease, tho-
racic spine involvement is due to contiguous spread
from the lungs. The presentation, clinical findings and
The back
radiographic features may mimic those of TB. The
chest X-ray may show a fungal ball or pneumonia. The
diagnosis is made by sputum examination and bron-
choscopy. The immunodiffusion test is specific for As-
pergillosis and the latex agglutination test for Crypto-
coccus. A biopsy is performed to confirm the diagnosis.
Treatment
Neurological deficit is an indication for operative
decompression. Specific treatment includes 5-flucyto-
sine and amphotericin B, which act synergistically.
Synthetic oral antifungals (ketoconazole, fluconazole,
(a) (b) itraconazole) are well absorbed and the serum and
cerebrospinal fluid (CSF) concentrations are high.
18.26 Spinal tuberculosis – operative treatment Concurrent treatment of the underlying immuno-
(a) Marked bone collapse and kyphosis, threatening
neurological complications. (b) After debridement and comprised state is essential.
spinal fusion with a rib strut graft.
PARASITIC INFESTATION
HUMAN IMMUNODEFICIENCY VIRUS AND
SPINAL TUBERCULOSIS The commonest parasitic infestation affecting the spine
is due to the cestode worm Echinococcus granulosis,
One of the main reasons for the resurgence of TB, which causes hydatid disease. It is encountered mainly
especially in the developing world, is the spread of in areas where sheep are raised: Australasia, South Amer-
HIV. Spinal TB, which is an extrapulmonary focus, is ica, parts of Africa, Wales and Iceland. The definitive
AIDS defining. host is the dog and as well as other canine animals.
These patients are prone to developing opportunis- The sheep is the intermediate host and humans are
tic infections and atypical mycobacterial infections affected by the ingestion of ova that are usually carried
(Mycobacterium intracellulare, M. avium, M. fortui- in the dog’s excreta or fur. The embryo worm enters
tum). The tuberculous infection usually involves mul- the human host by being either ingested through fae-
tiple vertebrae and results in severe deformity. A cal contamination or by inhalation of dessicated parti-
primary epidural abscess is not uncommon. cles in dust. In that way the embryos come to lodge in
Decompression and stabilization for neurological the liver and the lungs, but in about 10 per cent of cases
deficit are performed through an extrapleural pos- there is dissemination to other sites, including the
terolateral approach with instrumentation to mini- bones (mainly the spine, skull and long bones) where
mize pulmonary complications. A primary epidural hydatid cysts develop in about 1 per cent of cases.
abscess is drained through a laminectomy. Hydatid disease is usually picked up in childhood but
Postoperatively antituberculous therapy and anti- it may be many years before the diagnosis is made. The
retroviral treatment are commenced. Compliance presentation and clinical features are similar to those of
with treatment and regular monitoring of viral loads other forms of spondylitis. X-rays may reveal a translu-
and CD4/CD8 counts are essential to ensure a suc- cent area with a sclerotic margin in the affected verte-
cessful outcome. bral body. In untreated cases this can lead to bone de-
struction. Neurological deficit, the difficulty in
eradicating the disease and the tendency to recurrence
FUNGAL INFECTION make for significant morbidity and mortality.
Systemic treatment is with albendazole, which is
These are opportunistic infections occurring in an active against the larvae and the cysts; three cycles of
immunocompromised host (e.g. due to HIV, malig- 25 days each is the usual recommendation. Operative
nancy, steroid therapy or chronic granulomatous treatment to achieve spinal decompression may be
disease) and a patient with extensive burns; however, called for; spillage of cyst contents must be avoided. 475
18 The prognosis is generally poor when the liver and majority of cases the backache is associated with
lungs are affected. degeneration of the intervertebral discs in the lower
lumbar spine. This is an age-related phenomenon that
occurs in over 80 per cent of people who live for more
than 50 years and in most cases it is asymptomatic.
NON-INFECTIVE
INFLAMMATORY DISEASE
Pathology
Ankylosing spondylitis and seronegative spondy- With normal ageing the disc gradually dries out: the
loarthropathies are dealt with in Chapter 3. nucleus pulposus changes from a turgid, gelatinous
bulb to a brownish, desiccated structure. The annulus
fibrosus develops fissures parallel to the vertebral end-
plates running mainly posteriorly, and small hernia-
DEGENERATIVE DISORDERS OF tions of nuclear material squeeze into and through the
THE SPINE annulus. Disc cells proliferate and collect into clusters,
then die at an increased rate. Glycosaminoglycans pro-
duction is diminished, leading to poor water retention
INTERVERTEBRAL DISC
and gradual ‘drying out’ of the disc (Roberts et al.,
DEGENERATION 2006). This process begins surprisingly early in life
and increases gradually with age. The discs flatten down
Lumbar backache is one of the most common causes and bulge slightly beyond the margins of the vertebral
of chronic disability in western societies, and in the bodies. Where they protrude against the ligaments,
18.27 Disc lesions –

pathology (a,b) Transverse
and sagittal sections
through a young (teenage)
intervertebral disc. The
nucleus is soft,
homogeneous and almost
translucent. The annulus is
composed of regular
lamellae of fibrocartilage.
(c,d) Mature (50-year-old)
normal disc. The nucleus is
(a) (b) more fibrous and less
homogeneous. The annulus
is thickened and the
vertebral body and end-
plates are intact.
(e) Degenerating disc,
which is markedly flattened
with break-up of the
nucleus and disruption of
the vertebral body end-
plates. (f) Young disc
stained with analine blue
dye to demonstrate a fissure
(c) (d) extending posteriorly
through the annulus
fibrosis.
476 (e) (f)

18
The back
(a) (b) (c) (d)
18.28 Spondylosis and osteoarthritis Typical x-ray features are (a) narrowing of the intervertebral space and anterior
‘osteophytes’. (b) Other features are slight retrolisthesis and a dark (vacant) area in the disc space – the ‘vacuum sign’ –
better demonstrated in the axial CT (c), which also shows the hypertrophic osteoarthritis of the facet joints. (d) In advanced
cases there are well marked signs of osteoarthritis.
reactive new bone formation produces bony ridges marginal ‘osteophyte’ formation – appear relatively
(erroneously called ‘osteophytes’, because in two- late. Other secondary changes such as vertebral dis-
dimensional x-ray images they do indeed look like placement and facet joint osteoarthritis may also
osteophytic projections). In the adjacent vertebrae the become apparent, making it increasingly difficult to
end plates ossify and become sclerotic; fatty change ascribe the patient’s symptoms to any particular
occurs in the subchondral bone marrow. The picture as abnormality. Indeed, even if there are no overt signs
a whole is referred to as spondylosis. A classification of apart from the primary discogenic changes, it cannot
the age-related changes in lumbar discs appears in the be determined for certain that the disc pathology is
paper by Boos et al. (2002). the cause of a patient’s backache, because disc degen-
eration and non-specific low-back pain are both
extremely common in older people. It is also not pos-
Secondary effects
sible to prognosticate about whether an asymptomatic
Once the degenerative process gets going, secondary individual with clear x-ray signs of disc degeneration
changes ensue. Displacement of the facet joints and for- will in the future develop disabling backache.
ward or backward shifts of adjacent vertebral bodies (as
shown in x-ray images) are often interpreted as signs of
‘segmental instability’. This, combined with increased
stress in the facet joints, may ultimately lead to os-
teoarthritis of these small synovial joints. If the changes
are marked, new bone formation may narrow the lat-
eral recesses of the spinal canal and the intervertebral
foramina causing root canal stenosis and, in some cases,
spinal stenosis. Thickening of the ligamentum flavum
and bulging of the disc annulus contribute further to
the circumferential nature of acquired stenosis.
Clinical features
As noted earlier, disc degeneration of itself is usually
asymptomatic. When symptoms such as chronic back-
ache or low-back pain on strenuous effort do appear
they may well be due to the secondary effects of disc
collapse rather than the disc degeneration per se.
These are described later. (a) (b)
18.29 Conditions resembling spondylosis

X-Rays (a) Forestier’s disease: at first sight this looks like
osteoarthritic spondylosis; there are large spurs at multiple
Radiographic features of intervertebral disc degenera- levels, but the disc spaces are usually preserved.
tion – typically flattening of the disc ‘space’ and (b) Ochronosis: intervertebral calcification is characteristic. 477
18 For the early features of disc degeneration, includ- produce a classification through grades I to V of
ing those that can be demonstrated in asymptomatic increasing severity (Pfirrmann et al., 2001), yet even
individuals, more advanced imaging techniques must that method has been found to be wanting on the
be considered (Boos et al., 2002). grounds that it provides a discontinuous scale of pro-
gressive degeneration (Haughton, 2006).
A significant secondary change, evidently arising
MRI
from altered loading characteristics of the degenerat-

The most obvious change on MRI is bulging of the ing disc, was described by Modic et al. (1989).
annulus fibrosus in both sagittal and axial projections. Oedema, fatty infiltration of the marrow and fibrosis
However, subtle changes such as diminished thickness in the subchondral bone adjacent to the vertebral
and reduced signal intensity of the degenerating disc, end-plates produce varying imaging characteristics
or small tears and fissures can also be distinguished that now form the basis of the Modic classification
and counted. These appearances have been used to (Figure 18.30). Although these changes are rarely
encountered in asymptomatic individuals, it is also
true that most patients with proven disc pathology do
not show Modic changes; i.e. as diagnostic markers
the Modic signs have relatively low sensitivity.
Treatment
Asymptomatic lumbar disc degeneration (often dis-
covered incidentally during x-ray examination for
other conditions) does not necessarily presage the
future onset of symptoms and does not need any
treatment. The management of patients with chronic
‘non-specific’ low-back pain, with or without obvious
(a) (b) signs of disc degeneration, is discussed on page 487.
Secondary features of disc degeneration, such as ver-
tebral displacement and facet joint osteoarthritis may
need focussed management, sometimes including
operative treatment.
ACUTE INTERVERTEBRAL DISC

PROLAPSE
(c) (d)
Acute disc herniation (prolapse, rupture) is much less
common but considerably more dramatic than
chronic degeneration. Physical stress (a combination
of flexion and compression) is the proximate cause
but, even at L4/5 or L5/S1 (where stress is most
severe) it seems unlikely that a disc would rupture
unless there was also some disturbance of the
hydrophilic properties of the nucleus. A ‘protrusion’ is
a posteriorly bulging disc with some outer annulus
intact. When rupture does occur, fibrocartilaginous
disc material is extruded posteriorly (‘extrusion’) and
usually bulges to one or other side of the posterior
(e) (f)
longitudinal ligament. With a complete rupture, part
of the nucleus may sequestrate and lie free in the
18.30 MRI – Modic types of vertebral change Sagittal spinal canal or work its way into the intervertebral
T1 and T2 weighted images of (a,b) Type 1, (c,d) Type 2 foramen (sequestration).
and (e,f) Type 3 Modic changes in lumbar vertebral end-
plates. Type 1 suggests oedema, but this may also occur in A large central rupture may cause compression of
infection and metastatic disease. Type 2 suggests fatty the cauda equina. A posterolateral rupture presses on
478 change; Type 3 is due to bony sclerosis. the nerve root proximal to its point of exit through
Clinical features 18
Acute disc prolapse may occur at any age, but is
uncommon in the very young and the very old. The
patient is usually a fit adult aged 20–45 years. Typi-
cally, while lifting or stooping he has severe back pain
Normal disc
The back
and is unable to straighten up. Either then or a day or
two later pain is felt in the buttock and lower limb
(sciatica). Both backache and sciatica are made worse
Protrusion by coughing or straining. Later there may be paraes-
Increased nuclear thesia or numbness in the leg or foot, and occasion-
pressure causing ally muscle weakness. Cauda equina compression is
bulging
rare but may cause urinary retention and perineal
numbness.
Extrusion The patient usually stands with a slight list to one side
Ruptured annulus (‘sciatic scoliosis’). Sometimes the knee on the painful
and ligament side is held slightly flexed to relax tension on the sciatic
nerve; straightening the knee makes the skew back
more obvious. All back movements are restricted, and
during forward flexion the list may increase.
Sequestration There is often tenderness in the midline of the low
back, and paravertebral muscle spasm. Straight leg
raising is restricted and painful on the affected side;
dorsiflexion of the foot and bowstringing of the lateral
Degeneration and popliteal nerve may accentuate the pain. Sometimes
joint displacement
raising the unaffected leg causes acute sciatic tension
on the painful side (‘crossed sciatic tension’). With a
high or mid-lumbar prolapse the femoral stretch test
may be positive.
Neurological examination may show muscle weak-
ness (and, later, wasting), diminished reflexes and sen-
sory loss corresponding to the affected level. L5
18.31 Disc lesions – pathology From above, downwards: impairment causes weakness of knee flexion and big
an abnormal increase in pressure within the nucleus causes
toe extension as well as sensory loss on the outer side
splitting and bulging of the annulus; the posterior segment
may rupture, allowing disc material to extrude into the of the leg and the dorsum of the foot. Normal reflexes
spinal canal; with chronic degeneration (lowest level) the at the knee and ankle are characteristic of L5 root
disc space narrows and the posterior facet joints are compression. Paradoxically, the knee reflex may
displaced, giving rise to osteoarthritis. appear to be increased, because of weakness of the
antagonists (which are supplied by L5). S1 impair-
ment causes weak plantar-flexion and eversion of the
foot, a depressed ankle jerk and sensory loss along the
the intervertebral foramen; thus a herniation at L4/5 lateral border of the foot. Occasionally an L4/5 disc
will compress the fifth lumbar nerve root, and a her- prolapse compresses both L5 and S1. Cauda equina
niation at L5/S1, the first sacral root. Sometimes a compression causes urinary retention and sensory loss
local inflammatory response with oedema aggravates over the sacrum.
the symptoms.
Acute back pain at the onset of disc herniation
Imaging
probably arises from disruption of the outermost lay-
ers of the annulus fibrosus and stretching or tearing of X-rays are helpful, not to show an abnormal disc space
the posterior longitudinal ligament. If the disc pro- but to exclude bone disease. After several attacks the
trudes to one side, it may irritate the dural covering of disc space may be narrowed and small osteophytes
the adjacent nerve root causing pain in the buttock, appear.
posterior thigh and calf (sciatica). Pressure on the Myelography (radiculography) using iopamidol
nerve root itself causes paraesthesia and/or numbness (Niopam) is a fairly reliable method of confirming the
in the corresponding dermatome, as well as weakness nerve root distortion resulting from a disc protrusion,
and depressed reflexes in the muscles supplied by that localizing it and excluding intrathecal tumours; how-
nerve root. ever, it carries a significant risk of unpleasant side 479
18 Differential diagnosis
The full-blown syndrome is unlikely to be misdiag-
nosed, but with repeated attacks and with lumbar
spondylosis gradually supervening (see later), the fea-
tures often become atypical. There are two diagnostic
aphorisms:
• Lower limb pain is not always the sciatica of root
compression; frequently it is referred pain from back-
ache and can occur in other lumbar spine disorders.
• Disc rupture affects at most two neurological levels;
if multiple levels are involved, suspect a cauda equina
compression (see box) or a neurological disorder.
Inflammatory disorders such as infection or ankylosing
spondylitis, cause severe stiffness, a raised ESR and
erosive changes on x-ray.
Vertebral tumours cause severe pain, marked muscle
spasm and pain through the night. With metastases
the patient is ill, the ESR is raised and the x-rays show
bone destruction or sclerosis.
Nerve tumours such as a neurofibroma of the cauda
equina, may cause ‘sciatica’ but pain is continuous.
(a) (b)
Advanced imaging will confirm the diagnosis.
18.32 Lumbar disc – signs (a) The patient has a sideways
list or tilt. (b) If the disc protrudes medial to the nerve root
the tilt is towards the painful side (to relieve pressure on FEATURES OF CAUDA EQUINA SYNDROME
the root); with a far lateral prolapse (lower level) the tilt is
away from the painful side. Bladder and bowel incontinence
Perineal numbness
effects, such as headache (in over 30 per cent), nausea
Bilateral sciatica
and dizziness. Myelography is unsuitable for diagnos-
ing a far lateral disc protrusion (lateral to the inter- Lower limb weakness
vertebral foramen); if this is suspected CT or MRI is
Crossed straight-leg raising sign
essential.
CT and MRI are more reliable than myelography Note: Scan urgently and operate urgently if a large
and have none of its disadvantages. These are now the central disc is revealed.
preferred methods of spinal imaging.
(a) (b) (c)
18.33 Disc prolapse – imaging (a) Radiculogram in which the gap in the contrast medium (arrow) shows where a disc
has protruded. (b) CT scan showing how disc protrusion can obstruct the intervertebral foramen. (c) MRI, axial view,
480 showing the relationship of the disc protrusion to the dural sac and intervertebral foramen.
Treatment rare. Recurrent prolapse with sciatica is more com- 18
mon and may require revision decompression surgery.
Heat and analgesics soothe, and exercises strengthen Microdiscectomy is essentially similar to the standard
muscles, but there are only three ways of treating the posterior operation, except that the exposure is very
prolapse itself – rest, reduction or removal, followed by limited and the procedure is carried out with the aid
rehabilitation: of an operating microscope. Morbidity and length of
The back
Rest With an acute attack the patient should be kept hospitalization are certainly less than with conven-
in bed, with hips and knees slightly flexed. A non- tional surgery, but there are drawbacks: careful x-ray
steroidal anti-inflammatory drug is useful. control is needed to ensure that the correct level is
entered; intraoperative bleeding may be difficult to
Reduction Continuous bed rest and traction for 2 control; there is a considerable ‘learning curve’ and
weeks may reduce the herniation. If the symptoms the inexperienced operator risks injuring the dura or a
and signs do not improve during that period, an stretched nerve root, or missing essential pathology;
epidural injection of corticosteroid and local anaes- there is a slightly increased risk of disc space infection,
thetic may help. and prophylactic antibiotics are advisable.
Chemonucleolysis dissolution of the nucleus pulposus Rehabilitation After recovery from an acute disc rup-
by percutaneous injection of a proteolytic enzyme ture, or disc removal, the patient is taught isometric ex-
(chymopapain) – is in theory an excellent way of ercises and how to lie, sit, bend and lift with the least
reducing a disc prolapse. However, controlled studies strain. Ideally this should be done as part of an educa-
have shown that it is less effective (and potentially tion programme in a ‘back school’ (Zachrisson, 1981).
more dangerous) than surgical removal of the disc
material (Ejeskär et al., 1982).
Removal The indications for operative removal of a PERSISTENT POSTOPERATIVE
prolapse are: (1) a cauda equina compression BACKACHE AND SCIATICA
syndrome – this is an emergency; (2) neurological
deterioration while under conservative treatment;
(3) persistent pain and signs of sciatic tension (espe- Persistent symptoms after operation may be due to:
cially crossed sciatic tension) after 2–3 weeks of (1) residual disc material in the spinal canal; (2) disc
conservative treatment. The presence of a prolapsed prolapse at another level; (3) nerve root pressure by a
disc, and the level, must be confirmed by CT, MRI or hypertrophic facet joint or a narrow lateral recess
myelography before operating. Surgery in the absence (‘root canal stenosis’). After careful investigation, any
of a clear preoperative diagnosis is usually unreward- of these may call for re-operation; but second proce-
ing. The two operations most widely performed are dures do not have a high success rate – third and
laminotomy and microdiscectomy. fourth procedures still less.
Laminotomy is nowadays preferred to the older,
more destructive type of laminectomy. Ligamentum
flavum on the relevant side and at the relevant level is ARACHNOIDITIS
removed, if necessary with some margin of the bor-
dering laminae and medial third of the facet joint. The Diffuse back pain and vague lower limb symptoms
dura and nerve root are then gently retracted towards such as ‘cramps’, ‘burning’ or ‘irritability’ sometimes
the midline and the pea-like disc bulge or extrusion/ appear after myelography, epidural injections or disc
sequestration is displayed. If the outer layer of the operations. This diagnosis is now rarely made and is
annulus is seen still to be intact, it is incised and the believed to have been a complication of oil-based con-
mushy disc material plucked out piecemeal with pitu- trast media used in myelography 30 years ago. There
itary forceps. The nerve is traced to its point of exit in may also be sphincter dysfunction and male impo-
order to exclude other pathology. tence. Patients complain bitterly and many are
A far lateral disc protrusion is very difficult to labelled neurotic. However, in some cases there are
expose by the standard interlaminar approach without electromyographic abnormalities, and dural scarring
damaging the facet joint. An intertransverse approach with obliteration of the subarachnoid space can be
may be more suitable for these cases. demonstrated by MRI or at operation.
The main intraoperative complication is bleeding Treatment is generally unrewarding. Corticosteroid
from epidural veins. This is less likely to occur if the injections at best give only temporary relief, and sur-
patient is placed on his side or in the kneeling posi- gical ‘neurolysis’ may actually make matters worse.
tion, thus minimizing pressure on the abdomen and a Sympathetic management in a pain clinic, psycholog-
rise in venous pressure. The major postoperative com- ical support and a graduated activity programme are
plication is disc space infection, but fortunately this is the best that can be offered. 481
18 FACET JOINT DYSFUNCTION In the established case, the patient gives a history of
intermittent backache related to spells of hard work,
standing, bending, or walking a lot, or sometimes
Facet joint abnormalities that have been demonstrated after sitting in one position during a long journey.
at operation or necropsy are: (1) anatomical variations Most patients find relief by lying down, or sitting
that limit articular movement; (2) anatomical variations and resting when backache appears during strenuous
that permit excessive movement; (3) malapposition of activity. A suspicion of ‘instability’ is favoured inas-
the articular surfaces secondary to loss of disc height; much as the patient achieves relief through recum-
(4) softening and fibrillation of the facet articular car- bency. However, a large minority of patients describe
tilage; (5) loose bodies in the facet joint; (6) synovial a contrasting pattern: pain aggravated by rest and
thickening; (7) classical changes of osteoarthritis, pro- recumbency and partially relieved by movement; they
gressing from fibrillation to complete loss of articular usually manage full forward bending without discom-
cartilage and osteophytic thickening of the facets. fort but backward bending (which stresses the facet
Some of these abnormalities are associated with joints more) is dramatically halted by pain. This is
radiologically demonstrable vertebral shift; in others reminiscent of ‘arthritic’ pain in other synovial joints
the abnormal movement is considered to be more and could signify the onset of osteoarthritis (OA) in
subtle and it is not surprising that this has given rise the facet joints. Interestingly, pathological features of
to semantic arguments about the concept (and indeed OA have been described in specimens excised at sur-
the very existence) of a condition called ‘segmental gery during operations for intractable back pain of
instability’, which could give rise to otherwise inexpli- this pattern (Eisenstein and Parry, 1987).
cable low-back pain. With time, pain becomes more constant and can
The concept of ‘segmental instability’ was elabo- sometimes be temporarily relieved only by manipula-
rated on more than 25 years ago (Kirkaldy-Willis and tion, local warmth and anti-inflammatory drugs; at
Farfan, 1982) in an attempt to explain the back pain that stage there are likely to be x-ray signs of
on the basis of disordered biomechanics of the spine osteoarthritis in the facet joints.
(or a spinal segment). It was widely recognized that Examination during a painful episode may reveal
patients with chronic backache may develop intermit- muscle spasm, local tenderness and restriction of back
tent episodes of severe pain with radiation into the movements, but little else. Occasionally the patient
buttock and thighs in the absence of any sign of inter- presents with a ‘locked back’, which is dramatically
vertebral disc prolapse. These attacks are usually trig- relieved by skilful manipulation.
gered by fairly modest lifting strains, but they can also Between acute attacks, physical signs are less obvi-
occur ‘spontaneously’. Kirkaldy-Willis suggested that ous and often unconvincing. The range of movement
the symptoms are due to abnormal movement and may not be much restricted, but the pattern of move-
mechanical stress at the posterior facet joints, arising ment is often recognizable: characteristically the
from local injury or non-specific ‘dysfunction’ of the patient bends forward quite easily but when asked to
lower lumbar vertebral segments. The theory is con- return to the upright position he or she does so with
troversial, partly because of differences about the a noticeable ‘heave’ or ‘catch’, sometimes seeking
meaning of the word ‘instability’ in this context and support by pressing upon the thighs.
partly because some patients with demonstrably Straight-leg raising may be slightly restricted (in
abnormal vertebral motion have no symptoms at all. this case only because of back pain), but neurological
Radiological images that are interpreted as showing examination is normal.
instability may or may not be accepted by a bioengi-
neer as proof of instability in mechanical terms.
Imaging
Clinical features X-RAY
X-rays may look completely normal. However, in
Whatever the doubts about aetiology, the clinical many cases there are mild to moderately severe fea-
appearances of this syndrome are easily recognizable. tures of intervertebral disc degeneration, mainly flat-
The patient, usually a young adult engaged in bend- tening of the ‘disc space’ and marginal osteophytes. A
ing and/or lifting activities, experiences mild back- singular feature, which is held to be characteristic of
ache from time to time. Typically this culminates in a ‘segmental instability’, is the appearance of a ‘traction
particular episode of more severe back pain, possibly spur’, a bony projection anteriorly a little distance
accompanied by pain in the buttock or the back of the from the upper or lower rim of the vertebral body. In
thighs, but no true neurological symptoms. Pain is the lateral view, there may be slight displacement of
usually relieved by rest, mobilization exercises or chi- one vertebra upon another, either forwards (spondy-
ropractic manipulation, only to recur a few weeks or lolisthesis) or backwards (retrolisthesis); this may
482 months later after a similar episode of physical stress. become apparent only during flexion or extension.
General care and attention Poor understanding has led 18
to the condition being neglected and, unless there is
a very obvious abnormality that is amenable to sur-
gery, patients soon come to feel that the doctor has
lost interest in their complaints; little wonder that
many of them turn for help to ‘alternative’ practition-
The back
ers. They should be given a clear explanation of the
likely cause of their symptoms and an outline of the
proposed treatment. In more enlightened (and better
supported) centres patients are enrolled in a ‘back
school’.
Physical therapy Conventional physiotherapy, includ-
ing spinal ‘mobilization’, often relieves pain dramati-
(a) (b) cally – at least for a while. In the longer term, weight
18.34 Facet joint dysfunction X-ray features of control and strengthening of the vertebral and
spondylosis and facet joint dysfunction: (a) Narrowing of abdominal muscles will make for fewer recurrences.
the disc space and slight retrolisthesis at L4/5, with two There is also no reason why orthopaedic surgeons and
small traction spurs at the anterior borders of the adjacent chiropractors or osteopaths should not be able to col-
vertebrae. (b) Narrowing of the disc space at L3/4 and laborate in designing treatment programmes.
areas of subchondral sclerosis in the adjacent vertebral
bodies (reminiscent of the Modic type 3 MRI changes Drug treatment Mild analgesics may be needed for
shown in Fig.18.30). pain control. Long term non-steroidal anti-inflamma-
tory drug (NSAID) medication is still preferable to
the drastic remedy of spinal fusion surgery, but should
Discography and facetography may reveal disc be combined with an appropriate gut protector such
abnormalities, but these investigations are not rou- as omeprazole. However, beware the patient who
tinely available and in any case there is some contro- becomes dependent on increasing doses of medica-
versy about their reliability. tion.
Spinal support A soft lumbar support may give relief in
CT AND MRI some cases; obese patients benefit from having their
These investigations may reveal signs of disc degener- centre of gravity pulled in close to the spine.
ation as well as early features of OA in the facet joints
(loss of articular cartilage space and curling over of the Modification of activities One of the most important
joint surfaces). ‘Modic’ changes are worth noting (see aspects of treatment is modification of daily activities
Fig. 18.30). (bending, lifting, climbing, etc.) and specific activities
relating to work. The patient may need retraining for
a different job. The co-operation of employers is
Diagnosis essential.
Recurrent backache is often attributed to one particu- Psychological support Chronic back pain can be psy-
lar abnormal feature, such as ‘disc degeneration’ or ‘an chologically as well as physically debilitating. Coun-
annular tear’. It is difficult to prove a causative associ- selling and support are often welcomed by the
ation of this kind. The discovery of one abnormality patient. Perhaps the most successful treatment is the
should, however, prompt the clinician to look for oth- reassurance that the surgeon can provide for the vast
ers; it is the set of clinical and imaging features rather majority of patients, to the effect that the patient has
than any single sign that elucidates the diagnosis. no serious spinal disease.
Trigger point and facet joint injectionIf clinical and
Treatment x-ray signs point consistently to one or two facet
levels, injection of local anaesthetic and corticos-
Whatever pattern the back pain may present, the pain teroids may be carried out under fluoroscopic control.
may be sufficiently distressing or disabling to justify Most patients can be expected to obtain short-term
treatment in increasing degrees of invasiveness. benefit and some are relieved of symptoms for periods
of more than a year. Lumbosacral trigger points
CONSERVATIVE MEASURES (Travell, 1983) in the midline or along the iliac crests,
Initially the symptoms are neither severe nor dis- are a common finding in chronic low back pain. If
abling; conservative measures should be encouraged they are focal and consistent they may respond
for as long as possible: dramatically, if only temporarily, to deep soft tissue 483
18 local infiltration without the need for fluoroscopic Lytic or isthmic (50 per cent) In this, the commonest
control. variety, there are defects in the pars interarticularis
(spondylolysis), or repeated breaking and healing may
SURGERY lead to elongation of the pars. The defect (which
Only after all of the above measures have been tried occurs in about 5 per cent of people) is usually present
and found to be ineffectual should a spinal fusion be by the age of 7, but the slip may appear only some
considered. Even then very strict guidelines should be years later (Eisenstein, 1978; Fredrickson et al., 1984).
followed if embarking on a road already crowded with It is difficult to exclude a genetic factor because
patients labelled ‘failed back surgery’ is to be avoided: spondylolisthesis often runs in families, and is more
common in certain races, notably Eskimos; but the
1. Repeated examination should ensure that there is
incidence increases with age up to the late teenage
no other treatable pathology.
years, although clinical presentation with pain can con-
2. There should have been at least some response to
tinue into late middle age. An acquired factor proba-
conservative treatment; patients who ‘benefit from
bly supervenes to produce what is essentially an
nothing’ will not benefit from spinal fusion either.
ununited stress fracture. The condition is more com-
3. There should be unequivocal evidence of facet
mon than usual in those whose spines are subjected to
joint instability or osteoarthritis at a specific level.
extraordinary stresses (e.g. competitive gymnasts and
4. The patient should be emotionally stable and
weight-lifters).
should not exaggerate his symptoms nor display
inappropriate physical signs (see later). Degenerative (25 per cent) Degenerative changes in the
5. The patient should be warned that: (1) a ‘fusion’ facet joints and the discs permit forward slip (nearly
doesn’t always fuse (there is a 10–20 per cent always at L4/5 and mainly in women of middle age)
failure rate); (2) a fusion at one level does not despite intact laminae. Many of these patients have
preclude further pathology developing at another generalized osteoarthritis and pyrophosphate crystal
level; Lehmann et al (1987), in a long-term arthropathy.
follow-up of patients who had undergone spinal
Post-traumatic Unusual fractures may result in
fusion, found that after 10 years 40 per cent had
destabilization of the lumbar spine.
developed signs of instability elsewhere.
Pathological Bone destruction (e.g. due to tuberculosis
Whether the surgery is performed anteriorly or pos-
or neoplasm) may lead to vertebral slipping.
teriorly, or from both approaches combined, or
whether implants of one kind or another are employed, Postoperative (iatropathic) Occasionally, excessive opera-
seems not to affect the final result materially. The sur- tive removal of bone in decompression operations
geon must be allowed to perform the procedure in results in progressive spondylolisthesis.
which he/she has the most confidence. There is no
doubt however, that the more extensive and more
complex procedures carry a higher complication rate.
Pathology
In the common lytic type of spondylolisthesis the pars
SPONDYLOLISTHESIS interarticularis on both sides is disrupted, as in an
ununited fracture (spondylolysis), leaving the posterior
neural arch separated from the vertebral body anteri-
‘Spondylolisthesis’ means forward translation of one
orly; the gap is occupied by fibrous tissue. With stress,
segment of the spine upon another. The shift is nearly
the vertebral body and superior facets in front of the
always between L4 and L5, or between L5 and the
gap may subluxate or dislocate forwards, carrying the
sacrum. Normal discs, laminae and facets constitute a
superimposed vertebral column with it (spondylolisthe-
locking mechanism that prevents each vertebra from
sis); the isolated segment of neural arch maintains its
moving forwards on the one below. Forward shift (or
normal relationship to the sacral facets. When there is
slip) occurs only when this mechanism has failed.
no gap, the pars interarticularis is elongated or the
facets are defective.
Classification The degree of slip is measured by the amount of
overlap of adjacent vertebral bodies and is usually
Various classifications have been suggested. Basically
expressed as a percentage.
there are six types of spondylolisthesis:
With forward slipping there may be pressure on the
Dysplastic (20 per cent) The superior sacral facets are dura mater and cauda equina, or on the emerging
congenitally defective; slow but inexorable forward slip nerve roots; these roots may also be compressed in
leads to severe displacement. Associated anomalies the narrowed intervertebral foramina. Disc prolapse is
484 (usually spina bifida occulta) are common. liable to occur.
Clinical features 18
Spondylolysis, and even a well-marked spondylolisthe-
sis, may be discovered incidentally during routine
x-ray examination.
In children the condition is usually painless but the
The back
mother may notice the unduly protruding abdomen
and peculiar stance. In adolescents and adults backache
is the usual presenting symptom; it is often intermit-
tent, coming on after exercise or strain. Sciatica may
occur in one or both legs. Patients aged over 50 are usu-
ally women with degenerative spondylolisthesis. They
always have backache, some have sciatica and some
present because of claudication due to spinal stenosis.
On examination the buttocks look curiously flat,
the sacrum appears to extend to the waist and trans-
verse loin creases are seen. The lumbar spine is on a
plane in front of the sacrum and looks too short.
Sometimes there is a scoliosis.
A ‘step’ can often be felt when the fingers are run
down the spine. Movements are usually normal in the
younger patients but there may be ‘hamstring tight-
ness’; in the degenerative group the spine is often stiff.
X-RAYS
Lateral views show the forward shift of the upper part
of the spinal column on the stable vertebra below;
elongation of the arch or defective facets may be seen.
The gap in the pars interarticularis is best seen in the
oblique views. In doubtful cases, reversed gantry CT
may be helpful.
18.35 Spondylolisthesis – clinical appearance The
transverse loin creases, forwards tilting of the pelvis and
flattening of the lumbar spine are characteristic.
Prognosis
Dysplastic spondylolisthesis appears at an early age,
often goes on to a severe slip and carries a significant
risk of neurological complications.
(a) (b) (c)
18.36 Spondylolisthesis – x-rays (a) There is a break in the pars interarticularis of L5, allowing the anterior part of the
vertebra to slip forwards. In this case the gap is easily seen in the lateral x-ray, but usually it is better seen in the oblique view
(b). In degenerative spondylolisthesis there is no break in the pars – the degenerate disc and eroded facet joints permit one
vertebra to slide forwards on the other (c). There is no pars defect; the dehydrated disc permits slipping, usually at L4/5. 485
18 Lytic (isthmic) spondylolisthesis with less than 10 per present before injury and treatment is along the lines
cent displacement does not progress after adulthood, already indicated.
but may predispose the patient to later back problems. A detailed discussion of surgical options appears in
It is not a contraindication to strenuous work unless a review paper by Jones and Raj (2009).
severe pain supervenes (Wiltse et al., 1990). With slips
of more than 25 per cent there is an increased risk of
backache in later life.

Degenerative spondylolisthesis is rare before the age SPINAL STENOSIS
of 50, progresses slowly and seldom exceeds 30 per
cent.
The lumbar spinal canal is normally round or oval in
cross-section; in a minority of cases the L5 canal is
Treatment trefoil-shaped and the lateral recesses are narrower
than usual, yet still wide enough to allow free passage
Conservative treatment, similar to that for other types
of the nerve roots through the intervertebral foram-
of back pain, is suitable for most patients.
ina (Eisenstein, 1980). The term spinal stenosis is used
Operative treatment is indicated: (1) if the symp-
to describe abnormal narrowing of the central canal,
toms are disabling and interfere significantly with
the lateral recesses or the intervertebral foramina to
work and recreational activities; (2) if the slip is more
the point where the neural elements are compro-
than 50 per cent and progressing; (3) if neurological
mised. When this occurs the patient develops neuro-
compression is significant.
logical symptoms and signs in the lower limbs.
For children, posterior intertransverse fusion in situ
The causes of spinal stenosis are: (1) congenital
is almost always successful; if neurological signs
vertebral dysplasia (e.g. in achondroplasia or
appear, decompression can be carried out later. For
hypochondroplasia); (2) chronic disc protrusion and
adults, either posterior or anterior fusion is suitable.
peri-discal fibrosis or ossification; (3) displacement
However, in the ‘degenerative’ group, where neuro-
and hypertrophy, or osteoarthritis, of the apophyseal
logical symptoms predominate, decompression with-
(facet) joints; (4) hypertrophy, folding, or ossification
out fusion may suffice.
of the ligamentum flavum; (5) bone thickening due to
Note on post-traumatic spondylolisthesis The patient Paget’s disease; (6) spondylolisthesis. Unilateral nar-
found to have spondylolysis or spondylolisthesis after rowing of the intervertebral foramen (root canal steno-
recent back injury (usually hyperextension) may have sis) may result from an unresolved lateral disc
fractured the pars, or merely strained the fibrous tissue herniation, post-discectomy fibrosis or unilateral facet
of a pre-existing lesion. If doubt exists (and it usually joint ost.
does) a plaster jacket should be worn for 3 months; the What constitutes abnormal narrowing, or stenosis?
recent fracture may join spontaneously. If union does Two measurements are used: the mid-sagittal (antero-
not occur the assumption is that spondylolisthesis was posterior) diameter and the interpedicular (transverse)
18.37 Spinal stenosis (a) The shape of the lumbar

spinal canal varies from oval (with a large capacity)
to trefoil (with narrow lateral recesses); further
encroachment on an already narrow canal can cause
an ischaemic neuropathy and ‘spinal claudication’.
(b,c) Myelogram showing marked narrowing of the
radio-opaque column at the level of stenosis.
486 (a) (b) (c)

diameter of the spinal canal. On plain x-rays the lower Treatment 18
limits of normal for the lumbar vertebrae are usually
taken as 15 mm for the anteroposterior and 20 mm Conservative measures, including instruction in spinal
for the transverse diameters (Eisenstein, 1977; 1983). posture, may suffice. Most patients are prepared to
However, the boundaries of the canal are sometimes put up with their symptoms and simply avoid uncom-
difficult to define and more accurate measurements fortable postures. If discomfort is marked and activi-
The back
can be obtained from CT; anything less than 11 mm ties such as standing and walking are severely
for the anteroposterior diameter and 16 mm for the restricted, operative decompression is almost always
transverse diameter is considered abnormal. successful. A large laminotomy with flavectomy,
medial facetectomy and discectomy is performed at
every relevant level and on every relevant side, if nec-
Clinical features essary extending over several levels and laterally to
The patient, usually a man aged over 50, complains of clear the nerve root canals. This relieves the leg pain,
aching, heaviness, numbness and paraesthesia in the but not the back pain, and occasionally the surgery
thighs and legs; it comes on after standing upright or actually increases spondylolisthesis and back pain;
walking for 5–10 minutes, and is consistently relieved consequently in patients under 60 the operation is
by sitting, squatting or leaning against a wall to flex sometimes combined with spinal fusion (Eisenstein,
the spine (hence the term ‘spinal claudication’). The 2002).
patient may prefer walking uphill, which flexes the
spine (and maximizes the spinal canal capacity), to
downhill, which extends it. With root canal stenosis
the symptoms may be unilateral. The patient some- APPROACH TO DIAGNOSIS IN
times has a previous history of disc prolapse, chronic PATIENTS WITH LOW BACK PAIN
backache or spinal operation.
Examination, especially after getting the patient Chronic backache is such a frequent cause of disabil-
to reproduce the symptoms by walking, may (rarely) ity in the community that it has become almost a dis-
show neurological deficit in the lower limbs. Intact ease in itself. The following is a suggested approach to
pedal pulses would confirm the claudication as more specific diagnosis.
spinal rather than arterial, but beware of the older Careful history taking and examination will uncover
patient who could have both spinal and arterial clau- one of five pain patterns:
dication.
1. Transient backache following muscular activityThis
suggests a simple back strain that will respond to a
Imaging short period of rest followed by gradually increasing
X-rays will usually show features of disc degeneration exercise. People with thoracic kyphosis (of whatever
and proliferative osteoarthritis or degenerative origin), or fixed flexion of the hip, are particularly
spondylolisthesis. Measurement of the spinal canal can prone to back strain because they tend to compensate
be carried out on plain films, but more reliable infor- for the deformity by holding the lumbosacral spine in
mation is obtained from myelography, CT and MRI. hyperlordosis.
2. Sudden, acute pain and sciatica In young people
(those under the age of 20) it is important to exclude
infection and spondylolisthesis; both produce
recognizable x-ray changes. Patients aged 20–40 years
are more likely to have an acute disc prolapse:
diagnostic features are: (1) a history of a lifting strain,
(2) unequivocal sciatic tension; (3) neurological
symptoms and signs. Elderly patients may have
osteoporotic compression fractures, but metastatic
disease and myeloma must be excluded.
3. Intermittent low back pain after exertion Patients of
almost any age may complain of recurrent backache
(a) (b) following exertion or lifting activities and this is
relieved by rest. Features of disc prolapse are absent
18.38 Spinal stenosis – MRI T2-weighted sagittal and
axial images showing circumferential spinal stenosis at L4/5
but there may be a history of acute sciatica in the past.
in a middle-aged woman with marked osteoarthritis of the In early cases x-rays usually show no abnormality; later
facet joints. there may be signs of lumbar spondylosis in those over 487
18
(a) (b) (c) (d)
(e) (f)
18.39 Some causes of chronic back pain (a) Tuberculosis; (b) acute osteomyelitis – note the sclerosis that developed
within a few weeks; (c) discitis; (d) metastatic disease; (e) bilateral sacroiliac tuberculosis; (f) osteitis condensans ilii, which
is probably not the cause of the backache.
50 years and osteoarthritis of the facet joints is

common. These patients need painstaking examination
CHRONIC BACK PAIN SYNDROME
to: (1) uncover any features of radiological segmental
instability or facet joint osteoarthritis; (2) determine Patients with chronic backache may despair of finding
whether those features are incidental or are likely to a cure for their trouble (or, indeed, even a diagnosis
account for the patient’s symptoms. In the process, that everyone agrees on), and they often develop
disorders such as ankylosing spondylitis, chronic affective and psychosomatic ailments that subse-
infection, myelomatosis and other bone diseases must be quently become the chief focus of attention. This ‘ill-
excluded by appropriate imaging and blood ness behaviour’ is both self-perpetuating and
investigations. self-justifying. It is usually accompanied by ‘non-
organic’ (inappropriate) physical signs (Waddell et al.,
4. Back pain plus pseudoclaudication These patients are 1980; 1984), such as: (1) pain and tenderness of
usually aged over 50 and may give a history of previ- bizarre degree or distribution; (2) pain on performing
ous, longstanding back trouble. The diagnosis of impressive but non-stressful manoeuvres such as
spinal stenosis should be confirmed by CT and/or pressing vertically on the spine or passively rotating
MRI. the entire trunk; (3) variations in response to tests
such as straight leg raising while distracting the
5. Severe and constant pain localized to a particular site patient’s attention; (4) sensory and/or motor abnor-
This suggests local bone pathology, such as a malities that do not fit the known anatomical and
compression fracture, Paget’s disease, a tumour or physiological patterns; (5) overdetermined behaviour
infection. Spinal osteoporosis in middle-aged men is during physical examination (trembling, sweating,
pathological and calls for a full battery of tests to hyperventilating, inability to move, a tendency to fall
exclude primary disorders such as myelomatosis, and exaggerated withdrawal) – usually accompanied
carcinomatosis, hyperthyroidism, gonadal insufficiency, by loud groaning and exclamations of discomfort.
488 alcoholism or corticosteroid usage. Patients with these features are unlikely to respond to
surgery and they may require prolonged support and 18
management in a special pain clinic – but only after
every effort has been made to exclude organic
pathology.
The back
NOTES ON APPLIED ANATOMY
THE SPINE AS A WHOLE

The spine has to move, to transmit weight and to pro-
tect the spinal cord. In upright man the lumbar seg- (a) (b) (c)
ment is lordotic and the column acts like a crane; the
paravertebral muscles are the cables that counterbal- 18.40 Anatomy (a,b) The vertebral column has a series
of gentle curves that produce lordosis in the cervical and
ance any weight carried anteriorly. The resultant force, lumbar regions and kyphosis in the dorsal segment. The
which passes through the nucleus pulposus of the low- column functions like a crane, the weight in front of the
est lumbar disc, is therefore much greater than if the spine being counterbalanced by contraction of the
column were loaded directly over its centre; even at posterior muscles. (c) Relationship of nerve root to disc and
rest, tonic contraction of the posterior muscles balances facet joint.
the trunk, so the lumbar spine is always loaded.
Nachemson and Morris (1964) measured the intradis-
plates. In childhood these are covered by cartilage,
cal pressure in volunteers during various activities and
which contributes to vertebral growth. Later the
found it as high as 10–15 kg/cm2 while sitting, about
peripheral rim ossifies and fuses with the body, but the
30 per cent less on standing upright and 50 per cent
central area remains as a thin layer of cartilage adher-
less on lying down. Leaning forward or carrying a
ent to the intervertebral disc. The epiphyseal end-
weight produces much higher pressures, though when
plates may be damaged by disc pressure during
a heavy weight is lifted breathing stops and the abdom-
childhood, giving rise to irregular ossification and
inal muscles contract, turning the trunk into a tightly
abnormal vertebral growth (Scheuermann’s disease).
inflated bag that cushions the force anteriorly against
the pelvis. (Could it be that champion weight-lifters
benefit in this way from having voluminous bodies?)
Seen from the side, the dorsal spine is convex back- INTERVERTEBRAL DISC
wards (kyphosis); the cervical and lumbar regions are
The disc consists of a central avascular nucleus pulpo-
convex forwards (lordosis). In forward flexion the lor-
sus – a hydrophilic gel made of protein-polysaccha-
dotic curves straighten out. Lying supine with the legs
ride, collagen fibres, sparse chondroid cells and water
straight tilts the pelvic brim forwards; the lumbar
(88 per cent), surrounded by concentric layers of
spine compensates by increasing its lordosis. If the
fibrous tissue – the annulus fibrosus. If the physico-
hips are unable to extend fully (fixed flexion defor-
chemical state of the nucleus pulposus is normal, the
mity), the lumbar lordosis increases still more until
disc can withstand almost any load that the muscles
the lower limbs lie flat and the flexion deformity is
can support; if it is abnormal, even small increases in
masked.
force can produce sufficient stress to rupture the
annulus.
VERTEBRAL COMPONENTS
Each segment of the vertebral column transmits MOVEMENTS
weight through the vertebral body anteriorly and the
facet joints posteriorly. Between adjacent bodies (and The axis of movements in the thoracolumbar spine is
firmly attached to them) lie the intervertebral discs. the nucleus pulposus; the disposition of the facet
These compressible ‘cushions’, and the surrounding joints determine which movements occur. In the lum-
ligaments and muscles, act as shock-absorbers; if they bar spine these joints are in the anteroposterior plane,
are degenerate or weak their ability to absorb some of so flexion, extension and sideways tilting are free but
the force is diminished and the bones and joints suf- there is virtually no rotation. In the thoracic spine the
fer the consequences. facet joints face backwards and laterally, so rotation is
The vertebral body is cancellous, but the upper and relatively free; flexion, extension and tilting are possi-
lower surfaces are condensed to form sclerotic end- ble but are grossly restricted by the ribs. The cos- 489
18 tovertebral joints are involved in respiration and their pain of similar distribution. Pain down the thigh and
limitation is an early feature of ankylosing spondylitis. leg (‘sciatica’) does not necessarily signify root pres-
sure; it may equally well be referred from a facet joint
or any painful spinal tissue.
THE SPINAL CANAL
The shape of the canal changes from ovoid in the BLOOD SUPPLY
upper part of the lumbar spine to triangular in the
lower. Variations are common and include the trefoil In addition to the spinal arteries, which run the
canal, whose shape is mainly due to thickening of the length of the cord, segmental arteries from the aorta
laminae (Eisenstein, 1980). This shape is harmless in send branches through the intervertebral foramina at
itself, but further encroachment on the canal (e.g. by each level. Accompanying veins drain into the azygos
a bulging disc or hypertrophic facet joints) may cause system and inferior vena cava, and anastomose pro-
compression of the spinal contents (spinal stenosis). fusely with the extradural plexus, which extends
throughout the length of the spinal canal (Batson’s
plexus).
SPINAL CORD
The spinal cord ends at about L1 in the conus
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491
The hip
19
Louis Solomon, Reinhold Ganz, Michael Leunig, Fergal Monsell, Ian Learmonth
CLINICAL ASSESSMENT SIGNS WITH THE PATIENT UPRIGHT

Start by standing face to face with the patient and
SYMPTOMS note his or her general build and the symmetry of the
lower limbs. First impressions are important and can
Pain arising in the hip joint is felt in the groin, down be put to the test as the examination proceeds. The
the front of the thigh and, sometimes, in the knee; patient in Figure 19.1, for example, seems to have
occasionally knee pain is the only symptom! Pain at unusually short lower limbs in comparison to his
the back of the hip is seldom from the joint; it usually trunk length. Is it a mild type of bone dysplasia, or are
derives from the lumbar spine. the hips dislocated?
Limp is the next most common symptom. It may
simply be a way of coping with pain, or it may be due
to a change in limb length, weakness of the hip abduc- Trendelenburg’s sign
tors or joint instability. This is a test for postural stability when the patient
Snapping or clicking in the hip suggests a number stands on one leg. In normal two-legged stance the
of causes: slipping of the gluteus maximus tendon body’s centre of gravity is placed midway between the
over the greater trochanter, detachment of the acetab- two feet. Normally, in one-legged stance, the pelvis is
ular labrum or psoas bursitis. pulled up on the unsupported side and the centre of
Stiffness and deformity are late symptoms, and tend gravity is placed directly over the standing foot. If the
to be well compensated for by pelvic mobility. weightbearing hip is unstable, the pelvis drops on the
Walking distance may be curtailed; or, reluctantly, unsupported side; to avoid falling, the person has to
the patient starts using a walking stick. throw his body towards the loaded side so that the
centre of gravity is again over that foot.
If the difference between the two hips is marked
you can detect it by simply looking at the patient’s
stance. However, small differences are not so obvious.
In the classical Trendelenburg test the examiner
stands behind the patient and looks at the buttock-
folds. Normally in one-legged stance the buttock on
the opposite side rises as the person lifts that leg; in a
positive (abnormal) test the opposite buttock-fold
drops (Fig. 19.2).
The causes of a positive Trendelenburg sign are: (1)
pain on weightbearing; (2) weakness of the hip
abductors; (3) shortening of the femoral neck; and
(a) (b) (c) (4) dislocation or subluxation of the hip.
19.1 Trendelenburg’s sign (a) Standing normally on two

legs. (b) Standing on the right leg which has a normal hip Gait
whose abductor muscles ensure correct weight Now ask the patient to walk, and observe each phase
transference. (c) Standing on the left leg whose hip is
faulty, and so abduction cannot be achieved; the pelvis of the gait. The commonest abnormalities are a short-
drops on the unsupported side and the shoulder swings leg limp (a regular, even dip on the short side), an
over to the left. antalgic gait (an irregular limp, with the patient
19
19.3 Testing iliopsoas function This is best done with

the patient sitting. Ask him or her to lift the thigh (flex the
hip) against resistance. In this position the psoas is acting
while the other hip flexors are relaxed. Pain or weakness
suggests a local disorder such as a psoas bursitis.
superior iliac spines at the same level) and the legs

placed symmetrically. Limb length can be gauged by
looking at the ankles and heels, but measurement is
(a) (b)
more accurate. With the two legs in identical posi-
tions, measure the distance from the anterior superior
19.2 Trendelenburg’s test This man has a positive iliac spine to the medial malleolus on each side. The
Trendelenburg sign on the left due to osteoarthritis of the
hip. (a) He can steady himself perfectly well when limb may lie in an abnormal position; excessive rota-
balancing on the right hip; (b) when he attempts to stand tion is easy to detect but other deformities are often
on the left hip, his pelvis dips and the right buttock drops. masked by tilting of the pelvis.
Sometimes the real length, as determined by meas-
uring between two bony points, is quite different
moving more quickly off the painful side) and a Tren- from the apparent length with the patient lying in
delenburg lurch (a variant of Trendelenburg’s sign). repose. This happens when the pelvis is tilted and one
While the patient is upright, take the opportunity limb is hitched upwards. Almost invariably this is due
to examine the spine for deformity or limitation of to an uncorrectable deformity at the hip: with fixed
movement. adduction on one side, the limbs would tend to be
crossed; when the legs are placed side by side the
pelvis has to tilt upwards on the affected side, giving
the impression of a shortened limb. The exact oppo-
SIGNS WITH THE PATIENT SITTING site occurs when there is fixed abduction, and the limb
This is the best way to test for iliopsoas function. The seems to be longer on the affected side.
patient should be sitting on the edge of the examina- If real shortening is present it is usually possible to
tion couch. Place a hand firmly on his thigh and ask establish where the fault lies. With the knees flexed
him to lift the thigh (flex the hip) against resistance. and the heels together, it can be seen whether the dis-
This is a predominantly psoas action; pain or weakness crepancy is below or above the knee. If it is above, the
suggests a local disorder such as tendinitis or psoas next question is whether the abnormality lies above
bursitis. the greater trochanter. The thumbs are pressed firmly
against the anterior superior iliac spines and the mid-
dle fingers grope for the tops of the greater
trochanters; any elevation of the trochanter on one
SIGNS WITH THE PATIENT LYING side is readily appreciated.
Look
Scars or sinuses may be seen (or they may be at the
Feel
back of the hip). Compare the two sides for signs of Skin temperature and soft-tissue contours can be felt,
muscle wasting or swelling. but are unhelpful unless the patient is very thin.
494 Check that the pelvis is horizontal (both anterior Bone contours are felt when levelling the pelvis and
19.4 Measurement 19
(a,b) Make sure the
patient is lying straight
on the examination
couch and that the
pelvis is absolutely level
– the anterior superior
The hip
iliac spines at the same
level in relation to the
longitudinal axis of the
body (c). Then check
the medial malleoli
(d); discrepancy in leg
(a) (b) length will usually be
obvious. (e,f) Leg
length is most
accurately assessed by
measuring from the
anterior superior iliac
spine to the tip of the
medial malleolus on
each side.
(c) (d)
(e) (f)
judging the height of the greater trochanters. Tender- Similarly, when testing abduction the pelvis must be
ness may be elicited in and around the joint. prevented from tilting sideways. This is achieved by
placing the ‘sound’ hip (the hip opposite to the one
being examined) in full abduction and keeping it
Move
there. A hand is placed on one iliac crest to detect the
The assessment of hip movements is difficult because slightest movement of the pelvis. Then, after checking
any limitation can easily be obscured by movement of that the anterior superior iliac spines are level, the
the pelvis. Thus, even a gross limitation of extension, affected joint is moved gently into abduction. The
causing a fixed flexion deformity, can be completely normal range is about 40 degrees.
masked simply by arching the back into excessive lor- Adduction is tested by crossing one limb over the
dosis. Fortunately it can be just as easily unmasked by other; the pelvis must be watched and felt to deter-
performing Thomas’ test (Fig. 19.5): both hips are mine the point at which it starts to tilt. The normal
flexed simultaneously to their limit, thus completely range of adduction is about 30 degrees.
obliterating the lumbar lordosis; holding the ‘sound’ To test rotation both legs, lifted by the ankles, are
hip firmly in position (and thus keeping the pelvis rotated first internally (medially) and then externally
still), the other limb is lowered gently; with any flex- (laterally); the patellae are watched to estimate the
ion deformity the knee will not rest on the couch. amount of rotation. Rotation in flexion is tested with
Meanwhile the full range of flexion will also have been the hip and knee each flexed 90 degrees.
noted; the normal range is about 130 degrees. If internal rotation is full with the hip extended, but 495
19
(a) (b) (c)
(d) (e) (f)
(g) (h) (i)
19.5 Movement (a) Forcing one hip into full flexion will straighten out the lumbar spine; the other hip should still be
capable of full extension in this position. (b) Now the position is reversed; the right hip is held in full flexion. (c) If the hip
cannot straighten out completely, this is referred to as a fixed flexion deformity. (d) Testing for abduction. The pelvis is kept
level by placing the opposite leg over the edge of the examination couch with that hip also in abduction (the examiner’s left
hand checks the position of the anterior spines) before abducting the target hip. (e) Testing for adduction. (f–h) External
and internal rotation are assessed (f) first with the hips in full extension and then (g,h) in 90° of flexion. (i) Testing for
extension.
restricted in flexion, this suggests pathology in the

anterosuperior portion of the femoral head, probably
IMAGING
avascular necrosis (the so-called ‘sectoral sign’). How-
Plain x-rays The minimum required is an anteroposte-
ever, in a young person, pain on internal rotation with
rior x-ray of the pelvis showing both hips and a lateral
the hip flexed may indicate a torn acetabular labrum.
view of each hip separately. The two sides can be
Abnormal movement is rarely elicited. Telescoping
compared: any difference in the size, shape or position
(excessive movement when the limb is alternately
of the femoral heads is important. With a normal hip
pulled and pushed in its long axis) is a sign of gross
Shenton’s line, which continues from the inferior
instability.
border of the femoral neck to the inferior border of
Do not forget the back of the hip. Ask the patient to roll
the pubic ramus, looks continuous; any interruption
over into the prone position. Check for scars and
in the line suggests an abnormal position of the
sinuses. Feel for tenderness and test the range of hip
femoral head. Narrowing of the joint ‘space’ is a sign
extension. Rotation can also be assessed by flexing both
of articular cartilage loss, a feature of both inflamma-
knees and moving the legs, first away from each other
tory and non-inflammatory arthritis.
(producing internal rotation at the hips) and then
A lateral view is obligatory for assessing the shape,
towards or crossing each other (external rotation).
496 position and architecture of the femoral head; for
neonatal hip dysplasia, when the joint is entirely carti- 19
laginous.
Arthrography Arthrography may be used to show the
outline of the cartilaginous femoral head in young
(a) (b) children. It may also reveal loose bodies, a loose flap of
articular cartilage or a tear of the acetabular labrum.
The hip
Computed tomography CT is ideal for demonstrating
structural abnormalities of the joint, e.g. in the assess-
ment of fracture-dislocations of the hip.
Radioscintigraphy Radioisotope scans are helpful in

investigating the blood supply of the femoral head or
cellular activity in the subchondral bone (new bone
formation or an inflammatory ‘hot spot’).
Magnetic resonance imaging This is ideal for detecting

(c) (d) (e) changes in the marrow and is the only certain way of
19.6 Normal range of movements (a) The hip should diagnosing early avascular necrosis, in which the
flex until the thigh meets the abdomen, but (b) extends changes are confined to the marrow.
only a few degrees. (c) Abduction is usually greater than
adduction. The relative amounts of internal and external
rotation may vary according to whether the hip is in
(d) flexion or (e) extension. ARTHROSCOPY
Arthroscopy has come much later to the hip than to

example, when a slipped epiphysis or avascular necro-
other joints such as the knee and shoulder. The indi-
sis is suspected.
cations for its use are still being defined. In a review of
Special tangential views are helpful when assessing
328 patients presenting with pain in the hip and sub-
congruency between the acetabular socket and the
sequently undergoing arthroscopy, it was reported
femoral head.
that in over half the cases the procedure contributed
Ultrasonography Ultrasound scans are useful for to the diagnosis beyond the information derived from
demonstrating intra-articular effusions. This is also clinical and imaging studies. In 172 cases some type of
the ideal method of imaging in the early diagnosis of operation was performed as well, usually debridement
19.7 Imaging (a) Antero-

posterior x-ray of normal hips,
showing Shenton’s line. (b) X-ray
of a patient with secondary
osteoarthritis of the left hip due to
congenital subluxation. The joint
‘space’ is narrowed and Shenton’s
line is broken. (c,d) X-ray and
three-dimensional CT showing
how shallow the acetabula are,
and how much of the femoral
head is uncovered, especially in
(a) (b) this dysplastic left hip. (Courtesy of
Professor Kjeld Søballe. Århus
Universitetshopital.)
(c) (d) 497

19 Table 19.1 The diagnostic calendar: age of onset can
be a guide to probable diagnosis
1:60; however 60 per cent stabilized by one week and
88 per cent by 8 weeks. The incidence is considerably
higher in some ethnic groups – 25–50 cases per 1000
Age of onset (years) Probable diagnosis
live births in Lapps and Native Americans!
0 (birth) Developmental dysplasia Girls are much more commonly affected than boys,
0–5 Infections the ratio being about 7:1. The left hip is more often
5–10 Perthes’ disease affected than the right; in 1 in 5 cases the condition is
bilateral.
10–20 Slipped epiphysis
Adults Arthritis
Genetic factors must play a part in the aetiology, for
of osteoarthritic tissue, extraction of loose bodies, DDH tends to run in families and even in entire pop-
debridement of labral tears and biopsies (Baber et al., ulations (e.g. in countries along the northern and
1999). Arthroscopy is now considered to be more eastern Mediterranean seaboard). Wynne-Davies
reliable than MRI for the diagnosis of non-osseous (1970) identified two heritable features which could
loose bodies, labral tears and cartilage surface dam- predispose to hip instability: generalized joint laxity (a
age. dominant trait), and shallow acetabula (a polygenic
trait which is seen mainly in girls and their mothers).
However, this cannot be the whole story because in 4
out of 5 cases only one hip is dislocated.
THE DIAGNOSTIC CALENDAR Hormonal factors (e.g. high levels of maternal
oestrogen, progesterone and relaxin in the last few
Hip disorders are characteristically seen in certain weeks of pregnancy) may aggravate ligamentous laxity
well-defined age groups. While there are exceptions to in the infant. This could account for the rarity of
this rule, it is sufficiently true to allow the age at onset instability in premature babies, born before the hor-
to serve as a guide to the probable diagnosis (see mones reach their peak.
Table 19.1). Intrauterine malposition (especially a breech posi-
tion with extended legs) favours dislocation; this so-
called ‘packaging disorder’ is linked with the higher
DEVELOPMENTAL DYSPLASIA OF incidence in first-born babies, among whom sponta-
neous version is less likely. Unilateral dislocation usu-
THE HIP ally affects the left hip; this fits with the usual vertex
presentation (left occiput anterior) in which the left
The terminology used to describe abnormalities of hip is adjacent to the mother’s sacrum, placing it in an
the paediatric hip is imprecise and confusing. The adducted position. Other manifestations of intrauter-
term ‘congenital dislocation of the hip’ (CDH) has ine crowding, including plagiocephaly, congenital tor-
been largely superseded by developmental dysplasia of ticollis and postural foot deformities, are also
the hip (DDH) in an attempt to describe the range associated with a higher than usual incidence of DDH.
and evolution of abnormalities that occur in this con- Postnatal factors may contribute to persistence of
dition. This comprises a spectrum of disorders includ- neonatal instability and acetabular maldevelopment.
ing acetabular dysplasia without displacement, Dislocation is very common in Lapps and North
subluxation and dislocation. Teratological forms of American Indians who swaddle their babies and carry
malarticulation leading to dislocation are also them with legs together, hips and knees fully
included. extended, and is rare in southern Chinese and African
Normal hip development depends on proportion- Negroes who carry their babies astride their backs
ate growth of the acetabular triradiate cartilages and with legs widely abducted. There is also experimental
the presence of a concentrically located femoral head. evidence that simultaneous hip and knee extension
Whether the instability comes first and then affects leads to hip dislocation during early development
acetabular development because of imperfect seating (Yamamuro and Ishida, 1984).
of the femoral head, or is a result of a primary acetab-
ular dysplasia, is still uncertain. Both mechanisms
might be important. Pathology
The reported incidence of neonatal hip instability in At birth the hip, though unstable, is probably normal
northern Europe is approximately 1 per 1000 live in shape but the capsule is often stretched and redun-
births, but this is dependent on the definition of dant.
498 ‘instability’. Barlow (1962) described an incidence of During infancy a number of changes develop, some
instability. In Ortolani’s test, the baby’s thighs are held 19
with the thumbs medially and the fingers resting on
the greater trochanters; the hips are flexed to 90
degrees and gently abducted. Normally there is
smooth abduction to almost 90 degrees. In congeni-
tal dislocation the movement is usually impeded, but
The hip
if pressure is applied to the greater trochanter there is
a soft ‘clunk’ as the dislocation reduces, and then the
hip abducts fully (the ‘jerk of entry’). If abduction
stops halfway and there is no jerk of entry, there may
be an irreducible dislocation.
Barlow’s test is performed in a similar manner, but
here the examiner’s thumb is placed in the groin and,
by grasping the upper thigh, an attempt is made to
19.8 Developmental dysplasia of the hip (DDH) – lever the femoral head in and out of the acetabulum
early signs Position of the hands for performing Ortolani’s
test. during abduction and adduction. If the femoral head
is normally in the reduced position, but can be made
to slip out of the socket and back in again, the hip is
classed as ‘dislocatable’ (i.e. unstable).
of them perhaps reflecting a primary dysplasia of the Every hip with signs of instability – however slight –
acetabulum and/or the proximal femur, but most of should be examined by ultrasonography. This shows the
them from adaptation to persistent instability and shape of the cartilaginous socket and the position of the
abnormal joint loading. femoral head. If there is any abnormality, the infant is
The femoral head dislocates posteriorly but, with placed in a splint with the hips flexed and abducted (see
extension of the hips, it comes to lie first postero- under Management) and is recalled for re-examination
lateral and then superolateral to the acetabulum. – in the splint – at 2 weeks and at 6 weeks. By then it
The cartilaginous socket is shallow and anteverted. should be possible to assess whether the hip is reduced
The cartilaginous femoral head is normal in size but and stable, reduced but unstable (dislocatable by Bar-
the bony nucleus appears late and its ossification is low’s test), subluxated or dislocated.
delayed throughout infancy. Late features An observant mother may spot asym-
The capsule is stretched and the ligamentum teres metry, a clicking hip, or difficulty in applying the nap-
becomes elongated and hypertrophied. Superiorly the kin (diaper) because of limited abduction.
acetabular labrum and its capsular edge may be With unilateral dislocation the skin creases look
pushed into the socket by the dislocated femoral head; asymmetrical and the leg is slightly short (Galeazzi’s
this fibrocartilaginous limbus may obstruct any sign) and externally rotated; a thumb in the groin may
attempt at closed reduction of the femoral head. feel that the femoral head is missing. With bilateral
After weightbearing commences, these changes are dislocation there is an abnormally wide perineal gap.
intensified. Both the acetabulum and the femoral neck Abduction is decreased.
remain anteverted and the pressure of the femoral Contrary to popular belief, late walking is not a
head induces a false socket to form above the shallow marked feature; nevertheless, in children who do not
acetabulum. The capsule, squeezed between the edge walk by 18 months dislocation must be excluded.
of the acetabulum and the psoas muscle, develops an Likewise, a limp or Trendelenburg gait, or a waddling
hourglass appearance. In time the surrounding mus- gait could be a sign of missed dislocation.
cles become adaptively shortened.
Imaging
Clinical features
Ultrasonography Ultrasound scanning has replaced
The ideal, still unrealized, is to diagnose every case at radiography for imaging hips in the newborn. The
birth. For this reason, every newborn child should be radiographically ‘invisible’ acetabulum and femoral
examined for signs of hip instability. Where there is a head can, with practice, be displayed with static and
family history of congenital instability, and with dynamic ultrasound. Sequential assessment is straight-
breech presentations or signs of other congenital forward and allows monitoring of the hip during a
abnormalities, extra care is taken and the infant may period of splintage.
have to be examined more than once. Even then some
Plain x-rays X-rays of infants are difficult to interpret
cases are missed.
and in the newborn they can be frankly misleading.
In the neonate There are several ways of testing for This is because the acetabulum and femoral head are 499
19 19.9 DDH – late signs
(a,b) Unilateral dislocation of the left
hip. (c) The left hip does not abduct
more than half way, and (d) the
drawing shows why – the femoral
head is caught up on the rim of the
acetabulum. (e) X-ray showing
bilateral displacement of the hip.
(a) (b) (c)
(d) (e)
largely (or entirely) cartilaginous and therefore not ning is repeated at intervals until stability and normal
visible on x-ray. X-ray examination is more useful after anatomy are restored or a decision is made to aban-
the first 6 months, and assessment is helped by draw- don splintage in favour of more aggressive treatment.
ing lines on the x-ray plate to define three geometric If ultrasound is not available, the simplest policy is
indices (Fig. 19.10). to regard all infants with a high-risk background or a
positive Ortolani or Barlow test, as ‘suspect’ and to
nurse them in double napkins or an abduction pillow
Screening
for the first 6 weeks. At that stage they are re-exam-
Neonatal screening in dedicated centres has led to a ined: those with stable hips are left free but kept under
marked reduction in missed cases of DDH. Risk fac- observation for at least 6 months; those with persist-
tors such as family history, breech presentation, oligo- ent instability are treated by more formal abduction
hydramnios and the presence of other congenital splintage (see below) until the hip is stable and x-ray
abnormalities are taken into account in selecting new- shows that the acetabular roof is developing satisfac-
born infants for special examination and ultrasonogra- torily (usually 3–6 months)
phy. Ideally all neonates should be examined, but if There are two drawbacks to this approach: (1) the
the programme is to be effective those doing the sensitivity of the clinical tests is not high enough to
examining should receive special training (Harcke and ensure that all cases will be spotted (Jones, 1994); and
Kumar, 1991; Jones, 1994). (2) of those hips that are unstable at birth, 80–90 per
cent will stabilize spontaneously in 2–3 weeks. It
therefore seems more sensible not to start splintage
Management immediately unless the hip is already dislocated. This
THE FIRST 3–6 MONTHS reduces the small (but significant) risk of epiphyseal
Where facilities for ultrasound scanning are available, necrosis that attends any form of restrictive splintage
all newborn infants with a high-risk background or a in the neonate. Thus: if a hip is dislocatable but not
suggestion of hip instability are examined by ultra- habitually dislocated, the baby is left untreated but re-
sonography. If this shows that the hip is reduced and examined weekly; if at 3 weeks the hip is still unstable,
has a normal cartilaginous outline, no treatment is abduction splintage is applied (see below). If the hip
required but the child is kept under observation for is already dislocated at the first examination, it is gen-
3–6 months. In the presence of acetabular dysplasia or tly placed in the reduced position and abduction
hip instability, the hip is splinted in a position of flex- splintage is applied from the outset. Reduction is
500 ion and abduction (see below) and ultrasound scan- maintained until the hip is stable; this may take only a
This situation must be avoided; if the hip fails to 19
locate, splintage should be abandoned in favour of
closed or operative reduction at a later date.
Follow-up Whatever policy is adopted, follow-up is
continued until the child is walking. Sometimes, even
with the most careful treatment, the hip may later
The hip
show some degree of acetabular dysplasia.
(a)
PERSISTENT DISLOCATION: 6–18 MONTHS

If, after early treatment, the hip is still incompletely
reduced, or if the child presents late with a ‘missed’
dislocation, the hip must be reduced – preferably by
closed methods but if necessary by operation – and
held reduced until acetabular development is satisfac-
tory.
(b) Closed reduction Closed reduction is suitable after the
age of 3 months and is performed under general
anaesthesia with an arthrogram to confirm a concen-
tric reduction. To minimize the risk of avascular
necrosis, reduction must be gentle and may be pre-
ceded by gradual traction to both legs.
Failure to achieve concentric reduction should lead
to abandoning this method in favour of an operative
approach at approximately 1 year of age. The hips
(c) should be stable in a safe zone of abduction, which
may be increased with a closed adductor tenotomy.
19.10 DDH – X-rays (a) The left hip is dislocated, the
femoral head is underdeveloped and the acetabular roof Splintage The concentrically reduced hip is held in a
slopes upwards much more steeply than on the right side. plaster spica at 60 degrees of flexion, 40 degrees of
In this case the features are very obvious but lesser changes
can be gauged by geometrical tests. The epiphysis should abduction and 20 degrees of internal rotation. After 6
lie medial to a vertical line which defines the outer edge of weeks the spica is changed and the stability of the hips
the acetabulum (Perkins’ line) and below a horizontal line
which passes through the triradiate cartilages
(Hilgenreiner’s line). (b) The acetabular roof angle should
not exceed 30°. (c) Von Rosen’s lines: with the hips
abducted 45° the femoral shafts should point into the
acetabula. In each case the left side is shown to be
abnormal.
few weeks, but the safest policy is to retain some sort

of splintage until x-ray shows a good acetabular roof.
Splintage The object of splintage is to hold the hips
somewhat flexed and abducted; extreme positions are
avoided and the joints should be allowed some move-
ment in the splint. Von Rosen’s splint is an H-shaped (a) (b)
malleable splint that has the merit of being easy to
apply (and the demerit of being equally easy to take
off!). The Pavlik harness is more difficult to apply but
gives the child more freedom while still maintaining
position. The three golden rules of splintage are: (1)
the hip must be properly reduced before it is splinted;
(2) extreme positions must be avoided; (3) the hips
should be able to move. If the hip is splinted in a sub- (c) (d)
luxed/dislocated position, the posterior wall of the 19.11 DDH – early treatment (a,b) Various types of
acetabulum is at risk of growth disturbance, leading to abduction splint. (c,d) X-rays showing result of splintage
considerable difficulties with later reconstruction. for DDH of the right hip at 3 months and 18 months. 501
19 assessed under anaesthesia. Provided the position and Operation The joint capsule is opened anteriorly, any
stability are satisfactory the spica is retained for a fur- redundant capsule is removed along with any other
ther 6 weeks. Following plaster removal the hip is blocks to reduction including the hypertrophied liga-
either left unsplinted or managed in a removable mentum teres and transverse acetabular ligament and
abduction splint which is retained for up to 6 months the femoral head is seated in the acetabulum. Usually
depending on radiological evidence of satisfactory a derotation femoral osteotomy held by a plate and
acetabular development. screws will be required. At the same time a 1 cm seg-

ment can be removed from the proximal femur to
Operation If, at any stage, concentric reduction has
reduce pressure on the hip (Klisic and Jankovic,
not been achieved, open operation is needed. The
1976). If there is marked acetabular dysplasia, some
psoas tendon is divided; obstructing tissues (redun-
form of acetabuloplasty will also be needed – either a
dant capsule and thickened ligamentum teres) are
pericapsular reconstruction of the acetabular roof
removed and the hip is reduced. It is usually stable in
(Pemberton’s operation) or an innominate (Salter)
60 degrees of flexion, 40 degrees of abduction and 20
osteotomy which repositions the entire innominate
degrees of internal rotation. A spica is applied and the
bone and acetabulum (Fig. 19.12).
hip is splinted as described above.
If stability can be achieved only by markedly inter- Splintage After operation, the hip is held in a plaster
nally rotating the hip, a corrective subtrochanteric spica for 3 months and then left unsupported to allow
osteotomy of the femur is carried out, either at the recovery of movement. The child is kept under inter-
time of open reduction or 6 weeks later. In young mittent clinical and radiological surveillance until
children this usually gives a good result (Fig. 19.12a, skeletal maturity.
b).
DISLOCATION IN CHILDREN OVER 4 YEARS
PERSISTENT DISLOCATION: 18 MONTHS – 4 YEARS Reduction and stabilization become increasingly diffi-
In the older child, closed reduction is less likely to cult with advancing age. Nevertheless, in children
succeed; many surgeons would proceed straight to between 4 and 8 years – especially if the dislocation is
arthrography and open reduction. unilateral – it is still worth attempting, bearing in
mind that the risk of avascular necrosis and hip stiff-
Traction Even if closed reduction is unsuccessful, a
ness is reported as being in excess of 25 per cent. The
period of traction (if necessary combined with psoas
principles of treatment are as described immediately
and adductor tenotomy) may help to loosen the tis-
above.
sues and bring the femoral head down opposite the
Unilateral dislocation in the child over 8 years often
acetabulum.
leaves the child with a mobile hip and little pain. This
Arthrography An arthrogram at this stage will clarify is the justification for non-intervention, though in
the anatomy of the hip and show whether there is an that case the child must accept the fact that gait is dis-
inturned limbus or any marked degree of acetabular tinctly abnormal. If reduction is attempted it will
dysplasia. require an open operation and acetabular reconstruc-
19.12 Congenital hip dislocation – operative

treatment (a) Reduced open, but stable only in medial
rotation – 6 weeks later; (b) derotation osteotomy.
(c) Reduced open, but head poorly covered; (d)
innominate osteotomy. (e,f) X-rays after Salter
innominate osteotomy of the left hip.
(a) (b)
502 (c) (d) (e) (f)

19
The hip
(a) (b)
19.13 Untreated DDH (a) This patient, aged 35 years, had a short leg, a severe limp and back pain. (b) Hip replacement
restored her to near normality.
tion. These procedures are best undertaken in centres severe cases the epiphyseal and physeal growth plates
specializing in this area. also suffer; the ossific nucleus looks fragmented, the
With bilateral dislocation the deformity – and the epiphysis is distorted to greater or lesser extent and
waddling gait – is symmetrical and therefore not so metaphyseal changes lead to shortening and defor-
noticeable; the risk of operative intervention is also mity of the femoral neck.
greater because failure on one or other side turns this Prevention is the best cure: forced manipulative
into an asymmetrical deformity. Therefore, in these reduction should not be allowed; traction should be
cases, most surgeons avoid operation above the age of gentle and in the neutral position; positions of
6 years unless the hip is painful or deformity unusually extreme abduction must be avoided; soft-tissue
severe. The untreated patient walks with a waddle but release (adductor tenotomy) should precede closed
may be surprisingly uncomplaining. reduction; and if difficulty is anticipated open reduc-
tion is preferable.
Once the condition is established, there is no effec-
Complications tive treatment except to avoid manipulation and
weightbearing until the epiphysis has healed. In the
Failed reduction Multiple attempts at treatment, with
mildest cases there will be no residual deformity, or at
failure to achieve concentric reduction, may be worse
worst a femoral neck deformity which can be cor-
than no treatment. The acetabulum remains undevel-
rected by osteotomy. In severe cases the outcome may
oped, the femoral head may be deformed, the neck is
be flattening and mushrooming of the femoral head,
usually anteverted and the capsule is thickened and
shortening of the neck (with or without coxa vara),
adherent. It is important to enquire also why reduction
acetabular dysplasia and incongruency of the hip. Sur-
failed: is the dislocation part of a generalized condi-
gical correction of the proximal femur and pelvic
tion, or a neuromuscular disorder associated with mus-
osteotomy to reposition or deepen the acetabulum
cle imbalance? The principles of treatment for children
may be needed.
over 8 years are the same as those discussed above.
Avascular necrosis A much-feared complication of
Persistent dislocation in adults
treatment is ischaemia of the immature femoral head.
It may occur at any age and any stage of treatment Adults who appear to have managed quite well for
and is probably due to vascular injury or obstruction many years may present in their thirties or forties with
resulting from forceful reduction and hip splintage in increasing discomfort due to an unreduced congenital
abduction. The effects vary considerably: in the dislocation. Walking becomes more and more tiring
mildest cases the changes are confined to the ossific and backache is common. With bilateral dislocation,
nucleus, which appears to be slightly distorted and the loss of abduction may hamper sexual intercourse
irregular on x-ray. The cartilaginous epiphysis retains in women.
the shape and physical growth is normal. After 12–24 Disability may be severe enough to justify total joint
months the appearances return to normal. In more replacement. The operation is difficult and should be 503
19
(a) (b) (c) (d)
19.14 Congenital subluxation (a) The cardinal physical sign, restricted abduction; (b) X-ray in
childhood; (c) in adolescence; (d) degeneration in early adult life.
undertaken only by those with experience of hip dislocation, damage to the lateral acetabular epiphysis
reconstructive surgery. The femoral head is seated or maldevelopment of the femoral head (either con-
above the acetabulum, which is shallow or completely genital or, for example, after Perthes’ disease). The
obliterated. A new socket should be fashioned at the socket is unusually shallow, the roof is sloping and
normal anatomical site; however, the pelvic wall is there is deficient coverage of the femoral head super-
usually thin and it may be necessary to build up the olaterally and anteriorly; in some cases the hip sublux-
roof of the socket with bone grafts. It is then difficult ates. Faulty load transmission in the lateral part of the
to bring the femoral head down to the level of the joint may lead to secondary osteoarthritis (OA).
socket without risking damage to the sciatic nerve; if
necessary, an osteotomy should be performed and a
small segment of femoral bone removed to allow a
safe fit. The proximal femur is usually very narrow and Clinical features
the neck may be markedly anteverted; this also may
During infancy, dysplasia may be clinically silent and
need correction when the osteotomy is performed,
only apparent on ultrasound examination. If there is
and special implants are available to fit the small
associated instability, Barlow’s test may be positive,
medullary canal.
but other clinical indicators including loss of abduc-
tion may be absent.
In children the condition is usually asymptomatic
and discovered only when the pelvis is x-rayed for
ACETABULAR DYSPLASIA AND some other reason. Sometimes, however, the hip is
SUBLUXATION OF THE HIP painful – especially after strenuous activity – and the
child may develop a limp. If there is subluxation the
Acetabular dysplasia may be genetically determined or Trendelenburg sign is positive, leg length may be
may follow incomplete reduction of a congenital asymmetrical and the femoral head may be felt as a
(a) (b) (c)
19.15 Acetabular dysplasia (a) X-ray showing a dysplastic left acetabulum. The socket is shallow and the roof sloping,
leaving much of the femoral head uncovered. Note that the femoral neck–shaft angle is somewhat valgus on both sides.
(b) Measuring Wiberg’s centre–edge (CE) angle; the line C–C joins the centre of each femoral head; C–B is perpendicular to
this and C–E cuts the superior edge of the acetabulum. The angle BCE should not be less than 30°; in this case the left hip
is abnormal. (c) X-ray of another patient showing acetabular dysplasia on the right side and secondary osteoarthritis in an
504 untreated dysplastic left hip.
IMAGING 19
X-rays should be taken lying and standing (the latter
may show minor degrees of incongruity). In the
supine anteroposterior radiograph, the acetabulum
looks shallow, the roof is sloping and the femoral head
is uncovered. Subtle abnormalities are revealed by
The hip
measuring the depth of the socket and the relation-
ship between the centre of the femoral head and the
edge of the acetabulum – Wiberg’s centre–edge (CE)
angle. With subluxation, Shenton’s line is broken.
The faux profil (oblique view) of the hip in the stand-
ing position will demonstrate acetabular dysplasia and
19.16 Acetabular dysplasia – three-dimensional CT incipient OA in the young adult. Congruity and sta-
Three-dimensional CT shows the full extent of the hip bility of the hip may be best assessed by examination
dysplasia in several planes, which is ideal for planning and dynamic arthrography under anaesthesia (Catter-
reconstructive surgery. all, 1992).
CT and MRI are helpful in those who are consid-
ered for operative treatment. Three-dimensional CT
reconstruction is particularly useful in providing an
lump in the groin; movement – particularly abduction accurate picture of the anatomy.
in flexion – is restricted.
Older adolescents and young adults may complain of
pain over the lateral side of the hip, probably due to Diagnosis
muscle fatigue and/or segmental overload towards It is often difficult to be sure that the patient’s symp-
the edge of the acetabulum. Some experience toms are due to the dysplastic acetabulum; other con-
episodes of sharp pain in the groin, possibly the result ditions causing pain and limp must be excluded (see
of a labral tear or detachment. Box on page 514).
Older adults (predominantly in their thirties and Bilateral dysplasia is a feature of developmental dis-
forties) usually present with features of secondary OA. orders, such as multiple epiphyseal dysplasia.
Indeed, in southern Europe dysplasia of the hip is the
commonest cause of symptomatic OA.
Treatment
NOTE: It is worth emphasizing that most people
with mild acetabular dysplasia go through life without Infants with subluxation are treated as for dislocation:
knowing that they are in any way abnormal and the the hip is splinted in abduction until the acetabular
condition exists only as a ‘x-ray diagnosis’. roof looks normal.
(a) (b)
19.17 Acetabular dysplasia – peri-acetabular osteotomy (a) Bilateral acetabular dysplasia, symptomatic on the left.
(b) X-ray after peri-acetabular osteotomy. Cuts were made through the innominate, the ischium and the lateral part of the
superior pubic ramus; the entire segment containing the acetabulum was then rotated so as to cover the load-bearing part
of the femoral head superolaterally and anteriorly. (Courtesy of Professor Kjeld Søballe. Århus Universitetshopital.) 505
19 Young children (4–10) are treated with a Salter be completely absent; however, some part of it often
innominate osteotomy, provided the dysplastic survives, although it is too osteoporotic to be seen.
acetabulum remains congruent. It is often difficult to Although the infection may be overcome and some
recommend surgery for an asymptomatic condition, measure of bone regeneration later appears, the dislo-
but significant persistent dysplasia, without improve- cation persists and the child presents with signs resem-
ment of the acetabular index, in a child over 5 years bling those of DDH – plus the telltale scars of old
old merits serious discussion. sinuses or an operation. Total destruction of the

Older children and young adolescents, provided the femoral head often results in a completely unstable
hip is reducible and congruent, often manage with no joint (Tom Smith’s arthritis).
more than muscle-strengthening exercises. If symp- Treatment of the acute infection is discussed in
toms persist, they may need an operation to augment Chapter 2. If there is a threat of joint instability, a hip
the acetabular roof, either a lateral shelf procedure or spica should be applied until the soft-tissue infection
a limited pelvic osteotomy such as the Chiari opera- has settled completely. In the absence of a femoral
tion, either of which may be combined with a varus head, the greater trochanter can be placed in the
osteotomy of the proximal femur. acetabulum or stability can be restored by performing
Older adolescents and young adults with pain, a valgus (Shanz) osteotomy with limb lengthening to
weakness, instability and subluxation of the hip are equality. In later life the patient will require further
candidates for peri-acetabular osteotomy and three- reconstructive surgery or total joint replacement.
dimensional re-orientation of the entire hip (Ganz et
al., 1998).
Patients with secondary OA may need inter tro-
Dislocation due to muscle imbalance
chanteric osteotomy or total hip replacement. Unbalanced paralysis in childhood may result in the
hip abductors being weaker than the adductors. This
is seen in cerebral palsy, in myelomeningocele and after
poliomyelitis (see Chapter 10). The foetal anteversion
ACQUIRED DISLOCATION OF THE of the femoral neck persists, the greater trochanter
HIP fails to develop properly, the femoral neck becomes
valgus and the hip may subluxate or dislocate.
Dislocation occurring after the first year of life is Treatment is similar to that of congenital disloca-
usually due to one of three causes: pyogenic arthritis, tion, but in addition some muscle-rebalancing opera-
muscle imbalance or trauma. Rare causes of acquired tion is essential.
dislocation include tuberculosis and Charcot’s disease.
Persistent traumatic dislocation
Dislocation following sepsis Occasionally dislocation of the hip is missed while
Septic arthritis of the hip in early childhood, whether attention is focussed on some more distal (and more
from direct infection of the joint or via spread from obvious) injury. Reduction is essential, if necessary by
metaphyseal osteomyelitis, may result in partial or open operation; even if avascular necrosis or hip stiff-
complete destruction of the largely cartilaginous ness supervenes, a hip in the anatomical position pres-
femoral head and pathological dislocation of the hip ents an easier prospect for reconstructive surgery than
(see Chapter 2). On x-ray the femoral head appears to one that remains persistently dislocated.
19.18 Acquired
dislocation in children
(a) Almost complete
destruction of the femoral
head following neglected
septic arthritis. (b) Bilateral
dislocation in a child with
muscle imbalance due to
spina bifida.
506 (a) (b)

The arc of rotatory movement of the hip is assessed 19
FEMORAL ANTEVERSION AND with the child prone and knees flexed. An in-toeing
RETROVERSION (‘IN-TOEING’ AND gait is associated with greater medial rotation of the
‘OUT-TOEING’) hip than lateral, but is considered to be within the
normal range as long as there is 20 degrees of lateral
rotation. Similarly, an out-toeing child has a normal
The hip
Children with in-toeing (and less commonly out-toe-
range if there is at least 20 degrees of internal rota-
ing) are often ‘taken to the doctor’ because of an awk-
tion.
ward gait. Usually this is no more than one extreme of
The alignment of the sole of the foot to the thigh
the normal developmental spectrum.
is known as the thigh–foot angle and combines the
The in-toeing child tends to trip over his or her feet
effect of any foot deformity as well as tibial torsion.
when running. The cause is rarely serious but a pater-
Palpation of the positions of the malleoli demon-
nalistic assurance that the child ‘will grow out of it’
strates the presence of tibial torsion.
may fail to convince the parents and certainly will not
Rotational profiles in the normal child are variable
satisfy the grandparents.
and charts documenting normal values are available. A
Internal rotation of the tibia is common at birth
rotational profile which lies outside two standard
and is usually associated with an equivalent degree of
deviations of the mean is considered abnormal and a
genu varum. This may produce in-toeing in the tod-
pathological cause should be considered (Staheli et al,
dler, which gradually resolves over a period of 2–3
1985).
years. External tibial torsion, producing out-toeing, is
Femoral neck anteversion can also be assessed by
less common.
ultrasonography or by obtaining axial CT scans across
In children between 3 and 10 years, the cause of in-
the hips and the knees and measuring the angle
toeing is usually femoral anteversion. (‘Version’ in this
between the axis of the femoral neck and the trans-
context describes the angle in the axial plane sub-
verse axis across the femoral condyles.
tended by the femoral neck and the femoral shaft,
Physiological rotational abnormalities have not
with ‘anteversion’ being an anterior tilt and ‘retrover-
been shown to have any long-term consequences and
sion’ a posterior tilt of the femoral neck and head.) In
parental reassurance is the cornerstone of treatment.
the young child anteversion may be as much as 40
Shoe modifications and orthotics are unnecessary
degrees, thus requiring the rest of the leg to turn
(Kling and Hensinger, 1983; Staheli, 1994).
inwards in order to keep the femoral head within the
acetabulum. Femoral neck anteversion decreases to
approximately 20 degrees by the age of 10 years, and
this is associated with a gradual loss of in-toeing (Engel
and Staheli, 1974; Kling and Hensinger, 1983). PROTRUSIO ACETABULI (OTTO
The gait may look clumsy but that is no bar to ath- PELVIS)
letic prowess and usually improves with growth.
These children often sit on the floor in the ‘television In this condition the socket is too deep and bulges
position’ with the knees facing each other. With the into the cavity of the pelvis. The ‘primary’ form shows
child standing, the patellae are turned inwards a slight familial tendency. It affects females much
(‘squinting patellae’) and there may be compensatory more often than males and develops soon after
external torsion of the tibiae. puberty; at this stage there are usually no symptoms
(b)
19.19 In-toe gait (a) These two sisters

have excessive anteversion with an in-toe
gait. (b) This explains their sitting posture
when playing or watching television.
(a) 507
19
(a) (b)
19.20 Protrusio acetabuli (a) The early stage in a child. (b) In this adult with protrusio, degenerative changes have
developed in both hips.
although movements are limited. X-rays show the neck bends or develops a stress fracture, and with con-
sunken acetabulum, with the inner wall bulging tinued weightbearing it collapses increasingly into
beyond the ilio-pectineal line. Secondary OA may varus and retroversion. Sometimes there is also short-
develop in later life, but until then the condition does ening or bowing of the femoral shaft. As the child
not require treatment. grows, the proximal femur keeps elongating but the
Protrusio may occur in later life secondary to bone neck–shaft angle goes into increasing varus. The con-
‘softening’ disorders, such as osteomalacia or Paget’s dition is bilateral in about one-third of cases.
disease, and in longstanding cases of rheumatoid
arthritis. If pain is severe, or movements are markedly
restricted, joint replacement is indicated.
Clinical features
The condition is usually diagnosed when the child
starts to walk. The leg is short and the thigh may be
bowed. X-rays show that the femoral neck is in varus
COXA VARA and abnormally short. Often there is a separate frag-
ment of bone in a triangular notch on the inferome-
The normal femoral neck–shaft angle is 160 degrees dial surface of the femoral neck. Because of the
at birth, decreasing to 125 degrees in adult life. An distorted anatomy, it is difficult to measure the neck–
angle of less than 120 degrees is called coxa vara. The shaft angle. A helpful alternative is to measure Hilgen-
deformity may be either congenital or acquired. reiner’s epiphyseal angle – the angle subtended by a
horizontal line joining the centre (triradiate cartilage)
of each hip and another parallel to the physeal line;
CONGENITAL COXA VARA the normal angle is about 30 degrees (Fig. 19.21a)
while the angle on the abnormal side is much larger
This is a rare developmental disorder of infancy and (Fig. 19.21c). At maturity the deformity may be quite
early childhood. It is due to a defect of endochondral bizarre. With bilateral coxa vara the patient may not
ossification in the medial part of the femoral neck. be seen until he or she presents as a young adult with
When the child starts to crawl or stand, the femoral OA.
Normal Abnormal
(a) (b) (c) (d)
19.21 Infantile coxa vara In the normal hip (a) Hilgenreiner’s epiphyseal angle is well within the normal range of 30–40°.
The measurements are shown in (b). On the opposite side (c) the physis is too vertical: 45–60° calls for careful follow-up
and review, and more than 60° is an indication for Pauwels’ valgus osteotomy. In a neglected case (d) the trochanteric
508 physis allows further growth but the femoral neck may remain fixed in marked varus.
Treatment During childhood, coxa vara is seen in rickets and 19
bone dystrophies, and sometimes after Perthes’ dis-
If the epiphyseal angle is more than 40 but less than ease. Deformity presenting in adolescence is more
60 degrees, the child should be kept under observa- likely to be due to epiphysiolysis.
tion and re-examined at intervals for signs of progres- At any age bone ‘softening’ may result in coxa vara;
sion. If it is more than 60 degrees, or if shortening is causes include osteomalacia, fibrous dysplasia, patho-
The hip
progressive, the deformity should be corrected by a logical fracture or the aftermath of infection. Other
subtrochanteric or intertrochanteric valgus osteotomy. causes of deformity are malunited fractures and
Pauwels demonstrated that permanent correction Paget’s disease.
was possible if the plane of the physeal plate was Treatment in the form of a corrective (valgus)
restored to normal and the characteristic triangular osteotomy is needed only if there is marked shorten-
metaphyseal fragment and protruding femoral head ing or intolerable discomfort. If the problem is due to
were supported on the femoral neck. These objectives a disproportionately high greater trochanter, distal
are possible with a Y-shaped intertrochanteric transposition of the trochanter may suffice.
osteotomy of the proximal femur (Fig. 19.22). Cordes
et al. (1991) evaluated 14 hips at a mean follow-up of
11 years and reported good/excellent function in 78
per cent. Patients with the three hips rated ‘poor’ had PROXIMAL FEMORAL FOCAL
a persistent Trendelenburg gait and fatigue pain. DEFICIENCY
Proximal focal femoral deficiency (PFFD) or congenital

ACQUIRED COXA VARA femoral deficiency (CFD) is a rare (possibly teratogenic)
Coxa vara can develop if the femoral neck bends or if anomaly with a spectrum of presentation between
it breaks. A ‘mechanical’ coxa vara sometimes results femoral hypoplasia and virtual absence of the femur.
from severe shortening of the femoral neck and rela- The condition is easily recognized: the affected limb is
tive overgrowth of the greater trochanter; during abnormally short, sometimes bizarre in appearance with
weightbearing the abductor muscles are at a mechan- the foot on that side lying at the same level as the knee
ical disadvantage and the patient walks with a severe on the opposite limb; the hip is usually held flexed,
Trendelenburg gait. abducted and externally rotated; in many cases there are
also other anomalies, such as fibular deficiency.
CLASSIFICATION AND MANAGEMENT

The most useful anatomical classification is that of
Aitken.
• In type A there appears to be a gap in the femoral
neck or subtrochanteric region, which is in fact a
segment of unossified cartilage. This does eventu-
ally ossify, but by then the proximal femur has
developed a varus deformity and shortening. The
femoral head and acetabulum are present.
44° P • In type B the ‘gap’ persists, the femoral head and
44° acetabulum are dysplastic, the upper end of the
16° femur lies above the acetabulum and there is sig-
H
nificant shortening.
• In type C the femoral head is missing and the
acetabulum is undeveloped.
(a) (b) • In type D there is agenesis of the entire proximal
femur and acetabulum. Both hips may be affected
19.22 Pauwels’ valgus osteotomy (a) Preoperative
planning on a tracing of the preoperative radiograph. P = and in half the cases there are also distal anomalies.
the plane of the physis. H is a horizontal line drawn well The classification suggested by Gillespie (1998) is
below the lesser trochanter. In this case a 44° closing
wedge osteotomy is required to correct the inclination of probably more useful for planning treatment.
the physis to 16°. (b) After the osteotomy and removal of • Patients in Group A have short femurs but stable
the 44° wedge from the lateral side of the proximal femur,
the femoral head and adjacent neck are supported by the hips, functional knees and the foot below the level
calcar femorale. (From Cordes et al., 1991. With of the middle of the opposite tibia. These can be
permission from the Journal of Bone and Joint Surgery.) considered for limb reconstruction and lengthen- 509
19
A B C D
19.23 Proximal femoral focal deficiency – Aitken’s classification In types A and B the femoral head and acetabulum
are present, though showing varying degrees of dysplasia. Coxa vara may be marked and shortening is significant. In types
C and D there is no effective hip joint, shortening is severe and distal deficiencies may be present.
ing. Abnormalities of the knee and foot may have to mity; were it not for the cosmetic problem, they are
be addressed as well. probably best left alone.
• Patients in group B correspond more or less to On a personal note: ‘Rotationplasty’ (an operation
those in Aitken’s types B and C; most of them can to turn the foot around so that the ankle acts like a
be treated by rotationplasty and a prosthesis or knee knee) sounds better than it turns out to be in real life.
fusion combined with ankle disarticulation and a The operation is difficult and fraught with complica-
prosthesis. tions; patients often end up needing multiple proce-
• Those in Group C correspond to Aitken’s type D. dures; the limb without a prosthesis is cosmetically
They have total (or near-total) absence of the questionable; and patients have been known to suffer
femur, sometimes associated with dysplasia of the severe psychological trauma with a foot facing back-
hemipelvis and absence of any acetabular develop- wards (Fixen, 1983).
ment. These patients require a prosthesis and in the
most deficient cases retaining the foot may actually
be beneficial to the prosthetist.
THE IRRITABLE HIP (TRANSIENT
Patients with bilateral symmetrical anomalies are SYNOVITIS)
functionally better than those with unilateral defor-
This condition is defined as a non-specific, short-lived

synovitis, resulting in an effusion of the hip joint. It is
the most common cause of an acute limp or hip pain
in children, with a reported frequency of 14 per 1000.
The most commonly affected age group is 3–8-year-
olds with boys affected twice as often as girls. It affects
both hips in 5 per cent of cases, although this is rarely
simultaneous.
Aetiology
While viral infections, trauma and allergy have been
suggested, the exact aetiology remains unclear. The
pathological process involves a synovial effusion
(a) (b) (c) resulting in an increased intra-articular pressure.
19.24 Proximal femoral dysplasia (a) This man was
born with transverse deficiency of the right arm and
bilateral proximal femoral focal deficiency. Although Clinical features
unhappy with his appearance, because the lower limb The typical patient presents with pain and a limp,
defects were symmetrical he was able to get about
remarkably well. (b) By contrast, this young man with often intermittent and following activity. Pain is felt in
similar but unilateral dysplasia was severely disabled. the groin or front of the thigh, sometimes reaching as
510 (c) X-ray showing the proximal femoral deficiency. far as the knee. Slight wasting may be detectable but
the cardinal sign is restriction of all movements with Although this condition carries a good prognosis, 19
pain at the extremes of the range in all directions. The recurrence rates of up to 10 per cent have been
diagnosis is based primarily on the clinical features. reported. A causal relationship with Perthes’ disease
Standard laboratory investigations including white has been suspected but remains unproven.
cell count, erythrocyte sedimentation rate (ESR) and
C-reactive protein concentration are usually within
The hip
normal limits. X-rays do not demonstrate any bony PERTHES’ DISEASE
defects, but occasionally there may be a subtle widen-
ing of the medial joint space (1–2 mm) when com-
Perthes’ disease – or rather Legg–Calvé–Perthes dis-
pared with the unaffected side. This is caused by the
ease, for in 1910 the condition was described inde-
effusion which allows the femoral head to sublux
pendently by three different people – is a painful
slightly; it may be confirmed by ultrasonography.
disorder of childhood characterized by avascular
Characteristically, symptoms last for 1–2 weeks and
necrosis of the femoral head. It is uncommon in any
then subside spontaneously; hence the synonym ‘tran-
community – the quoted incidence is about 1 in
sient synovitis’. The child may experience more than
10 000 – with a higher incidence in Japanese, Inuits
one episode, with an interval of months between
and central Europeans and a lower incidence in native
attacks of pain.
Australians, native Americans, Polynesians and blacks.
Patients are usually 4–10 years old and boys are
Differential diagnosis affected four times as often as girls.
The condition may be part of a general disorder of
The condition is important largely because it resem-
growth. Epidemiological studies in the UK have
bles a number of serious disorders which have to be
shown that there is a higher than usual incidence in
excluded.
underprivileged communities. Affected children and
Perthes’ disease is the main worry. Acute symptoms
their siblings have slightly retarded growth of the
usually last longer than 2 weeks and x-rays may show
trunk and limbs.
an increased ‘joint space’. Later, of course, the x-ray
As in other forms of non-traumatic osteonecrosis,
features are unmistakable.
inherited thrombophilia has been postulated as a con-
Slipped epiphysis may present as an ‘irritable hip’. Ini-
tributory cause and antithrombotic factor deficiencies
tially the x-ray looks normal and this may lead to com-
and hypofibrinolysis have been reported in children
placency. If the age and general build are suggestive, or
with Perthes’ disease (Glueck et al., 1996). This
if the symptoms persist, the x-ray should be repeated.
hypothesis has been questioned by others (Editorial
Tuberculous synovitis produces a raised ESR and the
by R. J. Liesner, 1999).
Heaf test is positive.
Juvenile chronic arthritis and ankylosing spondylitis
may start with synovitis of one hip and it may take
Pathogenesis
months before other joints are affected. Look for sys-
temic features and a raised ESR. In doubtful cases, The precipitating cause of Perthes’ disease is unknown
synovial biopsy may be helpful. but the cardinal step in the pathogenesis is ischaemia of
Septic arthritis should always be borne in mind. the femoral head. Up to the age of 4 months, the
The early symptoms and signs are sometimes mislead- femoral head is supplied by (1) metaphyseal vessels
ing, especially if someone has already prescribed which penetrate the growth disc, (2) lateral epiphyseal
antibiotics ‘just in case!’ vessels running in the retinacula and (3) scanty vessels
in the ligamentum teres. The metaphyseal supply grad-
ually declines until, by the age of 4 years, it has virtu-
Treatment ally disappeared; by the age of 7, however, the vessels
Treatment involves bed rest, reduced activity and in the ligamentum teres have developed. Between 4 and
observation, which may be supervised at home or in 7 years of age the femoral head may depend for its
hospital. Most children recover within a few days and blood supply and venous drainage almost entirely on
any deterioration in signs or symptoms requires the lateral epiphyseal vessels whose situation in the
urgent reassessment. Traction, although popular in retinacula makes them susceptible to stretching and to
the past, is not currently recommended as it may pressure from an effusion. Although such pressure may
increase the intra-articular pressure. Joint aspiration is be insufficient to block off the arterial flow, it could eas-
ineffective; any relief in symptoms tends to be short- ily cause venous stasis resulting in a rise in intraosseous
lived as the effusion rapidly recurs. pressure and consequent ischaemia (Lin and Ho,
Ultrasonography is repeated at intervals and 1991). This may be enough to tip the balance towards
weightbearing is allowed only when the symptoms infarction and necrosis in children who are constitu-
disappear and the effusion resolves. tionally predisposed. 511
19
(a) (b)
(c) (d)
(e) (f)
(g) (h)
19.25 Perthes’ disease – Herring classification The Herring classification is based on the severity of structural
disintegration of the lateral pillar of the femoral epiphysis. Column 1 shows the changes in a boy with moderately severe
Perthes’ disease of the right hip. Although the central part of the epiphyseal ossific centre seems to be ‘fragmented’, the
lateral part remains intact throughout the progress of the disease. This is a favourable feature and serial x-rays show how
the femoral head has gradually re-formed. Column 2 shows progressive changes in another boy with severe Perthes’
disease of the left hip. The epiphysis is widely involved from the outset, ‘fragmentation’ extends to the most lateral portion
512 of the epiphysis and there is progressive flattening of the epiphysis resulting in permanent distortion of the femoral head.
The immediate cause of capsular tamponade may X-rays 19
be an effusion following trauma (of which there is a
history in over half the cases) or a non-specific syn- Although the condition may be suspected from the clin-
ovitis. Two or more such incidents may be needed to ical appearances, diagnosis hinges on the x-ray changes.
produce the typical bone changes. At first the x-rays may seem normal, though subtle
changes such as widening of the ‘joint space’ and
The hip
slight asymmetry of the ossific centres are usually pres-
Pathology ent. Radionuclide scanning may show a ‘void’ in the
The pathological process goes through several stages anterolateral part of the femoral head.
which in total may last up to 3 or 4 years. The classic feature of increased density of the ossific
nucleus occurs somewhat later. This is often referred
Stage 1: Ischaemia and bone death All or part of the
to as the ‘necrotic phase’, though the radiographically
bony nucleus of the femoral head is dead; it still looks dense areas must surely be due to the new bone for-
normal on plain x-ray but stops enlarging. The carti- mation that always follows bone necrosis. This pro-
laginous part of the femoral head, being nourished by gresses to the phase of radiographic ‘fragmentation’ –
synovial fluid, remains viable and becomes thicker alternating patches of density and lucency, or some-
than normal. There may also be thickening and times a crescentic subarticular fracture often best seen
oedema of the synovium and capsule. in the lateral view. Epiphyseal density increases (the
Stage 2: Revascularization and repair Within weeks phase of re-ossification) and scintigraphy shows
(possibly even days) of infarction, a number of increased activity. With healing the femoral head may
changes begin to appear. Dead marrow is replaced by regain its normal (or near-normal) shape; however, in
granulation tissue, which sometimes calcifies. The less fortunate cases the femoral head becomes mush-
bone is revascularized and new lamellae are laid down room-shaped, larger than normal and laterally dis-
on the dead trabeculae, producing the appearance of placed in a dysplastic acetabular socket.
increased density on x-ray. Some of the dead trabecu- The Catterall classification The radiographic picture
lar fragments are resorbed and replaced by fibrous tis- varies with the age of the child, the stage of the
sue; when this happens, the alternating areas of disease and the amount of head that is necrotic.
sclerosis and fibrosis appear on the x-ray as ‘fragmen- Catterall (1982) described four groups, based on the
tation’ of the epiphysis. The metaphysis may become appearances in both anteroposterior and lateral x-rays.
hyperaemic and on x-ray looks rarefied or cystic. In In group 1 the epiphysis has retained its height and
older children, and more severe cases, morphological less than half the nucleus is sclerotic. In group 2 up to
changes may also appear in the acetabulum. half the nucleus is sclerotic and there may be some
Stage 3: Distortion and remodelling If the repair process collapse of the central portion. In group 3 most of the
is rapid and complete, the bony architecture may be re- nucleus is involved, with sclerosis, fragmentation and
stored before the femoral head loses its shape. If it is collapse of the head. Metaphyseal resorption may be
tardy, the bony epiphysis may collapse and subsequent present. Group 4 is the worst: the whole head is
growth of the femoral head and neck will be distorted: involved, the ossific nucleus is flat and dense and
the head becomes oval or flattened – like the head of a metaphyseal resorption is marked.
mushroom – and enlarged laterally, while the neck is of- The Herring classification This classification embodies a
ten short and broad. Slowly the femoral head is dis- greater degree of predictive value for the outcome of
placed laterally in relation to the acetabulum. Any the Perthes changes and is therefore preferred by
residual deformity is likely to be permanent. many orthopaedic surgeons. The features are
described below and illustrated in Figure 19.25.
Clinical features
The patient – typically a boy of 4–8 years – complains
Prognostic features
of pain and starts limping. Symptoms continue for
weeks on end or may recur intermittently. The child ap- The outlook for children with Perthes’ disease, as a
pears to be well, though often somewhat undersized. In group, is well summarized by Herring (1994): ‘A small
4 per cent there is an associated urogenital anomaly. percentage of patients have a very difficult course, with
The hip looks deceptively normal, though there recurrent loss of motion, pain, and an eventual poor
may be a little wasting. Early on, the joint is irritable outcome. However, most children have moderate prob-
so that all movements are diminished and their lems in the active phase of the disease and then improve
extremes painful. Often the child is not seen till later, steadily, eventually having a satisfactory outcome.’
when most movements are full; but abduction (espe- This does not, of course, absolve one from under-
cially in flexion) is nearly always limited and usually taking careful analysis and planning in dealing with
internal rotation also. the individual case. Age is the most important 513
19 prognostic factor: in children under 6 years the out-
APPROACH TO THE LIMPING CHILD
look is almost always excellent; thereafter, the older
the child the less good is the prognosis. There is a 1. Measure limb length
poorer prognosis, too, for girls than for boys. 2. Check the foot
A widely used radiographic guide is the Catterall Splinter? Injury?
classification (see above). The greater the degree of Swollen ankle: Infection? Arthritis?
femoral head involvement, the worse the outcome. 3. Examine the knee
This is recognized in the simpler classification of Salter Swelling: Infection? Arthritis? Tumour?
and Thompson, into those with more and those with 4. Examine the hip
less than half the head involved (Simmons et al., Septic arthritis?
1990). There is also the concept of the head at risk – Dislocation? Subluxation? Coxa vara? Transient
radiographic signs which presage increasing deformity synovitis?
and displacement of the femoral head: (1) progressive Perthes’ disease? Arthritis? Tumour?
uncovering of the epiphysis; (2) calcification in the car- 5 General assessment
tilage lateral to the ossific nucleus; (3) a radiolucent Exclude non-accidental injury
area at the lateral edge of the bony epiphysis (Gage’s
sign); and (4) severe metaphyseal resorption.
Common to all these predictive systems is the im-
portance of the structural integrity of the superolateral Analgesia and modification of activities are often suffi-
(principal load-bearing) part of the femoral head. This cient, but hospitalization for bed rest and short periods
is reflected in Herring’s lateral pillar classification. In of traction are sometimes necessary. Wheelchair use and
the anteroposterior x-ray, the femoral head is divided crutch walking should be discouraged in order to avoid
into three ‘pillars’ by lines at the medial and lateral unnecessary joint stiffness and contracture. Once joint
edges of the central ‘sequestrum’. Group A are those irritability has subsided, which usually takes about 3
with normal height of the lateral pillar. Group B are pa- weeks, movement is encouraged, particularly cycling
tients with partial collapse (but still more than 50 per and swimming. Preservation of abduction is also im-
cent height) of the lateral pillar; those under 9 years of portant, with formal stretching used in some children.
age usually have a good outcome but older children are The clinical and radiographic features are then re-
likely to develop flattening of the femoral head. Group assessed and the bone age is determined from x-rays of
C cases show more severe collapse of the lateral pillar the wrist. The choice of further management is between
(less than 50 per cent of normal height); these take (a) symptomatic treatment and (b) containment.
longer to heal and usually end up with significant dis- Symptomatic treatment means pain control (if nec-
tortion of the femoral head. essary by further spells of traction), gentle exercise to
maintain movement and regular reassessment. During
asymptomatic periods the child is allowed out and
about but sport and strenuous activities are avoided.
The irritable hip of early Perthes’ disease must be dif- Containment means taking active steps to seat the
ferentiated from other causes of irritability; the child’s femoral head congruently and as fully as possible in
fitness, the increased joint space and the patchy bone the acetabular socket, so that it may retain its spheric-
density are characteristic. In transient synovitis the ity and not become displaced during the period of
x-ray is normal. healing and remodelling. This is achieved (a) by hold-
Morquio’s disease, cretinism, multiple epiphyseal ing the hips widely abducted, in plaster or in a remov-
dysplasia, sickle-cell disease and Gaucher’s disease may able brace (ambulation, though awkward, is just
resemble Perthes’ disease radiologically, especially if possible, but the position must be maintained for at
they are bilateral; however, in bilateral Perthes’ disease least a year); or (b) by operation, either a varus
the two sides are likely to be at different stages. More- osteotomy of the femur or an innominate osteotomy
over, in the other conditions general diagnostic fea- of the pelvis, or both.
tures are usually apparent. In earlier years there was a good deal of support for
‘Old Perthes deformities’ in adults, in the 10 per cent non-operative containment, and this is still applicable
of cases with bilateral involvement, may resemble those where specialized surgical facilities are unavailable.
of certain bone dysplasias, especially multiple epiphyseal However, this has been questioned by more recent
dysplasia. Look for changes in other epiphyses. outcome studies and the preferred approach is to
achieve containment by operative methods (Martinez,
1992; Meehan 1992).
Management
Operative reconstruction provides the advantages of
The initial management of the child with Perthes’ improved containment and early mobilization. Short-
514 disease is determined by the severity of symptoms. term studies also suggest an improvement in the
19
The hip
(a) (b)
19.26 Perthes’ disease – operative treatment (a) The x-ray shows advanced Perthes changes and lateral displacement
of the right femoral head. (b) Following an innominate osteotomy, the femoral head is much better ‘contained’ and,
although not normal, is developing reasonably well.
anatomy of the hip, but there is no convincing evi- capital femoral epiphysis (SCFE) – is uncommon (1–
dence of any alteration in the natural history of the 3 per 100 000) and virtually confined to children
disorder or (in particular) the likelihood of needing an going through the pubertal growth spurt. Boys (usu-
arthroplasty in later life. ally between 14 and 16 years old) are affected more
often than girls (who are, on average, 2–3 years
GUIDELINES TO TREATMENT younger). The left hip is affected more commonly
There is no general agreement on the ‘correct’ course than the right and if one side slips there is a 25–40 per
of treatment for all cases. Decisions are based on an cent risk of the other side also slipping.
assessment of the stage of the disease, the prognostic
x-ray classifications, the age of the patient and the
clinical features, particularly range of abduction and
Aetiology
extension. The following guidelines are derived from The slip occurs through the hypertrophic zone of the
the review by Herring (1994). cartilaginous growth plate. Why should the physis
give way during a period of accelerated growth? Many
Children under 6 years No specific form of treatment has
of the patients are either fat and sexually immature or
much influence on the outcome. Symptomatic treat-
excessively tall and thin. It is tempting to formulate a
ment, including activity modification, is appropriate.
theory of hormonal imbalance as the underlying cause
Children aged 6–8 years In this group the bone age is of physeal disruption. Normally, pituitary hormone
more important than the chronological age. activity, which stimulates rapid growth and increased
physeal hypertrophy during puberty, is balanced by
Bone age at or below 6 years
increasing gonadal hormone activity, which promotes
Lateral pillar group A and B (or Catterall stage I and
physeal maturation and epiphyseal fusion. A disparity
II) – symptomatic treatment.
between these two processes may result in the physis
Lateral pillar group C (or Catterall stage III and IV)
being unable to resist the shearing stresses imposed by
– abduction brace.
the increase in body weight. This occurs most obvi-
Bone age over 6 years ously in the hypogonadal ‘Frohlich type’ of child, and
Lateral pillar group A and B (Catterall stage I and II) it may be a factor in cases associated with juvenile
– abduction brace or osteotomy. hypothyroidism. There are also instances of epiphysi-
Lateral pillar group C (Catterall stage III and IV) – olysis occurring in children with craniopharyngioma
outcome probably unaffected by treatment, but some after successful treatment and sudden reactivation of
would operate. pituitary activity. Oestrogens produce a decrease in
physeal width and increased physeal strength, which
Children 9 years and older Except in very mild cases
may partly explain the lower incidence in girls and rare
(which is rare), operative containment is the treat-
occurrence after menarche.
ment of choice.
Other factors may also play a part. The perichondr-
ial ring (the retaining ‘collar’ around the physis) is rel-
atively thinned in this age group and provides less
SLIPPED CAPITAL FEMORAL support for the increased load transmitted through
EPIPHYSIS the physis during the growth spurt. Most patients
with SCFE have a greater than average body mass
Displacement of the proximal femoral epiphysis – also index. Adolescents with SCFE also have either relative
known as femoral capital epiphysiolysis or slipped or absolute femoral neck retroversion and the physis 515
19 protracted history leading to a severe climax – the
‘acute-on-chronic’ slip. An initial acute slip occurs in
only 15 per cent of cases. In over 50 per cent of cases
there is a history of injury. In sequential bilateral slips,
the second slip is diagnosed within 18 months of the
first slip in 82 per cent of cases (Loder et al., 1993).
The patient is usually a child around puberty, typi-

cally overweight or very tall and thin. The presenting
symptom is almost invariably pain, sometimes in the
groin, but often only in the thigh or knee – which can
be very misleading. It may be called a ‘sprain’; often,
and unfortunately, it is disregarded. It soon disappears
(a) (b) only to recur with further exercise. Limp also occurs
19.27 Slipped epiphysis – clinical features (a) This boy early and is more constant. Sometimes the child
complained only of pain in his right knee. His build is becomes aware that the leg is ‘turning out’.
unmistakable and the resting posture of his right lower On examination the leg is externally rotated and is
limb tends towards external rotation. (b) On examination, 1–2 cm short. Characteristically there is limitation of
abduction and medial rotation were restricted. flexion, abduction and medial rotation. A classic sign
is the tendency to increasing external rotation as the
hip is flexed.
has an increased obliquity – on average 11 degrees Following an acute slip, the hip is irritable and all
more vertical than in children who do not develop movements are accompanied by pain.
SCFE (Galbraith et al., 1987).
Trauma plays a part, especially in the 30 per cent of
cases with an ‘acute’ slip. In the other 70 per cent
Imaging
there is a slow, progressive displacement – or a series
of slight displacements – sometimes culminating in a X-rays In very early cases the x-ray may be reported
major slip after relatively mild mechanical stress (the as ‘normal’; changes can be extremely subtle. This
‘acute-on-chronic’ slip). should not be taken as a signal to forego further
examination if symptoms persist! In most cases, even
trivial slipping can be diagnosed. In the anteroposte-
Pathology rior view the epiphyseal plate seems to be too wide
In slipped epiphysis the femoral shaft rolls into exter- and too ‘woolly’. A line drawn along the superior sur-
nal rotation and the femoral neck is displaced for- face of the femoral neck should normally intersect the
wards while the epiphysis remains seated in the epiphysis. In an early slip the epiphysis may be flush
acetabulum. Disruption occurs through the hyper- with or even below this line (Trethowan’s sign). The
trophic zone of the physis and, relatively speaking, the metaphyseal blanch sign of Steel is a ‘double-density’
epiphysis slips posteriorly on the femoral neck. If the seen at the level of the metaphysis on an AP x-ray. It
slip is severe, the anterior retinacular vessels are torn. reflects the posterior cortical lip of the epiphysis as it
At the back of the femoral neck the periosteum is is beginning to slip posteriorly and becomes superim-
lifted from the bone with the vessels intact; this may posed on the metaphysis.
be the main – or the only – source of blood supply to Capener’s sign describes loss of the intracapsular
the femoral head, and damage to these vessels by area at the medial aspect of the femoral neck, which
manipulation or operation may result in avascular normally overlaps the posterior wall of the acetabulum
necrosis. creating a dense triangular shadow.
Physeal disruption leads to premature fusion of the Decreased epiphyseal height, physeal widening,
epiphysis – usually within 2 years of the onset of lesser trochanter prominence due to increased exter-
symptoms. This is accompanied by considerable bone nal rotation of the femur and new bone formation in
modelling and, although there may be a permanent the posterior femoral metaphysis, with anterior-
external rotation deformity and apparent coxa vara, remodelling, are also useful signs in diagnosis.
adaptive changes often ensure good joint function In the lateral view the femoral epiphysis is tilted
even without treatment. backwards; this is the most reliable x-ray sign and
minor abnormalities can be detected by measuring the
angle subtended by the epiphyseal base and the
Clinical features femoral neck; this is normally a right angle and any-
Slipping usually occurs as a series of minor episodes thing less than 87 degrees means that the epiphysis is
516 rather than a sudden, acute event; or there may be a tilted posteriorly.
19
The hip
(a)
(b)
19.28 Slipped epiphysis – x-rays (a) Anteroposterior and (b) lateral views of early slipped epiphysis of the right hip. The
upper diagrams show Trethowan’s line passing just above the head on the affected side, but cutting through it on the
normal side. The lateral view is diagnostically more reliable; even minor degrees of slip can be shown by drawing lines
through the base of the epiphysis and up the middle of the femoral neck – if the angle indicated is less than 90°, the
epiphysis has slipped posteriorly.
Ultrasonography Ultrasonography may detect a hip knee lasting more than 3 weeks; episodes of deterio-
effusion associated with an acute event, and may also ration and remission; loss of internal rotation, ab-
show metaphyseal remodelling in a chronic slip. duction and flexion of the hip and limb shortening.
• Acute-on-chronic slip: Long prodromal history and
Magnetic resonance imaging MRI has been used to
an acute, severe exacerbation.
detect and stage avascular necrosis (AVN) of the
femoral head. While this temporal classification is commonly
Computed tomography Three-dimensional CT scan- used, it does not correlate to the risk of avascular
ning has proved useful in the preoperative planning of necrosis or predict the outcome in the longer term.
realignment procedures for complex proximal femoral Loder et al. (1993) described a classification that
deformities. discriminated between the stable slipped epiphysis
when the child walked with or without crutches and
the unstable, when walking was not possible. This dis-
Grading tinction is clinically useful as it correlates with the risk
Slipped capital femoral epiphysis can be graded by the of avascular necrosis, which occurs in 0 per cent of sta-
clinical presentation and/or radiographic appearance. ble slips and 47 per cent of unstable slips.
The simplest classification is based on the timing of Radiological grading is based on measurement of
onset: pre-slip, acute, chronic or acute-on-chronic. the magnitude of the slip relative to the width of the
femoral neck, or the angle of the arc of the slip. The
• Pre-slip: The child complains of groin or knee pain, prognosis of a slip is associated with both the distance
particularly on exertion, and there may be a limp. of slippage and the degree of angulation.
Examination is often normal, but may demonstrate On a ‘frog lateral’ x-ray the slip is divided into three
reduced internal rotation. The x-ray may show stages according to the percentage slip of the epiph-
widening or irregularity of the physis. ysis in relation to the femoral neck.
• Acute slip: Symptoms present for less than 3 weeks;
painful hip movements with an external rotation • Mild: Displacement is less than one-third of the
deformity, shortening and marked limitation of width of the femoral neck.
rotation (the greater the limitation of motion, the • Moderate: Displacement is between one-third and
greater the degree of slip). Symptoms last for less a half.
than 3 months. • Severe: Displacement is greater than half of the
• Chronic slip: The child has pain in the groin, thigh or femoral neck width. 517
19
(a) (b) (c)
19.29 Moderate slip – treatment (a) A moderate slip can be accepted and fixed internally; it is essential that the
threaded pins or screws enter the femur anteriorly so as not to risk damaging the retinacular vessels on the back of the
femoral neck. (b) The femoral neck seen from behind and from above, showing the position of the vessels
posterosuperiorly. (c) An alternative method of fixation – the Heyman and Herndon epiphyseodesis.
Jerre and Billing (1994) described a classification

based on the magnitude of the epiphyseal–femoral
shaft angle seen on the ‘frog lateral’ view. This
requires precise placement of the limb in 90 degrees
of external rotation with neutral rotation of the hip
and the thigh elevated 25 degrees from the table. This
position is often painful and caution is advised in
unstable slips, which may displace further.
• Mild: Angle less than 30 degrees.
• Moderate: Angle 31–50 degrees. 19.30 Severe slip – open reduction Dunn’s operation
• Severe: Angle more than 50 degrees. for a severe slip. A small segment of the femoral neck is
removed so that the epiphysis can be reduced and pinned
without placing tension on the posterior vessels.
Treatment
The aims of treatment are (1) to preserve the epiphy-
seal blood supply, (2) to stabilize the physis and (3) to the epiphysis is easier said than done: because the
correct any residual deformity. Manipulative reduc- head is tilted backwards, pins driven up in the axis of
tion of the slip carries a high risk of avascular necrosis the femoral neck will either enter the most anterior
and should be avoided. The choice of treatment segment of the epiphysis (and be very insecure) or will
depends on the degree of slip. penetrate the posterior cortex of the femoral neck and
damage the retinacular vessels. Therefore, short
Minor slips (less than one-third of the width of the
threaded pins are inserted on the anterior femoral
epiphysis on the AP x-ray and less than 20 degrees tilt
neck and directed posteromedially into the centre of
in the lateral view). Deformity is minimal and needs
the epiphysis. Alternatively – and probably with less
no correction. The position is accepted and the physis
risk of complications – fusion can be achieved by bone
is stabilized by inserting one or two screws or
graft epiphyseodesis. At the same time any protruding
threaded pins along the femoral neck and into the epi-
bump on the anterosuperior metaphysis can be
physis, under fluoroscopic control.
trimmed to prevent impingement on the lip of the
Moderate slips (between one-third and two-thirds of acetabulum.
the width of the epiphysis on the AP x-ray and 20–40
Severe slips (more than two-thirds of the width of the
degrees of tilt in the lateral view). Deformity resulting
epiphysis on the AP x-ray and 40 degrees of tilt in the
from this degree of slip, though noticeable, is often
lateral view). This, the ‘unacceptable slip’, causes
tempered by gradual bone modelling and may in the
marked deformity which, untreated, will predispose to
end cause little disability. One can therefore accept the
secondary OA. Closed reduction by manipulation is
position, fix the epiphysis in situ and then wait: if,
dangerous and should not be attempted.
after a year or two, there is a noticeable deformity, a
corrective osteotomy is performed below the femoral Open reduction by Dunn’s method (Dunn and
518 neck (see below). This approach is safe, but ‘fixing’ Angel, 1978) gives good results, but should be
19
The hip
(a) (b) (c) (d) (e)
19.31 Severe slip – fixation and osteotomy (a–c) A severe slip can be treated by fixing it and then performing a
compensatory osteotomy. Wedges are cut based laterally and anteriorly so as to permit valgus, flexion and rotation at the
osteotomy. (d,e) The position after osteotomy and internal fixation.
reserved for the specialist. The greater trochanter is Avascular necrosis Death of the epiphysis used to be
elevated and the femoral neck exposed. By gentle sub- common. It is now recognized that it hardly ever
periosteal dissection, the posterior retinacular vessels occurs in the absence of treatment. This iatrogenic
are preserved while mobilizing the epiphysis (which is complication is minimized by avoiding forceful
usually stuck down by young callus). A small segment manipulation and operations which might damage the
of the femoral neck is then removed, so that the epi- posterior retinacular vessels.
physis can be repositioned without tension on the
Articular chondrolysis Cartilage necrosis probably
posterior structures; once reduced, it is held by two or
results from vascular damage (often iatrogenic), but in
three pins. In all but the most experienced hands, this
these cases bone changes are minimal. There is pro-
still carries a 5–10 per cent risk of avascular necrosis or
gressive narrowing of the joint space and the hip
chondrolysis.
becomes stiff.
The alternative – and the method recommended for
This is a recognized complication in SCFE, and
the less experienced surgeon – is to fix the epiphysis as
does not appear to be related to the method of treat-
for a ‘moderate slip’ and then, as soon as fusion is com-
ment. In some cases, the condition improves sponta-
plete, to perform a compensatory intertrochanteric
neously while in others it leads to loss of mobility and
osteotomy: the easiest is a triplane osteotomy with
OA.
simultaneous repositioning of the proximal femur in
valgus, flexion and medial rotation; more anatomical is Coxa vara A slipped epiphysis that goes unnoticed –
the geometric flexion osteotomy described by Griffith or is inadequately treated – may result in coxa vara.
(1976). However, the patient should be told that this Except in the most severe cases, this is more apparent
may result in 2–3 cm of shortening. than real; the head slips backwards rather than down-
General note: Most of the complications of slipped wards and the deformity is essentially one of femoral
epiphysis are related to treatment – injudicious neck retroversion. Secondary effects are external rota-
attempts at manipulative reduction of the slip, or fail- tion deformity of the hip, possibly shortening of the
ure to recognize the hazards of internal fixation (Riley femur and (still a point of contention) secondary OA.
et al., 1990). The first rule of surgical treatment is
‘thou shalt do no harm’!
Slipped epiphysis in adults
Epiphysiolysis is occasionally seen in young adults
Complications with endocrine disorders (hypogonadism, hypopitu-
Slipping at the opposite hip In at least 20 per cent of itarism or hypothyroidism). This is a risk to be borne
cases slipping occurs at the other hip – sometimes in mind in all patients with open physes in the proxi-
while the patient is still in bed. Forewarned is fore- mal femur, and especially those who are then treated
armed: the asymptomatic hip should be checked by with growth hormone and suddenly increase in
x-ray and at the least sign of abnormality the epiphysis stature before the physes stabilize. Treatment is the
should be pinned. same as in children. 519
19 PYOGENIC ARTHRITIS
be held absolutely still and all attempts at moving the
hip are resisted. With care and patience it may be pos-
(see also Chapter 2) sible to localize a point of maximum tenderness over
the hip; the diagnosis is confirmed by aspirating pus
Pyogenic arthritis of the hip is usually seen in children or fluid from the joint and submitting it for laboratory
under 2 years of age. The organism (usually a staphy- examination and bacteriological culture.
lococcus) reaches the joint either directly from a dis- In the acute stage x-rays are of little value but some-
tant focus or by local spread from osteomyelitis of the times they show soft-tissue swelling, displacement of
femur. Unless the infection is rapidly aborted, the the femoral head and a vacuum sign in the joint.
femoral head, which is largely cartilaginous at this age, Ultrasonography will reveal the joint effusion.
is liable to be destroyed by the proteolytic enzymes of Diagnosis can be difficult, especially in neonates
bacteria and pus. who may be almost asymptomatic. If the baby looks
Adults, also, may develop pyogenic hip infection, ei- ill and no cause is apparent, think of deep sepsis and
ther as a primary event in states of debilitation or (more look for a possible source (e.g. an intravascular line).
often) secondary to invasive procedures around the hip. A high index of suspicion is the best aid.
Clinical features Treatment

The child is ill and in pain, but it is often difficult to Intravenous antibiotics should be given as soon as the
tell exactly where the pain is! The affected limb may diagnosis is reasonably certain. The joint is aspirated
under general anaesthesia and, if pus is withdrawn,
anterior arthrotomy is performed; antibiotics are
instilled locally and the wound is closed without
drainage. Systemic antibiotics are essential, and the
hip is kept on traction or splinted in abduction until
all evidence of disease activity has disappeared.
Complications
(a) If the infection is unchecked the head and neck of the
femur may be destroyed and a pathological dislocation
result. The pus may escape and, when the child recov-
ers, the sinus heals. The hip signs then resemble those
of a congenital dislocation, but the telltale scar remains
and on x-ray the femoral head is completely absent.
TUBERCULOSIS
(b)
The disease may start as a synovitis, or as an

osteomyelitis in one of the adjacent bones. Once
arthritis develops, destruction is rapid and may result
in pathological dislocation. Healing usually leaves a
fibrous ankylosis with considerable limb shortening
and deformity.
Clinical features
The condition starts insidiously with aching in the
(c) (d) groin and thigh, and a slight limp; later, pain is more
severe and may wake the patient from sleep.
19.32 Pyogenic arthritis (a,b) In an infant: the left hip is
distended and the head is drifting out of the socket. Six With early disease (synovitis or osteomyelitis) the
months later the epiphysis appears to be necrotic. (c,d) In joint is held slightly flexed and abducted, and
520 an adult: rapid bone destruction over a period of 3 weeks! extremes of movement are restricted and painful, but
19
The hip
19.33 Hip tuberculosis – drug treatment In this patient, antituberculous drugs alone resulted in healing – though hip
movements were still restricted.
until x-ray changes appear the hip is merely ‘irritable’ necessary once all signs of activity have disappeared,
and diagnosis is difficult. If arthritis supervenes the but usually not before the age of 14.
hip becomes flexed, adducted and medially rotated, In older patients with residual pain and deformity,
muscle wasting becomes obvious, and all movements if the disease has clearly been inactive for a consider-
are grossly limited by pain and spasm. able time, total joint replacement is feasible and often
successful; with antituberculous drugs, which are
X-ray The earliest change is general rarefaction but
essential, the chances of recurrence are not great.
with a normal joint space and line; the femoral epiph-
Girdlestone’s excisional arthroplasty is occasionally
ysis may be enlarged or a bone abscess visible; with
the only option.
arthritis, in addition to the general rarefaction, there
is destruction of the acetabular roof (wandering
acetabulum) or the femoral head, usually both; the
joint may be subluxed or even dislocated. With heal- RHEUMATOID ARTHRITIS
ing the bones re-calcify. (see also Chapter 3)
Outcome The hip joint is frequently affected in rheumatoid

Early disease, if properly treated, may heal leaving a arthritis; occasionally the disease remains monarticular
normal or almost normal hip, but once the articular for several years, but eventually other sites are affected.
surface is destroyed the usual result is an unsound Persistent synovitis in a weightbearing joint soon leads
fibrous joint. In untreated cases, the leg becomes to the destruction of cartilage and bone; the acetabu-
scarred and thin; shortening is often severe because of lum is eroded and eventually the femoral head may per-
bone destruction, adduction and flexion deformity of
the hip and (in children) damage to the upper femoral
epiphysis and occasionally premature fusion of the
lower femoral epiphysis (especially if the child has
been in a spica for too long).
Treatment
Antituberculous drugs are essential, and these alone
may result in healing. Skin traction is applied and, for
a child, an abduction frame may be used. An abscess
in the femoral neck is best evacuated; if the arthritis
does not settle, joint ‘debridement’ is performed. As
the disease subsides, traction is discontinued and 19.34 Rheumatoid arthritis – treatment Severe erosive
movement is encouraged. arthritis treated by hip replacement with an uncemented
If the joint has been destroyed, arthrodesis may be socket and bone grafting of the acetabulum. 521
19 forate its floor. The hallmark of the disease is progres- Table 19.2 Causes of osteoarthritis of the hip
sive bone destruction on both sides of the joint with-
Abnormal stress Defective cartilage Abnormal bone
out any reactive osteophyte formation.
Subluxation Infection Fracture
Coxa magna Rheumatoid Necrosis
Clinical features Coxa vara Calcinosis Paget’s
Minor deformities Other causes
Usually the patient already has rheumatoid disease Protrusio of scelorosis

affecting many joints. Pain in the groin comes on
insidiously; limp, though common, may be ascribed
to pre-existing arthritis of the foot or knee. With
advancing disease the patient has difficulty getting immune to the disease. This may simply be because
into or out of a chair, and even movements in bed certain predisposing conditions (acetabular dysplasia,
may be painful. Occasionally the slow symptomatic Perthes’ disease, slipped epiphysis) show a similar dif-
progression is punctuated by acute flares with intense ferential incidence in these populations.
pain in the hip. Where there is an obvious underlying cause the
Wasting of the buttock and thigh is often marked, term ‘secondary osteoarthritis’ is applied (Table 19.2);
and the limb is usually held in external rotation and these patients are often in their third or fourth decade
fixed flexion. All movements are restricted and painful. and the appearance of the joint reflects the preceding
abnormality. Thus in regions where congenital dislo-
X-rays During the early stages there is osteoporosis and
cation and acetabular dysplasia are common (e.g. in
diminution of the joint space; later, the acetabulum and
southern Europe), women are more often affected
femoral head are eroded. Protrusio acetabuli is com-
than men, the hips may be the only joints affected and
mon. In the worst cases (and especially in patients on
lateral subluxation is common.
corticosteroids) there is gross bone destruction and the
When no underlying cause is apparent, the term
floor of the acetabulum may be perforated.
‘primary osteoarthritis’ is used. It is now believed that
even in these cases there is some preceding disorder
Treatment that leads to articular cartilage damage and subtle
abnormalities are being sought in patients who would
If the disease can be arrested by general treatment, hip
otherwise fall into the ‘primary’ category. In the case
deterioration may be slowed down. However, once
of the hip particular attention has been given to
cartilage and bone are eroded, no treatment will influ-
anatomical and mechanical factors that affect joint
ence the progression to joint destruction. Total joint
congruency and predispose to femoro-acetabular
replacement is then the best answer. It relieves pain
impingement and erosion of the articular surface. This
and restores a useful range of movement. It is advo-
comparatively new field of enquiry is explored on
cated even in younger patients, because the pol-
page 524.
yarthritis so limits activity that the implants are not
unduly stressed.
Care should be taken during operation to prevent Pathology
fracture or perforation of the osteoporotic bone. If
The articular cartilage becomes soft and fibrillated
the acetabular floor is deficient, a supportive cage and
while the underlying bone shows cyst formation and
bone grafting will be needed.
sclerosis. These changes are most marked in the area
Children with juvenile chronic arthritis may need
of maximal loading (chiefly the top of the joint); at
custom-made prostheses for their small and often del-
the margins of the joint there are the characteristic
icate bones.
osteophytes. Synovial hypertrophy is common and
Postoperative infection poses a greater risk in
capsular fibrosis may account for joint stiffness. The
rheumatoid patients than in others – more particularly
pathology of OA is discussed in greater detail in
if the patient is on corticosteroid therapy. Prophylaxis
Chapter 5.
is even more important than usual.
Sometimes articular destruction progresses very
rapidly, with erosion of the femoral head or acetabu-
lum (or both), occasionally going on to perforation of
the pelvis. This could be due to basic calcium crystal
OSTEOARTHRITIS deposition in the joint (see Chapter 4).
Clinical features
The hip joint is one of the commonest sites of OA,
though in some populations (e.g. African Negroes Pain is felt in the groin but may radiate to the knee.
522 and southern Chinese) this joint seems peculiarly Typically it occurs after periods of activity but later it
19
The hip
(a) (b) (c) (d)
19.35 Osteoarthritis – pathology (a) Normal ageing causes slight degeneration of the articular surface but the general
structure is well preserved. (b) By contrast, in progressive osteoarthritis the load-bearing area suffers increasing damage: in
this case the superior surface of the femoral head is completely denuded of cartilage and there are large osteophytes
around the periphery. In the coronal section (c,d) subarticular cysts are clearly revealed.
is more constant and sometimes disturbs sleep. Stiff- are restricted; internal rotation, abduction and exten-
ness at first is noticed chiefly after rest; later it sion are usually affected first and most severely.
increases progressively until putting on socks and
X-ray The earliest sign is a decreased joint space, usu-
shoes becomes difficult. Limp is often noticed early
ally maximal in the superior weightbearing region but
and the patient may think the leg is getting shorter.
sometimes affecting the entire joint. Later signs are
The patient is usually fit and over 50, but second-
subarticular sclerosis, cyst formation and osteophytes.
ary OA can occur at 30 or even 20 years of age. There
The shape of the femoral head or acetabulum may give
may be an obvious limp and, except in early cases, a
a clue to an underlying condition (e.g. old Perthes’ dis-
positive Trendelenburg sign. The affected leg usually
ease or a previous inflammatory arthritis). Bilateral cases
lies in external rotation and adduction, so it appears
occasionally show features of a generalized dysplasia.
short; there is nearly always some fixed flexion,
although this may only be revealed by Thomas’ test.
Muscle wasting is detectable but rarely severe. Deep
pressure may elicit tenderness, and the greater
Treatment
trochanter is somewhat high and posterior. Move- Analgesics and anti-inflammatory drugs may be help-
ments, though often painless within a limited range, ful, and warmth is soothing. The patient is encour-
19.36 Osteoarthritis – x-ray

(a–c) Cartilage softening and thinning are
greatest in the zone of maximal stress.
There is a vascular reaction and new-bone
formation in the subchondral bone as well
as osteophytic growth at the margins of
the joint. These changes, as well as
subchondral cyst formation, are reflected in
the sequential x-ray appearances (d–f).
(a) (b) (c)
(d) (e) (f) 523

19 19.37 Secondary
osteoarthritis (a) After Perthes’
disease. (b) After slipped upper
femoral epiphysis. (c) After
congenital subluxation. (d) After
rheumatoid disease. (e) Bilateral
in a patient with multiple
epiphyseal dysplasia.
(a) (b) (c)
(d) (e)
aged to use a walking stick and to try to preserve incidence of later backache, as well as deformity and
movement and stability by non-weightbearing exer- discomfort in other nearby joints (Solomon, 1998).
cises. In early cases physiotherapy (including manipu-
lation) may relieve pain for long periods. Activities are
adjusted so as to reduce stress on the hip.
FEMORO-ACETABULAR
Operative treatment The indications for operation are IMPINGEMENT AND
(1) progressive increase in pain, (2) severe restriction OSTEOARTHRITIS
of activities, (3) marked deformity and (4) progressive
loss of movement (especially abduction), together
with (5) x-ray signs of joint destruction.
Reinhold Ganz and Michael Leunig
In the usual case – a patient aged over 60 years with Although morphological abnormalities of the femoral
a long history of pain and increasing disability – the head and acetabulum have long been recognized in
preferred operation is total joint replacement (see patients with ‘secondary’ OA of the hip, the concept
below). In those between 40 and 60 years this may of femoro-acetabular impingement as a potent cause
still be the best operation if joint destruction is severe. of ‘primary’ OA is comparatively new and its patho-
In younger patients, particularly those with some genesis has been elaborated only in the last decade.
preservation of articular cartilage, an intertrochanteric The human hip is a ball-and-socket joint in which
realignment osteotomy may be considered. If per- the load-transmitting surfaces are covered by hyaline
formed early, it can arrest or delay further cartilage cartilage, thus offering minimal gliding resistance
destruction, and if the operation is well planned it even during peak loading while permitting sufficient
does not preclude later replacement arthroplasty. motion to serve the normal activities of daily living.
In recent years osteochondroplasty has gained atten- The range of motion of the hip joint is determined
tion following the realization that ‘primary’ or ‘idio- to a large extent by the head–neck ratio and the head
pathic’ OA of the hip is often associated with size. Other influences include the spatial orientation
malposition or malcongruency of this ball-and-socket of the acetabular socket and the proximal end of the
joint. This is discussed in the next section. femur as well as the femoral neck offset. A certain
Arthrodesis of the hip is a practical solution for amount of anteversion of the socket and the femoral
young adults with marked destruction of a single neck is necessary for the optimal amount of flexion
joint, and particularly when the conditions for and internal rotation of the hip. This combination of
advanced reconstructive surgery are less than ideal. If flexion and internal rotation represents the most
well executed, the operation guarantees freedom from important type of motion for optimal bipedal func-
pain and permanent stability, though it has the dis- tion. It is now known that if the combined angle of
524 advantages of restricted mobility and a significant anteversion is less than 40 degrees, flexion–internal
19
The hip
(a) (b)
19.38 The ‘pistol-grip’ deformity X-ray of the pelvis in a

man of 59 years who complained of pain in both hips.
There is a loss of sphericity of the femoral heads, and
unusual bony prominence of the uppermost outline at the
head–neck junction in both hips, producing an appearance
reminiscent of an old-fashioned pistol grip.
rotation is limited and may be painful, and that this

condition is often associated with early OA of the hip
(Tönnis and Heinecke, 1999).
Almost 100 years ago, Preisser recognized that lim- (c) (d)
ited internal rotation could be a precursor to OA of 19.39 The two types of FAI Pincer type of impingement:
the hip. During the 1960s–1980s Murray (1965), (a) Hip in neutral position. Femoral head ‘overcovered’ by
Solomon (1976), Harris et al. (1986) and Stuhlberg acetabular rim prominence. Arrow indicates intended
motion. (b) During internal rotation the femoral neck abuts
et al. (1975) noted a significant association between against the rim prominence; high shearing force
early hip OA and subtle morphological abnormalities posteriorly. Cam type of impingement: (c) Bony
of the proximal femur, such as retroversion and the prominence at anterior head–neck junction. During flexion,
so-called ‘pistol-grip’ deformity of the femoral head internal rotation and adduction (d) the abnormally
(Fig. 19.38). Tönnis and Heinecke (1999) explored prominent head/neck abuts against the acetabular rim; the
head is jammed in the acetabular cavity producing outside-
the relationship between acetabular and femoral in avulsion or abrasion of the cartilage from the labrum.
anteversion and OA and gave a detailed description of
how to measure these parameters.
These observations led to the theory that most, if not
all, cases of so-called ‘primary’ OA of the hip are sec-
ondary to minimal deformities previously unnoticed or
ignored, and that the initial cartilage damage is caused force, mostly in the posterior part of the joint during
by femoro-acetabular impingement (FAI) (Ganz et al., medial rotation of the hip.
2003; Beck et al., 2005; Ganz et al., 2008). In the cam mechanism bony thickening at the
femoral head–neck junction (i.e. a low head:neck
Pathomechanics of femoro-acetabular ratio) causes jamming of the femoral neck against the
front of the acetabulum and abrasion or delamination
impingement
of the acetabular cartilage. During the early phase of
There are two main subtypes of FAI: pincer and cam. cam FAI the acetabular labrum is normal in size and
In the pincer mechanism there is either global over- structure, but it may degenerate over time.
coverage of the femoral head (circumferentially as in The pattern of cartilage damage differs in these two
coxa profunda or protrusio) or local overcoverage of types of FAI; however the majority of FAI hips show
the femoral head by the anterior part of the acetabu- a mixed type of impingement with predominance of
lar rim if the acetabular opening is retroverted. As a the cam type. The peripheral anterosuperior part
consequence a bony ridge (or osteophyte) abuts of the joint is severely involved and the central portion
against the front of the femoral neck during joint of the joint is not involved until there is progression
motion. This results in fatiguing and degeneration of of more advanced OA. However, in the pincer FAI
the anterior part of the acetabular labrum along with pathology the postero-inferior joint cartilage may
a small zone of the adjacent articular cartilage develop damage rather early; this could represent a
(Fig. 19.39). There may also be an increased shearing contre-coup lesion. 525
19 Table 19.3 Characteristics of two types of femoro-acetabular impingement (FAI)
FAI type Pincer Cam
Typical patient Female, 30–40 years old Male, 20–30 years old, high activity
Level of deformity Acetabulum Proximal femur

Pathological anatomy Deep socket Non-spherical extension femoral head Prominent

Maloriented socket (idiopathic, metaphysis, retrotilted head
iatrogenic) Low anteversion femoral neck/low CCD angle (idiopathic,
iatrogenic)
Associated disorders Coxa profunda/ protrusio Idiopathic, Perthes’ disease, avascular necrosis, slipped
Acetabular retroversion (idiopathic, capital femoral epiphysis, retrotilt after neck fracture,
proximal focal femoral deficiency, iatrogenic
post-traumatic dysplasia, iatrogenic)
Structure of primary damage Labrum Acetabular cartilage with outside-in abrasion/delamination
Secondary changes Bone apposition acetabular rim, (double Labral degeneration

line), contre-coup lesion, postero-inferior
cartilage, bone apposition, femoral neck,
impingement cyst, head–neck junction
First radiological signs Postero-inferior joint space narrowing Anterolateral migration femoral head
Progression Slow – rather painful Rapid, although only mildly symptomatic
Aetiology of femoro-acetabular groin pain may be exacerbated by excessive demand

impingement on the hip or may present after sitting for a prolonged
period. Examination reveals a restriction of internal
Slipped capital femoral epiphysis (SCFE), Perthes’ rotation in flexion. This test indicates the presence of
disease and post-traumatic dysplasia are all associated abnormal morphology of the femoral neck and
with a high incidence of pincer and cam types of FAI acetabular rim with recreation of pain, particularly
(Ganz et al., 1991; Dora et al., 2000; Leunig et al., once there is a chondral or labral lesion. Occasionally
2000) (see Fig.19.39), but the aetiology of most cases pain is elicited with flexion–abduction and/or with
of ‘idiopathic’ FAI remains unknown. hyperextension and external rotation pointing
Retroversion of the acetabulum has been associated towards an impingement in these areas.
with hip pain and OA (Reynolds et al., 1999; Giori The typical patient with predominantly pincer FAI
and Trousdale, 2003). This common deformity is a is a woman of 30–40 years. The pain is generated
type of spatial malorientation, not just a deficiency in from the injured labrum when there is direct contact
the posterior wall, and a focal pincer type FAI is cre- between the femoral neck and the sensitive pain fibres
ated by excessive anterior coverage of the femoral of the labrum. Pain can be quite marked although the
head. Iatropathic retroversion has been reported fol- cartilage damage may be moderate.
lowing pelvic osteotomy (Dora et al., 2002), but the The typical patient with predominantly cam FAI is
aetiology in most cases has not been identified. a man, rather muscular and athletic, and about 10
Femoral malformation following undiagnosed years younger. Pain in this case is less dramatic, prob-
SCFE was initially thought to be the main cause of ably because FAI is more a jamming of the non-spher-
cam type FAI, but MRI has shown that most of the ical portion of the head into the cavity of the socket.
hips with cam FAI do not manifest the other typical Despite the lesser degree of pain, cartilage destruction
physeal abnormalities associated with SCFE (Sieben- is often substantial (Table 19.3).
rock et al., 2004). Standard methods of scoring hip function used for
It has also been suggested that rigorous physical total hip replacement patients are not suitable for
activity during skeletal development (causing young FAI patients with high athletic demands. New
increased physeal stresses) may play a part in the outcome scores have been developed and validated for
development of proximal femoral abnormalities. their potential use in hip disorders of the younger
patient but so far they have not been widely adopted.
Clinical features of femoro-acetabular
impingement Imaging
Groin pain and limited motion are the usual present- An orthograde anteroposterior radiograph of the
526 ing symptoms. During the initial stages of the disease, pelvis and a lateral radiograph of the hip are needed
for patients with suspected FAI. The pelvis should be 19
pictured with the coccyx pointing towards the symph-
ysis and a distance of 1–2 cm between them. Besides
demonstrating the ‘pistol-grip’ deformity of the prox-
imal femur, this projection is essential for assessing
acetabular version. The quality must be sufficient to
The hip
allow visualization of the anterior and posterior rims
of the acetabulum and to define the double contours
of the rim; in a retroverted acetabulum the line of the
anterior rim sweeps lateral to the line of the posterior
rim (Ganz et al., 2003; Jamali et al., 2007), a feature
associated with FAI (Fig. 19.40).
The best lateral view is a ‘cross-table lateral’, allow-
ing one to detect anterolateral abnormalities of the
head/neck contour (Fig. 19.41). MR arthrograms of
19.42 MR arthrography Section of the lateral joint space

of a retroverted left hip, showing the bony projection at
the acetabular rim (arrow), a slight signal alteration of the
rounded labrum and some irregularity of the adjacent
cartilage.
the hip, including radial cuts, are used to visualize the

labrum and cartilage as well as the femoral head/neck
morphology.
MR arthrograms of the hip are used routinely to
visualize the labrum and articular cartilage. These are
capable of detecting abnormal sphericity of the
femoral head, low offset of the neck, impingement
cysts and bone appositions of the rim, all related to
19.40 X-ray – Pincer FAI Anteroposterior radiograph of a FAI. MR arthrography is sensitive and specific for
23-year-old man with bilateral retroversion. One can see detecting labral and chondral lesions; however, there
that in each hip the anterior rim of the acetabulum projects
are limitations in detecting undisplaced delaminations
lateral to the posterior rim (arrow, left hip), a sign
suggesting retroversion of the hip. At the right hip there is of the acetabular cartilage.
a fatigue fracture of part of the anterior rim of the New advanced imaging techniques such as the 3-
acetabulum (arrow, right hip). Tesla system and delayed gadolinium-enhanced MRI
of cartilage (dGEMRIC) may in the future be able to
detect early cartilage lesions and capsular adhesions.
Treatment
Non-operative The benefits of non-surgical treatment
such as physical or anti-inflammatory therapy in FAI
are questionable. Restriction of athletic activities may
occasionally reduce symptoms; however, a delay in the
surgical correction of symptomatic bone dysmor-
phism may permit progression of articular cartilage
destruction, leading to the premature onset of
osteoarthritis.
Operative Arthroscopic procedures are suitable only for
minor and localized structural abnormalities (Fig.
19.41 X-ray – same case as in Figure 19.40 Lateral 19.43). Isolated treatment of labral lesions without
projection of the right proximal femur. Note the non-
correcting the underlying bony pathology is a major
spherical expansion of the anterolateral contour of the
femoral epiphysis (arrows point to the curved line of the cause of failure.
former growth plate). Open operation with dislocation of the hip is the pre- 527
19 OSTEONECROSIS
The femoral head is the commonest site of sympto-

matic osteonecrosis, mainly because of its peculiar
blood supply which renders it vulnerable to ischaemia

from arterial cut-off, venous stasis, intravascular
thrombosis, intraosseous sinusoidal compression, or a
combination of several of these. The pathogenesis and
pathological anatomy of the condition are discussed in
Chapter 6.
Post-traumatic osteonecrosis usually follows a dis-
placed fracture of the femoral neck or dislocation of
19.43 FAI – arthroscopy Arthroscopic view showing the hip. The main cause is interruption of the arterial
frayed acetabular articular cartilage blood supply, but contributory factors are venous sta-
sis and thrombosis of intramedullary arterioles and
capillaries.
ferred approach for treating FAI, offering the advan- Non-traumatic osteonecrosis is seen in association
tages of unrestricted access, a precise correction, with infiltrative disorders of the marrow, Gaucher’s
anatomical labral refixation and the possibility of disease, sickle-cell disease, coagulopathies, caisson
dynamic control of the correction (Fig 19.44). Osteo- disease, systemic lupus erythematosus and – com-
chondroplasty calls for debridement of damaged car- monest of all – high-dosage corticosteroid administra-
tilage and/or resection of the bony impediments that tion and alcohol abuse. Perthes’ disease is a special
are responsible for the malarticulation. For cam-type example which is dealt with elsewhere in this chapter.
impingement the bony excrescence on the anterior The pathogenesis and pathological anatomy of the
femoral neck needs to be resected; for pincer-type bone changes are discussed in Chapter 6.
impingement the abnormal overgrowth at the ante-
rior rim of the acetabulum must be removed. In some
cases corrective osteotomy of the proximal femur or
Clinical features
acetabulum may also be needed (Fig. 19.45). Post-traumatic osteonecrosis develops soon after injury
The morbidity of the procedure is low and the to the hip, but symptoms and signs may take months
short- to mid-term results are good to excellent (Beck to appear.
et al., 2004; Murphy et al., 2004; Espinosa et al., Non-traumatic osteonecrosis is more insidious.
2006; Beaulé et al., 2007). Children are affected in conditions such as Perthes’
(a) (b) (c)
(d) (e) (f)
19.44 FAI – open osteochondroplasty Series showing open surgical treatment of FAI. (a–c) Torn
528 acetabular labrum re-fixed and (d–f) non-spherical contour of the femoral head trimmed back.
19
The hip
(a) (b)
19.45 FAI – bony correction Bone deformities due to acetabular dysplasia, old Perthes’ disease or SCFE may need
corrective osteotomy as well. This 19-year-old female had an old Perthes deformity (a) a high-riding greater trochanter with
short neck producing extra-articular impingement against the posterosuperior acetabular wall. The prominent anterior
border of the femoral head (producing a ‘sagging rope sign’ on x-ray) led to intra-articular FAI. (b) Correction of the
complex impingement was achieved by trimming the femoral head contour and ‘lengthening’ the femoral neck, together
with advancement of both the greater and the lesser trochanter.
disease, sickle-cell disease and Gaucher’s disease. with the hip extended, internal rotation is almost full,
Adult patients come from both sexes and all ages. but with the hip flexed it is grossly restricted.
The presenting complaint is usually pain in the hip There may be symptoms or signs of an associated,
(or, in over 50 per cent of cases, both hips), which causative disorder or a history of having been treated
progresses over a period of 2–3 years to become quite with corticosteroids – remember that even a short
severe. However, in over 10 per cent of cases the con- course of high-dosage corticosteroids can result in
dition is asymptomatic and discovered incidentally osteonecrosis and the hip is the commonest target.
after x-ray or MRI during investigation of a systemic Another risk factor is high usage of alcohol.
disorder or longstanding symptoms in the other hip.
On examination, the patient walks with a limp and
may have a positive Trendelenburg sign. The thigh is
Imaging
wasted and the limb may be 1 or 2 cm short. Move- X-rays During the early stages of osteonecrosis plain
ments are restricted, particularly abduction and inter- x-rays are normal. The first signs appear only 6–9
nal rotation. A characteristic sign is a tendency for the months after the occurrence of bone death and are
hip to twist into external rotation during passive flex- due mainly to reactive changes in the surrounding
ion; this corresponds to the ‘sectoral sign’ in which, (live) bone. Thus, the classic feature of increased den-
sity (interpreted as sclerosis) is a sign of repair rather
than necrosis. With time, destructive changes do
appear in the necrotic segment: a thin subchondral
fracture line (the ‘crescent sign’), slight flattening of
the weightbearing zone and then increasing distor-
tion, with eventual collapse, of the articular surface of
the femoral head.
MRI MRI shows characteristic changes in the marrow
long before the appearance of x-ray signs – a mean of
3.6 months after the initiation of steroid treatment in
one published study (Sakamoto et al., 1997). The
diagnostic feature is a band of altered signal intensity
running through the femoral head (diminished
(a) (b) intensity in the T1 weighted SE image and increased
intensity in the STIR image). This ‘band’ represents
19.46 Osteonecrosis – imaging, early stage (a) This the reactive zone between living and dead bone and
patient had few symptoms and x-rays that were, at most,
equivocal. However, even at that early stage the MRI thus demarcates the ischaemic segment, the extent
(b) showed a clear-cut segment of osteonecrosis at the and location of which are important in staging the
dome of the femoral head. lesion. 529
19
(a)
19.47 Osteonecrosis – imaging, late stage (a) Plain

x-ray showing the typical features of bilateral
corticosteroid-induced osteonecrosis of the femoral heads.
The dense bands define the boundary between dead bone (b)
and new-bone formation. (b) The corresponding MRI in
the same patient.
Diagnosis Transient osteoporosis of the hip is sometimes con-

fused with avascular necrosis. The condition is
X-ray features of destructive or sclerotic forms of described below.
osteoarthritis are sometimes mistaken for those of
advanced osteonecrosis. There may, indeed, be The causative disorder Diagnosis should include eluci-
elements of bone necrosis in some types of OA, but dation of the causative disorder. There may be a his-
there is an important point of distinction between tory of trauma, a familial condition such as sickle-cell
these two conditions: in OA the articular ‘space’ disease or Gaucher’s disease, an occupational back-
diminishes before the bone breaks up, whereas in ground suggesting dysbaric ischaemia, an underlying
osteonecrosis the articular ‘space’ is preserved to the disease such as systemic lupus erythematosus, or a
last (because it is not primarily a disease of articular known background of corticosteroid administration
cartilage). or alcohol abuse. If there is no such history, the
patient should be fully investigated for these associ-
ated conditions (see Chapter 6).
It is important to recognize that pathogenic factors
are cumulative, so a patient with systemic lupus or a
moderately severe alcohol habit may develop
osteonecrosis following comparatively low doses of
cortisone, and occasionally even after prolonged or
excessive use of topical corticosteroids (Solomon and
Pearse, 1994).
STAGING (see Chapter 6)

In the past, Ficat and Arlet’s radiographic staging of
femoral head necrosis was widely used. In Stage 1
the patient has little or no pain and the plain x-ray
shows no abnormality. However, there are typical
(a) (b)
changes on MRI (see Fig. 19.46). In Stage 2 there
19.48 Diagnosis (a) Osteoarthritis sometimes shows are early x-ray signs but no distortion of the femoral
marked segmental sclerosis on x-ray. These features are head. Stage 3 is more advanced, with increasing
often mistaken for those of osteonecrosis. The clue lies in signs of bone destruction and femoral head distor-
the absent joint ‘space’, a cardinal sign in osteoarthritis.
Compare this with (b), an x-ray of severe osteonecrosis in tion. Stage 4 is characterized by collapse of the artic-
which the joint ‘space’ is preserved in the face of bone ular surface and joint disorganization. This is a
530 collapse. useful descriptive classification of the current state
19.49 Osteonecrosis
Femoral head necrosis due to
19
(a) femoral neck fracture,
(b) Gaucher’s disease and
(c) chronic alcohol abuse.
The hip
(a) (b) (c)
of affairs but it does not provide a guide to progno- ter and involving only the medial third of the weight-
sis (and therefore treatment) in the early stages of bearing surface rarely go on to collapse; (3) lesions
the condition. occupying up to one-half of the femoral head diame-
Shimizu et al. (1994) proposed a classification ter and involving between one-third and two-thirds of
based on MR images which defines the extent, loca- the weightbearing surface are likely to collapse in
tion and intensity of the abnormal segment in the about 30 per cent of cases; and (4) lesions occupying
femoral head. The risk of femoral head collapse (at more than one-quarter of the femoral head diameter
least over a period of 2–3 years) was related mainly to and involving more than two-thirds of the weight-
the extent (the area of the coronal femoral head image bearing surface will collapse within 3 years in over 70
involved) and location (the portion of the weightbear- per cent of cases. When discussing treatment, we shall
ing surface) in the initial MRI. In general terms, their refer to these three degrees of severity as Grade I,
findings suggested that: (1) the extent of the Grade II and Grade III.
ischaemic segment is determined at the outset and Note that although this classification is useful for
does not increase over time; (2) lesions occupying less predicting outcome and planning treatment, extent
than one-quarter of the femoral head coronal diame- (in this context) is not synonymous with volume; the
true volume of the necrotic segment is very difficult to
determine (Kim et al., 1998).
For purposes of comparing data from different
sources before and after treatment, the recommended
classification is the one proposed by the International
Association of Bone Circulation and Bone Necrosis
(Association Research Circulation Osseous – ARCO)
(Table 19.4).
Treatment of post-traumatic
osteonecrosis
Femoral head necrosis following fracture or disloca-
tion of the hip usually ends in collapse of the femoral
head. Very young patients (those under 40 years), in
whom one is reluctant to perform hip replacement,
can be treated by realignment osteotomy, with or
without bone grafting of the necrotic segment. They
will probably require hip replacement at a later stage.
Older patients will almost invariably opt for partial
or total joint replacement.
19.50 Predictive staging The likelihood of progression to Treatment of non-traumatic

collapse depends on the location and extent of the osteonecrosis
boundary changes on MRI. In this figure the risk of
progression is represented by + signs. The general scheme Early Shimuzu Grade I lesions (those restricted to the
is based on findings published by Shimuzu et al. (1994). medial part of the femoral head) progress very slowly 531
19 Table 19.4 ARCO staging of osteonecrosis the femoral head and more than two-thirds of the
weightbearing surface) have a poor prognosis.
Stage 0 Patient asymptomatic and all clinical investigations
Decompression is unlikely to have a lasting effect. For
‘normal’
Biopsy shows osteonecrosis younger patients, therefore, realignment osteotomy is
the treatment of choice. X-rays and CT will show
Stage 1 X-rays normal. MRI or radionuclide scan shows
osteonecrosis
exactly where the necrotic segment is and the angula-
tion osteotomy can be planned so as to displace the

Stage 2 X-rays and/or MRI show early signs of osteonecrosis
necrotic segment away from the maximal load-bearing
but no distortion of bone shape or subchondral
‘crescent sign’. trajectory. A flexion osteotomy will be needed for
Subclassification by area of articular surface involved: most cases. The more radical transtrochanteric rota-
A = less than 15 per cent tional osteotomy of Sugioka (Sugioka and Mohtai,
B = 15–30 per cent 1998) is difficult to perform and the results in most
hands are no better than those of the more conven-
Stage 3 X-ray shows ‘crescent sign’ but femoral head still tional osteotomies. Older patients with intrusive
spherical symptoms will be better served by partial or total joint
Subclassification by length of ‘crescent’/articular
surface:
replacement.
A = less than 15 per cent Late Patients with advanced osteonecrosis and bone
B = 15–30 per cent
collapse (Ficat stage 3 or 4) will need reconstructive
surgery: osteotomy, with or without bone grafting, or
Stage 4 Signs of flattening or collapse of femoral head
joint replacement.
A = less than 15 per cent of articular surface
B = 15–30 per cent of articular surface There is a limited place for arthrodesis in young
C = more than 30 per cent of articular surface men who are willing to accept the limitations of a
Stage 5 Changes as above plus loss of ‘joint space’
‘stiff’ hip in return for pain relief (Solomon, 1998).
(secondary OA)
Stage 6 Changes as above plus marked destruction of
articular surfaces
ARCO, Association Research Circulation Osseous; OA, osteoarthritis.
TRANSIENT OSTEOPOROSIS OF THE
HIP (MARROW OEDEMA
SYNDROME)
or not at all. Almost any treatment for this group is
This is a well-recognized, though uncommon, syn-
therefore liable to be assessed as ‘beneficial’. All that
drome characterized by pain and rapidly emerging
is needed is symptomatic treatment and reassurance,
osteoporosis of the femoral head and adjacent pelvis.
but it is wise to observe the patient over several years
Radionuclide scanning shows increased activity on
in case there should be a change.
both sides of the hip but not in the soft tissues. The
Grade II lesions (those occupying up to one-half of
condition was originally described in women in the
the femoral head and between one and two-thirds of
last trimester of pregnancy, but it is now seen in
the weightbearing surface) are liable to progress. If
patients of both sexes and all ages from early adult-
they are seen before there is any distortion of the
hood onwards. Typically the changes last for 6–12
femoral head, it would therefore be justifiable to
months, after which the symptoms subside and x-ray
advise conservative surgery (core decompression or
gradually returns to normal.
decompression and bone grafting of the femoral
The cause is unknown, but MRI features are char-
head). Coring of the femoral head was introduced by
acteristic of marrow oedema. It has been suggested
Ficat (1985) as a means of reducing the intraosseous
that the condition is a precursor (or forme fruste) of
pressure in patients with early non-traumatic
avascular necrosis, but there is little evidence to sup-
osteonecrosis. The intraosseous pressure is measured
port this (see Chapter 6).
and, if it is raised, a 7 mm core of bone is removed by
drilling up the femoral neck under image intensifica- Treatment The condition almost always resolves
tion fluoroscopy. It is impossible to say which cases spontaneously and most patients require no more
will respond favourably, but the attempt is worthwhile than symptomatic treatment. However, pain can be
and sustained symptomatic improvement is seen in rapidly abolished by operative decompression of the
30–50 per cent of patients. The alternative is realign- femoral head (drilling up the femoral neck), and some
ment osteotomy in younger patients and partial or would prefer this to the long wait for a natural ‘cure’.
total hip replacement in patients over 45 years old If there is any doubt about whether the MRI changes
with increasing symptoms. are due to osteonecrosis or marrow oedema, operative
532 Grade III lesions (those occupying a large part of decompression is recommended.
of local anaesthetic and corticosteroid. If a 19
haematoma is present it should be aspirated.
Gluteus medius tendinitis

Acute tendinitis may cause pain and localized tender-
The hip
ness just behind the greater trochanter. This is seen
particularly in dancers and athletes. The clinical and x-
ray features are similar to those of trochanteric bursi-
tis, and the differential diagnosis is the same.
Treatment is by rest and injection of local anaesthetic
and corticosteroid.
Adductor longus strain or tendinitis

This overuse injury is often seen in footballers and
athletes. The patient complains of pain in the groin
and tenderness can be to the adductor longus origin
close to the pubis. Swelling below this site may signify
19.51 Marrow oedema syndrome This patient an adductor longus tear.
complained of pain in the right hip. X-ray showed no Acute strains are treated by rest and heat. Chronic
obvious abnormality, although the area around the hip strains may need prolonged physiotherapy.
looked somewhat osteoporotic. MRI disclosed the typical
picture of diffuse signal reduction (in the right femoral
head) in the T1 weighted scans. This contrasts sharply with Iliopsoas bursitis
the localized bands which are characteristic of
osteonecrosis. Pain in the groin and anterior thigh may be due to an
iliopsoas bursitis. The site of tenderness is difficult to
define and there may be guarding of the muscles over-
lying the lesser trochanter. Hip movements are some-
BURSITIS AND TENDINITIS AROUND times restricted; indeed, the condition may arise from
THE HIP synovitis of the hip since there is often a potential
communication between the bursa and the joint. The
most typical feature is a sharp increase in pain on
Trochanteric bursitis adduction and internal rotation of the hip. Pain can
Pain over the lateral aspect of the hip and thigh may also be elicited by testing psoas contraction against
be due to local trauma or overuse, resulting in inflam- resistance (see Fig. 19.3).
mation of the trochanteric bursa which lies deep to The differential diagnosis of anterior hip pain
the tensor fasciae latae. There is local tenderness and includes inguinal lymphadenopathy, hernia, a psoas
sometimes crepitus on flexing and extending the hip. abscess, fracture of the lesser trochanter, slipped epi-
Swelling is unusual but post-traumatic bleeding can physis, local infection and arthritis.
produce a bursal haematoma. Treatment is by non-steroidal anti-inflammatory
X-rays may show evidence of a previous fracture, or drugs and injection of local anaesthetic and steroid;
a protruding metal implant or trochanteric wires dat- the injection is best performed under fluoroscopic
ing from some former operation. There may also be control.
calcification or shadows suggesting swelling of the
soft tissues. It is important to exclude underlying dis-
Snapping hip
orders such as gout, rheumatoid disease and infection
(including tuberculosis). ‘Snapping hip’ is a disorder in which the patient (usu-
Other causes of pain and tenderness over the ally a young woman) complains of the hip ‘jumping
greater trochanter are stress fractures (in athletes and out of place, or ‘catching’, during walking. The snap-
elderly patients), slipped epiphysis (in adolescents) ping is caused by a thickened band in the gluteus max-
and bone infection (in children). The commonest imus aponeurosis flipping over the greater trochanter.
cause of misdiagnosis is referred pain from the lumbar In the swing phase of walking the band moves anteri-
spine. orly; then, in the stance phase, as the gluteus maximus
The usual treatment of trochanteric bursitis is rest, contracts and pulls the hip into extension, the band
administration of non-steroidal anti-inflammatory flips back across the trochanter, causing an audible
drugs and (provided infection is excluded) injection ‘snap’. This is usually painless but it can be quite dis- 533
19 PAIN AROUND THE HIP
by detaching the origins of rectus femoris. This pro-
vides adequate exposure for many operations, includ-
Anteriorly (groin)
ing open reduction of the dislocated hip in infants and
Synovitis and arthritis
the various types of pelvic osteotomy. However, it is
Perthes’ disease
not ideal for major reconstructive surgery in adults.
Labral tear or detachment
The anterolateral (Watson-Jones) approach is also
Loose bodies in the joint

anterior to the gluteus medius, but behind the tensor
Stress fracture
fasciae femoris. It provides reasonable exposure of the
Osteitis pubis
hip joint, with minimal detachment of muscles, but
Other bone lesions
the gluteus medius is in the way and this makes hip
Inguinal hernia
replacement difficult.
Inguinal lymphadenopathy
Lateral approaches suffer from the fact that the glu-
Iliopsoas tendinitis or bursitis
teus medius and minimus obstruct the view of the
Iliopsoas abscess
acetabulum. The abductors are dealt with by (1)
Adductor longus strain or tendinitis
retracting them posterosuperiorly (a limited solution),
or (2) splitting them and raising the anterior portion
Laterally intact from the greater trochanter (Hardinge’s direct
Referred from spine lateral approach), or (3) osteotomizing the greater
Slipped epiphysis trochanter and retracting it upwards with the attached
Trochanteric bursitis abductors (as in the Charnley approach for total joint
Stress fracture replacement). This provides excellent exposure; how-
Trochanteric tuberculosis ever, there may be problems with reattachment of the
Posteriorly
trochanteric fragment.
Referred from spine
The posterior approach is the most direct. By split-
Gluteus medius tendinitis
ting the anterior part of gluteus maximus, the rotators
at the back of the hip are exposed and the sciatic nerve
is retracted safely beneath the bulk of the posterior
portion of gluteus maximus. Once the short rotators
are detached, the hip is entered directly. Many sur-
geons prefer this approach for joint replacement. It
tressing, especially if the hip gives way. Sometimes
has two minor disadvantages: orientation is more dif-
there is tenderness around the hip, and it may be pos-
ficult, especially for placing the acetabular cup; and it
sible to reproduce the peculiar sensation by flexing
is associated with an increased incidence of postoper-
and extending the hip while abducted.
ative dislocation.
The condition must be distinguished from other
causes of painful clicking, particularly a tear of the
PLANNING
acetabular labrum or an osteocartilaginous flap on the
Reconstructive surgery of the hip needs careful pre-
femoral head (similar to osteochondritis dissecans).
operative planning. Tracings of plain x-rays are useful
Contrast arthrography, or arthroscopy if this is avail-
for taking measurements and working out reposition-
able, will exclude these entities.
ing angles. For the most difficult cases, three-dimen-
Treatment of the snapping tendon is usually unnec-
sional imaging studies should be obtained.
essary; the patient merely needs an explanation and
reassurance. Occasionally, though, if discomfort is
marked the band can be either divided or lengthened Osteochondroplasty
by a Z-plasty.
Osteochondroplasty for early OA associated with
femoro-acetabular impingement (FAI) is dealt with
on page 528.
PRINCIPLES OF HIP OPERATIONS
Exposure of the hip Intertrochanteric osteotomy
Operative approaches to the hip can be broadly Rationale Intertrochanteric osteotomy has three
divided into anterior, anterolateral, lateral and poste- objectives: (1) to change the orientation of the
rior. femoral head in the socket so as to reduce mechanical
The anterior (Smith-Petersen) approach starts in the stress in a damaged segment; (2) realigning the prox-
plane between sartorius and rectus femoris medially imal femur, to improve joint congruity; and (3) tran-
and tensor fasciae femoris laterally and remains ante- secting the bone, to reduce intraosseous hypertension
534 rior to the gluteus medius. The hip capsule is exposed and relieve pain. An unintentional, and poorly under-
19.52 Osteoarthritis – 19
treatment by osteotomy
Following a varus type of
osteotomy this patient lost
most of her pain, and the
x-rays suggest articular
cartilage regeneration.
The hip
(a) (b) (c)
stood, consequence is (4) fibrocartilaginous repair of thus turning the femoral head through an arc of 90
the articular surface. degrees or more.
Indications In children osteotomy is used to correct Complications The main complication is malposition
angular or rotational deformities of the proximal of the bone. Only careful planning can prevent this.
femur (e.g. in congenital dislocation, coxa vara or Non-union of the osteotomy is rare.
severe slips of the capital epiphysis), or to produce Results Provided the indications are strictly observed,
‘containment’ of the femoral head in Perthes’ disease. the results are moderately good. In a series of 368
In adults, the main indication is osteoarthritis asso- osteotomies, survivorship analysis showed that 10
ciated with joint dysplasia, particularly in patients who years after osteotomy 47 per cent of patients had
are younger than 50 years. Pain is often relieved required no further surgery (Werners et al., 1990).
immediately (probably due to reduced vascular con- The operation has not been widely adopted, partly
gestion) and sometimes the articular space is gradually because of its technical complexity, partly because of
restored. The other prime indication is in localized the risk of complications and partly because of doubts
avascular necrosis of the femoral head; if only a small about its long-term effectiveness – particularly in
segment is involved, realignment can rotate this seg- comparison to the outcome of modern methods of
ment out of the path of maximum stress. total hip replacement.
Contraindications Osteotomy is unsuitable in elderly
patients and in those with severe stiffness; movement
may be even further decreased afterwards. It is also
contraindicated in rheumatoid arthritis, and even in
ARTHRODESIS
OA if there is widespread loss of articular substance; Rationale Fusion of the hip is guaranteed to relieve
reposition is useless if other parts of the femoral head pain and provide stability for a lifetime. But at what
are equally damaged. cost? Surprisingly, although the joint is fused, the
patient retains a great deal of ‘mobility’ because lum-
Technical considerations The osteotomy allows reposi-
bosacral tilting and rotation are preserved and often
tioning of the femoral head in valgus, varus or differ-
increased. Nevertheless, there are restrictions: for sit-
ent degrees of rotation. Exact placement and
ting comfortably the hip needs 60 degrees of flexion;
angulation can be ensured only by meticulous preop-
for climbing stairs, 45 degrees; and for walking, 20
erative planning and painstaking execution of the
degrees. In the stance phase of walking the normal hip
bone cuts. The fragments are fixed with suitably
is in slight abduction, but in the swing phase it is car-
angled plates and screws. Postoperatively the patient is
ried in slight adduction. No position of fusion can sat-
permitted only partial weightbearing for 3–6 months.
isfy all these demands, so one aims at a compromise.
About 15 per cent of patients will require some assis-
And sometimes it is wrong, with the result that func-
tance (a walking stick) for the rest of their lives.
tion is seriously impaired.
Sugioka (Sugioka and Mohtai, 1998) devised a
transtrochanteric rotational osteotomy for dealing Indications Arthrodesis should be considered for any
with anterosuperior segmental destructive lesions of destructive condition of the hip when there are seri-
the femoral head, such as localized osteonecrosis. This ous contraindications to osteotomy or arthroplasty:
allows the femoral neck to be rotated on its long axis, for example, a patient who is too young, a hip that is 535
19 already stiff but painful, and previous infection. Young
patients adapt well; those aged over 30–40 years
respond unpredictably.
Contraindications Elderly patients, and any patient
with a good range of movement, will resent a ‘stiff
hip’. Other contraindications are lack of bone stock
and abnormalities in the ‘compensating joints’ (lum-

bar spine, knees and opposite hip).
Technical considerations The recommended position for
arthrodesis is 20–30 degrees of flexion, 0–10 degrees
of adduction (unless the leg is short) and about 5 de-
grees of external rotation. However, in young people
there is a tendency for the ‘joint’ to drift into further (a) (b) (c)
flexion and by the age of 40 this may be as much as 40
19.53 Arthrodesis Stiffness of the hip is largely disguised
degrees. Some form of internal fixation is used to secure by mobility of the spine and knee.
the bones in the desired position. It is important to en-
sure that these implants do not destroy the abductors;
though they are not needed while the hip is
arthrodesed, they will be essential if ever the fusion is
converted to an arthroplasty. joint destruction. However, there are several prob-
lems to be overcome: (1) the prosthetic implants must
Complications The major complications are (1) failure be durable; (2) they must permit extraordinary low-
to fuse and (2) malposition, which hampers function friction movement at the articulation; (3) they must
and puts unwanted strain on other joints. Late com- be firmly fixed to the skeleton; and (4) they must be
plications are (3) compensatory deformities in other inert and not provoke any unwanted reaction in the
joints (knees and opposite hip) and (4) low backache, tissues. The usual combination is a metal femoral
which occurs in over 60 per cent of patients 20 years component (stainless steel, titanium or cobalt–
after fusion. Women may complain of (5) difficulty chrome alloy) articulating with a polyethylene socket.
with sexual intercourse, and (6) squatting is, of Ceramic components have better frictional character-
course, impossible. However, it should be remem- istics but are more easily broken. Fixation is either by
bered that total replacement is still possible after a hip embedding the implant in methylmethacrylate
has been arthrodesed. cement, which acts as a grouting material filling the
Results Provided the ‘compensating joints’ (lumbar
interstices, or by fitting the implant closely to the
spine, knee and opposite hip) are completely normal, bone bed without cement. The ‘bond’ between bone
young patients in particular may derive benefit from and the implant surface, or cement, is never perfect.
arthrodesis, with many years of reasonable comfort, a The best that can be hoped for is ingrowth of trabec-
well-disguised limp and the ability to walk long dis- ular bone on the implant or cement (osseointegra-
tances and play games. Older patients fare less well: tion). There are various ways of enhancing this
they find walking more difficult, tend to develop process: (1) if acrylic cement is used, it is applied
backache and seem more prone to degenerative under pressure and allowed to cure without move-
changes in other joints. ment or extrusion after the implant has been inserted;
In countries where advanced facilities and expertise (2) Ling and his co-workers have shown that a
are available, modern techniques of total hip replace- smooth, tapered and collarless femoral prosthesis will
ment – providing results as high as 90–100 per cent continue settling within the cement mantle even after
survival rate with excellent function at 10 years post- polymerization, thereby maintaining expansile pres-
operatively – have rendered arthrodesis more or less sure between cement and bone (Fowler et al., 1988);
obsolete. This attitude prevails ever more strongly as (3) uncemented implants may be covered with a mesh
each new generation of orthopaedic surgeons lacks any or porous coating that encourages bone ingrowth
sustained training in ‘old’ types of surgery such as this. (Engh et al., 1987); (4) the implant may be coated
with hydroxyapatite, an excellent substrate for
osteoblastic new-bone formation and osseointegra-
tion (Geesink, 1990).
TOTAL HIP REPLACEMENT – GENERAL
Indications Because of the tendency for implants to
PRINCIPLES
loosen with time, joint replacement was customarily
Rationale Total replacement of the articular surfaces reserved for patients over 60 years. However, with
536 seems the ideal way of treating any disorder causing improved cementing techniques and rapid advances in
19
The hip
(b)
(a) (a) (b)
19.55 Total hip replacement (a) X-ray of a Charnley hip

replacement system, forerunner of all the modern methods
19.54 Prosthetic fixation Fixation between cement and of total hip replacement. This comprises a collared femoral
bone is by (a) interlock (interdigitation of large irregularities prosthesis with a fairly wide stem and a polyethelene
in cement and bone) and, more completely, by acetabular cup, both implants fixed with acrylic cement.
(b) osseointegration (intimate penetration of cement (b) X-ray of a cemented Ling femoral prosthesis – collarless
between endosteal trabeculae). with a tapered stem – and an uncemented acetabular cup.
the design of uncemented prostheses, the operation is

Factors that may contribute to the development of
being offered to younger patients with destructive hip
complications include previous hip operations, severe
disorders, and occasionally even to children severely
deformity, lack of preoperative planning, inadequate
crippled with rheumatoid disease.
‘bone stock’, an insufficiently sterile operating envi-
Contraindications Overt or latent sepsis is the chief ronment and lack of experience or expertise on the
contraindication to joint replacement. An infected part of the surgical team.
arthroplasty spells disaster. Patients under 60 years of Intraoperative complications include perforation or
age are considered only if other operations are unsuit- even fracture of the femur or acetabulum. Special care
able. should be taken in patients who are very old or osteo-
porotic and in those who have had previous hip oper-
Technical considerations The fear of infection dictates a
ations.
host of prophylactic measures, including the use of
Sciatic nerve palsy (usually due to traction but occa-
special ultraclean-air operating theatres, occlusive the-
sionally caused by direct injury) may occur with any
atre clothing and perioperative antibiotic cover (Lid-
type of arthroplasty but is more common with a pos-
well et al., 1984; Marotte et al., 1987). In addition,
terior approach. Most cases recover spontaneously but
some surgeons routinely use antibiotic-laden cement.
if there is reason to suspect nerve damage the area
The choice of implant should depend on sound
should be explored.
biomechanical and biological testing. The array of
Postoperative dislocation is rare if the prosthetic
over 300 different mechanisms currently on the mar-
components are correctly placed. Reduction is easy
ket represents the triumph of hope over reason. The
and traction in abduction usually allows the hip to sta-
argument of ‘cemented versus cementless’ goes on.
bilize. If malposition of the femoral or acetabular
Sound technique is probably more important than
component is severe, revision may be needed, or pos-
anything else.
sibly augmentation of the socket.
Postoperatively the implant should be protected
Heterotopic bone formation around the hip is seen in
from full loading until osseointegration is advanced;
about 20 per cent of patients 5 years after joint
6 weeks on crutches is not unreasonable.
replacement. The cause is unknown, but patients with
Complications Hip replacements are often performed skeletal hyperostosis and ankylosing spondylitis are
on patients who are somewhat elderly; some have particularly at risk. In severe cases this is associated
rheumatoid disease and may be having steroid ther- with pain and stiffness. Ossification can be prevented
apy. Consequently the general complication rate is by in high-risk patients by giving either a course of non-
no means trivial; deep vein thrombosis is more com- steroidal anti-inflammatory drugs for 3–6 weeks post-
mon than with other elective operations. operatively or a single dose of irradiation to the hip. 537
19 19.56 Hip replacement –
aseptic loosening (a) Ten
years after a hip
replacement there is a
distinct radiolucent line
around this femoral implant
as well as resorption of the
calcar. (b) A further stage

shown in another patient.
Aggressive osteolysis. (c)
The end of the line. This
patient, after four
‘revisions’, ended up with
fragmentation of the
proximal femur, massive
resorption of the
acetabulum and fragments
of bone and acrylic cement
in the soft tissues. Happily,
cases such as this are,
nowadays, few and far
between but the risk is
(a) (b) (c) always there.
Aseptic loosening of either the acetabular socket or Infection is the most serious postoperative compli-
the femoral stem is the commonest cause of long- cation. With adequate prophylaxis the risk should be
term failure. Figures for its incidence vary widely, less than 1 per cent, but it is higher in the very old, in
depending on the criteria used. With modern patients with rheumatoid disease or psoriasis, and in
methods of implant fixation, there is likely to be those on immunosuppressive therapy (including cor-
radiographic evidence of loosening in less than 10 per ticosteroids).
cent of patients 15 years after operation; at micro- The large bulk of foreign material restricts the
scopic level many stable implants show cellular reac- access of the body’s normal defence mechanism; con-
tion and membrane formation at the bone–cement sequently, even slight wound contamination may be
interface (Linder and Carlsson, 1986). Fortunately, serious. Organisms may multiply in the postoperative
only a fraction of these are symptomatic. Pain may be haematoma to cause early infection, and, even many
a feature, especially when first taking weight on the years later, haematogenous spread from a distant site
leg after sitting or lying, but the diagnosis usually rests may cause late infection.
on x-ray signs of progressively increasing radiolucency Early wound infection sometimes responds to
around the implant, fracturing of cement, movement antibiotics. Later infection does so less often and may
of the implant or bone resorption (Gruen et al., need operative ‘debridement’ followed by irrigation
1979). Radionuclide scanning shows increased activ- with antibiotic solution for 3–4 weeks. Once the
ity, and it is claimed that the pattern of 99Tc-HDP and infection has cleared, a new prosthesis can be inserted,
67
Ga uptake can differentiate between aseptic loosen- preferably without cement. An alternative, more
ing and infection (Taylor et al., 1989). If symptoms applicable to ‘mild’ or ‘dubious’ infection, is a one-
are marked, and particularly if there is evidence of stage exchange arthroplasty using gentamicin-impreg-
progressive bone resorption, the implant and cement nated cement. The results of revision arthroplasty for
should be painstakingly removed and a new prosthe- infection are only moderately good. If all else fails the
sis inserted. Revision arthroplasty can be either prosthesis and cement may have to be removed, leav-
cemented or uncemented, depending on the condi- ing an excisional (Girdlestone) arthroplasty.
tion of the bone.
Aggressive osteolysis, with or without implant loos- Results The success rate of primary total hip replace-
ening, is sometimes seen. It is associated with granu- ment is now so high that only with a prolonged
loma formation at the interface between cement (or follow-up of a large number of cases can we evaluate
implant) and bone. This may be due to a severe histi- the relative merits of different models. It is important
ocyte reaction stimulated by cement, polyethylene or to compare like with like; present-day cementing
metal particles that find their way into the boundary (and non-cementing) techniques are far superior to
zone. Revision is usually necessary and this may have those of only a decade ago and implant survival rates
to be accompanied by impaction grafting with morsel- of more than 95 per cent at 15 years are being
538 lized bone. reported.
TOTAL HIP REPLACEMENT – PRESENT- Cemented implants Cemented stems embrace two 19
broad concepts: a taper-slip or force-closed design,
DAY PERSPECTIVE and a composite beam or shape-closed design.
The taper slip is a highly polished tapered stem
Total hip replacement is the second most commonly
designed to settle within the cement mantle and re-
performed elective surgical procedure in the UK; over
engage the taper. This connects shear stresses to radial
60 000 were performed in 2006.
The hip
hoop stresses, thus optimizing the load distribution to
Charnley (1979) revolutionized the management
the surrounding bone and cement. Taper slip stems,
of the arthritic hip with the development of low-fric-
such as the Exeter prosthesis, have gained increasing
tion arthroplasty. His three major contributions to the
popularity among cemented implants. A 100 per cent
evolution of hip replacement were: (1) the concept of
implant survivorship has been reported at 10-year fol-
low-friction torque arthroplasty; (2) the use of acrylic
low-up with aseptic loosening as the endpoint, and
cement to fix the components; and (3) the introduc-
good results have also been noted in younger patients
tion of high-density polyethylene as a bearing material.
(Yates et al., 2008).
Using this implant, several authors have reported sur-
Cement is a grout, not a glue, and fixation is
vivorship in the region of 80 per cent at a follow-up
achieved by a mechanical interlock in the bony inter-
of 25 years. Total hip replacement reproducibly allevi-
stices. Many surgeons today routinely use antibiotic-
ates pain and restores mobility while providing joint
loaded cement. The antibiotic elutes out of the
stability. It has been described as ‘the operation of the
cement and produces high local concentrations in
century’. There has been rapid progress in the tech-
the early weeks following the operation, thus reducing
nology relating to joint replacement over the last 50
the incidence of infection. Early methods of cementa-
years.
tion entailed little preparation of the bone bed: the
cement was introduced antegrade and no real attempt
INDICATIONS was made at pressurization beyond finger-packing.
The indications for hip replacement include pain, loss Contemporary cementing techniques include clearing
of movement and associated disability in the presence of the endosteal bone with pulsed lavage, retrograde
of radiographic evidence of joint destruction. For- insertion of cement and sustained pressurization to
merly patients had to earn their total hip replacement resist back-bleeding and enhance the mechanical
with severe pain – usually with sleep disturbance – and interlock. The Swedish Hip Registry demonstrates the
marked loss of function, with the patient often finally benefits of modern cementation techniques.
presenting on two crutches. The procedure was Cemented total hip replacement is technique-depen-
largely restricted to the elderly and the infirm. The dent, as the surgeon mixes the bone–cement–implant
success of the early implants and vastly improved composite at the time of surgery.
access to information have persuaded patients that an The design of cemented cups has not changed
unacceptable compromise in the quality of life repre- much over the years. The cement is pressurized into
sents a valid indication for joint replacement. These an acetabulum that has been cleaned and dried.
patients expect to return to a full profile of profes- Cemented cups still have the best results of the
sional and recreational activities. Given their increased designs recorded in the Norwegian Hip Registry.
expectations it is important that the risks and benefits Cemented total hip replacements are indicated for
of total hip replacement be fully discussed with them. older, less active patients, although very good results
Orthopaedic surgeons should avoid promoting unre- have also been reported in the younger patient.
alistic expectations as this leads to dissatisfaction with
the outcome if they are not achieved. Uncemented implants The use of uncemented
implants has become increasingly popular over the
IMPLANT SELECTION past two decades, particularly in North America. The
Technological advances have resulted in some of surface of these implants was often textured (with
today’s implants being very costly. It is essential that porous beads or titanium mesh) to enhance bone fix-
the implant selected is effective – that it will function ation by osseointegration. It is important to have ini-
satisfactorily for the individual patient. The objectives tial press-fit stability to allow bone on- or ingrowth
are to obtain durable fixation of both components into the textured surface. More recently bioactive sur-
with good orientation and to avoid instability. Care face coatings – such as hydroxyapatite – have been
must be taken to reproduce the centre of rotation of applied to accelerate bone ongrowth and improve the
the acetabulum, restore the offset and ensure that the extent of the osseointegration. Well-fixed uncemented
limb lengths end up equal. Health economics dictate hips provide a durable biological fixation which is
that the operation should also be cost-effective – the cyclically renewed with time.
lowest-cost implant that will do the job should be In the femur the most predictable geometry and
used. good quality bone were available in the diaphysis. 539
19
(a) (b)
19.57 Total hip replacement X-rays showing two modern types of total hip replacement: (a) a cemented collarless
tapered femoral prosthesis with an uncemented press-fit metal-backed acetabular implant; and (b) modular uncemented
implants. Some modular fittings allow a choice of femoral neck angles to overcome problems of severe anteversion or
retroversion of the femoral neck,.
Early uncemented implants – which were often exten- the polyethylene liner and to accelerated wear of the
sively textured – were cylindrical distally and gained thinner polyethylene liner. This problem has been
fixation in the diaphysis. As these stems were often addressed by improving the locking mechanism and
large, this led to thigh pain in up to 40 per cent of the bearing surfaces. A combination of a cemented
patients and stress protection in the proximal femur stem and an uncemented cup – the so-called ‘hybrid
with associated loss of bone. Subsequently tapered hip’ – has proved popular for use in the middle-aged
stems were designed in which the surface texturing patient.
was limited to the metaphyseal region to promote
proximal cancellous bone ingrowth. Three-point stem
BONE-CONSERVING FEMORAL ARTHROPLASTY
fixation provided immediate stability. Ten-year sur-
vivorship of 100 per cent has been reported with these Resurfacing arthroplasty Resurfacing arthroplasty was
tapered stems. popular in the 1970s. A large diameter head articu-
Uncemented acetabular components were intro- lated with a very thin polyethylene cup which was
duced to address the failure of fixation of cemented cemented. Catastrophic wear of the plastic occurred,
polyethylene cups, particularly in the younger patient. and implant failure of up to 33 per cent was reported
Most of these components are hemispherical and ini- in the short- to mid-term. Exploiting the evolving
tial stability and fixation is achieved by press-fitting technology of metal-on-metal bearings, McMinn
the cup into a slightly under-reamed acetabular demonstrated that very acceptable mid-term results
socket. Excellent survival of fixation has been could be achieved with metal-on-metal resurfacing
reported. Failures of these implants were often attrib- and hybrid fixation. Concerns remain about fracture
540 utable to malfunction of the locking mechanism of of the femoral neck – which occurs in 1–2 per cent of
all major series – and remodelling with narrowing of BEARING SURFACES 19
the femoral neck. Resurfacing is not suitable for all The issue of osteolysis had not been resolved by the
hips, and indications and limitations need to be rec- implantation of uncemented implants. Lytic lesions
ognized to reduce the number of technique-related have been reported with both stable and loose unce-
failures. The ideal indication is probably the need for mented prostheses, and micron or submicron particles
hip replacement in males younger than 60 years who of polyethylene have been identified as the main con-
The hip
have OA. tributing factors. Indeed this has been recognized as
the major limiting factor of conventional total hip
Short-stemmed implants Patients are now presenting
replacement and has led to the development of alter-
for total hip replacement at an increasingly younger
native bearing surfaces including highly cross-linked
age than in the past. These patients are likely to need
polyethylene and hard-on-hard couples.
at least one revision operation during their lifespan,
and one of the major challenges facing the surgeon
Highly cross-linked polyethylene (XLPE) Gamma irradia-
will be loss of bone stock beneath the cup. Conserva-
tion of polyethylene causes cross-linking, which
tive, short-stemmed prostheses have been developed
greatly improves the wear resistance compared to con-
which preserve bone. They are easily inserted through
ventional polyethylene. However, this comes at a
a minimally invasive approach, entail a smaller loss of
price, as the dose of irradiation is inversely propor-
bone at the time of surgery and conserve bone with
tional to the fracture toughness. Encouraging clinical
more physiological loading of the proximal femur.
results with markedly reduced wear have been
While excellent mid-term results have been reported
reported with XLPE. It should be noted that none of
with some of these implants, the concept should not
the commercially available XLPEs are the same – and
be widely embraced until longer-term follow-up has
the clinical performance is therefore likely to differ.
shown results similar to those of conventional
stemmed implants.
Ceramic-on-ceramic Alumina ceramics were intro-
duced as a bearing material in the 1970s. They are
APPROACHES
‘wettable’, have very low wear rates, are scratch-resis-
As noted earlier in this section, total hip replacement
tant and their particulate debris is not biologically
can be carried out through the standard approaches to
very active. However, ceramics are brittle and are sus-
the hip. The anterolateral and posterolateral
ceptible to fractures. Modern ceramics have been
approaches remain the most popular. The former is
strengthened and have much improved fracture tough-
associated with an increased incidence of abductor
ness. Excellent results have been reported with
dysfunction, while the latter is associated with an
ceramic–ceramic couples; however, because of their
increased risk of dislocation.
brittle nature it is still not possible to make safe ceramic
Minimally invasive surgery (MIS) Minimally invasive sur- liners with an inner diameter greater than 86 mm.
gery was initially advocated using the two-incision
technique – one anterior and one posterior – but this Metal-on-metal Metal bearing surfaces have very low
has been shown to be associated with an unacceptably wear rates and are self-polishing, which allows for self-
high incidence of complications including fractures, healing of surface scratches. Metal is not brittle,
component malposition and dislocation. It has now unlike ceramic, and components therefore do not
largely fallen into disuse. have to be as thick as their ceramic counterparts. Thus
Single-incision surgery, carried out through a skin large head diameters can be combined with mono-
incision of less than 10 cm, is reported to reduce pain, lithic cups. This gives a greater range of motion to
blood loss, rehabilitation time and length of hospital impingement, and thus greater mobility and greater
stay. An anterior or posterior approach is usually stability. The wear of these larger heads is dictated by
employed. The length of skin incision is a poor deter- the lubrication regimen, which is favourably influ-
minant of minimally invasive surgery, and will make enced by increasing the head size (thus increasing the
little difference to the morbidity and speed of rehabil- entrainment velocity of the lubricating fluid), and
itation if exactly the same soft-tissue dissection is car- optimizing the diametrical clearance and the spheric-
ried out deep to the skin as would have been done ity of the head. These durable couples allow patients
with a conventional incision. It is perhaps better to to return to vigorous recreational activities, and are
talk about ‘soft-tissue sparing surgery’; certainly this known as ‘high performance bearings’.
raised awareness of minimizing soft-tissue damage has Although these metal-on-metal couples have very
resulted in all incisions becoming very much smaller. low volumetric wear, they still generate twice the
Long-term follow-up is needed to show that the number of particles as metal-on-polyethylene bear-
proven durability of total hip replacement is not being ings. These particles are very small – in the nano range
lost by compromised exposure and malpositioning of – but do elicit a biological reaction. This is discussed
the implants. under Complications. 541
19 There are hundreds of different implants and bearing particularly vulnerable, and when this position results
options available on the market. This is not a reflec- from unbalanced paralysis the hip can slip unobtru-
tion of the requirement but rather of commercial sively out of position.
competition – yet another case of the tail wagging the During the swing phase of walking not only does
dog. the hip flex, it also rotates; this is because the pelvis
swivels forwards. As weight comes onto the leg, the
Rehabilitation The length of inpatient stay has been

abductor muscles contract, causing the pelvis to tilt
reduced to 4–6 days in most hospitals. Patients are
downwards on the weightbearing side; it is failure of
well mobilized on crutches or sticks before discharge,
this abductor mechanism which causes the Trendelen-
and will have negotiated stairs independently.
burg lurch.
Progress to full weightbearing without support will
The femoral head receives its arterial blood supply
usually take 6–8 weeks at the patient’s own pace.
from three sources: (1) intraosseous vessels running
up the neck, which are inevitably damaged with a dis-
placed cervical fracture; (2) vessels in the retinacula
NOTES ON APPLIED ANATOMY reflected from capsule to neck, which may be dam-
aged in a fracture or compressed by an effusion; and
The ball-and-socket arrangement of the hip combines (3) vessels in the ligamentum teres, which are unde-
stability for weightbearing with freedom of movement veloped in the early years of life and even later convey
for locomotion. A deeper acetabulum would confer only a meagre blood supply. The relative importance
greater stability but would limit the range of move- of these vessels varies with age, but at all ages avascu-
ment. Even with the fibrocartilaginous labrum the lar necrosis is a potential hazard.
socket is not deep enough to accommodate the whole The nerve supply of the hip, unlike the blood sup-
of the femoral head, whose articular surface extends ply, is plentiful. Sensory fibres, conveying propriocep-
considerably beyond a hemisphere. tion as well as pain, abound in the capsule and
The opening of the acetabulum faces downwards ligaments. The venous sinusoids of the bones also are
and forwards (about 30 degrees in each direction); supplied with sensory fibres; a rise in the intraosseous
the neck of the femur points upwards and forwards. venous pressure accounts for some of the pain in
Consequently, in the neutral position, the anterior osteoarthritis, and a reduction of this pressure for
portion of the head is not ‘contained’. The amount of some of the relief which may follow osteotomy.
forward inclination of the neck relative to the shaft The tensor fasciae femoris, though a relatively small
(the angle of anteversion) varies from 10 to 30 muscle, has, through its action in tightening the ili-
degrees in the adult. The upward inclination of the
neck is such that the neck–shaft angle is 125 degrees.
A neck–shaft angle of less than 125 degrees is
referred to as ‘coxa vara’ because, were the neck nor-
mally aligned relative to the pelvis, the limb would be
deviated towards the midline of the body – in varus; a
neck–shaft angle greater than 125 degrees (i.e. with
the neck unduly vertical) is coxa valga. The angle is
mechanically important because the further away the
abductor muscles are from the hip, the greater is their
leverage and their efficiency.
During standing and walking, the femoral neck acts
as a cantilever; the line of body weight passes medial
to the hip joint and is balanced laterally by the abduc-
tors (especially gluteus medius). The combination of
body weight, leverage effect and muscle action means
that the resultant force transmitted through the
femoral head can be very great – about five times the
body weight when walking slowly and much more
when running or jumping. It is easy to see why the hip
is so liable to suffer from cartilage failure – the essen-
tial feature of osteoarthritis. 19.58 Forces around the hip When standing on one leg
the pelvis is balanced on the femoral head. The vertical
The ligaments of the hip, though very strong in force due to the body weight (M) is counterbalanced by
front, are weak posteriorly; consequently, posterior contraction of the lateral muscles (F). The force borne by
dislocation is much more common than anterior. the femoral head is produced by the combined moments
542 When the hip is adducted and medially rotated it is M x A and F x B.
otibial tract, a surprisingly large range of functions. Espinosa N, Rothenfluh DA, Beck M, Ganz R, Leunig M. 19
This tract is anterior to the axis of knee flexion when Treatment of femoroacetabular impingement: prelimi-
the knee is straight, so its tension helps to hold the nary results of labral refixation. J Bone Joint Surg 2006;
knee slightly hyperextended while standing. It is also 88A: 925–35.
important in getting up from the sitting position, as Ficat RP. Idiopathic bone necrosis of the femoral head: early
well as during the phases of walking and running diagnosis and treatment. J Bone Joint Surg 1985; 67B:
The hip
when weight is being taken on the slightly flexed knee. 3–9.
Fish J. Cuneiform osteotomy of the femoral neck in the
treatment of slipped capital femoral epiphysis. A follow up
note. J Bone Joint Surg 1994; 76A: 46–59.
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545
The knee
20
Louis Solomon, Theo Karachalios
Locking is different from stiffness. The knee, quite

CLINICAL ASSESSMENT suddenly, cannot be straightened fully, although flex-
ion is still possible. This happens when a torn menis-
SYMPTOMS cus or loose body is caught between the articular
surfaces. By wiggling the knee around, the patient
Pain, either insidious in onset or more acute, is the may be able to ‘unlock’ it; sudden unlocking is the
most common knee symptom. With inflammatory or most reliable evidence that something mobile had
degenerative disorders it is usually diffuse, but with previously obstructed full extension. Do not be mis-
mechanical disorders (and especially after injury) it is led by ‘pseudo-locking’, when movement is suddenly
often localized – the patient can, and should, point to stopped by pain or the fear of impending pain.
the painful spot. Deformity is seldom a leading symptom; patients
If the patient can describe the mechanism of the are not keen to admit to having ‘knock knees’ or
injury, this is extremely useful: a direct blow to the ‘bandy legs’. However, a unilateral deformity, espe-
front of the knee may damage the patello-femoral cially if it is progressive, will be more worrying.
joint; a blow to the side may rupture the collateral lig- Giving way, a feeling of instability, or a lack of trust
ament; twisting injuries are more likely to cause a torn in the knee suggests a mechanical disorder caused by
meniscus or a cruciate ligament rupture. ligamentous, meniscal or capsular injury, or simple
Swelling may be diffuse or localized. If there was an muscle weakness. Giving way, particularly if it occurs
injury, it is important to ask whether the swelling when climbing up or down stairs, may also be due to
appeared immediately (suggesting a haemarthrosis) or patello-femoral pain or instability. Excessive use of an
only after some hours (typical of a torn meniscus). A unstable knee produces post-exercise swelling (effu-
complaint of recurrent swelling, with more or less sion or haemarthrosis) and diffuse pain within the
normal periods in between, suggests a longstanding joint.
internal derangement – possibly an old meniscal tear, Limp may be due to either pain or instability.
degeneration of the meniscus, a small osteoarticular Loss of function manifests as a progressively dimin-
fracture or loose bodies in the joint. Chronic swelling ishing walking distance, inability to run and difficulty
is typical of synovitis or arthritis. going up and down steps. Squatting or kneeling may
A small, localized swelling on the anteromedial or be painful, either because of pressure on the patello-
anterolateral side of the joint makes one think of a cyst femoral joint or because the knee cannot flex fully.
of the meniscus (always on the medial side) or a float-
ing loose body. Swelling over the front of the knee
could be due to a prepatellar bursitis; a localized bulge SIGNS WITH THE PATIENT UPRIGHT
in the popliteal fossa can also be caused by a bursal
swelling, but is more often due to ballooning of the For the examination, both lower limbs must be
synovial membrane and capsule at the back of the exposed from groin to toe; a mere hitching up of the
joint. skirt or rolling back of a trouser leg is not good
‘Stiffness’ is a common complaint, but it must be enough.
distinguished from inhibition of movement due to Deformity (valgus or varus or hyperextension) is
pain, or simple weakness of the extensor apparatus. best seen with the patient standing and bearing
Particularly characteristic is stiffness that appears reg- weight, lower limbs together (if possible!) and feet
ularly after periods of rest – so-called ‘post-inactivity pointing forward. Normally the knees and ankles can
stiffness’ – which suggests some type of chronic touch in the midline; this means that the knees must
arthritis. be in slight valgus (about 7 degrees in women and
20
(a) (b) (c) (d) (e)

20.1 Examination standing (a,b) Look at the general shape and posture, first from in front and then from behind.
Normally the knees are in slight valgus. Look for swelling of the joint or wasting of the thigh muscles; quadriceps wasting
occurs very quickly. (c) This patient has rheumatoid arthritis and bilateral valgus deformities; in contrast, osteoarthritis is
likely to lead to varus deformities (d). Unilateral deformity is easier to notice and almost always pathological – this man has
Paget’s disease of the tibia (e).
5 degrees in men), because the hips are wider than the remain centred over the femoral condyles or does it
knees. Genu valgum and genu varum are determined veer off towards one side in the early phase of knee
in relation to this normal anatomical alignment. But extension and then slide back to the centre with full
look carefully to see whether the deformity is really in extension – suggesting a tendency to subluxation?
the knee (often a sign of arthritis) and not in the lower Patellar alignment can also be assessed by measur-
end of the femur (a bone tumour?) or the upper end ing the Q-angle (quadriceps angle). This is the angle
of the tibia (e.g. a malunited fracture, or maybe subtended by a line drawn from the anterior superior
Paget’s disease (see Fig. 20.1e)). iliac spine to the centre of the patella and another
Alignment of the extensor mechanism (quadriceps, from the centre of the patella to the tibial tubercle
patella and patellar ligament) can also be measured (Fig. 20.2c); it normally averages about 14 degrees in
with the patient standing but is probably more conve- men and 17 degrees in women. An increased Q-angle
niently done with the patient seated (see below). is regarded as a predisposing factor in the develop-
Gait is important; the patient should also be ment of chondromalacia; however, small variations
observed walking with and without any support such from the norm are not a reliable indicator of future
as a stick or crutch. In the stance phase note whether pathology.
the knee extends fully (is there a fixed flexion defor-
mity or a hyperextension deformity?) and see if there
is any lateral or medial thrust signifying instability. In SIGNS WITH THE PATIENT LYING SUPINE
the swing phase note whether the knee moves freely or
The knees are the most visible and accessible of all the
is held in one position – usually because the joint is
large joints; with the legs lying side by side, features
painful but perhaps because it really is ankylosed!
on one side can be constantly compared with those on
When the patient walks, is there any sign of a limp?
the other.
And if so, does it stem from the knee? Or perhaps the
hip, or the foot?
Look
SIGNS WITH THE PATIENT SITTING The first things that strike one are the position of the
knee. Is it symmetrical with the normal side? Is it held
With the patient sitting sideways on the examination in valgus or varus, incompletely extended, or hyper-
couch, the outlines of the patellae and patellar liga- extended? Note also the presence of swelling, either of
ments, as well as the general shape and symmetry of the joint as a whole or as lumps or bumps in localized
the two knees and the tibial tubercles, can be made out areas.
quite easily. With the knees dangling at 90 degrees of Wasting of the quadriceps is a sure sign of joint dis-
flexion, the patellae should be facing straight forwards; order. The visual impression can be checked by meas-
note if they appear to be seated higher than usual uring the girth of the thigh at the same level (e.g. a
(patella alta) or lower than usual (patella baja). Patella fixed distance above the joint line or a hand’s breadth
alta is believed to be associated with a higher than nor- above the patella) in each limb.
mal incidence of chondromalacia patellae. Look more closely for signs of bruising, and for old
Next, ask the patient to straighten each knee in turn scars or sinuses, signifying previous infection or opera-
548 and observe how the patella moves upwards. Does it tions.
knee, the outlines of the joint margins, the patellar lig- 20
ament, the collateral ligaments, the iliotibial band and
Q the pes anserinus are then easily traced with the fin-
gers. The point of maximum tenderness will suggest
at least the anatomical site of pathology if not the pre-
cise diagnosis.
The knee
Synovial thickening is best appreciated as follows:
placing the knee in extension, the examiner grasps the
edges of the patella in a pincer made of the thumb and
middle finger, and tries to lift the patella forwards;
normally the bone can be grasped quite firmly, but if
the synovium is thickened the fingers simply slip off
the edges of the patella.
(a) (b)
20.2 Examination with the patient sitting The two Move
knees are compared for shape and symmetry. Note the Passive extension can be tested by the examiner simply
position of the patellae (a) in relaxation, (b) in full
extension and by measuring the Q-angle. holding both legs by the ankles and lifting them off
the couch; the knees should straighten fully (or even
into a few degrees of hyperextension) and symmetri-
cally. Active extension can be roughly tested by the
examiner slipping a hand under each knee and then
asking the patient to force the knees into the surface
of the couch; it is usually easy to feel whether the
hands are trapped equally strongly on the two sides.
Another way is to have the patient sitting on the edge
of the couch with his or her legs hanging over the side
and then asking them to extend each knee as far as
(a) (b) possible; the test can be repeated with the patient
extending the knees against resistance.
20.3 Examination with the patient supine (a) Wasting
of the quadriceps occurs rapidly after any internal
Passive and active flexion are tested with the patient
derangement of the knee. (b) The girth is measured at the lying supine. Normally the heel can be pulled up close
same level in both limbs, about a hand’s breadth above the to the buttock, with the knee moving through a range
patella. of 0–150 degrees. The ‘heel-to-buttock’ distance is
compared on the two sides.
Take note of the shape and position of the patella, Internal and external rotation, though normally no
both with the knee at rest and during movement. more than about 10 degrees, should also be assessed.
Always compare the symptomatic with the normal The patient’s hip and knee are flexed to 90 degrees;
side. one hand steadies and feels the knee, the other rotates
the foot.
Crepitus during movement may be felt with a hand
Feel placed on the front of the knee. It usually signifies
As with all joints, palpation of the knee – if it is to be patello-femoral roughness.
rewarding – demands a sound knowledge of the local Movement with compartmental loading is a useful
anatomy. test for localizing the site of joint pain; the medial or
Start by running your hand down the length of the lateral compartment of the knee can be loaded sepa-
limb, feeling for changes in skin temperature and com- rately by applying varus or valgus stress during flexion
paring the symptomatic with the normal side. There is and noting which manoeuvre is more painful.
normally a gradual decrease in skin temperature from
proximal to distal. Increased warmth over the knee
signifies increased vascularity, usually due to inflam-
Tests for intra-articular fluid
mation. Cross fluctuation This test is applicable only if there is
The soft tissues and bony outlines are then palpated a large effusion. The left hand compresses and emp-
systematically, feeling for abnormal outlines and local- ties the suprapatellar pouch while the right hand
ized tenderness. This is easier if the joint is flexed and straddles the front of the joint below the patella; by
the examiner sits on the edge of the couch facing the squeezing with each hand alternately, a fluid impulse
knee. By placing both hands over the front of the is transmitted across the joint. 549
20
(a) (b) (c)
2
5 3
4
6
(d) (e) (f)

20.4 Examination with the patient supine Swelling may involve either the whole joint, as in this patient (a) with acute
synovitis, or may be due to some localized lesion as in patient (b) with a large loose body in the joint and patient (c) with a
small meniscal cyst protruding at the medial joint line. (d) Feeling for synovial swelling: try to ‘lift’ the patella – if the
synovium is thickened your fingers will slip off the edges. (e) Feeling for tenderness: sit facing the patient’s knee and try to
identify the exact site of pain/tenderness. A superficial ‘map’ is shown in (f): 1, quadriceps tendon; 2, edge of patella; 3,
medial collateral ligament; 4, joint line; 5, lateral collateral ligament; 6, patellar ligament.
The patellar tap Again the suprapatellar pouch is com- is sharply compressed – a distinct ripple is seen on the
pressed with the left hand to squeeze any fluid from flattened medial surface as fluid is shunted across.
the pouch into the joint. With the other hand the
Normally, when the knee
The juxta-patellar hollow test
patella is then tapped sharply backwards onto the
is flexed, a hollow appears lateral to the patellar liga-
femoral condyles. In a positive test the patella can be
ment and disappears with further flexion; if there is
felt striking the femur and bouncing off again (a type
excess fluid, the hollow fills and disappears at a lesser
of ballottement).
angle of flexion (Mann et al., 1991). Compare this in
The bulge test This is a useful method of testing when the two knees.
there is very little fluid in the joint, though it takes
some practice to get it right! After squeezing any fluid
out of the suprapatellar pouch, the medial compart-
The patello-femoral joint
ment is emptied by pressing on the inner aspect of the The size, shape and position of the patella are noted.
joint; that hand is then lifted away and the lateral side The bone is felt, first on its anterior surface and then
(a) (b) (c)
20.5 Movement The knee should move from full extension (a) through a range of 150 degrees to full flexion (b). Small
degrees of flexion deformity (loss of full extension) can be detected by placing the hands under the knees while the patient
forces the legs down on the couch (c); if your hand can be extracted more easily on one side than the other, this indicates
550 loss of the final few degrees of complete extension.
excessive (compared to the normal side) it suggests a 20
torn or stretched collateral ligament. Sideways move-
ment in full extension is always abnormal; it may be
due to either torn or stretched ligaments and capsule
or loss of articular cartilage or bone, which allows the
affected compartment to collapse.
The knee
Cruciate ligaments Routine tests for cruciate ligament
(a) (b) stability are based on examining for abnormal gliding
movements in the sagittal plane. With both knees
flexed 90 degrees and the feet resting on the couch,
the upper tibia is inspected from the side; if its upper
end has dropped back, or can be gently pushed back,
this indicates a tear of the posterior cruciate ligament
(the ‘sag sign’). With the knee in the same position,
the foot is anchored by the examiner sitting on it
(provided this is not painful); then, using both hands,
the upper end of the tibia is grasped firmly and rocked
backwards and forwards to see if there is any antero-
posterior glide (the ‘drawer test’). Excessive anterior
(c) (d) (e) movement (a positive anterior drawer sign) denotes
anterior cruciate laxity; excessive posterior movement
20.6 Testing for intra-articular fluid (a) The juxta-
patellar hollow, which disappears in flexion if there is fluid (a positive posterior drawer sign) signifies posterior
in the knee. (b) Patellar tap test. (c,d,e) Doing the bulge cruciate laxity.
test: compress the suprapatellar pouch (c), empty the More sensitive is the Lachman test, but this is diffi-
medial compartment (d), push fluid back from the lateral cult if the patient has big thighs (or the examiner has
compartment and watch for the bulge on the medial
small hands). The patient’s knee is flexed 20 degrees;
side (e).
with one hand grasping the lower thigh and the other
the upper part of the leg, the joint surfaces are shifted
backwards and forwards upon each other. If the knee
is stable, there should be no gliding. In both the
along its edges and at the attachments of the quadri-
drawer test and Lachman test, note whether the end-
ceps tendon and the patellar ligament. Much of the
point of abnormal movement is ‘soft’ or ‘hard’.
posterior surface, too, is accessible to palpation if
the patella is pushed first to one side and then to the
other; tenderness suggests synovial irritation or artic-
ular cartilage softening.
Moving the patella up and down while pressing it
lightly against the femur (the ‘friction test’) causes
painful grating if the central portion of the articular
cartilage is damaged.
Pressing the patella laterally with the thumb while
flexing the knee slowly may induce anxiety and sharp
resistance to further movement; this, the ‘apprehen-
sion test’, is diagnostic of recurrent patellar subluxa- (a) (b)
tion or dislocation.
20.7 Patello-femoral
joint (a) Feeling under the
Tests for stability edge of the patella.
(b) Testing for patello-
Collateral ligaments The medial and lateral ligaments femoral tenderness. (c) The
are tested by stressing the knee into valgus and varus: patellar apprehension test.
this is best done by tucking the patient’s foot under
your arm and holding the extended knee firmly with
one hand on each side of the joint; the leg is then
angulated alternately towards abduction and adduc-
tion. The test is performed at full extension and again
at 30 degrees of flexion. There is normally some
medio-lateral movement at 30 degrees, but if this is (c) 551
20 20.8 Testing for instability
There are two ways of testing
the collateral ligaments (side-
to-side stability): (a) by
gripping the foot close to your
body and guiding the knee
alternately towards valgus and
varus; (b) by gripping the

femoral condyles (provided
your hand is big enough) and
then forcing the leg alternately
into valgus and varus. (c) In
(a) (b) (c)
this case there was gross
instability on the lateral side,
allowing the knee to be pulled
into marked varus. Cruciate
ligament instability can be
assessed by either the drawer
test (d) or the Lachman test
(e), as described in the text.
(d) (e)
Complex ligament injuries When only a collateral or A positive test is helpful but not pathognomonic; a
cruciate ligament is damaged the diagnosis is relatively negative test does not exclude a tear.
easy: the direction of unstable movement is either side-
Thessaly test This test is based on a dynamic repro-
ways or front-to-back. With combined injuries the
duction of load transmission in the knee joint under
direction of instability may be oblique or rotational.
normal or trauma conditions. With the affected knee
Special clinical tests have been developed to detect
flexed to 20 degrees and the foot placed flat on the
these abnormalities (see Chapter 30); the best known
ground, the patient takes his or her full weight on that
is the pivot shift test. The patient lies supine with the
leg while being supported (for balance) by the exam-
lower limb completely relaxed. The examiner lifts the
iner (Fig. 20.9). The patient is then instructed to twist
leg with the knee held in full extension and the tibia
his or her body to one side and then to the other three
internally rotated (the position of slight rotational sub-
times (thus, with each turn, exerting a rotational force
luxation). A valgus force is then applied to the lateral
in the knee) while keeping the knee flexed at 20
side of the joint as the knee is flexed; a sudden poste-
degrees. Patients with meniscal tears experience
rior movement of the tibia is seen and felt as the joint
medial or lateral joint line pain and may have a sense
is fully re-located. The test is sometimes quite painful.
of locking. The test has shown a high diagnostic accu-
racy rate at the level of 95 per cent in detecting menis-
Tests for meniscal injuries cal tears, with a low number of false positive and
negative recordings (Karachalios et al., 2005).
McMurray’s test This classic test for a torn meniscus is
seldom used now that the diagnosis can easily be
made by MRI. However, advanced imaging is not
always available and the clinical test has not been alto- SIGNS WITH THE PATIENT LYING PRONE
gether discarded.
The test is based on the fact that the loose meniscal Scars or lumps in the popliteal fossa are noted. If there
tag can sometimes be trapped between the articular sur- is a swelling, is it in the midline (most likely a bulging
faces and then induced to snap free with a palpable and capsule) or to one side (possibly a bursa)? A semi-
audible click. The knee is flexed as far as possible; one membranous bursa is usually just above the joint line,
hand steadies the joint and the other rotates the leg me- a Baker’s cyst below it.
dially and laterally while the knee is slowly extended. The popliteal fossa is carefully palpated. If there is a
The test is repeated several times, with the knee stressed lump, where does it originate? Does it pulsate? Can it
552 in valgus or varus, feeling and listening for the click. be emptied into the joint?
20
The knee
(a) (b)
(c) (d)
20.10 X-rays Anteroposterior views should always be

taken with the patient standing. (a,b) X-rays with the
20.9 Meniscal injury – Thessaly test Picture showing
patient lying down show only slight narrowing of the
how the patient is positioned during the Thessaly test.
medial joint space on each side; but with weightbearing
(c,d) it is clear that these changes are much more marked.
Apley’s test With the patient prone the knee is flexed

to 90 degrees and rotated while a compression force is
CT is useful for showing patello-femoral congru-
applied; this, the grinding test, reproduces symptoms if
ence at various angles of flexion.
a meniscus is torn. Rotation is then repeated while the
MRI provides a reliable means of diagnosing lateral
leg is pulled upwards with the surgeon’s knee holding
and medial meniscal tears and cruciate ligament
the thigh down; this, the distraction test, produces
injuries (Oei et al., 2003). It is also helpful in identi-
increased pain only if there is ligament damage.
fying the early stages of osteoarticular lesions and
Lachman’s test The Lachman test can be readily per- osteonecrosis of the femoral or tibial condyles.
formed with the patient prone.
IMAGING ARTHROSCOPY
X-RAYS Arthroscopy is useful: (1) to establish or refine the

Anteroposterior and lateral views are routine; it is accuracy of diagnosis; (2) to help in deciding whether
often useful also to obtain tangential (‘skyline’) to operate, or to plan the operative approach with
patello-femoral views and intercondylar (or tunnel) more precision; (3) to record the progress of a knee
views. The anteroposterior view should always be taken disorder; and (4) to perform certain operative proce-
with the patient standing; unless the femoro-tibial dures. Arthroscopy is not a substitute for clinical
compartment is loaded, narrowing of the articular examination; a detailed history and meticulous assess-
space may be missed. Both knees should be x-rayed, ment of the physical signs are indispensable prelimi-
so as to compare the abnormal with the normal side. naries and remain the sheet anchor of diagnosis.
Tibio-femoral alignment can be measured on full- However, arthroscopy is especially helpful in diagnos-
length standing views. Normal indices have also been ing meniscal injuries – and dealing with them at the
established for patellar height and patello-femoral same time. Full asepsis is essential.
congruence. These features are discussed in the rele-
vant sections of the chapter.
OTHER FORMS OF IMAGING THE DIAGNOSTIC CALENDAR

Radioscintigraphy may show increased activity in the
subarticular bone in early osteoarthritis. It is also While most disorders of the knee can occur at any age,
helpful in showing ‘hot spots’ due to infection after certain conditions are more commonly encountered
joint replacement. during specific periods of life. 553
20
(a) (b) (c)
(d) (e) (f)
20.11 MRI A series of sagittal T1 weighted images proceeding from medial to lateral show the normal appearances of
(a,b) the medial meniscus, (c) the posterior cruciate ligament, (d) the somewhat fan-shaped anterior cruciate ligament and
(e,f) the lateral meniscus.
Congenital knee disorders may be present at birth or

may become apparent only during the first or second
DEFORMITIES OF THE KNEE
decade of life.
Adolescents with anterior knee pain are usually By the end of growth the knees are normally in 5–7
found to have chondromalacia patellae, patellar insta- degrees of valgus. Any deviation from this may be
bility, osteochondritis or a plica syndrome. But regarded as ‘deformity’, though often it bothers no
remember – knee pain may be referred from the hip! one – least of all the possessor of the knees. The three
Young adults engaged in sports are the most fre- common deformities are bow leg (genu varum),
quent victims of meniscal tears and ligament injuries. knock knee (genu valgum) and hyperextension (genu
Examination should include a variety of tests for liga- recurvatum).
mentous instability that would be quite inappropriate
in elderly patients.
Patients above middle age with chronic pain and BOW LEGS AND KNOCK KNEES IN
stiffness probably have osteoarthritis. With primary CHILDREN
osteoarthritis of the knees, other joints also are often
affected; polyarthritis does not necessarily (nor even Deformity is usually gauged from simple observation.
554 most commonly) mean rheumatoid arthritis. Bilateral bow leg can be recorded by measuring the
20
The knee
20.12 Arthroscopy Arthroscopic images of the interior of the right knee from the lateral side, showing (1)
chrondomalacia patellae; (2) normal medial meniscus; (3) torn medial meniscus; (4) degenerate medial meniscus and
osteoarthritic femoral condyle; (5) rheumatoid synovium; (6) osteochondritis dissecans of medial femoral condyle.
distance between the knees with the child standing these children are normal in all other respects; the par-
and the heels touching; it should be less than 6 cm. ents should be reassured and the child should be seen
Similarly, knock knee can be estimated by measuring at intervals of 6 months to record progress.
the distance between the medial malleoli when the In the occasional case where, by the age of 10, the
knees are touching with the patellae facing forwards; deformity is still marked (i.e. the intercondylar distance
it is usually less than 8 cm. is more than 6 cm or the intermalleolar distance more
than 8 cm), operative correction should be advised.
Stapling of the physes on one or other side of the
Physiological bow legs and knock knees knee can be done to restrict growth on that side and
Bow legs in babies and knock knees in 4-year-olds are allow correction of the deformity (the staples are
so common that they are considered to be normal removed once the knee has over-corrected slightly);
stages of development. Other postural abnormalities there is a risk, however, that normal growth will not
such as ‘pigeon toes’ and flat feet may coexist but resume when the staples are removed. 555
20
(a) (b) (c) (d) (e) (f)
20.13 Physiological genu valgum ‘Knock-knees’ in young children usually correct spontaneously. These pictures of the
same child were obtained at various ages between 3 and 7 years.
Hemi-epiphysodesis (fusion of one-half of the growth

plate) on the ‘convex’ side of the deformity will
achieve similar correction; this requires careful timing
guided by charting the child’s bone age and estimat-
ing the corrective effect of arresting further growth
on one side of the bone.
Corrective osteotomy (supracondylar osteotomy for
valgus knees and high tibial osteotomy for varus
knees) may sound sensible; however, the child (and (a) (b) (c) (d)
the parents) will have to put up with the ‘deformity’
20.14 Persistent deformities (a,b) Persistent genu
until growth is complete before undergoing the oper-
varum before and after corrective osteotomy. (c,d) Before
ation, otherwise there is a risk of the deformity recur- and after osteotomy for severe genu valgum.
ring while the child is still growing.
Compensatory deformities Blount’s disease

Varus, valgus and rotational deformities of the proxi- This is a progressive bow-leg deformity associated
mal femur may give rise to complex compensatory with abnormal growth of the posteromedial part of
deformities of the knees and legs once the child starts the proximal tibia. The children are usually over-
to walk. Thus, persistent anteversion of the femoral weight and start walking early; the condition is
neck may come to be associated with ‘squinting
knees’ (the patellae face inwards when the hips are
fully located), genu valgum, tibial torsion and valgus
heels. It is essential to analyse all components of these
deformities before focussing on the knees. Often they
correct spontaneously by the end of growth, or if
some elements persist, they cause little or no problem;
only in severe cases – and after the most meticulous
preoperative planning – are osteotomies undertaken.
Pathological bow leg and knock knee

Disorders which cause distorted epiphyseal and/or
physeal growth may give rise to bow leg or knock
knee; these include some of the skeletal dysplasias and
the various types of rickets, as well as injuries of the
epiphyseal and physeal growth cartilage. A unilateral
deformity is likely to be pathological, but it is essen- (a) (b) (c)
tial in all cases to look for signs of injury or general-
ized skeletal disorder. If angulation is severe, operative 20.15 Pathological bow legs (a) Child with healed
rickets. (b) Growth deformity following a fracture involving
correction will be necessary, but it should be deferred the proximal tibial physis. (c) The deformity here was due
until near the end of growth lest the deformity recur to a ‘slipped’ proximal tibial epiphysis in a child with an
556 with further growth. endocrine disorder.
Slight over-correction should be aimed for as some 20
recurrence is inevitable. In severe cases it may be nec-
essary also to elevate the depressed medial tibial
plateau using a wedge of bone taken from the femur.
If a bony bar has formed, it can be excised and
replaced by a free fat graft. In older children it may be
The knee
easier to perform a surgical correction and then (if
necessary) lengthen the tibia by the Ilizarov method.
All these procedures should be accompanied by
fasciotomy to reduce the risk of a postoperative com-
partment syndrome.
DEFORMITIES OF THE KNEE IN

ADULTS
20.16 Blount’s disease In contrast to the children in Fig. GENU VARUM AND GENU VALGUM
20.15, this young boy developed progressive bow-legged
deformities from the time he started walking. X-rays Angular deformities are common in adults (usually
showed the typical features of Blount’s disease: marked bow legs in men and knock knees in women). They
distortion of the tibial epiphysis, as if one half of the
growth plate (physis) had fused and stopped growing.
may be the sequel to childhood deformity and if so usu-
Changes can be accurately assessed by measuring the ally cause no problems. However, if the deformity is
metaphyseo-diaphyseal angle: a line is drawn associated with joint instability, this can lead to
perpendicular to the long axis of the tibia and another osteoarthritis – of the medial compartment in varus
across the metaphyseal flare as shown on the x-ray; the knees and the lateral compartment in valgus knees.
acute angle formed by these two lines should normally not
exceed 11°.
Genu valgum may also cause abnormal tracking of the
patella and predispose to patello-femoral osteoarthri-
tis. Even in the absence of overt osteoarthritis, if the
patient complains of severe pain, or if there are clini-
bilateral in 80 per cent of cases. Children of negroid
cal or radiological signs of joint damage, a ‘prophy-
descent appear to be affected more frequently than
lactic’ osteotomy can be performed – above the knee
others. Deformity is noticeably worse than in physio-
for valgus deformity and below the knee for varus.
logical bow legs and may include internal rotation of
Preoperative planning should include radiographic
the tibia. The child walks with an outward thrust of
measurements to determine the mechanical and
the knee; in the worst cases there may be lateral sub-
anatomical axes of both bones and the lower limb, as
luxation of the tibia.
well as estimation of the centre of rotation of angula-
X-ray The proximal tibial epiphysis is flattened medi- tion.
ally and the adjacent metaphysis is beak-shaped. The Deformity may be secondary to arthritis – usually
medial cortex of the proximal tibia appears thickened; varus in osteoarthritis and valgus in rheumatoid
this is an illusory effect produced by internal rotation arthritis. In these cases the joint is often unstable and
of the tibia. The tibial epiphysis sometimes looks corrective osteotomy less predictable in its effect.
‘fragmented’; occasionally the femoral epiphysis also is Stress x-rays are essential in the assessment of these
affected. In the late stages a bony bar forms across the cases.
medial half of the tibial physis, preventing further Other causes of varus or valgus deformity are liga-
growth on that side. The degree of proximal tibia vara ment injuries, malunited fractures and Paget’s disease.
can be quantified by measuring the metaphyseo-dia- Where possible, the underlying disorder should be
physeal angle (see Fig. 20.16). dealt with; provided the joint is stable, corrective
In contrast to physiological bowing, abnormal osteotomy may be all that is necessary.
alignment occurs in the proximal tibia and not in the
joint.
GENU RECURVATUM (HYPEREXTENSION
Treatment Spontaneous resolution is rare and, once it OF THE KNEE)
is clear that the deformity is progressing, a corrective
osteotomy should be performed, addressing both the Congenital recurvatum This may be due to abnormal
varus and the rotational components. A preoperative intra-uterine posture; it usually recovers sponta-
(or peroperative) arthrogram, to outline the mis- neously. Rarely, gross hyperextension is the precursor
shapen epiphysis, will help in planning the operation. of true congenital dislocation of the knee. 557
20 20.17 Knee
deformities in
adults Genu varum
is usually associated
with osteoarthritis
(a); genu valgum
with rheumatoid
arthritis (b); and

genu recurvatum
(c) with severe
destructive arthritis
(e.g. Charcot’s
disease) or a flail
joint (e.g. post-
poliomyelitis).
(a) (b) (c)
Lax ligaments Normal people with generalized joint of the contact stresses are taken by the menisci when
laxity tend to stand with their knees back-set. Pro- the knee is loaded in extension, rising to almost 90
longed traction, especially on a frame, or holding the per cent with the knee in flexion. If the menisci are
knee hyperextended in plaster, may overstretch liga- removed, articular stresses are markedly increased;
ments, leading to permanent hyperextension defor- even a partial meniscectomy of one-third of the width
mity. Ligaments may also become overstretched of the meniscus will produce a threefold increase in
following chronic or recurrent synovitis (especially in contact stress in that area.
rheumatoid arthritis), the hypotonia of rickets, the The medial meniscus is much less mobile than the
flailness of poliomyelitis or the insensitivity of Char- lateral, and it cannot as easily accommodate to abnor-
cot’s disease. mal stresses. This may be why meniscal lesions are
In paralytic conditions such as poliomyelitis, recur- more common on the medial side than on the lateral.
vatum is often seen in association with fixed equinus Even in the absence of injury, there is gradual stiff-
of the ankle: in order to set the foot flat on the ening and degeneration of the menisci with age, so
ground, the knee is forced into hyperextension. In splits and tears are more likely in later life – particu-
moderate degrees, this may actually be helpful (e.g. in larly if there is any associated arthritis or chondrocal-
stabilizing a knee with weak extensors). However, if cinosis. In young people, meniscal tears are usually the
excessive and prolonged, it may give rise to a perma- result of trauma.
nent deformity. If bony correction is undertaken, the
knee should be left with some hyperextension to pre-
serve the stabilizing mechanism. If quadriceps power TEARS OF THE MENISCUS
is poor, the patient may need a caliper. Severe paralytic
hyperextension can be treated by fixing the patella The meniscus consists mainly of circumferential fibres
into the tibial plateau, where it acts as a bone block held by a few radial strands. It is, therefore, more
(Men et al., 1991). likely to tear along its length than across its width.
The split is usually initiated by a rotational grinding
Miscellaneous Other causes of recurvatum are growth
force, which occurs (for example) when the knee is
plate injuries and malunited fractures. These can be flexed and twisted while taking weight; hence the fre-
safely corrected by osteotomy. quency in footballers. In middle life, when fibrosis has
restricted mobility of the meniscus, tears occur with
relatively little force.
LESIONS OF THE MENISCI
Pathology
The menisci have an important role in (1) improving
articular congruency and increasing the stability of the The medial meniscus is affected far more frequently
knee, (2) controlling the complex rolling and gliding than the lateral, partly because its attachments to the
actions of the joint and (3) distributing load during capsule make it less mobile. Tears of both menisci may
558 movement. During weightbearing, at least 50 per cent occur with severe ligament injuries.
Most of the meniscus is avascular and spontaneous 20
repair does not occur unless the tear is in the outer
third, which is vascularized from the attached syn-
ovium and capsule. The loose tag acts as a mechanical
irritant, giving rise to recurrent synovial effusion and,
in some cases, secondary osteoarthritis.
The knee
(b) (c)
Clinical features
The patient is usually a young person who sustains a
twisting injury to the knee on the sports field. Pain
(usually on the medial side) is often severe and further
activity is avoided; occasionally the knee is ‘locked’ in
(a) (d) (e)
partial flexion. Almost invariably, swelling appears
20.18 Torn medial meniscus (a) The meniscus is usually some hours later, or perhaps the following day.
torn by a twisting force with the knee bent and taking With rest the initial symptoms subside, only to
weight; the initial split (b) may extend anteriorly
(c), posteriorly (d) or both ways to create a ‘bucket-handle’ recur periodically after trivial twists or strains. Some-
tear (e). times the knee gives way spontaneously and this is
again followed by pain and swelling.
It is important to remember that in patients aged
In 75 per cent of cases the split is vertical in the over 40 the initial injury may be unremarkable and the
length of the meniscus. If the separated fragment main complaint is of recurrent ‘giving way’ or ‘locking’.
remains attached front and back, the lesion is called a ‘Locking’ – that is, the sudden inability to extend
bucket-handle tear. The torn portion sometimes dis- the knee fully – suggests a bucket-handle tear. The
places towards the centre of the joint and becomes patient sometimes learns to ‘unlock’ the knee by
jammed between femur and tibia, causing a block to bending it fully or by twisting it from side to side.
extension (‘locking’). If the tear emerges at the free On examination the joint may be held slightly
edge of the meniscus, it leaves a tongue based anteri- flexed and there is often an effusion. In longstanding
orly (an anterior horn tear) or posteriorly (a posterior cases the quadriceps will be wasted. Tenderness is
horn tear). localized to the joint line, in the vast majority of cases
Horizontal tears are usually ‘degenerative’ or due to on the medial side. Flexion is usually full but exten-
repetitive minor trauma. Some are associated with sion is often slightly limited.
meniscal cysts (see below). Between attacks of pain and effusion there is a
20.19 Torn medial

meniscus – tests
(a,b) McMurray’s
test is performed at
varying angles of
flexion. (c,d) The
grinding test relaxes
the ligaments but
compresses the
meniscus – it causes
pain with meniscus
lesions. (e,f) The
(a) (b) distraction test
releases the
meniscus but
stretches the
ligaments and
causes pain if these
are injured.
(c) (d) (e) (f) 559

20 grinding test gives less pain and the distraction test
more pain.
A torn anterior cruciate ligament can cause chronic
instability, with a sense of the knee ‘giving way’ or
buckling when the patient turns sharply towards the
side of the affected knee. Careful examination should
reveal signs of rotational instability, a positive Lach-

man test or a positive anterior drawer sign. MRI or
arthroscopy will settle any doubts.
Treatment
Dealing with the locked knee Usually the knee
‘unlocks’ spontaneously; if not, gentle passive flexion
and rotation may do the trick. Forceful manipulation
is unwise (it may do more damage) and is usually
unnecessary; after a few days’ rest the knee may well
20.20 Torn meniscus – MRI Sagittal MRI showing a tear
in the medial meniscus. unlock itself. However, if the knee does not unlock, or
if attempts to unlock it cause severe pain, arthroscopy
is indicated. If symptoms are not marked, it may be
better to wait a week or two and let the synovitis set-
tle down, thus making the operation easier; if the tear
disconcerting paucity of signs. The history is helpful, is confirmed, the offending fragment is removed.
and McMurray’s test, Apley’s grinding test or the
Conservative treatment If the joint is not locked, it is
Thessaly test may be positive.
reasonable to hope that the tear is peripheral and can
therefore heal spontaneously. After an acute episode,
Investigations
the joint is held straight in a plaster backslab for 3–4
Plain x-rays are usually normal, but MRI is a reliable weeks; the patient uses crutches and quadriceps exer-
method of confirming the clinical diagnosis, and may cises are encouraged. Operation can be put off as long
even reveal tears that are missed by arthroscopy. as attacks are infrequent and not disabling and the
Arthroscopy has the advantage that, if a lesion is patient is willing to abandon those activities that pro-
identified, it can be treated at the same time. voke them. MRI will show if the meniscus has healed.
Operative treatment Surgery is indicated (1) if the
joint cannot be unlocked and (2) if symptoms are
Loose bodies in the joint may cause true locking. The recurrent. For practical purposes, the lesion is often
history is much more insidious than with meniscal dealt with as part of the ‘diagnostic’ arthroscopy.
tears and the attacks are variable in character and Tears close to the periphery, which have the capacity
intensity. A loose body may be palpable and is often to heal, can be sutured; at least one edge of the tear
visible on x-ray. should be red (i.e. vascularized). In appropriate cases
Recurrent dislocation of the patella causes the knee the success rate for both open and arthroscopic repair
to give way; typically the patient is caught unawares is almost 90 per cent.
and collapses to the ground. Tenderness is localized Tears other than those in the peripheral third are
to the medial edge of the patella and the apprehension dealt with by excising the torn portion (or the bucket
test is positive. handle). Total meniscectomy is thought to cause
Fracture of the tibial spine follows an acute injury more instability and so predispose to late secondary
and may cause a block to full extension. However, osteoarthritis; certainly in the short term it causes
swelling is immediate and the fluid is blood-stained. greater morbidity than partial meniscectomy and has
X-ray may show the fracture. no obvious advantages.
A partial tear of the medial collateral ligament may Arthroscopic meniscectomy has distinct advantages
heal with adhesions where it is attached to the medial over open meniscectomy: shorter hospital stay, lower
meniscus, so that the meniscus loses mobility. The costs and more rapid return to function. However, it
patient complains of recurrent attacks of pain and giv- is by no means free of complications (Sherman et al.,
ing way, followed by tenderness on the medial side. 1986).
Sleep may be disturbed if the medial side rests upon Postoperative pain and stiffness are reduced by pro-
the other knee or the bed. As with a meniscus injury, phylactic non-steroidal anti-inflammatory drugs.
560 rotation is painful; but unlike a meniscus lesion, the Quadriceps-strengthening exercises are important.
20.21 Torn meniscus – 20
operation (a) Removal of a
torn medial meniscus.
(b) Repair is appropriate if at
least one edge of the tear is
vascularized. This can be done
arthroscopically.
The knee
(a) (b)
Outcome DISCOID LATERAL MENISCUS

Neither a meniscal tear by itself nor removal of the In the fetus the meniscus is not semilunar but disc-
meniscus necessarily leads to secondary osteoarthritis. like; if this shape persists, symptoms are likely. A
However, the likelihood is increased if the patient has young patient complains that, without any history of
(a) a pre-existing varus deformity of the knee, (b) injury, the knee gives way and ‘thuds’ loudly. A char-
signs of cruciate ligament insufficiency or (c) features acteristic clunk may be felt at 110 degrees as the knee
elsewhere of a generalized osteoarthritis. is bent and at 10 degrees as it is being straightened.
The diagnosis is easily confirmed by MRI.
If there is only a clunk, treatment is not essential. If
MENISCAL DEGENERATION pain is disturbing, the meniscus may be excised,
though a more attractive procedure is arthroscopic
Patients over 45 years old may present with symptoms partial excision leaving a normally shaped meniscus
and signs of a meniscal tear. Often, though, they can (Dimakopoulos and Patel, 1990).
recall no preceding injury. At arthroscopy there may
be a horizontal cleavage in the medial meniscus – the MENISCAL CYSTS
characteristic ‘degenerative’ lesion – or detachment of
the anterior or posterior horn without an obvious Cysts of the menisci are probably traumatic in origin,
tear. Associated osteoarthritis or chrondrocalcinosis is arising from either a small horizontal cleavage tear or
common. repeated squashing of the peripheral part of the menis-
A detached anterior or posterior horn can be cus. It is also suggested that synovial cells infiltrate into
sutured firmly in place. Meniscectomy is indicated the vascular area between meniscus and capsule and
only if symptoms are marked or if, at arthroscopy, there multiply. The multilocular cyst contains gelati-
there is a major tear causing mechanical block. nous fluid and is surrounded by thick fibrous tissue.
20.22 Meniscal cyst (a) Typical

appearance of a small, firm swelling
at or just below the joint line.
(b) MRI showing the cyst arising
from the edge of the meniscus
(arrow).
(a) (b) 561

20 Clinical features nied by pain and recurrent episodes of swelling. There
may be a meniscal tear, but meniscectomy is likely to
The lateral meniscus is affected much more frequently make matters worse; sometimes patients present with
than the medial. The patient complains of an ache or meniscectomy scars on both sides of the knee!
a small lump at the side of the joint. Symptoms may Examination should include special tests for liga-
be intermittent, or worse after activity. mentous instability as well as radiological investiga-
On examination the lump is situated at or slightly tion and arthroscopy. It is important not only to
below the joint line, usually anterior to the collateral establish the nature of the lesion but also to measure
ligament. It is seen most easily with the knee slightly the level of functional impairment against the needs
flexed; in some positions it may disappear altogether. and demands of the individual patient before advocat-
Lateral cysts are often so firm that they are mistaken ing treatment.
for a solid swelling. Medial cysts are usually larger and The subject is dealt with in detail in Chapter 30.
softer.
RECURRENT DISLOCATION OF THE
Apart from cysts, various conditions may present with PATELLA
a small lump along the joint line.
A ganglion is quite superficial, usually not as ‘hard’
Acute dislocation of the patella is dealt with in Chap-
as a cyst, and unconnected with the joint.
ter 30. In 15–20 per cent of cases (mostly children)
Calcific deposits in the collateral ligament usually
the first episode is followed by recurrent dislocation
appear on the medial side, are intensely painful and
or subluxation after minimal stress. This is due, in
tender, and often show on the x-ray.
some measure, to disruption or stretching of the liga-
A prolapsed, torn meniscus occasionally presents as a
mentous structures which normally stabilize the
rubbery, irregular lump at the joint line. In some cases
extensor mechanism. However, in a significant pro-
the distinction from a ‘cyst’ is largely academic.
portion of cases there is no history of an acute strain
Various tumours, both of soft tissue (lipoma,
and the initial episode is thought to have occurred
fibroma) and of bone (osteochondroma), may pro-
‘spontaneously’. It is now recognized that in all cases
duce a medial or lateral joint lump. Careful examina-
of recurrent dislocation, but particularly in the latter
tion will show that the lump does not arise from the
group, one or more predisposing factors are often pres-
joint itself.
ent: (1) generalized ligamentous laxity; (2) under-
development of the lateral femoral condyle and
Treatment flattening of the intercondylar groove; (3) maldevel-
If the symptoms warrant operation, the cyst may be opment of the patella, which may be too high or too
removed. In the past this was usually combined with small; (4) valgus deformity of the knee; (5) external
total meniscectomy, in order to prevent an inevitable tibial torsion; or (6) a primary muscle defect.
recurrence of the cyst. However, it is quite feasible to Repeated dislocation damages the contiguous artic-
examine the meniscus by arthroscopy, remove only ular surfaces of the patella and femoral condyle; this
the torn or damaged portion and then decompress may result in further flattening of the condyle, so
the cyst from within the joint. The recurrence rate fol- facilitating further dislocations.
lowing such arthroscopic surgery is negligible Dislocation is almost always towards the lateral side;
(Parisien, 1990). medial dislocation is seen only in rare iatrogenic cases
following overzealous lateral release or medial trans-
position of the patellar tendon.
CHRONIC LIGAMENTOUS
INSTABILITY Clinical features
Girls are affected more commonly than boys and the
The knee is a complex hinge which depends heavily condition may be bilateral. Dislocation occurs unex-
on its ligaments for medio-lateral, anteroposterior and pectedly when the quadriceps muscle is contracted with
rotational stability. Ligament injuries, from minor the knee in flexion. There is acute pain, the knee is
strains through partial ruptures to complete tears, are stuck in flexion and the patient may fall to the ground.
common in sportsmen, athletes and dancers. What- Although the patella always dislocates laterally, the
ever the nature of the acute injury, the victim may be patient may think it has displaced medially because the
left with chronic instability of the knee – a sense of the uncovered medial femoral condyle stands out promi-
joint wanting to give way, or actually giving way, dur- nently. If the knee is seen while the patella is dislo-
562 ing unguarded activity. Sometimes this is accompa- cated, the diagnosis is obvious. There is a lump on the
20.23 Patello-femoral instability 20
(a,b) This young girl presented with
recurrent subluxation of the right
patella. The knee looks abnormal and
the x-ray shows the patella riding on
top of the lateral femoral condyle.
(c) The apprehension test: Watch the
The knee
patient’s face!
(a) (b) (c)
lateral side, while the front of the knee (where the episodes of dislocation and for these patients surgical
patella ought to be) is flat. The tissues on the medial reconstruction is indicated.
side are tender, the joint may be swollen and aspira-
tion may reveal a blood-stained effusion. OPERATIVE TREATMENT
More often the patella has reduced by the time the The principles of operative treatment are (a) to repair
patient is seen. Tenderness and swelling may still be or strengthen the medial patello-femoral ligaments,
present and the apprehension test is positive: if the and (b) to realign the extensor mechanism so as to
patella is pushed laterally with the knee slightly flexed, produce a mechanically more favourable angle of pull.
the patient resists and becomes anxious, fearing This can be achieved in several ways (see Fig. 20.24).
another dislocation. The patient will normally volun-
teer a history of previous dislocation. Direct medial patello-femoral ligament repair Occasion-
Between attacks the patient should be carefully ally it is possible to perform a direct repair of an atten-
examined for features that are known to predispose to uated medial patello-femoral ligament.
patellar instability (see above). Suprapatellar realignment (Insall) The lateral retinacu-
lum and capsule are divided. The quadriceps tendon
adjacent to the vastus medialis is split longitudinally to
Imaging the level of the tibial tubercle; the free edge is then
X-rays may reveal loose bodies in the knee, derived sutured over the middle of the patella, thus bringing
from old osteochondral fragments. A lateral view with vastus medialis distally and closer to the midline.
the knee in slight flexion may show a high-riding Infrapatellar soft-tissue realignment (Goldthwait) The lat-
patella and tangential views can be used to measure eral half of the patellar ligament is detached, threaded
the sulcus angle and the congruence angle. under the medial half and reattached more medially
MRI is helpful and may show signs of the previous and distally. This operation is seldom used by itself but
patello-femoral soft-tissue disruption. may be combined with suprapatellar realignment.
Infrapatellar bony realignment (Elmslie–Trillat) The tibial
Treatment tubercle is osteotomized and moved medially, thus
improving the angle of pull on the patella. This proce-
If the patella is still dislocated, it is pushed back into
dure is only appropriate after closure of the proximal
place while the knee is gently extended. The only indi-
tibial physis; if growth is incomplete, damage to the
cations for immediate surgery are (1) inability to
physis may result in a progressive recurvatum deformity.
reduce the patella (e.g. with a rare ‘intra-articular’ dis-
NOTE: All these procedures can be combined with
location), and (2) the presence of a large, displaced
repair or tightening of the medial patello-femoral lig-
osteochondral fragment.
ament. At the end of the operation it is essential to
A plaster cylinder or splint is applied and retained
check that the patella moves smoothly to at least 60
for 2–3 weeks; isometric quadriceps-strengthening
degrees of knee flexion; excessive tightening or
exercises are encouraged and the patient is allowed to
uneven tension may cause maltracking (and, occasion-
walk with the aid of crutches.
ally, even medial subluxation!) of the patella.
Exercises should be continued for at least 3
months, concentrating on strengthening the vastus Patellectomy Occasionally the patello-femoral carti-
medialis muscle. If recurrences are few and far lage is so damaged that patellectomy is indicated, but
between, conservative treatment may suffice; as the this operation should be avoided if possible. There is
child grows older the patellar mechanism tends to sta- a small risk that after patellectomy the patellar tendon
bilize. However, about 15 per cent of children with may continue to dislocate and require realignment by
patellar instability suffer repeated and distressing the tibial tubercle transfer. 563
20 20.24 Realignment for
recurrent patellar
dislocation There are
several methods popularly
used. Most involve a lateral
release of the capsule and
some form of ‘tether’
medially. This check-rein

may be created from
(a) vastus medialis (Insall),
(b) transposing the lateral
half of the patellar ligament
medially (Roux–Goldthwait)
or by (c) the semitendinosus
tendon (Galleazzi). (d) In
adults, bony operations
which shift the position of
the patellar tubercle may be
tried (Elmslie–Trillat).
(a) (b) (c) (d)
RECURRENT SUBLUXATION common among active adolescents and young adults.

It is often (but not invariably) associated with soften-
Patellar dislocation is sometimes followed by recurrent ing and fibrillation of the articular surface of the
subluxation rather than further episodes of complete patella – chondromalacia patellae. Having no other
displacement. This is the borderline between frank pathological label, orthopaedic surgeons have tended
instability and maltracking of the patella (see below). to regard chondromalacia as the cause (rather than
one of the effects) of the disorder. Against this are the
facts that (1) chondromalacia is commonly found at
OTHER TYPES OF NON-TRAUMATIC arthroscopy in young adults who have no anterior
DISLOCATION knee pain, and (2) some patients with the typical clin-
ical syndrome have no cartilage softening.
Congenital dislocation, in which the patella is perma-
nently displaced, is fortunately very rare. Reconstruc-
tive procedures, such as semitendinosus tenodesis,
Pathogenesis and pathology
have been tried but the results are unpredictable. Pain over the anterior aspect of the knee occurs as one
Habitual dislocation differs from recurrent disloca- of the symptoms in a number of well-recognized dis-
tion in that the patella dislocates every time the knee orders, the commonest of which are bursitis,
is bent and reduces each time it is straightened. In Osgood–Schlatter disease, a neuroma, plica syn-
longstanding cases the patella may be permanently dromes, patello-femoral arthritis and tendinitis affect-
dislocated. ing either the insertion of the quadriceps tendon or
The probable cause is contracture of the quadriceps, the patellar ligament – Sinding-Larsen’s disease.
which may be congenital or may result from repeated When these are excluded and no other cause can be
injections (usually antibiotics) into the muscle. found, one is left with a clinically recognizable syn-
Treatment requires lengthening of the quadriceps. drome that has earned the unsatisfactory label of
Additionally a lateral capsular release and medial pli- ‘anterior knee pain’ or ‘patello-femoral pain syn-
cation may be needed to hold the patella in the inter- drome’.
condylar groove. The basic disorder is probably mechanical overload
of the patello-femoral joint. Rarely, a single injury
(sudden impact on the front of the knee) may damage
the articular surfaces. Much more common is repeti-
PATELLO-FEMORAL PAIN tive overload due to either (1) malcongruence of the
SYNDROME (CHONDROMALACIA patello-femoral surfaces because of some abnormal
shape of the patella or intercondylar groove, (2)
OF THE PATELLA; PATELLO-
malalignment of the lower extremity and/or the patella,
FEMORAL OVERLOAD SYNDROME) (3) muscular imbalance of the lower extremity with
decreased strength due to atrophy or inhibition, or
There is no clear consensus concerning the terminol- relative weakness of the vastus medialis, which causes
ogy, aetiology and treatment of pain and tenderness in the patella to tilt, or subluxate, or bear more heavily
564 the anterior part of the knee. This syndrome is on one facet than the other during flexion and
Table 20.1 Causes of anterior knee pain neously (Bentley, 1985). It is not a precursor of pro- 20
gressive osteoarthritis in later life. Occasionally the
1. Referred from hip
lateral facet is involved – Ficat’s ‘hyperpression zone’
2. Patellofemoral disorders syndrome – and this may well be progressive (Ficat
Patellar instability and Hungerford, 1977).
Patello-femoral overload
Osteochondral injury
The knee
Patello-femoral osteoarthritis Clinical features
3. Knee joint disorders
Osteochondritis dissecans The patient, often a teenage girl or an athletic young
Loose body in the joint adult, complains of pain over the front of the knee or
Synovial chondromatosis ‘underneath the knee-cap’. Occasionally there is a his-
Plica syndrome tory of injury or recurrent displacement. Symptoms
4. Peri-articular disorders are aggravated by activity or climbing stairs, or when
Patellar tendinitis standing up after prolonged sitting. The knee may
Patellar ligament strain give way and occasionally swells. It sometimes
Bursitis
Osgood–Schlatter disease
‘catches’ but this is not true locking. Often both
knees are affected.
At first sight the knee looks normal but careful
extension, and (4) overactivity. ‘Overload’, as used examination may reveal malalignment or tilting of the
here, means either direct stress on a load-bearing facet patellae. Other signs include quadriceps wasting, fluid
or sheer stresses in the depths of the articular cartilage in the knee, tenderness under the edge of the patella
at the boundary between high-contact and low-con- and crepitus on moving the knee.
tact areas (Goodfellow et al., 1976). Personality and Patello-femoral pain is elicited by pressing the
chronic pain response issues must also be considered patella against the femur and asking the patient to
(Thomee et al., 1999). contract the quadriceps – first with central pressure,
Patello-femoral overload leads to changes in both then compressing the medial facet and then the lat-
the articular cartilage and the subchondral bone, not eral. If, in addition, the apprehension test is positive,
necessarily of parallel degree. Thus, the cartilage may this suggests previous subluxation or dislocation.
look normal and show only biochemical changes such Patellar tracking can be observed with the patient
as overhydration or loss of proteoglycans, while the seated on the edge of the couch, flexing and extend-
underlying bone shows reactive vascular congestion (a ing the knee against resistance; in some cases sublux-
potent cause of pain). Or there may be obvious carti- ation is obvious.
lage softening and fibrillation, with or without subar- With the patient sitting or lying supine, patellar
ticular intraosseous hypertension. This would account alignment can be gauged by measuring the quadriceps
for the variable relationship between (1) malalign- angle, or Q-angle – the angle subtended by the line of
ment syndrome, (2) cartilage softening, (3) subchon- quadriceps pull (a line running from the anterior
dral vascular congestion and (4) anterior knee pain. superior iliac spine to the middle of the patella) and
Cartilage fibrillation usually occurs on the medial the line of the patellar ligament. It normally averages
patellar facet or the median ridge, remains confined to 14–17 degrees and an angle of more than 20 degrees
the superficial zones and generally heals sponta- is regarded as a predisposing factor in the develop-
(a) (b) (c)

20.25 Chondromalacia of the patella There is no pathognomonic feature on which to base the diagnosis of
chondromalacia, but several signs are suggestive. (a) Hold the patella against the femoral condyles and ask the patient to
tighten the thigh muscles; even in normal people this may be uncomfortable, but patients with chondromalacia experience
sudden acute pain in the patello-femoral joint. (b) A ‘skyline’ x-ray with the knee in partial flexion may show obvious tilting
of the patella. (c) In the lateral x-ray, with the knee flexed to 45°, the lengths of the patella and the patellar ligament are
normally about equal (a ratio of 1:1); in patella alta the ratio is less than 1:1. 565
20 ment of anterior knee pain. Another predisposing fac- alignment and patello-femoral congruence and to
tor is a high-riding patella (patella alta); compressive reduce patello-femoral pressure. Various measures are
force on the patellar articular surface during flexion employed: lateral release, with or without one of the
and extension is likely to be greater than normal. realignment procedures illustrated in Figure 20.24,
Patella alta is best measured on the lateral x-ray). may be needed if there is any sign of patellar instabil-
Lastly, the structures around the knee should be ity; other operations are the patellar ligament eleva-
carefully examined for other sources of pain, and the tion procedure of Maquet and – as a last resort –
hip is examined to exclude referred pain. patellectomy.
Lateral release The lateral knee capsule and extensor
Imaging retinaculum are divided longitudinally, either open or
X-ray examination should include skyline views of the arthroscopically. This sometimes succeeds on its own
patella, which may show abnormal tilting or subluxa- (particularly if significant patellar tilting can be
tion, and a lateral view with the knee half-flexed to see demonstrated on x-ray or MRI), but more often
if the patella is high or small. patello-femoral realignment will be needed as well.
The most accurate way of showing and measuring Proximal realignment This is achieved by a combined
patello-femoral malposition is by CT or MRI with the open release of the lateral retinaculum and reefing of
knees in full extension and varying degrees of flexion. the oblique part of the vastus medialis.
Distal realignment The distal soft-tissue and bony
Arthroscopy realignment procedures are described on page 563.
Cartilage softening is common in asymptomatic They will improve the tracking angle but run the risk
knees, and painful knees may show no abnormality. of increasing patello-femoral contact pressures and
However, arthroscopy is useful in excluding other thus aggravating the patient’s symptoms.
causes of anterior knee pain; it can also serve to gauge
Distal elevation of the patellar ligament In Maquet’s
patello-femoral congruence, alignment and tracking.
(1976) tibial tubercle advancement operation the
tubercle, with the attached patellar ligament, is
Differential diagnosis hinged forwards and held there with a bone-block.
Other causes of anterior knee pain must be excluded This has the effect of reducing patello-femoral contact
before finally accepting the diagnosis of patello- pressures. Some patients resent the bump on the front
femoral pain syndrome (see Table 20.1). Even then, part of the tibia and the operation may substitute a
the exact cause of the syndrome must be established new set of complaints for the old. Alternatively, the
before treatment: e.g. is it abnormal posture, overuse, Fulkerson anteromedial tibial tubercle transfer and
patellar malalignment, subluxation or some abnormal- elevation can be used with satisfactory mid-term
ity in the shape of the bones? results.
Chondroplasty Shaving of the patellar articular surface
Treatment is usually performed arthroscopically using a power
tool. Soft and fibrillated cartilage is removed, in severe
CONSERVATIVE MANAGEMENT
cases down to the level of subchondral bone; the hope
In the vast majority of cases the patient will be helped
is that it will be replaced by fibrocartilage. The opera-
by adjustment of stressful activities and physiotherapy,
tion should be followed by lavage and can be com-
combined with reassurance that most patients eventu-
bined with any of the realignment procedures.
ally recover without physiotherapy. Exercises are
directed specifically at strengthening the medial Patellectomy This is a last resort, but patients with
quadriceps so as to counterbalance the tendency to severe discomfort are grateful for the relief it brings
lateral tilting or subluxation of the patella. Some after other operations have failed.
patients respond to simple measures such as providing
support for a valgus foot. Aspirin does no more than
reduce pain, and corticosteroid injections should be OSTEOCHONDRITIS DISSECANS
avoided.
The prevalence of osteochondritis dissecans is
OPERATIVE TREATMENT between 15 and 30 per 100 000 with males being
Surgery should be considered only if (1) there is a affected more often than females (ratio 5:3). An
demonstrable abnormality that is correctable by oper- increase in its incidence has been observed in recent
ation, or (2) conservative treatment has been tried for years, probably due to the growing participation of
at least 6 months and (3) the patient is genuinely inca- young children of both genders in competitive sports.
566 pacitated. Operation is intended to improve patellar A small, well-demarcated, avascular fragment of
bone and overlying cartilage sometimes separates 20
from one of the femoral condyles and appears as a
loose body in the joint. The most likely cause is
trauma, either a single impact with the edge of the
patella or repeated microtrauma from contact with an
adjacent tibial ridge. The fact that over 80 per cent of
The knee
lesions occur on the lateral part of the medial femoral
condyle, exactly where the patella makes contact in
full flexion, supports the first of these. There may also
be some general predisposing factor, because several
joints can be affected, or several members of one fam-
ily. Lesions are bilateral in 25 per cent of cases. (a) (b)
20.26 Osteochondritis dissecans – imaging The lesion

Pathology is often missed in the standard anteroposterior x-ray and is
better seen in the ‘tunnel view’, usually along the lateral
The lower, lateral surface of the medial femoral side of the medial femoral condyle (a). Here the
condyle is usually affected, rarely the lateral condyle, osteochondral fragment has remained in place but
and still more rarely the patella. An area of subchon- sometimes it appears as a separate body elsewhere in the
joint. (b) MRI provides confirmatory evidence and shows a
dral bone becomes avascular and within this area an much wider area of involvement than is apparent in the
ovoid osteocartilaginous segment is demarcated from plain x-ray.
the surrounding bone. At first the overlying cartilage
is intact and the fragment is stable; over a period of
months the fragment separates but remains in posi- the fragment has become detached, the empty hollow
tion; finally the fragment breaks free to become a may be seen – and possibly a loose body elsewhere in
loose body in the joint. The small crater is slowly filled the joint.
with fibrocartilage, leaving a depression on the articu- Radionuclide scans show increased activity around
lar surface. the lesion, and MRI consistently shows an area of low
signal intensity in the T1 weighted images; the adja-
Classification cent bone may also appear abnormal, probably due to
oedema. These investigations usually indicate whether
Osteochondritis dissecans of the knee is classified
the fragment is ‘stable’ or ‘loose’. MRI may also allow
according to anatomical location, arthroscopic
early prediction of whether the lesion will heal or not.
appearance, scintigraphic or MRI findings and
chronological age. For prognostic and treatment pur-
poses it is divided into juvenile and adult forms, either Arthroscopy
stable or unstable (Kocher et al., 2006).
With early lesions the articular surface looks intact,
but probing may reveal that the cartilage is soft. Loose
Clinical features segments are easily visualized.
The patient, usually a male aged 15–20 years, presents
with intermittent ache or swelling. Later, there are Differential diagnosis
attacks of giving way such that the knee feels unreli-
able; ‘locking’ sometimes occurs. Avascular necrosis of the femoral condyle – usually
The quadriceps muscle is wasted and there may be associated with corticosteroid therapy or alcohol
a small effusion. Soon after an attack there are two abuse – may result in separation of a localized osteo-
signs that are almost diagnostic: (1) tenderness local- cartilaginous fragment. However, it is seen in an older
ized to one femoral condyle; and (2) Wilson’s sign: if age group and on x-ray the lesion is always on the
the knee is flexed to 90 degrees, rotated medially and dome of the femoral condyle, and this distinguishes it
then gradually straightened, pain is felt; repeating the from osteochondritis dissecans.
test with the knee rotated laterally is painless.
Treatment
Imaging For the purposes of management, it is useful to ‘stage’
Plain x-rays may show a line of demarcation around a the lesion; hence the importance of radionuclide scan-
lesion in situ, usually in the lateral part of the medial ning, MRI and arthroscopy. Lesions in adults have a
femoral condyle. This site is best displayed in special greater propensity to instability whereas juvenile
intercondylar (tunnel) views, but even then a small osteochondritis is typically stable. Those lesions with
lesion or one situated far back may be missed. Once an intact articular surface have the greatest potential 567
20
(a) (b)
20.27 Osteochondritis dissecans Intraoperative pictures showing the articular lesion (a) and the defect left after removal
of the osteochondral fragment (b).
to heal with non-operative treatment if repetitive and the floor drilled before replacing the loose frag-
impact loading is avoided. ment and fixing it with Herbert screws. If the frag-
In the earliest stage, when the cartilage is intact and ment is in pieces or ill-shaped, it is best discarded; the
the lesion is ‘stable’, no treatment is needed but activ- crater is drilled and allowed to fill with fibrocartilage.
ities are curtailed for 6–12 months. Small lesions often In recent years attempts have been made to fill the
heal spontaneously. residual defects by articular cartilage transplantation:
If the fragment is ‘unstable’, i.e. surrounded by a either the insertion of osteochondral plugs harvested
clear boundary with radiographic ‘sclerosis’ of the from another part of the knee or the application of
underlying bone, or showing MRI features of separa- sheets of cultured chondrocytes. This approach
tion, treatment will depend on the size of the lesion. should still be regarded as in the ‘experimental’ stage.
A small fragment should be removed by arthroscopy After any of the above operations the knee is held
and the base drilled; the bed will eventually be cov- in a cast for 6 weeks; thereafter movement is encour-
ered by fibrocartilage, leaving only a small defect. A aged but weightbearing is deferred until x-rays show
large fragment (say more than 1 cm in diameter) signs of healing.
should be fixed in situ with pins or Herbert screws. In
addition, it may help to drill the underlying sclerotic
bone to promote union of the necrotic fragment. For LOOSE BODIES
drilling, the area is approached from a point some dis-
tance away, beyond the articular cartilage. The knee – relatively capacious, with large synovial
If the fragment is completely detached but in one piece folds – is a common haven for loose bodies. These
and shown to fit nicely in its bed, the crater is cleaned may be produced by: (1) injury (a chip of bone or
(a) (b) (c)
20.28 Loose bodies (a) This loose body slipped away from the fingers when touched; the term ‘joint mouse’ seems
appropriate. (b) Which is the loose body here? Not the large one (which is a normal fabella), but the small lower one
opposite the joint line. (c) Multiple loose bodies are seen in synovial chondromatosis, a rare disorder of cartilage metaplasia
568 in the synovium.
cartilage); (2) osteochondritis dissecans (which may 20
produce one or two fragments); (3) osteoarthritis
THE PLICA SYNDROME
(pieces of cartilage or osteophyte); (4) Charcot’s dis-
ease (large osteocartilaginous bodies); and (5) syn- A plica is the remnant of an embryonic synovial parti-
ovial chondromatosis (cartilage metaplasia in the tion which persists into adult life. During develop-
synovium, sometimes producing hundreds of loose ment of the embryo, the knee is divided into three
The knee
bodies). cavities – a large suprapatellar pouch and beneath this
the medial and lateral compartments – separated from
each other by membranous septa. Later these parti-
Clinical features tions disappear, leaving a single cavity, but part of a
Loose bodies may be symptomless. The usual com- septum may persist as a synovial pleat or plica (from
plaint is attacks of sudden locking without injury. the Latin plicare = fold). This is found in over 20 per
The joint gets stuck in a position which varies from cent of people, usually as a median infrapatellar fold
one attack to another. Sometimes the locking is only (the ligamentum mucosum), less often as a suprap-
momentary and usually the patient can wriggle the atellar curtain draped across the opening of the
knee until it suddenly unlocks. The patient may be suprapatellar pouch or a mediopatellar plica sweeping
aware of something ‘popping in and out of the down the medial wall of the joint.
joint’.
In adolescents, a loose body is usually due to osteo-
chondritis dissecans, rarely to injury. In adults Pathology
osteoarthritis is the most frequent cause. The plica in itself is not pathological. But if acute
Only rarely is the patient seen with the knee still trauma, repetitive strain or some underlying disorder
locked. Sometimes, especially after the first attack, (e.g. a meniscal tear) causes inflammation, the plica
there is synovitis or there may be evidence of the may become oedematous, thickened and eventually
underlying cause. A pedunculated loose body may be fibrosed; it then acts as a tight bowstring impinging
felt; one that is truly loose tends to slip away during on other structures in the joint and causing further
palpation (the well-named ‘joint mouse’). synovial irritation.
X-ray Most loose bodies are radio-opaque. The films
also show an underlying joint abnormality.
Clinical features
Treatment An adolescent or young adult complains of an ache in
the front of the knee (occasionally both knees), with
A loose body causing symptoms should be removed
intermittent episodes of clicking or ‘giving way’.
unless the joint is severely osteoarthritic. This can usu-
There may be a history of trauma or markedly
ally be done through the arthroscope, but finding the
increased activity. Symptoms are aggravated by exer-
loose body may be difficult; it may be concealed in a
cise or climbing stairs, especially if this follows a long
synovial pouch or sulcus and a small body may even
period of sitting.
slip under the edge of one of the menisci.
On examination there may be muscle wasting and a
small effusion. The most characteristic feature is ten-
derness near the upper pole of the patella and over the
SYNOVIAL CHONDROMATOSIS femoral condyle. Occasionally the thickened band can
be felt. Movement of the knee may cause catching or
This is a rare disorder in which the joint comes to con- snapping.
tain multiple loose bodies, often in pearly clumps resem-
bling sago (‘snowstorm knee’). The usual explanation is
that myriad tiny fronds undergo cartilage metaplasia at Diagnosis
their tips; these tips break free and may ossify. It has,
There is still controversy as to whether ‘plica syndrome’
however, been suggested that chondrocytes may be cul-
constitutes a real and distinct clinical entity. In some
tured in the synovial fluid and that some of the products
quarters, however, it is regarded as a significant cause of
are then deposited onto previously normal synovium, so
anterior knee pain. It may closely resemble other con-
producing the familiar appearance (Kay et al., 1989).
ditions such as patellar overload or subluxation; indeed,
X-rays reveal multiple loose bodies; on arthrography
the plica may become troublesome only when those
they show as negative defects.
other conditions are present. The diagnosis is often not
Treatment The loose bodies should be removed made until arthroscopy is undertaken. The presence of
arthroscopically. At the same time an attempt should a chondral lesion on the femoral condyle secondary to
be made to remove all abnormal synovium. plica impingement confirms the diagnosis. 569
20 20.29 Tuberculosis (a) Lateral views
of the two knees. On one side the
bones are porotic and the epiphyses
enlarged, features suggestive of a
severe inflammatory synovitis. (b) Later
the articular surfaces are eroded.
(a) (b)
Treatment because cartilage lysis is prevented by the presence of

a plasmin inhibitor in the synovial exudate.
The first line of treatment is rest, anti-inflammatory
drugs and adjustment of activities. If symptoms per- Late features If the disease is allowed to persist the
sist, the plica can be divided or excised by arthroscopy. joint surfaces will gradually be eroded and the knee
joint will become deformed. The classical picture in
neglected cases is a composite deformity: posterior
and lateral subluxation or dislocation, external rota-
TUBERCULOSIS tion and fixed flexion.
Tuberculosis of the knee may appear at any age, but it Diagnosis

is more common in children than in adults. Monarticular rheumatoid synovitis, or juvenile
chronic arthritis, may closely resemble tuberculosis. A
Clinical features synovial biopsy may be necessary to establish the diag-
nosis.
Early presentation Pain and limp are early symptoms;
or the child may present with a swollen joint and a
low-grade fever. The thigh muscles are wasted, thus
Treatment
accentuating the joint swelling. The knee feels warm General antituberculous chemotherapy should be
and there is synovial thickening. Movements are given for 12–18 months (see page 49).
restricted and often painful. The Mantoux test is pos- In the active stage the knee is rested in a bed splint.
itive and the erythrocyte sedimentation rate (ESR) The synovitis usually subsides, but if it does not do so
may be increased. after a few weeks’ treatment, then surgical debride-
X-rays show marked osteoporosis and, in children, ment will be needed. All obviously diseased and
enlargement of the bony epiphyses. Unlike pyogenic necrotic tissue is removed and bone abscesses are
arthritis, joint space narrowing is a late sign; this is evacuated.
(a) (b) (c) (d)
20.30 Rheumatoid arthritis (a) Patient with rheumatoid arthritis showing the typical valgus deformity of the right knee;
the feet and toes also are affected. (b) X-ray showing marked erosive arthritis resulting in joint deformity. (c) This patient
presented with a painful swelling of the left calf. She was thought at first to have developed deep vein thrombosis – until
570 we examined her knee and recognized this as a posterior synovial rupture, later confirmed by arthrography (d).
In the healing stage the patient is allowed up wear- and the muscles are reasonably strong, there is a dan- 20
ing a weight-relieving caliper. Gradually this is left off, ger of rupturing the posterior capsule; the joint con-
but the patient is kept under observation for any sign tents are extruded into a large posterior bursa or
of recurrent inflammation. If the articular cartilage between the muscle planes of the calf, causing sudden
has been spared, movement can be encouraged and pain and swelling which closely mimic the features of
weightbearing is slowly resumed. However, if the calf vein thrombosis.
The knee
articular surface is destroyed, immobilization is con- As the disease progresses the knee becomes increas-
tinued until the joint stiffens. ingly unstable, muscle wasting is marked and there is
In the aftermath the joint may be painful; it is then some loss of flexion and extension.
best arthrodesed, but in children this is usually post- X-rays may show diminution of the joint space,
poned until growth is almost completed. The ideal osteopaenia and marginal erosions. The picture is eas-
position for fusion is 10–15 degrees of flexion, 7 ily distinguishable from that of osteoarthritis by the
degrees of valgus and 5 degrees of external rotation. complete absence of osteophytes.
In some cases, once it is certain that the disease is In the late stage pain and disability are usually
quiescent, joint replacement may be feasible. severe. In some patients stiffness is so marked that the
patient has to be helped to stand and the joint has
only a jog of painful movement. In others, cartilage
and bone destruction predominate and the joint
RHEUMATOID ARTHRITIS becomes increasingly unstable and deformed, usually
in fixed flexion and valgus. X-rays reveal the bone
Occasionally, rheumatoid arthritis starts in the knee as destruction characteristic of advanced disease.
a chronic monarticular synovitis. Sooner or later,
however, other joints become involved.
Treatment
The majority of patients can be managed by conser-
Clinical features vative measures. In addition to general treatment with
The general features of rheumatoid disease are anti-inflammatory and disease-modifying drugs, local
described in Chapter 3. splintage and injection of triamcinolone usually help
The early stage is characterized by synovitis; to reduce the synovitis. A more prolonged effect may
rheumatoid disease occasionally starts with involve- be obtained by injecting radiocolloids such as yttrium-
ment of a single joint. The patient complains of pain 90 (90Y).
and chronic swelling of the knee; there is usually an
effusion and the thigh muscles may be wasted. The OPERATIVE TREATMENT
thickened synovium is often palpable. Synovectomy and debridement Only if other measures
Unless there are obvious signs of an inflammatory fail to control the synovitis (which nowadays is rare) is
polyarthritis, the condition has to be distinguished synovectomy indicated. This can be done very effec-
from other types of inflammatory monarthritis, such tively by arthroscopy. Articular pannus and cartilage
as gout, Reiter’s disease and tuberculosis; biopsy and tags are removed at the same time. Postoperatively,
microbiological investigations may be needed. any haematoma must be drained and movements are
During this early stage, while the joint is still stable commenced as soon as pain has subsided.
(a) (b) (c) (d)
20.31 Osteoarthritis (a,b) Varus deformity of the left knee suggesting loss of cartilage thickness in the medial
compartment. X-ray shows diminished joint space and peripheral osteophytes on the medial side of the knee.
(c) Sometimes it is the patello-femoral joint that is mainly affected. (d) Patello-femoral osteoarthritis with long trailing
osteophytes is typical of calcium pyrophosphate arthropathy.
571
20 changes are most marked in the medial compartment.
The characteristic features of cartilage fibrillation,
sclerosis of the subchondral bone and peripheral
osteophyte formation are usually present; in advanced
cases the articular surface may be denuded of cartilage
and underlying bone may eventually crumble.
Chondrocalcinosis is common, but whether this is

cause or effect – or quite unrelated – remains
(a) (b)
unknown.
Clinical features
Patients are usually over 50 years old; they tend to be
overweight and may have longstanding bow-leg
deformity.
Pain is the leading symptom, worse after use, or (if
(c) (d) the patello-femoral joint is affected) on stairs. After
rest, the joint feels stiff and it hurts to ‘get going’ after
20.32 Osteoarthritis – x-rays Always obtain weightbearing sitting for any length of time. Swelling is common,
views of the knees. X-rays taken with the patient lying down
(a,b) suggest only minor cartilage loss on the medial side of
and giving way or locking may occur.
each knee. (c,d) Weightbearing views show the true On examination there may be an obvious deformity
position: there is severe loss of articular cartilage. (usually varus) or the scar of a previous operation. The
quadriceps muscle is usually wasted.
Except during an exacerbation, there is little fluid
Supracondylar osteotomy Realignment osteotomy is and no warmth; nor is the synovial membrane thick-
unlikely to have any protective effect in a disease ened. Movement is somewhat limited and is often
which is marked by generalized cartilage erosion. accompanied by patello-femoral crepitus.
However, if the knee is stable and pain-free but trou- It is useful to test movement applying first a varus
blesome because of valgus and flexion deformity, a and then a valgus force to the knee; pain indicates
corrective supracondylar osteotomy is useful. which tibio-femoral compartment is involved. Pres-
Arthroplasty Total joint replacement is useful when joint sure on the patella may elicit pain.
destruction is advanced. However, it is less successful if The natural history of osteoarthritis is one of alter-
the knee has been allowed to become very unstable or nating ‘bad spells’ and ‘good spells’. Patients may
very stiff; timing of the operation is important. experience long periods of lesser discomfort and only
moderate loss of function, followed by exacerbations
of pain and stiffness (perhaps after unaccustomed
OSTEOARTHRITIS activity).
The knee is the commonest of the large joints to be X-ray

affected by osteoarthritis (see Chapter 5). Often there
is a predisposing factor: injury to the articular surface, The anteroposterior x-ray must be obtained with the
a torn meniscus, ligamentous instability or pre- patient standing and bearing weight; only in this way
existing deformity of the hip or knee, to mention a can small degrees of articular cartilage thinning be
few. However, in many cases no obvious cause can be revealed. The tibio-femoral joint space is diminished
found. Underlying all of these, there may also be a (often only in one compartment) and there is sub-
genetic component. Curiously, while the male:female chondral sclerosis. Osteophytes and subchondral cysts
distribution is more or less equal in white (Caucasian) are usually present and sometimes there is soft-tissue
peoples, black African women are affected far more calcification in the suprapatellar region or in the joint
frequently than their male counterparts. itself (chondrocalcinosis).
Osteoarthritis is often bilateral and in these cases If only the patello-femoral joint is affected, suspect
there is a strong association with Heberden’s nodes a pyrophosphate arthropathy.
and generalized osteoarthritis.
Treatment
Pathology
If symptoms are not severe, treatment is conservative.
Cartilage breakdown usually starts in an area of Joint loading is lessened by using a walking stick.
572 excessive loading. Thus, with longstanding varus the Quadriceps exercises are important. Analgesics are
prescribed for pain, and warmth (e.g. radiant heat or site is the dome of one of the femoral condyles, but 20
shortwave diathermy) is soothing. A simple elastic occasionally the medial tibial condyle is affected. Two
support may do wonders, probably by improving pro- main categories are identified: (1) osteonecrosis associ-
prioception in an unstable knee. ated with a definite background disorder [e.g. corticos-
Intra-articular corticosteroid injections will often teroid therapy, alcohol abuse, sickle-cell disease,
relieve pain, but this is a stopgap, and not a very good hyperbaric decompression sickness, systemic lupus
The knee
one, because repeated injections may permit (or even erythematosus (SLE) or Gaucher’s disease], and (2)
predispose to) progressive cartilage and bone destruc- ‘spontaneous’ osteonecrosis of the knee, popularly
tion. known by the acronym SONK, which is due to a small
New forms of medication have been introduced insufficiency fracture of a prominent part of the
in recent years, particularly the oral administration osteoarticular surface in osteoporotic bone; the vascu-
of glucosamine and intra-articular injection of lar supply to the free fragment is compromised
hyalourans. There is, as yet, no agreement about the (Yamamoto and Bullough, 2000).
long-term efficacy of these products. A third type, postmeniscectomy osteonecrosis, has been
reported; its prevalence and pathophysiology are still
OPERATIVE TREATMENT
unclear (Patel et al., 1998).
Persistent pain unresponsive to conservative treat-
ment, progressive deformity and instability are the
usual indications for operative treatment.
Clinical features
Arthroscopic washouts, with trimming of degenerate
meniscal tissue and osteophytes, may give temporary Patients are usually over 60 years old and women are
relief; this is a useful measure when there are con- affected three times more often than men. Typically
traindications to reconstructive surgery. they give a history of sudden, acute pain on the
Patellectomy is indicated only in those rare cases medial side of the joint. Pain at rest also is common.
where osteoarthritis is strictly confined to the patello- On examination there is usually a small effusion,
femoral joint. However, bear in mind that extensor but the classic feature is tenderness on pressure upon
power will be reduced and if a total joint replacement the medial femoral or tibial condyle rather than along
is later needed pain relief will be less predictable than the joint line proper.
usual (Paletta and Laskin, 1995). The patient may offer a history of similar symptoms
Realignment osteotomy is often successful in reliev- in the hip or the shoulder. Whether or not this is the
ing symptoms and staving off the need for ‘end-stage’ case, those joints should be examined as well.
surgery. The ideal indication is a ‘young’ patient
(under 50 years) with a varus knee and osteoarthritis
confined to the medial compartment: a high tibial val- Imaging
gus osteotomy will redistribute weight to the lateral
X-ray The x-ray appearances are often unimpressive
side of the joint. The degree and accuracy of angular
at the beginning, but a radionuclide scan may show
correction are the most important determinants of
increased activity on the medial side of the joint. Later
mid- and long-term clinical outcome.
the classic radiographic features of osteonecrosis
Replacement arthroplasty is indicated in older
appear (see Chapter 6). On the femoral side, it is
patients with progressive joint destruction. This is
always the dome of the condyle that is affected, unlike
usually a ‘resurfacing’ procedure, with a metal femoral
the picture in osteochondritis dissecans.
condylar component and a metal-backed polyethylene
table on the tibial side. If the disease is largely con- Magnetic resonance imaging MRI enhances the ability
fined to one compartment, a unicompartmental to visualize bone marrow and to separate necrotic
replacement can be done as an alternative to from viable areas with a high level of specificity. It
osteotomy. With modern techniques, and meticulous shows the area of reactive bone surrounding the
attention to anatomical alignment of the knee, the osteonecrotic lesion and can demonstrate the integrity
results of replacement arthroplasty are excellent. of the overlying cortical shell of bone and articular
Arthrodesis is indicated only if there is a strong con- cartilage. It is also helpful in determining prognosis
traindication to arthroplasty (e.g. previous infection) concerning the natural course of the condition.
or to salvage a failed arthroplasty.
OSTEONECROSIS
Once the diagnosis is confirmed, investigations
Osteonecrosis of the knee, though not as common as should be carried out to exclude generalized disorders
femoral head necrosis, has the same aetiological and known to be associated with osteonecrosis (see
pathogenetic background (see Chapter 6). The usual Chapter 6). 573
20 Differential diagnosis drilling with or without bone grafting, core decom-
pression of the femoral condyle at a distance from the
Osteonecrosis of the knee should be distinguished lesion, and (for patients with persistent symptoms and
from osteochondritis dissecans, though in truth the well-marked articular surface damage) a valgus
two conditions are closely related; however, the age osteotomy or unicompartmental arthroplasty. Resur-
group, aetiology, site of the lesion and prognosis are facing with osteochondral allografts has also been
different and these factors may influence treatment. employed, with variable results.
Other conditions that have a sudden, painful onset
and tenderness at the joint line are fracture of an
osteoarthritic osteophyte, disruption of a degenerative
meniscus, a stress fracture, pes anserinus bursitis and a CHARCOT’S DISEASE
local tendonitis.
Charcot’s disease (neuropathic arthritis) is a rare cause
Prognosis of joint destruction. Because of loss of pain sensibility
and proprioception, the articular surface breaks down
Symptoms and signs may stabilize and the patient be and the underlying bone crumbles. Fragments of
left with no more than slight distortion of the articu- bone and cartilage are deposited in the hypertrophic
lar surface; or one of the condyles may collapse, lead- synovium and may grow into large masses. The cap-
ing to osteoarthritis of the affected compartment. sule is stretched and lax, and the joint becomes pro-
The clinical progress depends on the radiographic gressively unstable.
size of the lesion, the ratio of size of the lesion to the
size of the condyle (>40 per cent carries a worse
prognosis) and the stage of the lesion (Patel et al., Clinical features
1998). The patient chiefly complains of instability; pain
(other than tabetic lightning pains) is unusual. The
Treatment joint is swollen and often grossly deformed. It feels
like a bag of bones and fluid but is neither warm nor
Treatment is conservative in the first instance and tender. Movements beyond the normal limits, with-
consists of measures to reduce loading of the joint and out pain, are a notable feature. Radiologically the
analgesics for pain. If symptoms or signs increase, joint is subluxated, bone destruction is obvious and
operative treatment may be considered. irregular calcified masses can be seen.
Surgical options include arthroscopic debridement,
Treatment
Patients often seem to manage quite well despite the
bizarre appearances. However, marked instability may
demand treatment – usually a moulded splint or
caliper will do – and occasionally pain becomes intol-
erable. Arthrodesis is feasible but fixation is difficult
and fusion is very slow. Replacement arthroplasty is
not indicated.
HAEMOPHILIC ARTHRITIS
The knee is the joint most commonly involved in

bleeding disorders. Repeated haemorrhage leads to
(a) (b) chronic synovitis and articular cartilage erosion.
20.33 Osteonecrosis (a) X-ray showing the typical Movement is progressively restricted and the joint
features of subarticular bone fragmentation and may end up deformed and stiff.
surrounding sclerosis situated in the highest part (the
dome) of the medial femoral condyle. (In osteochondritis
dissecans, the necrotic segment is almost always on the Clinical features
lateral surface of the medial femoral condyle.) (b) In this
case the medial compartment was ‘unloaded’ by Fresh bleeds cause pain and swelling of the knee, with
performing a high tibial valgus osteotomy. The patient the typical clinical signs of a haemarthrosis (see Chap-
574 remained pain-free for 6 years before dying of leukaemia. ter 5). Between episodes of bleeding the knee often
20
The knee
(a) (b) (c) (d) (e)
20.34 Extensor mechanism lesions These follow resisted action of the quadriceps; they usually occur at a progressively
higher level with increasing age (a). (b) Osgood-Schlatter’s disease – the only one that usually does not follow a definite
accident; (c) gap fracture of patella; (d) ruptured quadriceps tendon (note the suprapatellar depression); (e) ruptured rectus
femoris causing a lump with a hollow below.
continues to be painful and somewhat swollen, with age. In the elderly the injury is usually above the
restricted mobility. There is a tendency to hold the patella; in middle life the patella fractures; in young
knee in flexion and this may become a fixed deformity. adults the patellar ligament can rupture. In adoles-
cents the upper tibial apophysis is occasionally
X-rays Radiographic examination may show little
avulsed; much more often it is merely ‘strained’.
abnormality, apart from local osteoporosis. In more
Tendon rupture sometimes occurs with minimal
advanced cases the joint space is narrowed and large
strain; this is seen in patients with connective tissue
‘cysts’ or erosions may appear in the subchondral
disorders (e.g. SLE) and advanced rheumatoid dis-
bone.
ease, especially if they are also being treated with cor-
ticosteroids.
Treatment
Both the haematologist and the orthopaedic surgeon
should participate in treatment. The acute bleed may RUPTURE ABOVE THE PATELLA
need aspiration, but only if this can be ‘covered’ by
giving the appropriate clotting factor; otherwise it is Rupture may occur in the belly of the rectus femoris.
better treated by splintage until the acute symptoms The patient is usually elderly, or on long-term corti-
settle down. costeroid treatment. The torn muscle retracts and
Flexion deformity must be prevented by gentle forms a characteristic lump in the thigh. Function is
physiotherapy and intermittent splintage. If the joint usually good, so no treatment is required.
is painful and eroded, operative treatment may be Avulsion of the quadriceps tendon from the upper
considered. However, although replacement arthro- pole of the patella is seen in the same group of peo-
plasty is feasible, this should be done only after the ple. Sometimes it is bilateral. Operative repair is essen-
most searching discussion with the patient, where all tial.
the risks are considered, and only if a full haemato-
logical service is available.
RUPTURE BELOW THE PATELLA
This occurs mainly in young people. The ligament
may rupture or may be avulsed from the lower pole of
RUPTURES OF THE EXTENSOR the patella. Operative repair is necessary. Pain and ten-
APPARATUS derness in the middle portion of the patellar ligament
may occur in athletes; CT or ultrasonography will
Resisted extension of the knee may tear the extensor reveal an abnormal area. If rest fails to provide relief
mechanism. The patient stumbles on a stair, catches the paratenon should be stripped (King et al., 1990).
his or her foot while walking or running, or may only Partial rupture or avulsion sometimes leads to a
be kicking a muddy football. In all these incidents, traction tendinitis and calcification in the patellar lig-
active knee extension is prevented by an obstacle. The ament – the Sinding–Larsen Johansson syndrome (see
precise location of the lesion varies with the patient’s below). 575
20 PELLEGRINI–STIEDA DISEASE
X-rays sometimes show a plaque of bone lying next to
the femoral condyle under the medial collateral liga-
ment. Occasionally this is a source of pain. It is gener-
ally ascribed to ossification of a haematoma following
a tear of the medial ligament, though a history of injury

is not always forthcoming. Treatment is rarely needed.
PATELLAR ‘TENDINOPATHY’ (SINDING–

(a) (b)
LARSEN JOHANSSON SYNDROME).
20.35 Osgood–Schlatter’s disease This boy complained This condition was described independently by Sind-
of a painful bump below the knee. X-ray shows the ing-Larsen in 1921 and Johansson in 1922. Following
traction injury of the tibial apophysis. a strain or partial rupture of the patellar ligament the
patient (usually a young athletic individual) develops
a traction ‘tendinitis’ characterised by pain and point
tenderness at the lower pole of the patella. Some-
times, if the condition does not settle, calcification
OSGOOD–SCHLATTER DISEASE appears in the ligament (Medlar and Lyne, 1978). CT
(‘APOPHYSITIS’ OF THE TIBIAL TUBERCLE) or ultrasonography may reveal the abnormal area in
the ligament. A similar disorder has been described at
In this common disorder of adolescence the tibial the proximal pole of the patella.
tubercle becomes painful and ‘swollen’. Although The condition is comparable to Osgood-Schlatter’s
often called osteochondritis or apophysitis, it is noth- disease and usually recovers spontaneously. If rest fails to
ing more than a traction injury of the apophysis into provide relief, the abnormal area is removed and the
which part of the patellar tendon is inserted (the paratenon stripped (King et al., 1990; Khan et al., 1998).
remainder is inserted on each side of the apophysis
and prevents complete separation).
There is no history of injury and sometimes the
condition is bilateral. A young adolescent complains SWELLINGS OF THE KNEE
of pain after activity, and of a lump. The lump is ten-
der and its situation over the tibial tuberosity is diag- The knee is prone to a number of disorders which
nostic. Sometimes active extension of the knee against present essentially as ‘swelling’; and, because it is such
resistance is painful and x-rays may reveal fragmenta- a large joint with a number of synovial recesses, the
tion of the apophysis. swelling is often painless until the tissues become tense.
Spontaneous recovery is usual but takes time, and it Conditions to be considered can be divided into four
is wise to restrict such activities as cycling, jumping groups: swelling of the entire joint; swellings in front of
and soccer. Occasionally, symptoms persist and, if the joint; swellings behind the joint; and bony swellings.
patience or wearing a back-splint during the day are
unavailing, a separate ossicle in the tendon is usually
responsible; its removal is then worthwhile. ACUTE SWELLING OF THE ENTIRE JOINT
POST-TRAUMATIC HAEMARTHROSIS
Swelling immediately after injury means blood in the
TENDINITIS AND CALCIFICATION joint. The knee is very painful and it feels warm, tense
AROUND THE KNEE and tender. Later there may be a ‘doughy’ feel. Move-
ments are restricted. X-rays are essential to see if there
is a fracture; if there is not, then suspect a tear of the
CALCIFICATION IN THE MEDIAL anterior cruciate ligament.
LIGAMENT The joint should be aspirated under aseptic conditions.
If a ligament injury is suspected, examination under anaes-
Acute pain in the medial collateral ligament may be thesia is helpful and may indicate the need for operation;
due to a soft calcific deposit among the fibres of the otherwise a crepe bandage is applied and the leg cradled
ligament. There may be a small, exquisitely tender in a back-splint. Quadriceps exercises are practised from
lump in the line of the ligament. Pain is dramatically the start. The patient may get up when comfortable, re-
576 relieved by operative evacuation of the deposit. taining the back-splint until muscle control returns.
20
The knee
(a) (b)
(c) (d) (e) (f)
20.36 Swollen knees Some causes of chronic swelling in the absence of trauma: (a) tuberculous arthritis; (b) rheumatoid
arthritis; (c) Charcot’s disease; (d) villous synovitis; (e) haemophilia; (f) malignant synovioma.
BLEEDING DISORDERS ASEPTIC NON-TRAUMATIC SYNOVITIS

In patients with clotting disorders, the knee is the most Acute swelling, without a history of trauma or signs of
common site for acute bleeds. If the appropriate clot- infection, suggests gout or pseudogout. Aspiration will
ting factor is available, the joint should be aspirated and provide fluid which may look turbid, resembling pus,
treated as for a traumatic haemarthrosis. If the factor is but it is sterile and microscopy (using polarized light)
not available, aspiration is best avoided; the knee is reveals the crystals. Treatment with anti-inflammatory
splinted in slight flexion until the swelling subsides. drugs is usually effective.
ACUTE SEPTIC ARTHRITIS

Acute pyogenic infection of the knee is not uncommon.
The organism is usually Staphylococcus aureus, but in CHRONIC SWELLING OF THE JOINT
adults gonococcal infection is almost as common.
The joint is swollen, painful and inflamed; the white The diagnosis can usually be made on clinical and
cell count and ESR are elevated. Aspiration reveals x-ray examination. The more elusive disorders should
pus in the joint; fluid should be sent for bacteriologi- be fully investigated by joint aspiration, synovial fluid
cal investigation, including anaerobic culture. examination, arthroscopy and synovial biopsy.
Treatment consists of systemic antibiotics and
drainage of the joint – ideally by arthroscopy, with ARTHRITIS
irrigation and complete synovectomy; if fluid reaccu- The commonest causes of chronic swelling are
mulates, it can be aspirated through a wide-bore nee- osteoarthritis and rheumatoid arthritis. Other signs,
dle. As the inflammation subsides, movement is such as deformity, loss of movement or instability,
begun, but weightbearing is deferred for 4–6 weeks. may be present and x-ray examination will usually
show characteristic features.
TRAUMATIC SYNOVITIS
Injury stimulates a reactive synovitis; typically the SYNOVIAL DISORDERS
swelling appears only after some hours, and subsides Chronic swelling and synovial effusion without artic-
spontaneously over a period of days. There is inhibi- ular destruction should suggest conditions such as
tion of quadriceps action and the thigh wastes. The synovial chondromatosis and pigmented villonodular
knee may need to be splinted for several days but synovitis. The diagnosis will usually be obvious on
movement should be encouraged and quadriceps arthroscopy and can be confirmed by synovial biopsy.
exercise is essential. If the amount of fluid is consider- The most important condition to exclude is tuber-
able, its aspiration hastens muscle recovery. In addi- culosis. There has been a resurgence of cases during the
tion, any internal injury will need treatment. last ten years and the condition should be seriously 577
20
20.37 Lumps around the knee In front:

(a) prepatellar bursa; (b) infrapatellar bursa;
(c) Osgood–Schlatter disease.

(a) (b) (c)
On either side: (d) cyst of lateral meniscus;

(e) cyst of medial meniscus; (f) cartilage-
capped exostosis.
(d) (e) (f)
Behind: (g) semimembranosus bursa;

(h) arthrogram of popliteal cyst;
(i) leaking cyst.
(g) (h) (i)
considered whenever there is no obvious alternative SWELLINGS AT THE BACK OF THE KNEE
diagnosis. Investigations should include Mantoux test-
ing and synovial biopsy. The ideal is to start antituber- SEMIMEMBRANOSUS BURSA
culous chemotherapy before joint destruction occurs. The bursa between the semimembranosus and the
medial head of gastrocnemius may become enlarged
SWELLINGS IN FRONT OF THE JOINT in children or adults. It presents usually as a painless
lump behind the knee, slightly to the medial side of
PREPATELLAR BURSITIS (‘HOUSEMAID’S KNEE’) the midline and most conspicuous with the knee
The fluctuant swelling is confined to the front of the straight. The lump is fluctuant but the fluid cannot be
patella and the joint itself is normal. This is an unin- pushed into the joint, presumably because the muscles
fected bursitis due not to pressure but to constant compress and obstruct the normal communication.
friction between skin and bone. It is seen mainly in The knee joint is normal. Occasionally the lump
carpet layers, paving workers, floor cleaners and min- aches, and if so it may be excised through a transverse
ers who do not use protective knee pads. Treatment incision. However, recurrence is common and, as the
consists of firm bandaging, and kneeling is avoided; bursa normally disappears in time, a waiting policy is
occasionally aspiration is needed. In chronic cases the perhaps wiser.
lump is best excised.
Infection (possibly due to foreign body implantation) POPLITEAL ‘CYST’
results in a warm, tender swelling. Treatment is by rest, Bulging of the posterior capsule and synovial hernia-
antibiotics and, if necessary, aspiration or excision. tion may produce a swelling in the popliteal fossa. The
lump, which is usually seen in older people, is in the
INFRAPATELLAR BURSITIS (‘CLERGYMAN’S KNEE’) midline of the limb and at or below the level of the
The swelling is below the patella and superficial to the joint. It fluctuates but is not tender. Injection of
patellar ligament, being more distally placed than radio-opaque medium into the joint, and x-ray, will
prepatellar bursitis; it used to be said that one who show that the ‘cyst’ communicates with the joint.
prays kneels more uprightly than one who scrubs! The condition was originally described by Baker,
Treatment is similar to that for prepatellar bursitis. whose patients were probably suffering from tubercu-
Occasionally the bursa is affected in gout. lous synovitis. Nowadays it is more likely to be caused
by rheumatoid or osteoarthritis, but it is still often
OTHER BURSAE called a ‘Baker’s cyst’. Occasionally the ‘cyst’ ruptures
Occasionally a bursa deep to the patellar tendon or and the synovial contents spill into the muscle planes
the pes anserinus becomes inflamed and painful. causing pain and swelling in the calf – a combination
578 Treatment is non-operative. which can easily be mistaken for deep vein thrombosis.
The swelling may diminish following aspiration and possible. Before withdrawing the instrument, saline is 20
injection of hydrocortisone; excision is not advised, squeezed out. A firm bandage is applied; the arthro-
because recurrence is common unless the underlying scopic portals are often small enough not to require
condition is treated. sutures. Postoperative recovery is remarkably rapid.
POPLITEAL ANEURYSM COMPLICATIONS
The knee
This is the commonest limb aneurysm and is some- Intra-articular effusions and small haemarthroses are
times bilateral. Pain and stiffness of the knee may pre- fairly common but seldom troublesome.
cede the symptoms of peripheral arterial disease, so it Reflex sympathetic dystrophy (which may resemble a
is essential to examine any lump behind the knee for low-grade infection during the weeks following
pulsation. A thrombosed popliteal aneurysm does not arthroscopy) is sometimes troublesome. It usually
pulsate, but it feels almost solid. settles down with physiotherapy and treatment with
non-steroidal anti-inflammatory drugs; occasionally it
requires more radical treatment (see pages 261 and
BONY SWELLINGS AROUND THE KNEE 723).
Because the knee is a relatively superficial joint, bony

swellings of the distal femur and proximal tibia are LIGAMENT RECONSTRUCTION
often visible and almost always palpable. Common
examples are cartilage-capped exostoses (osteochon- The collateral and cruciate ligaments and the knee
dromata) and the characteristic painful swelling of capsule are important constraints which allow normal
Osgood–Schlatter disease of the tibial tubercle (see knee function; laxity or rupture of these structures,
below). either singly or in combination, is often the source of
recurrent episodes of ‘giving way’. Although a signifi-
cant proportion of such injuries are treated non-oper-
atively, complete ruptures may require surgery in
PRINCIPLES OF KNEE OPERATIONS ‘high-demand’ individuals.
Surgery for ligament reconstruction includes:
ARTHROSCOPY 1. Repair, usually for collateral ligament mid-
substance ruptures when they are found in
Arthroscopy is useful: (1) to establish or refine the combination with cruciate ligament injuries. This
accuracy of diagnosis; (2) to help in deciding whether repair can be a simple end-to-end suture.
to operate, and (3) to perform certain operative pro- 2. Substitution, usually for anterior cruciate ruptures:
cedures. Arthroscopy is not a substitute for clinical the semitendinosus and gracilis, either one or two
examination; a detailed history and meticulous assess- bundle technique, can be carefully anchored to
ment of the physical signs are indispensable prelimi- the femur and tibia ensuring that stability is
naries and remain the sheet anchor of diagnosis. restored without loss of knee movement. Another
method is to use an autologous graft from the
TECHNIQUE patellar tendon.
Full asepsis in an operating theatre is essential. The pa- 3. Tenodesis, using a variety of tendons which are
tient is anaesthetized (though local anaesthesia may suf- passed either through bony or soft-tissue tunnels
fice for short procedures) and a thigh tourniquet ap- to ‘check’ the abnormal movement resulting from
plied. Through a tiny incision, a trocar and cannula is ligament rupture.
introduced; sometimes, saline is injected to distend
the joint before it is punctured. Entry into the joint is
confirmed when saline flows easily into the joint or, if OSTEOTOMY
the joint was distended previously, by the outflow when
the trocar is withdrawn. A fibreoptic viewer, light Osteotomy above or below the joint used to be a pop-
source and irrigation system are attached; a small tele- ular method of treating arthritis of the knee, especially
vision camera and monitor make it much easier for the when articular destruction was more or less limited to
operator to concentrate on manipulating the instru- one compartment and the knee had developed a varus
ments with both hands (‘triangulation’). All compart- or valgus deformity. With the development of joint
ments of the joint are now systematically inspected; replacement techniques, the operation gradually fell
with special instruments and, if necessary, through into disuse, or at best was seen as a temporizing meas-
multiple portals, biopsy, partial meniscectomy, patellar ure to buy time for patients who would ultimately
shaving, removal of loose bodies, synovectomy, liga- undergo some form of arthroplasty. However,
ment replacement and many other procedures are improvements in technique and the introduction of 579
20 operations for meniscal and articular cartilage repair end of the tibia, whereas a valgus deformity should be
have led to renewed interest in this procedure. corrected by a varus osteotomy at the femoral supra-
The rationale for osteotomy is based on both bio- condylar level.
mechanical and physiological principles. Malalign- Angles must be accurately measured and the posi-
ment of the limb results in excessive loading and stress tion of correction carefully mapped out on x-rays
in part of the joint and consequently increased dam- before starting the operation.
age to the articular cartilage in that area – the medial A high tibial valgus osteotomy can be performed
compartment if the knee is in varus and the lateral either by removing a pre-determined wedge of bone
compartment in a valgus knee. As the articular surface based laterally and then closing the gap (closing wedge
is destroyed, the deformity progressively increases. technique) or by opening a wedge-shaped gap on the
Osteotomy and repositioning of the bone fragments, medial side (opening wedge technique).
by correcting the deformity, will improve the load- In the lateral closing wedge method the fibula must
bearing mechanics of the joint. Furthermore, it will first be released either by dividing it lower down or by
reduce the intraosseous venous congestion, and this disrupting the proximal tibio-fibular joint. The tibia is
may relieve some of the patient’s pain. divided just above the insertion of the patellar liga-
ment. Two transverse cuts are made, one parallel to
INDICATIONS the joint surface and another just below that, angled
Deformity of the kneeSevere varus or valgus deformity to create the desired laterally based wedge. The wedge
(e.g. due to a growth defect, epiphyseal injury or a of bone is removed and the fragments are then
malunited fracture) may of itself call for a corrective approximated and fixed in the corrected position
osteotomy, and the operation may also prevent or either with staples or with compression pins. The limb
delay the development of osteoarthritis. is immobilized in a cast for 4–6 weeks, by which time
Localized articular surface destruction Patients with uni-
the osteotomy should have started to unite.
compartmental osteoarthritis or advanced localized An opening wedge valgus osteotomy on the medial
osteonecrosis, particularly when this is associated with side offers some advantages: the ability to adjust the
deformity in the coronal plane, may benefit from an degree of correction intra-operatively and the option
osteotomy which offloads the affected area. Provided to correct deformities in the sagittal plane as well as the
the joint is stable and has retained a reasonable range coronal plane; it also makes it unnecessary to disrupt
of movement, this offers an acceptable alternative to a the tibio-fibular joint. However, there are also disad-
unicompartmental arthroplasty. Usually it is the vantages: the newly-created gap must be filled with a
medial compartment that is affected and the knee bone graft and a long period of restricted weightbear-
exhibits a varus deformity. By realigning the joint, ing is needed after the procedure; there is also a higher
load is transferred from the medial compartment to rate of non-union or delayed union. These drawbacks
the centre or a little towards the lateral side. Slight can be mitigated by stabilizing the fragments with an
over-correction may further offload the medial com- external fixator applied to the medial side, waiting for
partment but marked valgus should be avoided as this about 5 days and then opening the gap very gradually,
will rapidly lead to cartilage loss in the lateral com- allowing it to fill with callus (hemicallotasis). Cast
partment. immobilization is unnecessary. The external fixator
Published results suggest that the operation pro- usually remains in place for 10–12 weeks.
vides substantial improvements in pain and function If a varus osteotomy is required – usually for active
over a 7–10-year period (Dowd et al., 2006). patients with isolated lateral compartment disease and
valgus deformity of the knee – this is performed at the
Intra-articularreconstructions The introduction of supracondylar level of the femur. The method most
meniscal and articular cartilage reconstruction tech- commonly employed is a medial closing wedge
niques has led to considerable interest in applying the osteotomy, designed to place the mechanical axis at
favourable biomechanical effects of osteotomy to the zero. The fragments should be firmly fixed with a
younger patient who has a full-thickness chondral blade-plate; in many cases postoperative cast immobi-
lesion or an absent meniscus. Similarly, osteotomy in lization will also be needed.
conjunction with either simultaneous or staged cruci-
ate ligament reconstruction appears to be beneficial in RESULTS
patients who have a combination of instability and High tibial valgus osteotomy, when done for
pain from limb malalignment (Giffin and Fintan, osteoarthritis, gives good results provided (1) the dis-
2007). ease is confined to the medial compartment, and (2)
the knee has a good range of movement and is stable.
TECHNIQUE Relief of pain is good in 85 per cent of cases in the first
For sound biomechanical reasons, a varus deformity is year but drops to approximately 60 per cent after 5
580 best corrected by a valgus osteotomy at the proximal years. A recent review has shown that modern medial
opening wedge osteotomy techniques can achieve sat- INDICATIONS 20
isfactory postoperative alignment in 93 per cent of In the past – and even today in some parts of the
patients and survivorship rates of 94 per cent at 5- world – the main indications for arthrodesis of the
year, 85 per cent at 10-year, and 68 per cent at 15- knee were (and are) irremediable instability due to the
year follow-up, with conversion to total knee late effects of poliomyelitis and painful loss of mobility
arthroplasty as the end point (Brower et al., 2007; due to tuberculosis or chronic pyogenic infection.
The knee
Virolainen and Aro, 2004). In countries with advanced medical facilities the
The clinical results of distal femoral varus commonest indication is failed total knee replacement
osteotomy have been good in selected patients. Sub- (either septic or aseptic).
stantial improvements in pain and function can be
expected in approximately 90 per cent of patients CONTRAINDICATIONS
(Preston et al., 2005). Contraindications include severe general disability
because of age or multiple joint disease, especially if
COMPLICATIONS associated with problems in the ipsilateral hip or
Compartment syndrome in the leg This is the most ankle; amputation or knee fusion of the opposite limb;
important early complication of tibial osteotomy. and persistent non-union of a peri-articular fracture or
Careful and repeated checks should be carried out massive peri-articular bone loss. Finally, patient reluc-
during the early postoperative period to ensure that tance may be an important factor. A short period in a
there are no symptoms or signs of impending plaster cylinder before operation may convince the
ischaemia. Early features of compartment compres- patient that a rigidly stiff leg is better than a painful
sion in the leg are sometimes mistaken for those of a and unstable knee.
deep vein thrombosis; this mistake should be avoided
at all costs because the consequent delay in starting TECHNIQUE
treatment could make the difference between com- A vertical midline incision is used. If the operation is
plete recovery and permanent loss of function. for tuberculosis the diseased synovium is excised; oth-
erwise it is disregarded. The posterior vessels and
Peroneal nerve palsy Overzealous attempts at correct- nerves are protected and the ends of the tibia and
ing a longstanding valgus deformity can stretch and femur removed by means of straight saw cuts, aiming
damage the peroneal nerve. Poor cast techniques may to end with 15 degrees of flexion and 7 degrees of val-
do the same, which is a good reason why postopera- gus as the position of fusion. Charnley’s method,
tive cast application should not be left to an unsuper- using thick Steinman pins inserted parallel through
vised junior assistant. the distal femur and proximal tibia, and connecting
Failure to correct the deformity Under- or overcorrec- these with compression clamps, was for many years
tion of the deformity are really failures in technique. the standard method. Nowadays, multiplanar external
With medial compartment osteoarthritis, unless a fixation is used, or if the joint is not infected, a long
slight valgus position is obtained, the result is liable to intramedullary nail which may be unlocked or locked.
be unsatisfactory. However, marked overcorrection is
not only mechanically unsound but the cosmetic
defect is liable to be bitterly resented by the patient. KNEE REPLACEMENT
Delayed union and non-union These complications can INDICATIONS
be avoided by ensuring that fixation of the bone frag- The main indication for knee replacement is pain,
ments is stable and secure. especially when this is combined with deformity and
instability. Most replacements are performed for
ARTHRODESIS rheumatoid arthritis or osteoarthritis.
Arthrodesis of the knee has long been considered a TYPES OF OPERATION

demanding procedure that is subject to a variety of Partial replacement The role of unicompartmental
postoperative complications and often results in mar- replacement has yet to be firmly established. Early
ginal or unacceptable outcomes. A stiff knee is a con- results for medial compartment osteoarthritis were
siderable disability; it makes climbing difficult and promising but longer-term studies have highlighted
sitting in crowded areas distinctly awkward. Conse- the need for meticulous and exacting surgical tech-
quently, it is not often performed. For these reasons, nique to avoid high revision rates. Following a suc-
arthrodesis has typically been held in reserve as a final cessful operation, relief of pain and restoration of
salvage procedure for patients with irretrievably failed function can be impressive, but for the present it is
total knee arthroplasties and other comparable condi- reserved for older patients; tibial and femoral
tions. osteotomies are used in the younger population. 581
20 Patellar resurfacing, a kind of partial replacement, is high incidence of deep vein thrombosis. Prophylaxis,
rarely performed alone; usually it is combined with either pharmacological (anticoagulants) or mechanical
surface replacement of the condyles. (foot pumps, compression stockings), is recom-
Minimally constrained total replacement The term ‘min- mended.
imally constrained’ is used for prostheses where some Infection The methods of preventing and treating
of the stability after replacement is provided by the
infection are similar to those used in hip replacement.

prosthesis and some through preservation of the knee For established and intractable infection, treatment by
ligaments. Most modern minimally constrained de- debridement and antibiotics, or by exchange replace-
signs allow sacrifice of the anterior cruciate ligament; ment in one or two stages, are obvious possibilities,
some even allow both cruciates to be removed without though probably the safest salvage operation is
detriment to the long-term survival of the prosthesis. arthrodesis; this is especially applicable in immuno-
‘Totally unconstrained’ devices, where both cruciates suppressed patients and in those with resistant
are preserved, are rarely used because results are poor bacteria.
compared to the minimally constrained group.
At operation all the articular surfaces are replaced – Loosening Covert infection is only one cause of
with metal on the femoral side, polyethylene on a implant loosening. Aseptic loosening results from
metal tray on the tibial side and polyethylene alone on faulty prosthetic design, inaccurate bone shaping,
the patella. It is important to ensure correct place- incorrect placement of the implants or a combination
ment of the implants so as to reproduce the normal of these factors. Revision surgery for loose prostheses
mechanics of the knee as closely as possible. must deal with the cause, be it malposition of the
The tibial and patellar components are fixed with prosthesis, accumulation of wear debris or infection.
cement, whereas the femoral component may be A loose prosthesis can be re-cemented, but unless the
press-fitted. Bone defects may be filled either with cause is dealt with, loosening will recur.
bone graft, metal augmentation wedges or cement. Patellar problems Although relatively uncommon,
The development of suitable prostheses and instru- these can be very disabling. They include: (1) recur-
mentation in recent years has led to vast improve- rent patellar subluxation or dislocation, which may
ments in technique, so the results are now similar to need realignment; and (2) complications associated
those of hip replacement. with patellar resurfacing, such as loosening of the
Constrained joints Artificial joints with fixed hinges prosthetic component, fracture of the remaining bony
are used when there is marked bone loss and severe patella, and catching of soft tissues between the
instability. Their main value nowadays is to provide a patella and the femur.
mobile joint following resection of tumours at the Patellar tracking as assessed on the operating table
bone ends. The lack of rotation in these implants after implantation of the prosthesis is important. Any
places severe stresses on the bone/implant interfaces tendency to sublux must be corrected: common
and they are liable to loosen, to break or to erode the causes are unequal soft-tissue tension (for which a lat-
tibial or femoral shafts unless physical activity is eral release will be needed), a tibial component placed
severely restricted. Moreover, a considerable amount in internal rotation and/or a femoral component
of bone has to be removed, and this makes a subse- placed in internal rotation.
quent arthrodesis difficult. The risk of patellar fracture postoperatively can also
Minimally invasive total knee replacement This is in its be lessened if care is taken not to divide the geniculate
early stage of development and is not yet widely used. vessels when performing a lateral release.
Early results suggest that it provides some benefits
over conventional total joint replacement: less pain,
faster recovery, better quadriceps strength and a bet- NOTES ON APPLIED ANATOMY
ter range of movement.
TECHNIQUE The knee joint combines two articulations – tibio-
It is important: (1) to overcome deformity (the knee femoral and patello-femoral. The bones of the tibio-
should finally be about 7 degrees valgus); (2) to pro- femoral joint have little or no inherent stability; this
mote stability (by tailoring the bone cuts so that the depends largely upon strong static and dynamic stabi-
collateral ligaments are equally tense in both flexion lizers such as ligaments and muscles. The patello-
and extension); and (3) to permit rotation (otherwise femoral joint is so shaped that the patella moves in a
cemented prostheses are liable to loosen). shallow path (or track) between the femoral condyles;
if this track is too shallow the patella readily dislocates,
COMPLICATIONS and if its line is faulty the patellar articular cartilage is
General As with all knee operations (except subject to excessive wear. One important function of
582 arthroscopy) in which a tourniquet is used, there is a the patella is to increase the power of extension; it lifts
the quadriceps forwards, thereby increasing its does not occur medially) but remains stable concern- 20
moment arm. ing spinning kinematics, while the contact area trans-
The patellar tendon is inserted into the upper pole of fers from an anterior pair of tibio-femoral surfaces at
the patella. It is in line with the shaft of the femur, 10 degrees to a posterior part at about 30 degrees.
whereas the patellar ligament is in line with the shaft of Thus, because of the shapes of the bones, the
the tibia. Because of the angle between them (the Q- medial contact area moves backwards with flexion to
The knee
angle) quadriceps contraction would pull the patella lat- 30 degrees but the condyle does not. On the lateral
erally were it not for the fibres of vastus medialis, which side a variable spinning motion in mid-flexion (60
are transverse. This muscle is therefore important and degrees) and a rolling motion up to 120 degrees of
it is essential to try to prevent the otherwise rapid flexion are observed. Laterally, the femoral condyle
wasting that is liable to follow any effusion. and the contact area move posteriorly but to a variable
The shaft of the femur is inclined medially, while extent in the mid-flexion (roll-back) causing tibial
the tibia is vertical; thus the normal knee is slightly internal rotation to occur with flexion around a
valgus (average 7 degrees). This amount is physiolog- medial axis. Flexion beyond 120 degrees can only be
ical and the term ‘genu valgum’ is used only when the achieved passively. Medially, the femur rolls back onto
angle exceeds 7 degrees; significantly less than this the posterior horn. Laterally, the femur and the pos-
amount is genu varum. terior horn drop over the posterior tibia. New knee
During walking, weight is necessarily taken alter- prostheses have been designed to reflect contempo-
nately on each leg. The line of body weight falls rary data regarding knee kinematics.
medial to the knee and must be counterbalanced by Situated as they are between these complexly mov-
muscle action lateral to the joint (chiefly the tensor ing surfaces, the fibrocartilaginous menisci are prone
fascia femoris). To calculate the force transmitted to injury, particularly during unguarded movements
across the knee, that due to muscle action must be of extension and rotation on the weightbearing leg.
added to that imposed by gravity; moreover, since The medial meniscus is especially vulnerable because,
with each step the knee is braced by the quadriceps, in addition to its loose attachments via the coronary
the force that this imposes also must be added. ligaments, it is firmly attached at three widely sepa-
Clearly the stresses on the articular cartilage are (as rated points: the anterior horn, the posterior horn and
they also are at the hip) much greater than considera- to the medial collateral ligament. The lateral meniscus
tion only of body weight would lead one to suppose. more readily escapes damage because it is attached
It is also obvious that a varus deformity can easily only at its anterior and posterior horns and these are
overload the medial compartment, leading to carti- close to each other.
lage breakdown; similarly, a valgus deformity may The function of the menisci is not known for certain,
overload the lateral compartment. but they certainly increase the contact area between
For several decades, the prevailing opinion was that femur and tibia. They play a significant part in weight
the movements of the knee are guided by the cruciate transmission and this applies at all angles of flexion and
ligaments functioning as a crossed four-bar link. For a extension; as the knee bends they glide backwards, and
knee guided by a four-bar link, this implies that the as it straightens they are pushed forwards.
axis of rotation of the tibia relative to the femur must The deep portion of the medial collateral ligament,
be at the crossing point of the cruciate ligaments. An to which the meniscus is attached, is fan-shaped and
important kinematic consequence of the four-bar link blends with the posteromedial capsule. It is, therefore,
is the phenomenon known as ‘roll-back’. Roll-back is not surprising that medial ligament tears are often
a progressive movement of the femur backward on associated with tears of the medial meniscus and of
the tibia with flexion. The opposite – roll-forward – the posteromedial capsule. The lateral collateral liga-
would then occur during knee extension. ment is situated more posteriorly and does not blend
However, recent published work on normal knee with the capsule; nor is it attached to the meniscus,
kinematics has shown that the knee does not work as a from which it is separated by the tendon of popliteus.
crossed four-bar link. Modern knee kinematics are bet- The two collateral ligaments resist sideways tilting
ter understood by dividing the flexion arc into three of the extended knee. In addition, the medial liga-
parts (Freeman and Pinskerova, 2005). From full ment prevents the medial tibial condyle from sublux-
extension to 20 to 30 degrees of flexion, tibial internal ating forwards. Forward subluxation of the lateral
rotation is coupled with flexion and on the lateral side tibial condyle, however, is prevented, not by the lat-
a counter-translation nearing full extension is eral collateral ligament but by the anterior cruciate.
observed. Knee activities take place mainly between 20 Only when the medial ligament and the anterior cru-
degrees and 120 degrees. Over this arc, the articulat- ciate are both torn can the whole tibia subluxate for-
ing surfaces of the femoral condyles are circular in wards (giving a marked positive anterior drawer sign).
sagittal section and rotate around a centre. The medial Backward subluxation of the tibia is prevented by the
condyle does not move anteroposteriorly (roll-back powerful posterior cruciate ligament in combination 583
20 with the arcuate ligament on its lateral side and the Inone M, Shino K, Hirose H et al. Subluxation of the
posterior oblique ligament on its medial side. patella. Computed tomography analysis of patellofemoral
The cruciate ligaments are crucial, in the sense that congruence. J Bone Joint Surg 1988; 70A: 1331–7.
they are essential for stability of the knee. The anterior Insall JN, Salvati E. Patella position in the normal knee
cruciate ligament prevents forward displacement of joint. Radiology 1971; 101: 101.
the tibia on the femur and, in particular, it prevents Karachalios T, Hantes M, Zibis AH et al. Diagnostic accu-
forward subluxation of the lateral tibial condyle, a racy of a new clinical test (the Thessaly test) for early
movement that tends to occur if a person who is run- detection of meniscal tears. J Bone Joint Surg 2005; 87A:
ning twists suddenly. The posterior cruciate ligament 955–62.
prevents backward displacement of the tibia on the Kay PR, Freemont AJ, Davies DRA. The aetiology of
femur and its integrity is therefore important when multiple loose bodies. J Bone Joint Surg 1989; 71B:
progressing downhill. 501–4.
Khan KM, Maffulli N, Coleman BD et al. Patellar
tendinopathy: some aspects of basic science and clinical
management. Br J Sports Med 1998; 32: 346–55.
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clinical methods. J Bone Joint Surg 1947; 29: 78–84. of osteochondritis dissecans of the knee. Current con-
Bentley G. Articular cartilage changes in chondromalacia cepts review. Am J Sports Med 2006; 34: 1181–91.
patellae. J Bone Joint Surg 1985; 67B: 769–774. Liu SH, Mirzayan R. Current review. Functional knee brac-
Bowen JR, Leahy JL, Zhang Z, MacEwen GD. Partial epi- ing. Clin Orthop Res 1995; 317: 273–81.
physeodesis at the knee to correct angular deformity. Clin Mann G, Finsterbush A, Franfkl U et al. A method of
Orthop 1985; 198: 184–90. diagnosing small amounts of fluid in the knee. J Bone
Brower RW, van Raaij TM, Bierma-Zeinstra SM et al. Joint Surg 1991; 73B: 346–7.
Osteotomy for treating knee osteoarhtritis. Cochrane Maquet PGJ. Biomechanics of the Knee. Springer, Berlin,
Database Syst Rev 2007; 18(3): CD004019. 1976.
Coventry MB. Upper tibial osteotomy for osteoarthritis. Medlar RC, Lyne ED. Sinding-Larsen Johansson disease.
J Bone Joint Surg 1985; 67A: 1136–40. J Bone Joint Surg 1978; 60A: 1113–6.
Crotty JM, Monu JU, Pope TL Jr. Magnetic resonance Men HX, Bian CH, Yang CD et al. Surgical treatment of
imaging of the musculoskeletal system. Part 4. The knee. the flail knee after poliomyelitis. J Bone Joint Surg 1991;
Clin Orthop Relat Res 1996; 330: 288–303. 73B: 195–8.
Dandy DJ. Chronic patellofemoral instability. J Bone Joint Merchant AC, Mercer RL, Jacobsen RH, Cool CR.
Surg 1995; 78B: 328–35. Roentgenographic analysis of patellofemoral congruence.
Dimakopoulos P, Patel D. Partial excision of discoid J Bone Joint Surg 1974; 56A: 1391–6.
meniscus. Acta Orthop Scand 1990; 61: 1–40. Oei EHG, Nikken JJ, Verstijen ACM et al. MR Imaging of
Dowd GS, Somayaji HS, Uthukuri M. High tibial the menisci and cruciate ligaments: A systematic review.
osteotomy for medial compartment osteoarthritis. Knee Radiology 2003; 226: 837–48.
2006; 13: 87–92 Paletta GA Jr, Laskin RS. Total knee arthroplasty after a
Ficat RP, Hungerford DS. Disorders of the Patello-femoral previous patellectomy. J Bone Joint Surg 1995; 77A:
Joint, Williams & Wilkins, Baltimore, 1977. 1708–12.
Freeman MA, Pinskerova V. The movement of the normal Parisien JS. Arthroscopic treatment of cysts of the menisci.
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Goodfellow J, Hungerford DS, Zindel M. Patello-femoral Traumatol Arthrosc 1998; 6: 2–11.
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Goodfellow J, Hungerford DS, Woods C. Patellofemoral Salenius P, Vankka E. The development of the tibiofemoral
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Goodfellow JW, Kershaw CJ, Benson MKD’A, O’Connor J Bone Joint Surg 1996; 78A: 439–56.
JJ. The Oxford knee for unicompartmental osteoarthritis. Sherman OH, Fox JM, Snyder SJ, et al. Arthroscopy – ‘No
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Thomee R, Augustsson J, Karlsson J. Patellofemoral pain the literature and meta-analysis of follow up studies. Arch 20
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The knee
585
The ankle and foot
21
Gavin Bowyer
assessing the impact of the condition on function and

CLINICAL ASSESSMENT deciding on treatment in foot and ankle problems are:
Have you any pain or stiffness in your muscles, joints
SYMPTOMS or back? Can you dress yourself completely without
any difficulty? Can you walk up and down stairs with-
Adults with foot and ankle problems often present out any difficulty?
complaining of pain, swelling, deformity and impaired
function including difficulties with work, social and
domestic activities. Questions should include those
that flag up the possibility of neoplastic or generalized SIGNS WITH PATIENT UPRIGHT
inflammatory disease and diabetes.
It is important to see the patient stand, as deformities
Pain over a bony prominence or a joint is probably
will often be much better shown once the patient is
due to some local disorder; ask the patient to point to
weightbearing. The patient, whose lower limbs
the painful spot. Symptoms tend to be well localized
should be exposed from the knees down, stands first
to the structures involved, but vague pain across the
facing the surgeon, then with his or her back to the
forefoot (metatarsalgia) is less specific and is often
surgeon. Ask the patient to rise up on tiptoes and then
associated with uneven loading and muscle fatigue.
settle back on the heels. Note the posture of the feet
Often the main complaint is of shoe pressure on a ten-
throughout this movement. Normally the heels are in
der corn over a toe joint or a callosity on the sole.
slight valgus while standing and inverted on tiptoes;
Osteoarthritic pain at the first metatarsophalangeal
the degree of inversion should be equal on the two
(MTP) joint is often better in firm-soled shoes; hallux
sides, showing that the subtalar joint is mobile and the
valgus/bunions will be exacerbated by close-fitting
tibialis posterior functioning. Viewed from behind, if
shoes; a functionally or mechanically unstable ankle
there is excessive eversion of one foot, the lateral toes
often feels better in boots; metatarsalgia is worse in
are more easily visible on that side (the ‘too-many-toes’
shoes with a higher heel. Morton’s neuroma or a
sign).
prominent metatarsal head feels like a marble or peb-
ble in the shoe. Gait Observing the gait also helps to identify dynamic
Deformity is sometimes the main complaint; the problems and pathology on other lower limb joints.
patient may abhor a ‘crooked toe’ or a ‘twisted foot’, The patient is asked to walk normally. Note whether
even if it is not painful, and parents often worry about the gait is smooth or halting and whether the feet are
their children who are ‘flat-footed’ or ‘pigeon-toed’. well balanced. Gait is easier to analyze if concentrating
Elderly patients may complain chiefly of having diffi- on the sequence of movements that make up the
culty fitting shoes. walking cycle. It begins with heel-strike, then moves
Swelling is common, even in normal people, but it into stance, then push-off and finally swing-through
gains more significance if it is unilateral or strictly before making the next heel-strike. The stance phase
localized. itself can be further divided into three intervals: (1)
Instability of the ankle or subtalar joint produces from heel-strike to flat foot; (2) progressive ankle
repeated episodes of the joint ‘giving way’. Ask about dorsiflexion as the body passes over the foot; (3) ankle
any previous injury (a ‘twisted ankle’). plantarflexion leading to toe-off.
Numbness and paraesthesia may be felt in all the Gait may be disturbed by pain, muscle weakness,
toes or in a circumscribed field served by a single deformity or stiffness. The position and mobility of
nerve or one of the nerve roots from the spine. each ankle is of prime importance. A fixed equinus
General questions that help in reaching a diagnosis, deformity results in the heel failing to strike the
21
(a) (b) (c) (d)
(e) (f) (g)
21.1 Examination with patient standing Look at the patient as a whole, first from in front and from behind. (a,b) The
heels are normally in slight valgus and should invert equally when a patient stands on his/her toes. (c) This patient has flat
feet (pes planus), while the patient in (d) has the opposite deformity, varus heels and an abnormally high longitudinal arch
– pes cavus (e). From the front you can again notice (f) the dropped longitudinal arch in the patient with pes planus, as
well as the typical deformities of bilateral hallux valgus and overriding toes. (g) Corns on the top of the toes are common.
ground at the beginning of the walking cycle; some- Hindfoot and midfoot deformities may interfere
times the patient forces heel contact by hyperextend- with level ground-contact in the second interval of
ing the knee. stance; the patient walks on the inner or outer border
If the ankle dorsiflexors are weak, the forefoot may of the foot.
hit the ground prematurely, causing a ‘slap’; this is re- Toe contact, especially of the great toe, is also
ferred to as foot-drop (or drop-foot). During swing- important; pain or stiffness in the first MTP joint may
through the leg is lifted higher than usual so that the prevent normal push-off.
foot can clear the ground (a high-stepping gait).
SIGNS WITH PATIENT SITTING OR LYING

A systematic approach to examination, following the
‘look, feel, move’ steps, will lead to a diagnosis in the
majority of cases.
Next the patient is examined lying on a couch, or it
may be more convenient if he or she sits opposite the
examiner and places each foot in turn on the exam-
iner’s lap.
21.2 Gait – the three rockers of ankle-stance phase Look

The first rocker begins with heel-strike – if the anterior
compartment muscles are weak, a ‘foot-slap’ is noticeable; The heel is held square so that any foot deformity can
or if the ankle is in fixed equinus, this rocker may be absent be assessed. The toes and sole should be inspected for
altogether. In mid-stance, the centre of gravity of the body skin changes. The foot shows areas of overload by pro-
(and ground reaction force) moves from a position ducing callosities, and there are often corresponding
posterior to the ankle joint to anterior (second rocker). The
third rocker produces an acceleration force that shifts the
areas of wear and signs of overload on the footwear.
fulcrum of the pivot forwards to the metatarsal heads, just Thickening and keratosis may be seen over the proxi-
588 prior to toe-off (Gage, 1991). mal toe joints or on the soles. Atrophic changes in the
21
The ankle and foot

h
(a) (b) (c)
21.3 Foot – surface anatomy Medial aspect: a, tendon of tibialis anterior; b, medial malleolus; c, tendon of tibialis
posterior; d, sulcus behind medial malleolus; e, extensor tendons of toes; f, lateral malleolus; g, peroneal tendons curving
behind the lateral malleolus; h, anterior metatarsal arch.
skin and toe-nails are suggestive of a neurological or tion sense, protective sensation and sense of position
vascular disorder. in the toes.
Deformity may be in the ankle, the foot or toes. A
foot that is set flat on the ground at a right angle to
the tibia is described as plantigrade; if it is set in fixed Move
plantarflexion (pointing downwards) it is said to be in The foot comprises a series of joints that should be
equinus; a dorsiflexed position is called calcaneus. examined methodically:
Common defects are a ‘flat-footed’ stance (pes val-
gus); an abnormally high instep (pes cavus); a down- • Ankle joint – With the heel grasped in the left hand
ward-arched forefoot (pes plantaris); lateral deviation and the midfoot in the right, the ranges of plan-
of the great toe (hallux valgus); fixed flexion of a sin- tarflexion (flexion) and dorsiflexion (extension) are
gle interphalangeal (IP) joint (hammer toe) or of all estimated. Beware not to let the foot go into valgus
the toes (claw toes). during passive dorsiflexion as this will give an erro-
Swelling may be diffuse and bilateral, or localized; neous idea of the range of movement.
unilateral swelling nearly always has a surgical cause • Subtalar joint – It is important to ‘lock’ the ankle
and bilateral swelling is more often ‘medical’ in origin. joint when assessing subtalar inversion and ever-
Swelling over the medial side of the first metatarsal sion. This is done simply by ensuring that the ankle
head (a bunion) is common in older women. is plantigrade when the heel is moved. It is often
Corns are usually obvious; callosities must be looked easier to record the amount of subtalar movement
for on the soles of the feet. if the patient is examined prone. Inversion is nor-
mally greater than eversion.
• Midtarsal joint – One hand grips the heel firmly to
Feel stabilize the hindfoot while the other hand moves
Pain and tenderness in the foot and ankle localize very the forefoot up and down and from side to side.
well to the affected structures – the patient really does • Toes – The MTP and IP joints are tested separately.
show us where the problem is. The skin temperature Extension (dorsiflexion) of the great toe at the
is assessed and the pulses are felt. Remember that one MTP joint should normally exceed 70 degrees and
in every six normal people does not have a dorsalis flexion 10 degrees.
pedis artery. If all the foot pulses are absent, feel for
the popliteal and femoral pulses; the patient may need
further evaluation by Doppler ultrasound.
Stability
If there is tenderness in the foot it must be precisely Stability is assessed by moving the joints across the
localized, for its site is often diagnostic. Any swelling, normal physiological planes and noting any abnormal
oedema or lumps must be examined. ‘clunks’. Ankle stability should be tested in both coro-
Sensation may be abnormal; the precise distribution nal and sagittal planes, always comparing the two
of any change is important. If a neuropathy is sus- joints. Patients with recent ligament injury may have
pected (e.g. in a diabetic patient) test also for vibra- to be examined under anaesthesia. 589
21 WHERE DOES IT HURT; WHERE IS IT TENDER?
Anterior ankle joint line – impingement from osteophytes in OA
Anterolateral angle of ankle joint – lateral gutter impingement in post-traumatic ankle with soft tissue
problems
Bony tip/lateral malleolus – ankle fracture (Ottawa guidelines)
Posterior/inferior to lateral malleolus – peroneal tenosynovitis or tear
Posterior to medial malleolus/line of tibialis posterior – tibialis posterior tendinitis or tear, and in plano-valgus
collapse of hindfoot
Base of fifth metatarsal – fracture/insertional problem with peroneus brevis
Achilles tendon – Achilles tendinitis/paratendinitis
Achilles insertion – insertional tendinitis
Retrocalcaneal bursa – bursitis
Plantar fascia – plantar fasciitis
Medial to first MTP joint – bunion
Dorsal to first MTP joint – OA, hallux limitus/rigidus
Beneath first MTP joint – sesamoiditis
Beneath metatarsal heads – ‘metatarsalgia’
In third interspace – Morton’s neuroma
Medial and lateral stability are checked by stressing Muscle power

the ankle first in valgus and then in varus. Anteropos-
terior stability is assessed by performing an anterior Power is tested by resisting active movement in each di-
‘drawer test’: the patient lies on the examination rection. The patient will be more cooperative if the
couch with hips and knees flexed and the feet resting movement required is demonstrated precisely. While
on the couch surface; the examiner grasps the distal the movement is held, feel the muscle belly and tendon
tibia with both hands and pushes firmly backwards, to establish whether they are intact and functioning.
feeling for abnormal translation of the tibia upon the
talus. Another way of doing this is to stabilize the dis- Shoes
tal tibia with one hand while the other grasps the heel
and tries to shift the hindfoot forwards and back- Footwear often adds additional clues when examining
wards. the foot and ankle, providing valuable information
The same tests can be performed under x-ray and about faulty stance or gait.
the positions of the two ankles measured and com-
pared. General examination
If there are any symptoms or signs of vascular or neu-
rological impairment, or if multiple joints are affected,
a more general examination is essential.
IMAGING
There are practical problems with imaging in children,
21.4 Normal range of movement All movements are and babies in particular because: (1) babies tend not
measured from zero with the foot in the ‘neutral’ or
‘anatomical’ position: thus, dorsiflexion is 0–15 degrees to keep still during examination; (2) their bones are
and plantarfexion 0–40 degrees. Inversion is about 30 not completely ossified and their shape and position
590 degrees and eversion 15 degrees. may be hard to define.
X-rays In the adult, the standard views of the ankle are force plate; sensors in the plate produce a dynamic 21
anteroposterior (AP), mortise (an AP view with the map of the peak pressures and the time over which
ankle internally rotated 15–20 degrees) and lateral. these are recorded can be obtained. Although this is
Although the subtalar joint can be seen in a lateral view sometimes helpful in clinical decision making, or for
of the foot, medial and lateral oblique projections allow comparing pre- and postoperative function, the inves-
better assessment of the joint. These views are often tigation is used mainly as a research tool.
The ankle and foot

used to check articular congruity after treatment of cal-
caneal fractures. The calcaneum itself is usually x-rayed
in axial and lateral views, but a weightbearing view is
helpful in defining its relationship to the talus and CONGENITAL DEFORMITIES
tibia. The foot, toes and intertarsal joints are well dis-
played in standing anteroposterior and medial oblique
Congenital deformities of the foot are common.
views, but occasionally a true lateral view is needed.
Many appear as part of a more widespread genetic dis-
Stress x-rays These complement the clinical tests for order; only those in which the foot is the main (or
ankle stability. The patient should be completely only) problem are considered in this section. Isolated
relaxed; if the ankle is too painful, stress x-rays can be abnormalities of the toes are also dealt with elsewhere.
performed under regional or general anaesthesia. Both
ankles should be examined, for comparison.
CT scan CT provides excellent coronal views and is TALIPES EQUINOVARUS (IDIOPATHIC
important in assessing fractures and congenital bony CLUB-FOOT)
coalitions.
The term ‘talipes’ is derived from talus (Latin = ankle
Radioscintigraphy Radioisotope scanning, though non-
bone) and pes (Latin = foot). Equinovarus is one of
specific, is excellent for localizing areas of abnormal
several different talipes deformities; others are talipes
blood flow or bone remodelling activity; it is useful in
calcaneus and talipes valgus.
the diagnosis of covert infection.
In the full-blown equinovarus deformity the heel is
MRI and ultrasound These methods are used to in equinus, the entire hindfoot in varus and the mid-
demonstrate soft tissue problems, such as tendon and and forefoot adducted and supinated. The abnormal-
ligament injuries. ity is relatively common, the incidence ranging from
1–2 per thousand births; boys are affected twice as
often as girls and the condition is bilateral in one-third
PEDOBAROGRAPHY of cases.
The exact cause is not known, although the resem-
A record of pressures beneath the foot can be blance to other disorders suggests several possible
obtained by having the patient stand or walk over a mechanisms. It could be a germ defect, or a form of
(a) (b) (c) (d)
21.5 X-rays (a) AP view of the ankle in a young woman who complained that after twisting her right ankle it kept giving
way in high-heeled shoes. The x-ray looks normal; the articular cartilage width (the ‘joint space’) is the same at all aspects
of the joint. The inversion stress view (b) shows that the talus tilts excessively; always x-ray both ankles for comparison and
in this case the left ankle (c) does the same. She has generalized joint hypermobility, not a torn lateral ligament. (d) X-rays
of the feet should be taken with the feet flat on the ground. 591
21 arrested development. Its occurrence in neurological border and through that of the calcaneum parallel to
disorders and neural tube defects (e.g. myelo- its lateral border; they normally cross at an angle of
meningocele and spinal dysraphism) points to a neu- 20–40 degrees (Kite’s angle) but in club-foot the two
romuscular disorder. Severe examples of club-foot are lines may be almost parallel. Incomplete ossification
seen in association with arthrogryposis, tibial defi- makes it difficult to decide exactly where to draw
ciency and constriction rings. In some cases it is no these lines and this means that there is a considerable
more than a postural deformity caused by tight pack- degree of interobserver variation.
ing in an overcrowded uterus. The lateral film is taken with the foot in forced dor-
siflexion. Lines drawn through the midlongitudinal
axis of the talus and the lower border of the calca-
neum should meet at an angle of about 40 degrees.
The neck of the talus points downwards and deviates Anything less than 20 degrees shows that the calca-
medially, whereas the body is rotated slightly out- neum cannot be tilted up into true dorsiflexion; the
wards in relation to both the calcaneum and the ankle foot may seem to be dorsiflexed but it may actually
mortise (Herzenberg et al., 1988). The posterior part have ‘broken’ at the midtarsal level, producing the so-
of the calcaneum is held close to the fibula by a tight called rocker-bottom deformity.
calcaneo-fibular ligament, and is tilted into equinus
and varus; it is also rotated medially beneath the
ankle. The navicular and entire forefoot are shifted
medially and rotated into supination (the composite
varus deformity).
The skin and soft tissues of the calf and the medial
side of the foot are short and underdeveloped. If the
condition is not corrected early, secondary growth
changes occur in the bones; these are permanent.
Even with treatment the foot is liable to be short and
the calf may remain thin. (a) (b)
Clinical features
The deformity is usually obvious at birth; the foot is
both turned and twisted inwards so that the sole faces
posteromedially. More precisely, the ankle is in equi-
nus, the heel is inverted and the forefoot is adducted
and supinated; sometimes the foot also has a high
medial arch (cavus), and the talus may protrude on
the dorsolateral surface of the foot. The heel is usually (c) (d)
small and high, and deep creases appear posteriorly
and medially; some of these creases are incomplete
constriction bands. In some cases the calf is abnor-
mally thin.
In a normal baby the foot can be dorsiflexed and
everted until the toes touch the front of the leg. In
club-foot this manoeuvre meets with varying degrees
of resistance and in severe cases the deformity is fixed.
The infant must always be examined for associated
disorders such as congenital hip dislocation and spina
bifida. The absence of creases suggests arthrogryposis;
look to see if other joints are affected.
(e)
X-rays 21.6 Talipes equinovarus (club-foot) (a) True club-foot
X-rays are used mainly to assess progress after treat- is a fixed deformity, unlike (b) postural talipes, which is
easily correctable by gentle passive movement. (c,d) With
ment. The anteroposterior film is taken with the foot
true club-foot, the poorly developed heel is higher than the
30 degrees plantarflexed and the tube likewise angled forefoot, which points downwards and inwards (varus).
30 degrees perpendicular. Lines can be drawn (e) Always examine the hips for congenital dislocation and
592 through the long axis of the talus parallel to its medial the back for spina bifida (as in the case shown here).
21
The ankle and foot

(b) (d)
(a) (c) (e)
21.7 Talipes equinovarus – x-rays The left foot is abnormal. In the anteroposterior view (a) the talocalcaneal angle is 5
degrees, compared to 42 degrees on the right. In the lateral views, the left talocalcaneal angle is 10 degrees in
plantarflexion (b) and 15 degrees in dorsiflexion (c). In the normal foot the angle is unchanged at 44 degrees, whatever the
position of the foot (d,e).
Treatment tendo Achillis lengthening in order to overcome the

equinus (Ponsetti, 1992).
The aim of treatment is to produce and maintain a The objective (ideally) is to achieve not only cor-
plantigrade, supple foot that will function well. There rection but overcorrection. The position should be
are several methods of treatment but relapse is com- checked by x-ray in order to ensure that there is no
mon, especially in babies with associated neuromus- rocker-bottom defect; attempts to overcome equinus
cular disorders. before the other deformities are corrected may ‘break’
the foot in the midtarsal region.
CONSERVATIVE TREATMENT Resistant cases will usually declare themselves after
Treatment should begin early, preferably within a day 8–12 weeks of serial manipulations and strapping. The
or two of birth. This consists of repeated manipula- surgeon then faces a choice of early surgery or con-
tion and adhesive strapping that maintains the correc- tinued conservative treatment. The results of early
tion; the manipulations are taught to the child’s operation, in particular neonatal surgery, have not
mother, who is then able to carry out gentle stretches been shown to be better than those of late surgery.
on a regular basis with the strapping still in place. Delaying surgery until the child is near walking age
Treatment is supervised by a physiotherapist, who has the advantages of operating on a larger foot (mak-
alters the strapping as correction is gradually ing surgery easier) and using the forces in normal
obtained. If this level of care is not available, it may be walking to help maintain the correction obtained at
better to hold position by applying a light plaster cast surgery.
(over a protective layer of strapping), which is soaked This delayed operative approach is suitable for
off and changed every week. severe, rigid deformities; however, for less severe cases
The three main components of the deformity are it may be preferable to operate at around 6 months of
always corrected in the following order. First the fore- age, but manipulation and splintage must still be con-
foot must be brought into rotational alignment with tinued until the child is walking.
the hindfoot; paradoxically this is done by increasing
the supination deformity of the forefoot so that it cor-
responds with the relatively more supinated hindfoot. OPERATIVE TREATMENT
Next, both hindfoot and forefoot are together gradu- The objectives of club-foot surgery are: (1) the com-
ally brought out of varus and supination; correction is plete release of joint ‘tethers’ (capsular and ligamen-
assisted by keeping the fulcrum on the lateral side of tous contractures and fibrotic bands); (2) lengthening
the head of the talus. Finally, equinus is corrected by of tendons so that the foot can be positioned normally
bringing the heel down and dorsiflexing the foot. It without undue tension. A detailed knowledge of the
may be necessary, en route, to perform percutaneous pathological anatomy is a sine qua non. 593
21 Access to the involved structures is through either often have to be divided to allow adequate correction
an extended posteromedial incision (Turco, 1971), a of hindfoot equinus. Sometimes flexor digitorum
posterior curved transverse incision extended anteri- longus and flexor hallucis longus also require atten-
orly on both medial and lateral sides (‘Cincinatti’ – tion. The calcaneo-fibular ligament, a key structure in
Crawford et al., 1982), or a posterolateral incision keeping the calcaneum malrotated, is then released. A
combined with a separate curved medial incision complete subtalar release is performed to allow the
(Caroll, 1994). The tendo Achillis and tibialis poste- hindfoot to be corrected. The superficial deltoid liga-
rior tendons are lengthened through Z-divisions; the ment is freed on the medial side but the deep part is
posterior capsules of the ankle and subtalar joints preserved to prevent ankle instability.
21.8 Congenital talipes

equinovarus – treatment First-
line treatment is non-operative.
This may be by manipulation and
strapping (a) or serial casting (b).
If insufficient correction is
achieved, a formal open release
may be needed (c). Severe
relapses need more radical forms
of treatment such as the Ilizarov
fixator (d). After successful
correction of deformity, relapses
may be prevented by using
Dennis Browne boots in infants
(e) or moulded ankle–foot
orthoses (f) in older children.
(a) (d)
(e)
(b)
594 (c) (f)

Correction of the forefoot deformity is carried out METATARSUS ADDUCTUS 21
by releasing the contractures around the talonavicular
and calcaneocuboid joints. The interosseous ligament Metatarsus adductus varies from a slightly curved
in the sinus canal should be preserved, especially in forefoot to something resembling a mild club-foot.
children with ligamentous laxity, as division may lead The majority (90 per cent) either improve sponta-
to overcorrection. Finally, the origin of the intrinsic neously or can be managed non-operatively using
The ankle and foot

muscles and plantar fascia from the calcaneum may serial corrective casts followed by straight-last shoes.
need to be divided to reduce any cavus or plantaris The more severe examples need operation. Extensive
deformity. capsulectomies of the tarso-metatarsal joints followed
The foot, in its corrected position, is immobilized by prolonged splintage have fallen out of favour
in a plaster cast. K-wires are sometimes inserted across because of the risk of early degenerative arthritis in the
the talonavicular and subtalar joints to augment the repositioned joints. Variations of the Dilwyn Evans
hold. The wires and cast are removed at 6–8 weeks, procedure (which aims to balance the lengths of the
after which hobble boots or a custom-made ankle– medial and lateral columns of the foot), often in com-
foot orthosis is used, depending on whether the child bination with basal metatarsal osteotomies, are suit-
has started walking. Stretching exercises that were able for the small percentage of children who require
performed prior to surgery are continued. The period surgical treatment.
of splintage varies: some surgeons wait until active
dorsiflexion and eversion are established whereas oth-
ers recommend some form of splintage until skeletal TALIPES CALCANEOVALGUS
maturity.
Calcaneovalgus is a common deformity that presents
LATE OR RELAPSED CLUB-FOOT in the newborn as an acutely dorsiflexed foot. There is
Late presenters often have severe deformities with sec- a deep crease (or several wrinkles) on the front of the
ondary bony changes, and the relapsed club-foot is ankle, and the calcaneum juts out posteriorly. Unlike
complicated by scarring from previous surgery. If the congenital vertical talus (which also presents as an
child is young (aged 4–7), a revision of the soft tissue acutely dorsiflexed foot) this deformity is flexible. In
releases may be considered together with a shortening addition, the anterior creases in congenital vertical
of the lateral side of the foot by calcaneo-cuboid talus are located over the midfoot.
fusion or cuboid enucleation (The Dilwyn–Evans Calcaneovalgus is usually bilateral. There is an asso-
operation – Evans, 1961). Calcaneal osteotomies, in ciation with hip dysplasia, especially if it presents on
the form of lateral closing wedges or lateral transla- one side only; examination of the hips followed by
tions, improve heel varus. Tendon transfers, once ultrasound or x-ray examination is therefore recom-
popular, now have a more limited role; a split tibialis mended.
anterior tendon transfer to the dorsum of the base of This is a postural deformity, probably due to abnor-
the fourth metatarsal may help balance weak evertors, mal intrauterine positioning, and it often corrects
whereas a transfer of tibialis posterior through the spontaneously in the neonatal period. Severe deformi-
interosseous membrane to the dorsum will act as a ties occasionally require serial casts for correction.
dorsiflexor in neurological cases. Tendon transfers
work well only if the joints are mobile, and this is sel-
dom the case in these patients.
Gradual correction by means of a circular external
fixator (the Ilizarov method) has gained popularity in
treating difficult relapsed cases and severe deformities;
the early results are encouraging. Full corrections can
be achieved even in feet severely scarred from previous
surgery, and there is often an increase in the size of
the foot, which is thought to be due to an increase in
the blood supply during distraction. The procedure
can be painful and long and for the time being it is
best reserved for these very difficult cases.
Despite initially successful surgery, deformities do
still recur. A deformed, stiff and painful foot in an
adolescent is best salvaged by corrective osteotomies
and fusions. The distorted anatomy makes triple
arthrodesis a real challenge but it is possible to end up 21.9 Metatarsus adductus In contrast to club-foot, the
with a plantigrade, stable and pain-free foot. deformity here is limited to the forefoot. 595
21 foot the dorsally subluxated navicular returns to the
normal position.
Treatment The only effective treatment is by
operation, ideally before the age of 2 years. Correction
is done in one stage through separate incisions. The
tendo Achillis is lengthened, with capsulotomies of the

ankle and subtalar joints; via a medial approach the
talonavicular joint is reduced and the tibialis anterior
tendon is transferred to the neck of the talus; if
necessary, the lateral structures are lengthened or
released. The reduced position is held with a K-wire
transfixing the talonavicular joint and plaster
immobilization for 8–12 weeks (the wire can be
removed at 6 weeks). Reasonably good results have
21.10 Talipes calcaneovalgus Bilateral calcaneovalgus. been reported with this method (Duncan and Fixsen,
This benign ‘deformity’ can be easily corrected without 1999).
hurting the baby. Over time it usually corrects
spontaneously.
PES PLANUS AND PES VALGUS

(‘FLAT-FOOT’)
CONGENITAL CONVEX PES VALGUS
(CONGENITAL VERTICAL TALUS) “Our feet are no more alike than our faces.” This tru-
ism from a British Medical Journal editorial sums up the
This rare condition is seen in infants, usually affecting problem of ‘normally abnormal’ feet. The medial arch
both feet. Superficially it resembles other types of val- may be normally high or normally low. The term ‘flat-
gus foot, but the deformity is more severe; the medial foot’ applies when the apex of the arch has collapsed
arch is not only flat, it is the most prominent part of and the medial border of the foot is in contact (or
the sole, producing the appearance of a rocker-bot- nearly in contact) with the ground; the heel becomes
tom foot. The hindfoot is in equinus and valgus and valgus and the foot pronates at the subtalar-midtarsal
the talus points almost vertically towards the sole; the complex. The problems associated with flat-foot differ
forefoot is abducted, pronated and dorsiflexed, with in babies, children and adults and these three categories
subluxation of the talonavicular joint. Passive correc- will therefore be considered separately.
tion is impossible; by the time the child is seen, the
tendons and ligaments on the dorsolateral side of the
foot are usually shortened. FLAT-FOOT IN CHILDREN AND
X-ray features The calcaneum is in equinus and the ADOLESCENTS
talus points into the sole of the foot, with the navicular
dislocated dorsally onto the neck of the talus. It is Flat-foot is a common complaint among children. Or
important to repeat the lateral x-ray with the foot rather their parents, grandparents, and assistants in
maximally plantarflexed; in congenital vertical talus the the shoe-shop – the children themselves usually don’t
appearance will be unchanged, whereas in flexible flat- seem to notice it!
21.11 Congenital vertical talus

(a) The infant’s foot is in marked
valgus and has a rocker-bottom shape.
The deformity is rigid and cannot be
corrected. (b) X-ray shows the vertical
talus pointing downwards towards the
sole and the other tarsal bones rotated
around the head of the talus.
596 (a) (b)

Flexible flat-foot Flexible pes valgus appears in toddlers 21
as a normal stage in development, and it usually
disappears after a few years, when medial arch
development is complete; occasionally, though, it
persists into adult life. The arch can often be restored
by simply dorsiflexing the great toe (Jack’s test), and
The ankle and foot

during this manoeuvre the tibia rotates externally
(Rose et al., 1985). Many of these children have
ligamentous laxity and there may be a family history of
both flat feet and joint hypermobility.
Stiff (or ‘rigid’) flat-foot A deformity that cannot be
corrected passively should alert the examiner to an (a) (b)
underlying abnormality. Congenital vertical talus is
dealt with earlier. In older children, conditions to be 21.12 Mobile flat feet (a) Standing with the feet flat on
the floor, the medial arches appear to have dropped and
considered are: (1) tarsal coalition; (2) an
the heels are in valgus. (b) When the patient goes up on
inflammatory joint disorder; (3) a neurological disorder. his toes, the medial arches are restored, indicating that
these are ‘mobile’ flat feet. If this does not occur, look
Compensatory flat-foot This is a spurious deformity
carefully for a tarsal coalition.
that occurs in order to accommodate some other
postural defect. For example, a tight tendo Achillis (or
a mild fixed equinus) may be accommodated by
everting the foot; or if the lower limbs are externally extensor tendons are in spasm. X-rays and computed
rotated the body weight falls anteromedial to the ankle tomography (CT) may show one or several of a vari-
and the feet go into valgus – the Charlie Chaplin look. ety of unions or partial unions between adjacent tarsal
bones; the commonest are talocalcaneal, calcaneonav-
icular and talonavicular coalitions. The anomaly is
Clinical assessment inherited as an autosomal dominant condition and is
Although there is usually nothing to worry about, the present at birth but it becomes symptomatic only
parents’ concerns should not be dismissed without a when the abnormal fibrous syndesmosis matures into
proper assessment of the child. Enquire about neona- a stiffer, cartilaginous synchondrosis that later ossifies
tal problems and a family history. to become a rigid bar.
Watch the child stand and note the position of the The child, usually at puberty or during early ado-
heels from behind. Are they in neutral or valgus, and lescence, develops an increasingly stiff flat-foot defor-
do they invert when the child stands on tiptoe? The mity. Pain may be due to abnormal tarsal stress or
tiptoe test will confirm a mobile subtalar joint and even fracture of an ossified bar. The picture differs
functioning tibialis posterior tendon. Let the child from that of the more common ‘idiopathic’ flat-foot
walk: is the gait normal for the child’s age? Are the in that the deformity is more or less rigid, with spasm
heels set flat during the stance phase, or does the child of the peroneal muscles. The diagnosis is confirmed
have tight Achilles tendons? by x-ray and/or CT, but other causes of rigid flat-foot
Examine the foot and note its shape. In the neonate, must be excluded (e.g. inflammatory arthritis and
the rare congenital vertical talus presents as a stiff, acutely infection of the hind- or midfoot).
dorsiflexed and very flat (almost rocker-bottom) foot.
Palpate for tenderness: are there signs of arthritis or in-
fection? Test the movements in the ankle as well as the
subtalar and midtarsal joints: a tight Achilles tendon may
be ‘constitutional’ or part of a neuromuscular problem.
Try to correct the flat-foot by gentle passive manip-
ulation. Perform Jack’s test (see earlier) to distinguish
between a flexible and a stiff (‘rigid’) deformity.
The spine, hips and knees also should be examined.
The clinical assessment is completed by a swift general
examination for joint hypermobility and signs of neu-
romuscular abnormalities.
(a) (b)
PERONEAL SPASTIC FLAT-FOOT (TARSAL COALITION) 21.13 Tarsal coalition (a) X-ray appearance of a
Older children and teenagers sometimes present with calcaneonavicular bar. (b) CT image showing incompletely
a painful, rigid flat-foot in which the peroneal and ossified talocalcaneal bars bilaterally (arrows). 597
21 Imaging be conservative. A walking plaster is applied with the
foot plantigrade and is retained for 6 weeks; splintage
X-rays are unnecessary for asymptomatic, flexible flat with an outside iron and inside T-strap may have to be
feet. For pathological flat feet (which are usually continued for another 3–6 months. Obviously if an
painful or stiff) standing anteroposterior, lateral and inflammatory joint disorder is discovered, this will have
oblique views may help to identify underlying disor- to be treated. If symptoms do not settle, operative
ders. On the lateral view, ‘beaking’ of the head of the treatment is needed. A calcaneonavicular bar can be
talus suggests the presence of a tarsal coalition. Nar- resected without much difficulty through a lateral
rowing of the talocalcaneal joint, which is sometimes approach, and the operation may be performed before
seen in talocalcaneal coalition, is easily mistaken for puberty; a portion of the bar is removed and the gap
‘arthritis’. Calcaneonavicular bars, if ossified, can be filled with fat or a piece of muscle (e.g. extensor
seen in oblique views of the foot. digitorum brevis) to prevent recurrence. Talocalcaneal
CT scanning is the most reliable way of demon- coalitions are more difficult to deal with and it may be
strating tarsal coalitions. wiser to wait until after the patient reaches puberty and
Radioscintigraphy is occasionally used if a covert then perform a triple arthrodesis.
infection or osteoid osteoma is suspected. It may also
help to identify a ‘hot’ accessory navicular before
advocating its removal.
FLAT-FOOT IN ADULTS
As in children, the usual picture is of a flexible flat-
Treatment foot with no obvious cause. However, underlying dis-
orders are common enough to always warrant a
Physiological flat-foot Young children with flexible flat
careful search for abnormal ligamentous laxity, tarsal
feet require no treatment. Parents need to be reassured
coalitions, disorders of the tibialis posterior tendon,
and told that the ‘deformity’ will probably correct itself
post-traumatic deformity, degenerative arthritis, neu-
in time; even if it does not fully correct, function is
ropathy and conditions resulting in muscular imbal-
unlikely to be impaired. Some parents will cite
ance.
examples of other children who were helped by insoles
Painful acquired flat-foot often results from tibialis
or moulded heel-cups. These appliances serve mainly
posterior dysfunction. Tibialis posterior tendon dys-
to alter the pattern of weightbearing and hence that of
function affects predominantly women in later
shoe wear; simply put, they are more effective in
midlife. It is usually of insidious onset, affecting one
treating the shoes than the feet.
foot much more than the other, and with identifiable
Tight tendo AchillisFlat-foot associated with a tight systemic factors such as obesity, diabetes, steroids or
tendo Achillis and restricted dorsiflexion at the ankle surgery. There may be recollection of a minor episode
may benefit from tendon-stretching exercises. of trauma, such as a twisting injury to the foot. The
patient experiences aching discomfort in the line of
Accessory navicular Sometimes the main complaint
the tibialis posterior tendon, often radiating up the
(with a flexible flat-foot) is tenderness over an
inner aspect of the lower leg. The foot often feels
unusually prominent navicular on the medial border of
‘tired’. As the tendon stretches out the foot drifts into
the midfoot. X-rays may show an extra ossicle at this
plano-valgus, producing the typical acquired flat-foot
site – the accessory navicular. Symptoms are due to
deformity. As the tendon ruptures the ache or pain
pressure (and possibly a ‘bursitis’) over the bony
will often improve, temporarily, but as the foot defor-
prominence, or repetitive strain at the synchondrosis
mity then worsens the plantar fascia becomes painful
between the accessory ossicle and the navicular proper.
and there may be lateral hindfoot pain as the fibula
If symptoms warrant it, the accessory bone can be
starts to impinge against the calcaneum.
shelled out from within the tibialis posterior tendon.
If the medial arch has ‘dropped’ significantly, the
tibialis posterior tendon can be used as a ‘hitch’ by re- Pathology
inserting it through a hole drilled in the navicular and
The tibialis posterior is a powerful muscle, with a
suturing the loop with the foot held in maximum
short excursion of its tendon and a strong mechanical
inversion (Kidner’s operation).
advantage as a foot inverter acting to help maintain
Rigid flat-foot (tarsal coalition) One of the problems with the medial longitudinal arch of the foot. This tendon
treatment of this condition is that the presence of a is probably inflamed more commonly and ruptures
tarsal coalition is not necessarily the cause of the more frequently than the Achilles tendon. There is
patient’s symptoms; the anomaly is sometimes usually an initial tenosynovitis. Tendon elongation
discovered as an incidental finding in asymptomatic and rupture are probably related to an area of hypo-
598 feet. For this reason the initial treatment should always vascularity in the tendon. Once the tendon elongates
21
The ankle and foot

(a) (b) (c)
21.14 Flat-foot in adults – clinical features (a) The medial arches have dropped and the feet appear to be pronated.
(b) The medial border of the foot is flat and the tuberosity of the navicular looks prominent. (c) The heels are in valgus and
the toes are visible lateral to the outer edge of the heel on the left side (the ‘too-many-toes’ sign).
the pathology is then related to the loss of powerful posterior cannot stabilize and invert the heel, impair-
hindfoot inversion, probably confounded by associ- ing the heel-raise action of the Achilles tendon.
ated stretching of the related ligaments, in particular
the spring ligament and the plantar fascia.
Imaging
Weightbearing x-rays show the altered foot axes. The
Examination tendon can be assessed with ultrasound or magnetic
There is usually swelling and tenderness in the line of resonance imaging (MRI) scan.
tibialis posterior, at and distal to the medial malleolus.
The hindfoot collapse is best appreciated by viewing
Treatment
the patient from behind, when the valgus deformity of
the heel is appreciated, and the forefoot abduction The key point is to recognize the condition. If it is in
leads to ‘too many toes’ being seen from this position, the early stages then relative rest (sticks or crutches),
compared to the contralateral foot. It is difficult for support with a temporary insole, elasticated foot/
the patient to do a single leg heel raise, as the tibialis ankle support and oral non-steroidal anti-inflamma-
tory drugs (NSAIDs) may be effective. Whether or
not to inject the tendon sheath with corticosteroid is
contentious; but to inject the tendon itself is just plain
wrong! These temporary measures may offer the
opportunity to institute more permanent solutions,
such as modification of weight and activity, and assess-
ment for definitive orthotics.
ORTHOSES
Functional foot orthoses (FFOs) have a role to play in
the adult flexible but symptomatic flat-foot. These
appliances (usually called orthotics) are used to correct
abnormal foot function or biomechanics and, in so
doing, they also correct for abnormal lower extremity
function; they are very much more than an ‘arch sup-
port’.
Orthotics are useful in the treatment of a range of
painful conditions of the foot and lower extremities,
(a) (b)
in particular first MTP joint arthritis, metatarsalgia,
21.15 Footprints Footprints made with the aid of an ink arch and instep pain, ankle pain and heel pain.
pad show the difference between normal sole contact and Since abnormal foot function may cause abnormal
flat-footed contact. (a) Normal footprint, showing the leg, knee and hip function, orthotics can be used to
main contact areas across the anterior metatarsal arch, the
lateral border of the foot and the heel, with a ‘hollow’ treat painful tendinitis and bursitis conditions in the
corresponding to the medial arch. (b) Flat-footed contact, ankle, knee and hip, as well as exercise-induced leg
across the sole to the medial side of the foot. pain (‘shin splints’). Some types of FFOs are also 599
21 designed to accommodate painful areas on the soles of neuropathies and spinal cord abnormalities (tethered
the feet (like accommodative foot orthoses). cord syndrome, diastematomyelia) are the common-
Orthoses may be made of flexible, semi-rigid or est in Western countries but poliomyelitis is the most
rigid plastic or graphite materials. They are relatively common cause worldwide. Occasionally the deformity
thin and fit easily into several types of shoe. They are follows trauma – burns or a compartment syndrome
fabricated from a three-dimensional model of the foot resulting in Volkmann’s contracture of the sole.
or scanning the foot with a mechanical or optical

scanner.
NEUROMUSCULAR CAUSES OF PES CAVUS
Assessment for orthotics can be performed by a
podiatrist, who can also advise on whether the Muscular dystrophies Duchenne, Becker
usual/intended footwear will accommodate such a
device and offer the support needed for it to be effec- Neuropathies HMSN I and II
tive. Cord lesions Poliomyelitis,
‘Off-the-shelf’ insoles are cheaper, but there are syringomyelia, diastom-
several advantages to prescription foot orthoses. They atomyelia, tethered cord
are custom-made to precisely fit each foot, and are
made in relatively rigid, durable materials with a min- Cerebral disorders Cerebral palsy, Friedreich’s
imal chance of discomfort or irritation to the foot and ataxia
a greater potential to relieve pain.
PHYSIOTHERAPY
Local treatment of the associated inflammation with
Pathology
physiotherapy might be of benefit. Assessment of the The toes are drawn up into a ‘clawed’ position, the
hindfoot biomechanics by a podiatrist might help to metatarsal heads are forced down into the sole and the
prevent progression, and could offer protection to the arch at the midfoot is accentuated. Often the heel is
contralateral side, which is often much less severely inverted and the soft tissues in the sole are tight.
affected. Under the prominent metatarsal heads callosities may
form.
SURGERY
If the condition does not improve with a few weeks of
conservative treatment, or the patient presents several
Clinical features
months after onset of the symptoms, then surgical Patients usually present at the age of 8–10 years.
intervention should be considered. Options include Deformity may be noticed by the parents or the
surgical decompression and tenosynovestomy, or school doctor before there are any symptoms. There
reconstruction of the tendon. The latter is often com- may be a past history of a spinal disorder, or a family
bined with a calcaneal osteotomy to help to protect history of neuromuscular defects. As a rule both feet
the tendon and improve the axis. If there is already are affected.
degeneration in the hindfoot joints then triple Pain may be felt under the metatarsal heads or over
arthrodesis might be indicated (fusing the subtalar, the toes where shoe pressure is most marked. Callosi-
calcaneo-cuboid and talonavicular joints – the ankle ties appear at the same sites and walking tolerance is
joint is not arthrodesed in this procedure, so foot reduced. Enquire about symptoms of neurological
plantarflexion and dorsiflexion are maintained). disorders, such as muscle weakness and joint instabil-
ity.
The overall cavus deformity is usually obvious; in
addition the toes are often clawed and the heel may be
PES CAVUS (HIGH-ARCHED FEET) varus. Closer inspection will show the components of
the high arch; this is important because it leads to an
In pes cavus the arch is higher than normal, and often understanding of the responsible deforming forces.
there is also clawing of the toes. The close resem- Rang (1993) presented a tripod analogy that simpli-
blance to deformities seen in neurological disorders fies the problem. The foot is likened to a tripod of
where the intrinsic muscles are weak or paralyzed sug- which the calcaneus, fifth metatarsal and first
gests that all forms of pes cavus are due to some type metatarsal form the legs. Combinations of deformities
of muscle imbalance. There are rare congenital causes, affecting one or more of these ‘legs’ produce the
such as arthrogryposis, but in the majority of cases pes common types of high arch, namely plantaris, cavo-
cavus results from an acquired neuromuscular dis- varus, calcaneus and calcaneo-cavus (Fig. 21.17).
order see Box opposite. A specific abnormality can The toes are held cocked up, with hyperextension at
600 often be identified; hereditary motor and sensory the MTP joints and flexion at the IP joints. There may
21
The ankle and foot

(a) (b) (c)
21.16 Pes cavus and claw-toes (a) Typical appearance of ‘idiopathic’ pes cavus. Note the high arch and claw-toes.
(b) This is associated with varus heels. (c) Look for callosities under the metatarsal heads.
be callosities under the metatarsal heads and corns on individual joints. On the lateral view, measurement of
the toes. Early on the toe deformities are ‘mobile’ and the calcaneal pitch and Meary’s angle help to deter-
can be corrected passively by pressure under the mine the components of the high arch (Fig. 21.19).
metatarsal heads; as the forefoot lifts, the toes flatten In a normal foot the calcaneal pitch is between 10 and
out automatically. Later the deformities become fixed, 30 degrees, whereas Meary’s angle, formed by the
with the MTP joints permanently dislocated. axes of the talus and first metatarsal, is zero, i.e. these
Mobility in the ankle and foot joints is important. axes are parallel. In a calcaneus deformity, the cal-
In the cavo-varus foot, the heel is inverted. The block caneal pitch is increased; in a plantaris deformity,
test (Coleman et al., 1984) is useful to check if the Meary’s lines meet at an angle.
deformity is reversible (Fig. 21.18); if it is, this signi- MRI scans of the spine will exclude a structural dis-
fies that the subtalar joint is mobile. If the cavus defor- order, especially if this is more common than polio as
mity has been present for a long time, then a cause of high-arched feet in the region.
movements of the ankle, subtalar and midtarsal joints
are usually limited.
A neurological examination is important to try to Treatment
identify a reason for the deformity. Disorders such as
Often no treatment is required; apart from the diffi-
hereditary sensory and motor neuropathy and
culty of fitting shoes, the patient has no complaints.
Friedreich’s ataxia must always be excluded, and the
spine should be examined for signs of dysraphism. Foot deformity In general, patients need treatment
only if they have symptoms. However, the problem
with high-arched feet is that it is often a progressive
Imaging disorder that becomes more difficult to treat when the
Weightbearing x-rays of the foot contribute further to deformities are fixed; therefore treatment should start
the assessment of the deformity and the state of the before the feet become stiff. Non-operative treatment
normal plantaris cavo-varus calcaneus calcaneo-cavus
Tibia
5th MT
1st MT
OS Calcis
(a) (b) (c) (d) (e)
21.17 The tripod analogy for high-arched feet This simplifies understanding of the various types of pes cavus. (a) The
calcaneum, first and fifth metatarsals of the foot are likened to the spokes of a tripod. (b) When the first and fifth rays are
drawn closer to the heel, a plantaris deformity is present. In a cavo-varus deformity (c), the first ray alone is drawn towards
the heel, which itself is in varus. In calcaneus (d), the heel is pushed plantarwards. Finally, a calcaneo-cavus deformity is
present (e) when the heel is in calcaneus and the first ray is drawn in. 601
21 21.18 Coleman’s block test This simple test is
used on a high-arched foot to see if the heel is
flexible. (a) Normal stance showing the varus
position of the heel. (b) With the patient standing
on a low block to permit the depressed first
metatarsal to hang free, the heel varus is
automatically corrected if the subtalar joint is
mobile.
(a) (b)
An equinus contracture is dealt with by lengthening

of the tendo Achillis and posterior capsulotomies of
the ankle and subtalar joints. The varus hindfoot, if
shown to be reversible by Coleman’s block test, may
benefit from a release of the plantar fascia (the tight
fascia acts as a contracted windlass on weightbearing,
accentuating the deformity). However, if the subtalar
joint is stiff, then calcaneal osteotomy will be needed;
two types are commonly used: (1) the lateral closing
wedge (an opening wedge on the medial side is a
comparable operation but is fraught with wound
problems); (2) a lateral translation osteotomy.
Treatment of a calcaneo-cavus deformity (which is
21.19 Weightbearing x-rays in foot deformities Non- the least common type of high arch) differs according
weightbearing films are notorious for ‘hiding’ the true to the age of the child. In young children (who usu-
components of foot deformities. In standing lateral views,
some measurements are useful in describing the type of
ally have a neurological problem) tendon transfers,
high-arched foot: (a) the axes of the talus and first e.g. transferring the tibialis anterior through the
metatarsal are parallel in normal feet but cross each other interosseous membrane to the calcaneum, may be
in a plantaris deformity (Meary’s angle); (b) the calcaneal combined with tenodesis of the ankle using the tendo
pitch is greater than 30 degrees in calcaneus deformities. Achillis (Banta et al., 1981). Older children may need
crescentic calcaneal osteotomies, which will correct
in the form of custom-made shoes with moulded both varus and calcaneus deformities (Samilson,
inserts may provide some relief but does not alter the 1976) or variations of a triple arthrodesis (Cholmeley,
deformity or influence its progression. Surgery is often 1953).
needed and the type of procedure will depend on the Midfoot deformities are usually cavus (plantarflexed
child’s age, underlying cause, site and flexibility of the first metatarsal) or plantaris (plantarflexed first and
individual deformities and type of muscle imbalance. fifth metatarsals). The Jones tendon transfer helps ele-
The aim of surgery is to provide a pain-free, planti- vate the depressed first metatarsal by using the exten-
grade, supple but stable foot. The methods available sor hallucis longus tendon as a sling through the neck
are soft tissue releases, osteotomies and tendon trans- of the first metatarsal. Often the peroneus longus is
fers. However, the deformity first needs to be cor- overactive and is partly responsible for pulling the first
rected before a tendon transfer is considered; metatarsal down; some balance is restored by dividing
additionally, the transfer only works if the joints are this tendon on the lateral side of the foot and attach-
mobile. ing the proximal end to the peroneus brevis, thereby
21.20 Treatment of pes cavus 1 In

a normal foot (a), the point of
contact of the heel is slightly lateral
to the centre of the ankle, producing
an eversion lever when weight is
borne. In a varus heel (b) excising a
wedge of bone from the lateral side,
or (c) performing a lateral translation
osteotomy.
602 (a) (b) (c)

21
The ankle and foot

(a) (b) (c) (d)
21.21 Treatment of pes cavus 2 (a,b) If the great toe is clawed and the first metatarsal depressed, reducing the
subluxation at the metatarsophalangeal joint by simply elevating the neck of the metatarsal often reduces the severity of
the cavus deformity. The surgical equivalent of this effect is (c,d) the Robert Jones tendon transfer: the extensor hallucis
longus tendon is detached distally and transferred to the neck of the first metatarsal; the interphalangeal joint is then either
fused or tenodesed.
removing the deforming force and improving the all in those who wear high-heeled shoes. Metatarsus
power of eversion simultaneously. Occasionally the primus varus may be congenital, or it may result from
deformity affecting the first metatarsal is fixed, in loss of muscle tone in the forefoot in elderly people.
which case a dorsal closing wedge osteotomy at the Hallux valgus is also common in rheumatoid arthritis,
base of the metatarsal is needed. A plantaris deformity probably due to weakness of the joint capsule and
is treated along similar lines for the first ray, and com- ligaments. Heredity plays an important part; a positive
bined with a plantar fascia release if the deformity is family history is obtained in over 60 per cent of
mobile, but basal metatarsal osteotomies or even a cases.
wedge resection and arthrodesis across the midfoot
are needed for rigid deformities.
In severe examples and in those who have either
relapsed or who have responded poorly with soft tis- The elements of the deformity are lateral deviation
sue releases and osteotomies, salvage surgery in the and rotation of the hallux, together with a promi-
form of a triple arthrodesis is recommended; it pro- nence of the medial side of the head of the first
duces a stiff but plantigrade and pain-free foot. metatarsal (a bunion). Lateral deviation of the hallux
may lead to overcrowding and deformity of the other
Clawed toes Correction of a clawed first toe is by the
toes and sometimes overriding of adjacent toes. When
Jones tendon transfer, which involves either a tenodesis
the valgus deformity exceeds 30 or 40 degrees, the
or fusion of the IP joint. Clawing of the lesser toes is
great toe rotates into pronation so that the nail faces
treated with a flexor tendon transfer to the extensor
medially and the sessamoid bones of flexor hallucis
hood of each toe, and MTP joint capsulotomies if the
brevis are displaced laterally; in severe deformities the
toes are still passively correctable; however, if the
tendons of flexor and extensor hallucis longus bow-
deformities are fixed, proximal IP fusion is needed.
string on the lateral side, thus adding to the deform-
ing forces. The contracted adductor hallucis and
lateral capsule contribute further to the fixed valgus
deformity.
HALLUX VALGUS Prominence of the first metatarsal head is due to
subluxation of the MTP joint; increasing shoe pres-
Hallux valgus is the commonest of the foot deformi- sure on the medial side leads to the development of an
ties (and probably of all musculoskeletal deformities). overlying bursa and thickened soft tissues, additional
In people who have never worn shoes the big toe is in changes that combine to form the defining ‘bunion’
line with the first metatarsal, retaining the slightly fan- that eventually accompanies the great-toe deformity.
shaped appearance of the forefoot. In people who When exposed at operation, the medial prominence
habitually wear shoes the hallux assumes a valgus looks like an exostosis (because of a deep sagittal sul-
position; but only if the angulation is excessive is it cus on the head of the metatarsal) but there is no true
referred to as ‘hallux valgus’. exostosis.
Splaying of the forefoot, with varus angulation of In longstanding cases the MTP joint becomes
the first metatarsal, predisposes to lateral angulation osteoarthritic and osteophytes may then add to the
of the big toe in people wearing shoes – and most of prominence of the metatarsal head. 603
21 21.22 Hallux valgus (a,b) This
girl’s feet are well on the way to
becoming as deformed as those
of her mother (c,d). Hallux valgus
is not uncommonly familial.
X-rays should be taken with the
patient standing to show the true
metatarsal and digital angulation.
(a) (b)
(c) (d)
Clinical features (2) those in whom the articular surfaces are not con-
gruent, the phalangeal surface being tilted towards
The commonest complaints are pain over the bunion, valgus; (3) those in whom the joint is both incongru-
worries about cosmesis and difficulty fitting shoes. ent and slightly subluxated (Fig. 21.23). Type 1 is a
Often there is also deformity of the lesser toes and stable joint and any deformity is likely to progress very
pain in the forefoot. With the patient standing, plano- slowly or not at all. Type 2 is somewhat unstable and
valgus hindfoot collapse may become apparent. likely to progress. Type 3 is even more unstable and
The great toe is in valgus and the bunion varies in almost certain to progress.
appearance from a slight prominence over the medial
side of the first metatarsal head to a red and angry-
looking bulge that is tender. The MTP joint often Treatment
retains a good range of movement, but in longstand- ADOLESCENTS
ing cases it may be osteoarthritic. Many young patients are asymptomatic, but worry
Always check the circulation and sensation. over the shape of the toe and an anxious mother keen
not to let the condition become as severe as her own
will bring the patient to the clinic. It is wise to try
X-rays
conservative measures first, mainly because surgical
Standing views will show the degree of metatarsal and correction in this age group carries a 20–40 per cent
hallux angulation. Lines are drawn along the middle recurrence rate. This consists essentially of encourag-
of the first and second metatarsals and the proximal ing the patient to wear shoes with wide and deep toe-
phalanx of the great toe; normally the intermetatarsal boxes, soft uppers and low heels – ‘trainers’ are a good
angle is less than 9 degrees and the valgus angle at the choice. If x-rays show a type 1 (congruous) deformity,
MTP joint less than 15 degrees. Any greater degree of the patient can be reassured that it will progress very
angulation should be regarded as ‘hallux valgus’. slowly, if at all. If there is an incongruous deformity,
Not all types of valgus deformity are equally pro- surgical correction will sooner or later be required.
gressive and troublesome. Based on the x-ray appear- There are a number of non-operative strategies that
ances, patients with hallux valgus can be divided into may be adopted to deal with the deformity and the
three types (Piggott, 1960): (1) those in whom the resulting limitations, but none that will get rid of the
MTP joint is normally centred but the articular sur- bunion itself. Accommodating, comfortable shoes can
604 faces, though congruent, are tilted towards valgus; help, but are not acceptable for some patients (or
21
The ankle and foot

(a) (b) (c) (d) (e)
21.23 X-rays (a) The intermetatarsal angle (between the first and second metatarsals) as well as the metatarsophalangeal
angle of the hallux are recorded. Piggott (1960) defined three types of hallux valgus, based on the position and tilt of the
first MTP articular surfaces: In normal feet (b) the articular surfaces are parallel and centred upon each other. In congruent
hallux valgus (c) the lines across the articular surfaces are still parallel and the joint is centred, but the articular surfaces are
set more obliquely to the long axes of their respective bones. In (d) the deviated type of hallux valgus, the lines are not
parallel and the articular surfaces are not congruent. In the subluxated type (e) the surfaces are neither parallel nor centred.
professions). Lace-up or Velcro-fastening shoes are metatarsal osteotomy. If the x-ray shows a congruent
better than slip-ons, and flat shoes are probably better articulation, the deformity is largely bony and therefore
than those with a raised heel. amenable to correction by a distal osteotomy.
Bunion pads (like a Polo/doughnut shape) can If the MTP articulation is incongruent the defor-
help to offload the tender bunion, but strapping and mity is in the joint and soft-tissue realignment is indi-
overnight splints are probably a waste of money with cated. The tight structures on the lateral side
no quality research to support their use. (adductor hallucis, transverse metatarsal ligament, and
Chiropody can help by taking care of the callosities lateral joint capsule) are released; the prominent bone
and skin compromise. on the medial side of the metatarsal head is pared
Podiatrists may help to correct the foot biome- down and the capsule on the medial side is reefed.
chanics, but there is no good evidence that anti- In moderate and severe deformities the hallux valgus
pronatory orthoses are effective in the longer term angle may be greater than 30 degrees and inter-
management of the bunion. Diabetic services often metatarsal angle wider than 15 degrees. If the MTP
provide specialized foot-care. joint is congruent, a distal osteotomy combined with
a corrective osteotomy of the base of the proximal
Operative treatment In the adolescent with mild phalanx (Aikin’s osteotomy) is recommended. For
deformities, where the hallux valgus angle is less than greater deformities, if the joint is subluxed, a soft-
25 degrees, correction can be obtained by either a soft- tissue adjustment is needed as well as a proximal
tissue rebalancing operation (see later) or by a metatarsal osteotomy. This basal osteotomy is carried
(a) (b) (c) (d) (e) (f)
21.24 Hallux valgus – treatment (a) Basal osteotomy with bone graft inserted. (b) Mitchell’s osteotomy. (c) Wilson’s
osteotomy. (d) Before and after basal osteotomy and capsulorrhaphy. (e) Keller’s operation. (f) Arthrodesis. 605
21 out to reduce a wide intermetatarsal angle; care is functional demands are low, treatment by excision
needed not to injure an open physis or else growth of arthroplasty is usually successful. In the classic Keller’s
the metatarsal will be stunted. operation, the proximal third of the proximal phalanx,
as well as the bunion prominence, are removed. This
ADULTS used to be the most common operation for hallux val-
In the adult, when self-care is insufficient and the gus but it has fallen into disuse because of the high
bunion is causing pain and difficulty with footwear, rate of recurrent deformity and complications such as
surgical options are appropriate. Recurrent infection loss of control over great toe movement, overload of
or ulceration are also indications for operative treat- the other metatarsals, metatarsalgia and dubious cos-
ment. metic improvement.
The type of surgery proposed will depend on the
level and extent of the deformity. This will usually
Complications
comprise: (1) an osteotomy to re-align the first
metatarsal; (2) soft tissue procedures to rebalance the Recurrent infection and ulceration are particular prob-
joint. lems in the diabetic foot and are an indication for sur-
A number of different osteotomy patterns have gery, rather than a contraindication.
been described and named after their ‘inventors’ or Transfer metatarsalgia may occur if the realign-
the pattern of bone cut (chevron, scarf etc.), or the ment or shortening of the first ray does not take
part of the metatarsal that is osteotomized (distal account of the relative lengths of the lesser
usually if there is less deformity, proximal or basal for metatarsals, which then become prominent and over-
greater deformity). These procedures are reviewed in loaded; a metatarsal stress fracture sometimes occurs.
a paper by Robinson and Limbers (2005). Forefoot corrective surgery should strive to produce a
There is convincing evidence to show that a distal balanced forefoot with appropriately distributed
osteotomy is associated with reduced pain and weightbearing.
increased ability to work in the medium to long term; Complex regional pain syndrome is a potential com-
the safety profile is good, with a less than 10 per cent plication of all foot operations.
complication rate and with many procedures being
performed as day-case operations and without plaster
immobilization in the postoperative period. Patient
satisfaction with bunion surgery is generally good, HALLUX RIGIDUS
with 75 per cent being satisfied with the outcome.
‘Rigidity’ (or stiffness) of the first MTP joint occurs at
ELDERLY PATIENTS almost any age from adolescence onwards. In young
Hallux valgus in the elderly is best treated by shoe people it may be due to local trauma or osteochron-
modifications; where this fails, and in those whose dritis dissecans of the first metatarsal head. In older
people it is usually caused by longstanding joint dis-
orders such as gout, pseudogout or osteoarthritis
(OA), and is very often bilateral. In contrast to hallux
valgus, men and women are affected with equal fre-
quency. A family history is common.
Clinical features
Pain on walking, especially on slopes or rough
ground, is the predominant symptom. The patient
eventually develops an altered gait, trying to offload
the first MTP joint by transferring weight across to
the lesser toes; there is also impaired power in toe-off
during the gait cycle. The great toe is straight and
often has a callosity under the medial side of the dis-
tal phalanx. The MTP joint feels knobbly; a tender
dorsal ‘bunion’ (actually a large osteophyte) is diag-
nostic. Dorsiflexion is restricted and painful, and there
may be compensatory hyperextension at the interpha-
(a) (b) langeal joint. The outer side of the sole of the shoe
21.25 Hallux valgus – treatment (a) X-ray before may be unduly worn – the result of rolling the foot
606 operation. (b) X-ray after distal osteotomy. outwards to avoid pressing on the big toe.
21
The ankle and foot

(a) (b) (c)
21.26 Hallux rigidus (a) In normal walking, the big toe dorsiflexes (extends) considerably. With rigidus (b), dorsiflexion is
limited. (c) The usual cause is OA of the first MTP joint.
It is important to check the state of the other joints

in the foot in order to rule out a polyarthropathy.
X-rays The features are essentially those of OA:
narrowing of the joint space, subchondral sclerosis and
marginal osteophytes. There may be signs of recent or
old osteochondritis (‘squaring’ of the metatarsal head).
(a) (b)
Treatment 21.27 ‘Bunions’ Compare the two types of bunion:

(a) Dorsal bunion in hallux rigidus and (b) medial bunion
If the condition is not interfering with activity then it in hallux valgus.
can be left alone and the patient reassured. Intermit-
tent attacks of pain can be relieved by an intra-articu-
lar injection of corticosteroid and local anaesthetic. If, pressing against the shoe upper. Female patients may
however, the condition is painful and restricting of be concerned that they will be unable to wear shoes
activity then the risks of long-term NSAIDs must be with a higher heel if the toe is fused, but in fact the
balanced against those of surgical intervention. majority are able to wear footwear that can include
Some orthotic devices will offload or reduce move- moderate heels.
ment at the first MTP joint, but these are usually full- Arthroplasty is more controversial. Keller’s opera-
length insoles and relatively bulky – they may not fit tion (an excisional arthroplasty), carries a high risk of
in a smart shoe (at least not when the foot is in it as complications and seldom brings improvement in
well!) A rocker-soled shoe can abolish pain by allow- function; the procedure is no longer recommended.
ing the foot to ‘roll’ without the necessity for dorsi- Interposition arthroplasty has from time to time been
flexion at the MTP joint; many people are unwilling popular and can provide excellent pain relief, espe-
to wear such shoes. cially in patients with advanced OA. A simple capsular
arthroplasty is probably the safest. Silicone implants
OPERATIVE TREATMENT were often used in the past, but silicone-related com-
Pain at the first MTP joint that is intrusive or limits plications were common and the operation is no
activity should be an indication for referral. In limited longer recommended for hallux rigidus. Metallic
arthritic disease, simply removing the dorsal osteo- implants have fared better (in experienced hands) but
phyte (cheilectomy) might be effective, and may be these also produce variable long-term results.
coupled with an extension osteotomy in the proximal
phalanx, to alter the loadbearing region of the articu-
lation.
If the joint is more arthritic then a fusion or DEFORMITIES OF LESSER TOES
arthrodesis offers a good chance of returning the
patient to function, walking comfortably without a The commonest deformities of the lesser toes are
limp. The joint should be fused in 10 degrees of val- ‘claw’, ‘hammer’ and ‘mallet’. These terms are often
gus and 10–15 degrees of dorsiflexion in relation to used interchangeably, leading to confusion.
the sole of the foot, or with about 5–10 mm clearance Claw toe is characterized by hyperextension at the
between the line of the sole of the foot and the pulp MTP joint and flexion at both IP joints.
of the great toe. Too little dorsiflexion will cause pain Hammer toe is an acute flexion deformity of the
during toe-off and too much will result in the toe proximal IP joint only; in severe examples there may 607
21 be some extension at the MTP joint. The distal IP FIXED DEFORMITY
joint is either straight or hyperextended. When the deformity is fixed, it may either be accepted
Mallet toe is a flexion deformity of the distal IP joint. and accommodated by special footwear or treated by
one of the following operations:
1. Interphalangeal arthrodesis – If there is no joint
CLAW TOES
disease, proximal IP arthrodesis and dorsal

capsulotomy of the MTP joints permits active
The IP joints are flexed and the MTP joints hyperex-
flexion of the MTP joints by the long flexors. This
tended. This is an ‘intrinsic-minus’ deformity that is
is sometimes combined with transfer of the
seen in neurological disorders (e.g. peroneal muscular
extensor hallucis longus to the first metatarsal,
atrophy, poliomyelitis and peripheral neuropathies)
thus removing a deforming force while retaining
and in rheumatoid arthritis. Usually, however, no
the muscle as a forefoot stabilizer.
cause is found. The condition may also be associated
2. Joint excision – Fixed claw deformities, usually
with pes cavus.
associated with destruction of the MTP joints
(e.g. in rheumatoid arthritis), can be dealt with by
Clinical features excision arthroplasties of the MTP joints –
preferably removal of only the bases of the
The patient complains of pain in the forefoot and
proximal phalanges and trimming of the
under the metatarsal heads. Usually the condition is
metatarsal heads. This can usually be achieved
bilateral and walking may be severely restricted. At
through two longitudinal incisions on the dorsum
first the joints are mobile and can be passively cor-
of the foot. If the great toe is affected, a modified
rected; later the deformities become fixed and the
Keller’s operation is performed. The base of the
MTP joints subluxed or dislocated. Painful corns may
proximal phalanx is excised and the plantar pad
develop on the dorsum of the toes and callosities
(which is often displaced in these deformities) is
under the metatarsal heads. In the most severe cases
returned to its normal position beneath the
the skin ulcerates at the pressure sites.
metatarsal head; the space between the metatarsal
and phalanx is then filled by suturing the long
Treatment extensor tendon to the flexor.
3. Amputation – Toes that are severely contracted,
FLEXIBLE DEFORMITY
dislocated and ulcerated are worse than none. If
So long as the toes can be passively straightened the
the circulation is satisfactory and the patient is
patient may obtain relief by wearing a metatarsal sup-
willing to accept the appearance, amputation of all
port or by having a transverse metatarsal bar fitted to
ten toes is a useful palliative operation.
the shoe. A daily programme of intrinsic muscle exer-
cises is important. If these measures fail to relieve dis-
comfort, an operation is indicated. ‘Dynamic’ HAMMER TOE
correction is achieved by transferring the long toe
flexors to the extensors. The operation at one stroke The proximal IP joint is fixed in flexion, while the dis-
removes a powerful IP flexor and converts it to a tal joint and the MTP joint are extended. The second
MTP flexor and IP extensor. toe of one or both feet is commonly affected, and
(a) (b) (c) (d)
21.28 Disorders of the lesser toes (a) Hammer-toe deformity. (b,c) Claw toes. This patient suffered from peroneal
muscular atrophy, a neurological disorder causing weakness of the intrinsic muscles and cavus feet. (d) Overlapping fifth
608 toe.
hyperextension of the MTP joint may go on to dorsal transfer of the long extensor tendon beneath the 21
dislocation. Shoe pressure may produce painful corns proximal phalanx to the abductor digiti minimi
or callosities on the dorsum of the toe and under the (Lapidus, 1942).
prominent metatarsal head.
The cause is obscure: the similarity to boutonnière COCK-UP DEFORMITY
deformity of a finger suggests an extensor dysfunc- The MTP joint is dislocated and the little toe sits on
The ankle and foot

tion, a view supported by the frequent association the dorsum of the metatarsal head. Operative treat-
with a dropped metatarsal head, flat anterior arch and ment is usually successful: through a longitudinal
hallux valgus. A simpler explanation is that the toe was plantar incision, the proximal phalanx is winkled out
too long or the shoe too short. and removed; the wound is closed transversely, thus
pulling the toe out of the hyperextended position.
Treatment
TAILOR’S BUNION
Operative correction is indicated for pain or for diffi- An irritating or painful bunionette may form over an
culty with shoes. The toe is shortened and straight- abnormally prominent fifth metatarsal head. If the
ened by excising the joint. An ellipse of tissue shoe cannot be adjusted to fit the bump, the bony
(including the corn and the underlying extensor ten- prominence can be trimmed, taking care not to sever
don) is removed and the proximal IP joint is entered; the tendon of the fifth toe abductor. If the metatarsal
the articular surfaces are nibbled away and the raw shaft is bowed laterally (as is often the case), it can be
ends of the proximal and middle phalanges are straightened by performing either a distal osteotomy
brought together with the toe almost straight. The or a varus correction at the base of the metatarsal.
position is held by a longitudinally placed K-wire,
which is retained for 6 weeks. An alternative (and
some would say preferable) operation is simple exci-
sion of the head of the proximal phalanx, or excision TUBERCULOUS ARTHRITIS
of both articular surfaces, without formal arthrodesis; (see also Chapter 2)
the toe is splinted for 3 weeks to allow healing in the
corrected position. Tuberculous infection of the ankle joint begins as a
If the MTP joint is dislocated, a dorsal capsulotomy synovitis or as an osteomyelitis and, because walking
and elongation of the extensor tendon may be neces- is painful, may present before true arthritis super-
sary; the toe is held in position by driving the K-wire venes. The ankle is swollen and the calf markedly
more proximally, or by inserting a second wire. wasted; the skin feels warm and movements are
restricted. Sinus formation occurs early.
MALLET TOE
21.29 Tuberculous
In mallet toe it is the distal IP joint that is flexed. The arthritis of the ankle
toe-nail or the tip of the toe presses into the shoe, (a) The swelling of the
left ankle is best seen
resulting in a painful callosity.
from behind; (b) shows
If conservative treatment (chiropody and padding) regional osteoporosis
does not help, operation is indicated. The distal IP and joint destruction.
joint is exposed, the articular surfaces excised and the
toe straightened; flexor tenotomy may be needed. A
thin K-wire is inserted across the joint and left in posi-
tion for 6 weeks.
(a)
FIFTH TOE DEFORMITIES
OVERLAPPING FIFTH TOE
This is a common congenital anomaly (Fig. 21.28d).
If symptoms warrant, the toe may be straightened by
a dorsal V/Y-plasty, reinforced by transferring the
flexor to the extensor tendon. Tight dorsal and medial
structures may have to be released. The toe is held in
the overcorrected position with tape or K-wire for 6
weeks. Severe deformities or relapses may need a (b) 609
21 X-rays show regional osteoporosis, sometimes a Dorsal corns and plantar callosities also may break
bone abscess and, with late disease, narrowing and down and become infected. In the worst cases the
irregularity of the joint space. toes are dislocated, inflamed, ulcerated and useless.
X-rays show osteoporosis and peri-articular erosion
at the MTP joints. Curiously – in contrast to the situ-
Treatment
ation in the hand – the smaller digits (fourth and fifth
In addition to general treatment (Chapter 2) a remov- toes) are affected first.

able splint is used to rest the foot in neutral position.
If the disease is arrested early, the patient is allowed up
non-weightbearing in a calliper; gradually taking
Treatment
more weight and then discarding the calliper alto- During the stage of synovitis, corticosteroid injections
gether. Following arthritis, weightbearing is harmless, and attention to footwear may relieve symptoms;
but stiffness is inevitable and usually arthrodesis is the operative synovectomy is occasionally needed. Once
best treatment. deformity is advanced, treatment is that of the claw
toes and hallux valgus. Sometimes specially made
shoes will accommodate the toes in relative comfort.
If this does not help, the most effective operation is
excision arthroplasty in order to relieve pressure in the
RHEUMATOID ARTHRITIS sole and to correct the toe deformities. For the hallux,
(see also Chapter 3) an alternative is MTP fusion.
Forefoot surgery is more likely to succeed if the
The ankle and foot are affected almost as often as the hindfoot is held in the anatomical position. It is
wrist and hand. Early on there is synovitis of the MTP, important, therefore, to treat the foot as a whole and
intertarsal and ankle joints, as well as of the sheathed attend also to the proximal joints.
tendons (usually the peronei and tibialis posterior). As
the disease progresses, joint erosion and tendon dys-
function prepare the ground for increasingly severe ANKLE AND HINDFOOT
deformities.
The earliest symptoms are pain and swelling around
the ankle. Walking becomes increasingly difficult and,
FOREFOOT later, deformities appear. On examination, swelling
and tenderness are usually localized to the back of the
Pain and swelling of the MTP joints are among the medial malleolus (tenosynovitis of tibialis posterior)
earliest features of rheumatoid arthritis. Shoes feel or the lateral malleolus (tenosynovitis of the peronei).
uncomfortable and the patient walks less and less. Less often the ankle swells (joint synovitis) and its
Tenderness is at first localized to the MTP joints; later movements are restricted. Inversion and eversion may
the entire forefoot is painful on pressing or squeezing. be painful and limited; subtalar erosion is common. In
With increasing weakness of the intrinsic muscles and the late stages the tibialis posterior may rupture (all
joint destruction, the characteristic deformities too often this is missed), or become ineffectual with
appear: a flattened anterior arch, hallux valgus, claw progressive erosion of the tarsal joints, and the foot
toes and prominence of the metatarsal heads in the gradually drifts into severe valgus deformity. X-rays
sole (patients say it feels like walking on pebbles). show osteoporosis and, later, erosion of the tarsal and
Subcutaneous nodules are common and may ulcerate. ankle joints. Soft tissue swelling may be marked.
(a) (b) (c)

21.30 Rheumatoid arthritis (a,b) Forefoot deformities are similar to those in non-rheumatoid feet but more severe. They
are due to a combination of joint erosion and tendon attrition. (c) Swelling and deformity of the hindfoot due to a
610 combination of arthritis and tenosynovitis. In this case, both the ankle and the subtalar joints are affected.
loarthropathies. This appears in the foot as plantar 21
fasciitis and Achilles tendinitis. Splintage and local
injection of triamcinolone are helpful.
GOUT (see also Chapter 4)
The ankle and foot

Swelling, redness, heat and exquisite tenderness of the
MTP joint of the great toe (‘podagra’) is the epitome
of gout. The ankle joint, or one of the toes, may be
similarly affected – especially following a minor injury.
The condition may closely resemble septic arthritis,
but the systemic features of infection are absent. The
serum uric acid level may be raised.
(a) (b) Treatment with anti-inflammatory drugs will abort
21.31 Rupture of tibialis posterior tendon (a) This the acute attack of gout; until the pain subsides the
patient with rheumatoid arthritis suddenly developed a foot should be rested and protected from injury.
painful valgus foot on the left. (b) The deformity was well
controlled by a lightweight orthosis, and operative repair Chronic tophaceous gout Tophi may appear around any
was unnecessary. of the joints. The diagnosis is suggested by the
characteristic x-ray features and confirmed by
identifying the typical crystals in the tophus. Treatment
may require local curettage of the bone lesions.
Treatment
Plantar fasciitis Pain under the heel due to plantar
In the stage of synovitis, splintage is helpful (to allow fasciitis is another manifestation of gout, though the
inflammation to subside and to prevent deformity) association may be hard to prove in any particular case.
while waiting for systemic treatment to control the
disease. Initially, tendon sheaths and joints may be
injected with methylprednisolone, but this should not OSTEOCHONDRITIS DISSECANS OF THE
be repeated more than two or three times because of
the risk of tendon rupture. A lightweight below-knee TALUS
calliper with an inside supporting strap restores stabil-
Unexplained pain and slight limitation of movement
ity and may be worn almost indefinitely.
in the ankle of a young person may be due to a small
If the synovitis does not subside, operative synovec-
osteochondral fracture of the upper surface of the
tomy is advisable. Frayed tendons cannot be repaired
talus, though the injury may have been forgotten.
and, although tendon replacement is technically feasi-
X-rays taken at appropriate angles to produce tan-
ble, progressive erosion of the hindfoot joints will
gential views of the talar surface show the small bony
countervail any improvement this might achieve.
separation (no more than a few millimetres in dia-
In the very late stage, arthrodesis of the ankle and
meter) at either the anteromedial or posterolateral
tarsal joints can still restore modest function and abol-
part of the superior surface of the talus. MRI is also
ish pain. The place of arthroplasty is not yet firmly
helpful and the lesion may be visualized directly by
established.
arthroscopy.
SERONEGATIVE ARTHROPATHIES
The seronegative arthropathies are dealt with in
Chapter 3. These conditions are similar to rheumatoid
arthritis, but there are differences in the pattern of
joint involvement, the severity of the changes and the
soft tissue features.
The clinical features are often asymmetrical and the
ankle and hindfoot tend to be more severely affected
than the forefoot. However, in psoriatic arthritis the (a) (b)
toe joints are sometimes completely destroyed. 21.32 Gout (a) The classical image of gout in the big
An inflammatory reaction around the insertions of toe. An inflamed 1st MTP joint. (b) X-ray showing large
tendons and ligaments is a feature of the spondy- erosions due to tophi at the first metatarsal head. 611
21 ANKLE OSTEOARTHRITIS
OA of the ankle is far less common than OA of the hip

or knee; when it does occur it is almost always sec-
ondary to some underlying disorder: a malunited frac-

ture, recurrent instability, osteochondritis dissecans of
the talus, avascular necrosis of the talus or repeated
(a) (b) bleeding with haemophilia. Sometimes, however, the
21.33 Osteochondritis dissecans (a) Osteochondritis ankle is involved in generalized OA and crystal
dissecans at the common site, the anteromedial part of the arthropathy (See Chapter 4).
articular surface of the talus. (b) More extensive lesions can
lead to secondary OA of the ankle.
Clinical features
The presentation is usually with pain and stiffness
localized to the ankle, particularly noticed at ‘start
up’, when first standing up from rest. Patients often
indicate the site of pain as being transversely across
Treatment depends on the degree of cartilage dam- the front of the ankle. The ankle is usually swollen,
age. As long as the articular cartilage is intact, it is suf- with palpable anterior osteophytes and tenderness
ficient to restrict activities. Once it is softened, along the anterior joint line. Dorsiflexion (extension)
arthroscopic drilling may be helpful. A loose fragment and plantarflexion at the ankle are often restricted. If
may need to be removed, but often the symptoms are heel inversion and eversion movements are restricted
insufficient to warrant intervention. then suspect subtalar joint involvement. Gait is often
anatalgic, offloading the affected leg; the foot is often
turned outwards as the patient walks through on the
ATRAUMATIC OSTEONECROSIS OF affected ankle, to compensate for the loss of ankle
movement.
THE TALUS (see also Chapter 6) X-rays show the typical features of OA; the predis-
posing disorder is almost always easily detected.
Osteonecrosis of the talus is a well-recognized com-
plication of trauma (dislocation or fracture of the neck
of the talus). Atraumatic osteonecrosis, though less Treatment
common than its counterpart in the femoral head, is When the condition flares up, minor, generally non-
associated with the same group of systemic disorders intrusive symptoms can be managed with analgesia or
as the latter (hypercortisonism, alcoholism, systemic NSAIDs. Relative rest of the joint might be achieved
lupus erythematosus, Gaucher’s disease, sickle-cell with the use of a walking stick; weight loss might be
disease etc.) and is often one of multiple sites affected. appropriate. Activity such as walking, cycling and
Patients complain of pain, which is often aggravated swimming can be encouraged.
by weightbearing, and gradually increasing restriction
of movement. X-rays and MRI show the typical fea-
tures of osteonecrosis, almost always involving the
posterolateral part of the talar dome. Lesions can be
staged according to Ficat’s radiographic classification
(see Chapter 6). For purposes of treatment, it is
important to distinguish between ‘pre-collapse’ and
‘collapse’ of the talar dome.
Conservative treatment is sometimes effective; the
ankle is more forgiving than the hip and patients may
cope for some years on simple analgesics and
restricted weightbearing. If symptoms persist and
interfere significantly with function, operative treat- (a) (b)
ment may be needed. During the pre-collapse phase,
21.34 OA (a) The obvious malalignment that followed an
core decompression is worth trying as a first old injury has led to OA. (b) In this ankle the narrowed
approach. If this fails, ankle arthrodesis is indicated joint space and subarticular cysts are characteristic of OA;
612 (Delanois et al., 1998). the cause is not clear, though it may have been trauma.
Physiotherapy can be helpful in improving the ulceration in the foot. The skin feels smooth and cold, 21
range of movement, correcting gait and ensuring cor- the nails show trophic changes and the pulses are weak
rect use of walking aids. An ankle support or brace or absent. Doppler studies should corroborate the
may help. clinical findings. Superficial ulceration occurs on the
toes, deep ulceration typically under the heel; unlike
OPERATIVE TREATMENT neuropathic ulcers, these are painful and tender. Digital
The ankle and foot

Ankle arthritis that is interfering with the activities of vessel occlusion may cause dry gangrene of one or
daily living and limiting work, social or domestic func- more toes; proximal vascular occlusion is less common
tion warrants consideration for operative treatment. but more serious, sometimes resulting in extensive wet
Depending on the severity of the condition, ankle sur- gangrene.
gery such as arthroscopic or open removal of anterior
Peripheral neuropathy Early on, patients are usually
osteophytes (cheilectomy) might be offered, and con-
unaware of the abnormality but clinical tests will
sideration may be given to ankle arthrodesis; the ideal
discover loss of vibration and joint position sense and
position for fusion is at zero in the sagittal plane (the
diminished temperature discrimination in the feet.
foot therefore plantigrade) and 5 degrees of valgus.
Symptoms, when they occur, are mainly due to sensory
Total ankle arthroplasty is not as well established as
impairment: symmetrical numbness and paraesthesia,
hip and knee arthroplasty, but encouraging results are
dryness and blistering of the skin, superficial burns and
being reported.
skin cracks or ulceration due to shoe scuffing or
localized pressure. Motor loss usually manifests as claw
toes with high arches and this, in turn, may predispose
to plantar ulceration.
DIABETIC FOOT
Neuropathic joint disease ‘Charcot joints’ occur in less
than 1 per cent of diabetic patients, yet diabetes is the
The complications of longstanding diabetes mellitus
commonest cause of a neuropathic joint in Europe
often appear in the foot, causing chronic disability.
and America (leprosy and tertiary syphilis being the
More than 30 per cent of patients attending diabetic
other common causes worldwide). The mid-tarsal
clinics have evidence of peripheral neuropathy or vas-
joints are the most commonly affected, followed by
cular disease and about 40 per cent of non-trauma-
the MTP and ankle joints. There is usually a provoca-
related amputations in British hospitals are for
tive incident, such as a twisting injury or a fracture,
complications of diabetes.
following which the joint collapses relatively pain-
Factors affecting the foot are: (1) a predisposition
lessly. X-rays show marked and fairly rapid destruction
to peripheral vascular disease; (2) damage to periph-
of the articular surfaces. These changes are easily mis-
eral nerves; (3) reduced resistance to infection;
taken for infection but the simultaneous involvement
(4) osteoporosis.
of several small joints and the lack of systemic signs
Peripheral vascular disease Atherosclerosis affects mainly point to a neuropathic disorder. Joint aspiration and
the medium-sized vessels below the knee. The patient microbiological investigation will also help to exclude
may complain of claudication or ischaemic changes and infection.
(a) (b) (c)

21.35 The diabetic foot 1 (a) Ulceration in a patient with poorly controlled diabetes. (b,c) Despite the severe changes in
these two patients with diabetic neuropathy, the feet were relatively painless. 613
21 In late cases there may be severe deformity and loss tial, it should be retained for the shortest possible
of function. A rocker-bottom deformity from collapse period.
of the midfoot is diagnostic. Neuropathic joint disease is a major challenge.
Arthrodesis is fraught with difficulty, not least a very
Osteoporosis There is a generalized loss of bone
poor union rate, and sometimes is simply not feasible.
density in diabetes. In the foot the changes may be
‘Containment’ of the problem in a weight-relieving
severe enough to result in insufficiency fractures

orthosis may be the best option.
around the ankle or in the metatarsals.
Bone or joint infection is an ever-present risk and
Infection Diabetes, if not controlled, is known to have should be borne in mind in the differential diagnosis
a deleterious effect on white cell function. This, of insufficiency fractures and neuropathic joint ero-
combined with local ischaemia, insensitivity to skin sion. This will require urgent treatment.
injury and localized pressure due to deformity, makes
sepsis an ever-recurring hazard.
DISORDERS OF THE TENDO ACHILLIS

Management
The orthopaedic surgeon will usually be one member ACHILLES TENDINITIS
of a multidisciplinary team comprising a physician (or
endocrinologist), surgeon, chiropodist and orthotist. Athletes, joggers and hikers often develop pain and
The best way of preventing complications is to insist swelling around the tendo Achillis, due to local irrita-
on regular attendance at a diabetic clinic, full compli- tion of the tendon sheath or the paratenon.
ance with medication, examination for early signs of
vascular or neurological abnormality, advice on foot
care and footwear and a high level of skin hygiene.
Pathology
Examination for early signs of neuropathy should The condition usually affects the ‘watershed’ area
include the use of Semmes–Weinstein hairs (for test- about 4 cm above the insertion of the tendon, an area
ing skin sensibility) and a biothesiometer (for testing where the blood supply to the tendon is poorer than
vibration sense). Peripheral vascular examination is elsewhere. The tendon sheath or the flimsy tissue
enhanced by using a Doppler ultrasound probe. around it may become inflamed. In a minority of cases
Ulcers must be swabbed for infecting organisms; fre- the changes appear at the tendon insertion, or there
quently, multiple bacterial types are isolated (anaer- may be inflammation of the retrocalcaneal bursa just
obes make a regular appearance). X-ray examination above the calcaneum and deep to the tendon;
may reveal periosteal reactions, osteoporosis, cortical anatomical deformity of the posterior part of the cal-
defects near the articular margins and osteolysis – caneum may contribute to the pathogenesis.
often collectively described as ‘diabetic osteopathy’.
Great care is needed with nail trimming; skin cracks
should be kept clean and covered and ulcers should be
Clinical features
treated with local dressings and antibiotics if neces- The condition may come on gradually, or rapidly fol-
sary. Occasionally, septicaemia calls for admission to lowing a change in sporting activity (or a change of
hospital and treatment with intravenous antibiotics. sports footwear). Less commonly there is a history of
Ischaemic changes need the attention of a vascular direct trauma to the Achilles tendon. The area above
surgeon who can advise on ways of improving the the heel may look inflamed and function is inhibited
local blood supply. Arteriography may show that because of pain in the heel-cord, especially at push-
bypass surgery is feasible. Dry gangrene of the toe can off. The tendon feels thickened in the watershed area
be allowed to demarcate before local amputation; about 4 cm above its insertion. In chronic cases an
severe occlusive disease with wet gangrene may call ultrasound scan may be helpful in confirming the
for immediate amputation. diagnosis.
Indolent neuropathic ulcers require patient dress- If the onset is very sudden, suspect tendon rupture
ing and, if infected, antibiotic treatment. Total con- (see later).
tact casts may avoid the need for prolonged inpatient
stays or bed rest (Coleman et al., 1984). If a bony
‘high spot’ is identified, it should be trimmed or
Treatment
excised. Custom-made shoes with total contact If the condition starts acutely, it will often settle
insoles must follow the successful healing of these within about 6 weeks if treated appropriately. Referral
ulcers to avoid recurrence. for early physiotherapy is important. In the interim,
Insufficiency fractures should be treated, if possible, advice on rest, ice, compression and elevation (RICE)
614 without immobilizing the limb; or, if a cast is essen- and the use of an NSAID (oral or topical) are helpful.
When the symptoms improve, stretching exercises, up and stretch before sport, previous injury or ten- 21
followed by a muscle strengthening programme, dinitis and corticosteroid injection.
should be advised. The use of a removeable in-shoe
heel-raise might be helpful. If there is a plano-valgus
hindfoot, correction with orthotics will often bring Examination
about improvement and reduce the risk of recurrence.
Plantarflexion of the foot is usually inhibited and weak
The ankle and foot

When the onset is insidious and treatment is started
(although it may be possible, as the long flexors of the
late, symptoms will be prolonged and may last for 9
toes are also ankle flexors). There is often a palpable
months or longer.
gap at the site of rupture; bruising comes out a day or
Operative treatment is seldom necessary but if
two later. The calf squeeze test (Thompson’s or Sim-
symptoms fail to settle with physiotherapy then sur-
mond’s test) is diagnostic of Achilles tendon rupture:
gery may be appropriate – even more so if there is sus-
normally, with the patient prone, if the calf is
picion of an acute (or missed) tendon rupture. This
squeezed the foot will plantarflex involuntarily; if the
will involve some type of ‘decompressive’ operation.
tendon is ruptured the foot remains still.
Treatments such as radiofrequency coblation or
Clinical assessment is often sufficient. Ultrasound
extracorporeal shockwave lithotripsy are now showing
scans must be used to confirm or refute the diagnosis.
some promise.
Potential pitfalls Differential diagnosis

Injection with corticosteroids should be avoided. Incomplete tear A complete rupture is often mistaken
Tendon rupture is a real risk and could well give rise for a partial tear (which is rare). The mistake arises
to litigation. because, if a complete rupture is not seen within 24
Do not diagnose ‘partial rupture’ of the Achilles hours, the gap is difficult to feel; moreover, the patient
tendon; this should only be entertained if there is may by then be able to stand on tiptoe (just), by using
clearly some discontinuity of the tendon on ultra- his or her long toe flexors.
sound scan.
Tear of soleus muscle A tear at the musculotendinous
junction causes pain and tenderness halfway up the calf.
This recovers with the aid of physiotherapy and raising
ACHILLES TENDON RUPTURE the heel of the shoe.
A ripping or popping sensation is felt, and often

Treatment
heard, at the back of the heel. This most commonly
occurs in sports requiring an explosive push-off: If the patient is seen early, the ends of the tendon may
squash, badminton, football, tennis, netball. The approximate when the foot is passively plantarflexed.
patient will often report having looked round to see If so, a plaster cast or special boot is applied with the
who had hit them over the back of the heel, the pain foot in equinus; rehabilitation and physiotherapy
and collapse are so sudden. regimes vary, but it is probably safe, and may be bet-
The typical site for rupture is at the vascular water- ter for eventual tendon strength, to commence phys-
shed about 4 cm above the tendon insertion onto the iotherapy within 4–6 weeks. A shoe with a raised heel
calcaneum. The condition is often associated with should be worn for a further 6–8 weeks. The ‘re-rup-
poor muscle strength and flexibility, failure to warm ture rate’ is about 10 per cent.
(a) (b) (c)

21.36 Tendo Achillis (a) The soleus may tear at its musculotendinous junction (1), but the tendo Achillis itself ruptures
about 5 cm above its insertion (2). (b) The depression seen in this picture at the site of rupture later fills with blood. (c)
Simmonds’ test: both calves are being squeezed but only the left foot plantarflexes – the right tendon is ruptured. 615
21 Operative repair is associated with an earlier return Treatment
to function, better tendon and calf muscle strength
and a lower re-rupture rate. Supported rehabilitation The weakness may need no treatment at all, or only a
and physiotherapy are commenced early (within a drop-foot splint.
week or two of repair) There are, however, risks Drop-foot due to nerve palsy can be treated by
associated with operative tendon repair, including transferring the tibialis posterior through the
wound healing problems and sural nerve neuroma. interosseous membrane to the midtarsal region.
For ruptures that present late, reconstruction using Spastic paralysis is treated by tendon release and
local tendon substitutes (e.g. flexor hallucis longus transfer, but great care is needed to prevent overac-
tendon) or strips of fascia lata is still possible. tion in the new direction. Thus, a spastic equinovarus
deformity may be converted to a severe valgus defor-
mity by transferring the tibialis anterior to the lateral
side; this is avoided if only half the tendon is trans-
ferred.
Fixed deformities must be corrected first before
PARALYZED FOOT doing tendon transfers. If no adequate tendon is avail-
able to permit dynamic correction, the joint may be
Weakness or paralysis of the foot may be symptomless, reshaped and arthrodesed; at the same time muscle
or may present in one of three characteristic ways: the rebalancing (even of weak muscles) is necessary, oth-
patient may: (1) complain of difficulty in walking; erwise the deformity will recur.
(2) ‘catch his toe’ on climbing stairs (due to weak
dorsiflexion); (3) stumble and fall (due to instability).
Clinical features PAINFUL ANKLE

Upper motor neuron lesions Spastic paralysis may occur Except after trauma or in rheumatoid arthritis, persist-
in children with cerebral palsy or in adults following a ent pain around the ankle usually originates in one of
stroke. Muscle imbalance usually leads to equinus or the peri-articular structures or the talus rather than
equinovarus deformity. The reflexes are brisk but the joint itself. Conditions to be looked for are chronic
sensation is normal. The entire limb (or both lower ligamentous instability, tenosynovitis of the tibialis pos-
limbs) is usually abnormal. terior or peroneal tendons, rupture of the tibialis pos-
terior tendon, osteochondritis dissecans of the dome of
Lower motor neuron lesions Poliomyelitis was (and in
the talus or avascular necrosis of the talus.
some parts of the world still is) a common cause of
foot paralysis. If all muscle groups are affected, the Tenosynovitis Tenderness and swelling are localized to
foot is flail and dangles from the ankle; if knee the affected tendon, and pain is aggravated by active
extension is also weak, the patient cannot walk with- movement – inversion or eversion against resistance.
out a calliper. With unbalanced weakness, the foot Local injection of corticosteroid usually helps.
develops fixed deformity; it may also be smaller and
Rupture of tibialis posterior tendon Pain starts quite
colder than normal, but sensation is normal. Other
suddenly and sometimes the patient gives a history of
lower motor neuron disorders such as spinal cord
having felt the tendon snap. The heel is in valgus
tumours, peroneal muscular atrophy and severe nerve
during weightbearing; the area around the medial
root compression are rare causes of foot weakness or
malleolus is tender and active inversion of the ankle is
deformity.
both painful and weak. In physically active patients,
operative repair or tendon transfer using the tendon of
Peripheral nerve injuries The sciatic, lateral popliteal or
flexor digitorum longus is worthwhile. For poorly
peroneal nerve may be affected. The commonest
mobile patients, or indeed anyone who is prepared to
abnormalities are drop-foot and weakness of peroneal
put up with the inconvenience of an orthosis, splintage
action. Postoperative or postimmobilization drop-foot
may be adequate (see Fig. 21.31).
may be due to pressure on the lateral popiteal or on
the peroneal nerve as the leg rolls into external Osteochondritis dissecans of the talus Unexplained pain
rotation. In addition to motor weakness there is an area and slight limitation of movement in the ankle of a
of sensory loss. Unless the nerve is divided, recovery is young person may be due to a small osteochondral
possible but may take many months. fracture of the dome of the talus. Tangential x-rays will
‘Peroneal nerve lesion’ is sometimes diagnosed usually show the tiny fragment. MRI is also helpful and
after a hip operation. Beware! This is more often due the lesion may be visualized directly by arthroscopy. If
616 to injury of the peroneal portion of the sciatic nerve. the articular surface is intact, it is sufficient to simply
21
The ankle and foot

(a) (b) (c)
21.37 The paralyzed foot (a) In spina bifida – the small ulcer is an indication of insensitive skin. (b) Poliomyelitis and
(c) peroneal muscular atrophy, in both of which sensation is normal.
restrict activities. If the fragment has separated, it may a little and strenuous activities restricted for a few
have to be removed. weeks.
Avascular necrosis of the talus The talus is one of the Calcaneal bursitis Older girls and young women often
preferred sites of ‘idiopathic’ necrosis. The causes are complain of painful bumps on the backs of their heels.
the same as for necrosis at other more common sites The posterolateral portion of the calcaneum is
such as the femoral head. If pain is marked, arthrodesis prominent and shoe friction causes retrocalcaneal
of the ankle may be needed. bursitis. Symptoms are worse in cold weather and
when wearing high-heeled shoes (hence the use of
Chronic instability of the ankle This subject is dealt with
colloquial labels such as ‘winter heels’ and ‘pump-
in Chapter 3.
bumps’).
Treatment should be conservative – attention to
footwear (open-back shoes are best) and padding of
PAINFUL FEET the heel. Operative treatment – removal of the bump
“My feet are killing me!” This complaint is common

but the cause is often elusive. Pain may be due to:
(1) mechanical pressure (which is more likely if the
foot is deformed or the patient obese); (2) joint
inflammation or stiffness; (3) a localized bone lesion;
(4) peripheral ischaemia; (5) muscular strain – usually
secondary to some other abnormality. Remember,
too, that local disorders may be part of a generalized
disease (e.g. diabetes or rheumatoid arthritis), so (a) (b)
examination of the entire patient may be indicated.
Specific foot disorders that cause pain are consid-
ered later.
POSTERIOR HEEL PAIN

Two common causes of heel pain are traction
‘apophysitis’ and calcaneal bursitis: (c) (d)
Traction ‘apophysitis’ (Sever’s disease) This condition 21.38 Painful heel (a) Sever’s disease – the apophysis is
usually occurs in boys aged about 10 years. It is not a dense and fragmented. (b) Bilateral ‘heel bumps’. (c) The
‘disease’ but a mild traction injury. Pain and tenderness usual site of tenderness in plantar fasciitis. (d) X-ray in
are localized to the tendo Achillis insertion. The x-ray patients with plantar fasciitis often shows what looks like a
spur on the undersurface of the calcaneum. In reality this is
report usually refers to increased density and a two-dimensional view of a small ridge corresponding to
fragmentation of the apophysis, but often the painless the attachment of the plantar fascia. It is doubtful whether
heel looks similar. The heel of the shoe should be raised the ‘spur’ is responsible for the pain and local tenderness. 617
21 or dorsal wedge osteotomy of the calcaneum – is fea- ative arthropathy) then blood tests may be indicated.
sible but the results are unpredictable; despite the An ultrasound scan shows the thickening and some-
reduction in the size of the bumps, patients often con- times the Doppler test shows increased local blood
tinue to experience discomfort, potentially added to flow and neovascularization, but this investigation is
by an operation scar. not indicated in every case.
A plain lateral x-ray can help to exclude a stress frac-
ture, and will often show what looks like a bony spur
INFERIOR HEEL PAIN on the undersurface of the calcaneum. The ‘spur’ is,
in fact, a bony ridge that looks sharp and localized in
Calcaneal bone lesions Any bone disorder in the the two-dimensional x-ray image; it is an associated,
calcaneum can present as heel pain: a stress fracture, not a causative, feature in plantar fasciitis. Patients,
osteomyelitis, osteoid osteoma, cyst-like lesions and and sometimes doctors, can become fixated on the
Paget’s disease are the most likely. X-rays usually idea of a spur of bone causing the symptoms by dig-
provide the diagnosis. ging into the plantar fascia, and cannot conceive of
how the condition could possibly resolve whilst the
spur remains – but it can and does get better.
PLANTAR FASCIITIS MRI can be helpful in excluding a calcaneal stress
fracture, which is an important differential diagnosis.
This is an annoying and painful condition that limits
function. There is pain and tenderness in the sole of
the foot, mostly under the heel, with standing or
Treatment
walking. The condition usually comes on gradually, Relative rest and NSAIDs can be helpful in settling
without any clear incident or injury but sometimes the condition in the early stages, with NSAIDs either
there is a history of sudden increase in sporting orally or topically. An analysis of causative factors
activity, or a change of footwear, sports shoes or run- (footwear, sports and exercise factors) can help the
ning surface. There may be an associated tightness of patient to overcome the condition. There is an impor-
the Achilles tendon. The pain is often worse when first tant role for the patient in managing the condition,
getting up in the morning, with typical hobbling with stretching exercises and massage; self-help advice
downstairs, or when first getting up from a period of sheets are available.
sitting – the typical start-up pain and stiffness. The Patients might expect (or dread!) an injection into
pain can at times be very sharp, or it may change to a the plantar fascia, and they are right to be apprehen-
persistent background ache as the patient walks about. sive. There is no convincing research to support this,
The condition can take 18–36 months or longer to and there is evidence to show that it can lead to
resolve, but is generally self-limiting, given time. rupture of the plantar fascia (which will often imme-
diately ease the symptoms, but leads to a painful flat-
foot and impairs sporting function).
Pathology A physiotherapist can help to educate the patient
The plantar fascia or aponeurosis is a dense fibrous about the condition and its likely progress, and can
structure that originates from the calcaneum, deep to emphasize the need for a regular stretching regime for
the heel fat pad, and runs distally to the ball of the 8–12 weeks, supplemented with local massage (for
foot, with slips to each toe. The plantar fascia stiffens instance with a foot roller, golf ball, frozen water
and becomes less pliable with age. The fascia is prob- bottle). Local manual treatments from the physio-
ably not actually inflamed in this condition, at least therapist can help, as can the use of taping and a cush-
not beyond the first week or two of onset. There may ioned heel pad.
be micro-tears in the fascia, and the fascia thickens. Night splints have been tried, to keep the foot up in
The term ‘plantar fasciitis’ is apt in some cases, as a plantigrade position overnight, preventing stiffening
the condition is sometimes associated with inflamma- in the Achilles and plantar fascia; there is logic in this,
tory disorders such as gout, ankylosing spondylitis but no clear evidence for its efficacy, and trials have
and Reiter’s disease, in which enthesopathy is one of been hampered by poor compliance.
the defining pathological lesions. Podiatric assessment of the hindfoot biomechanics
may identify predisposing factors such as plano-valgus
hindfoot alignment, which can be corrected with
Clinical features orthotics.
There is localized tenderness, usually at the medial
aspect beneath the heel and sometimes in the mid- OPERATIVE TREATMENT
foot. This is essentially a clinical diagnosis. If there are Patients may lose heart and demand that something
618 features suggesting an inflammatory disease (seroneg- be done. However, there is no reliable surgical proce-
dure for this condition. Limited fasciotomy to release 21
part of the plantar fascia can help in some cases, but
there is a significant risk of complications including
worsening of the condition.
Promising new interventions include shockwave
lithotripsy and localized radiofrequency (coblation)
The ankle and foot

therapy, but these have yet to be fully tested in rigor-
ous and large-scale studies.
Potential pitfalls (a) (b)

It is important not to miss a manifestation of a sys-
temic disease such as an inflammatory arthropathy
(often seronegative), a peripheral neuropathy (usually
diabetic) or a stress fracture.
If a corticosteroid steroid injection is used it should
be done cautiously with a small dose into a limited
area, and after appropriate warnings to the patient.
Excising a ‘spur’ is usually a vain endeavour.
Differential diagnosis (c) (d)

Painful fat pad Chronic pain and tenderness directly 21.39 Pain over the midfoot (a) Köhler’s disease
over the fat pad under the heel sometimes follows a compared with (b) the normal foot. (c,d) The bump on the
direct blow to the area, e.g. in a fall from a height. The dorsum of the foot due to OA of the first cuneiform-
condition is also seen in athletes and has been metatarsal joint.
attributed variously to separation of the fat pad from
the bone, loss of its normal shock-absorbing effect and
atrophy. Non-specific ‘inflammation’ has also been has a painful limp and a tender warm thickening over
blamed. Treatment is palliative: wearing soft-soled the navicular. Usually no treatment is needed as the
shoes or shock-absorbing heel cups, foot baths and condition resolves spontaneously. If symptoms are
anti-inflammatory agents. severe, a short period in a below-knee plaster helps.
A comparable condition occasionally affects mid-
Nerve entrapment Entrapment of the first branch of
dle-aged women (Brailsford’s disease); the navicular
the lateral plantar nerve has been reported as a cause of
becomes dense, then altered in shape, and later the
heel pain. The commonest complaint is pain after
midtarsal joint may degenerate.
sporting activities. Characteristically, tenderness is
In adults, especially if the arch is high, a ridge of
maximal on the medial aspect of the heel, where the
bone sometimes develops on the adjacent dorsal sur-
small nerve branch is compressed between the deep
faces of the medial cuneiform and the first metatarsal
fascia of abductor hallucis and the edge of the
(the ‘overbone’). A lump can be seen, which feels bony
quadratus plantae muscle. Diagnosis is not easy,
and may become bigger and tender if the shoe presses
because the symptoms and signs may mimic those of
on it. If shoe adjustment fails to provide relief the
plantar fasciitis.
lump may be bevelled off.
Treatment, in the first instance, is conservative: a
long trial (6–8 months) of shock-absorbing orthoses,
foot baths, anti-inflammatory preparations and one or
two corticosteriod injections. Only if these measures GENERALIZED PAIN IN THE FOREFOOT
fail to give relief should surgical decompression of the
Metatarsalgia Generalized ache in the forefoot is a
nerve be considered.
common expression of foot strain, which may be due
to a variety of conditions that give rise to faulty weight
distribution (e.g. flattening of the metatarsal arch, or
PAIN OVER THE MIDFOOT undue shortening of the first metatarsal), or merely the
result of prolonged or unaccustomed walking,
In children, pain in the midtarsal region is rare: one marching, climbing or standing. These conditions have
cause is Kohler’s disease (osteochondritis of the navic- this in common: they give rise to a mismatch between
ular). The bony nucleus of the navicular becomes the loads applied to the foot, the structure on which
dense and fragmented. The child, under the age of 5, those loads are acting, and the muscular effort required 619
21 to maintain the structure so that it can support those flexion of the great toe, may be due to sesamoiditis.
loads. This term is a misnomer: symptoms usually arise from
Aching is felt across the forefoot and the anterior irritation or inflammation of the peritendinous tissues
metatarsal arch may have flattened out. There may around the sesamoids – more often the medial (tibial)
even be callosities under the metatarsal heads. sesamoid, which is subjected to most stress during
Treatment involves: (1) dealing with the mechanical weightbearing on the ball of the foot.
disorder (correcting a deformity if it is correctable, Acute sesamoiditis may be initiated by direct trauma
supplying an orthosis that will redistribute the load, fit- (e.g. jumping from a height) or unaccustomed stress
ting a shoe that will accommodate the foot); and (2) (e.g. in new athletes and dancers). Chronic sesamoid
performing regular muscle strengthening exercises, pain and tenderness should signal the possibility of
especially for the intrinsic muscles that maintain the sesamoid displacement, local infection (particularly in
anterior (metatarsal) arch of the foot. A good ‘do-it- a diabetic patient) or avascular necrosis.
yourself’ exercise is for the patient to stand barefoot on Sesamoid chondromalacia is a term coined by Apley
the floor, feet together, and then drag their body for- (1966) to explain changes such as fragmentation and
wards by repeatedly crimping the toes to produce trac- cartilage fibrillation of the medial sesamoid. X-rays in
tion upon the floor. Ten minutes a day should suffice. these cases may show a bipartite or multipartite
medial sesamoid, which is often mistaken for a frac-
Pain in metatarsophalangeal joints Inflammatory arth-
ture.
ritis (e.g. rheumatoid disease) may start in the foot
Treatment, in the usual case, consists of reduced
with synovitis of the MTP joints. Pain in these cases is
weightbearing and a pressure pad in the shoe. In
associated with swelling and tenderness of the forefoot
resistant cases, a local injection of methylprednisolone
joints and the features are almost always bilateral and
and local anaesthetic often helps; otherwise the
symmetrical.
sesamoid should be shaved down or removed, taking
great care not to completely interrupt the flexor hal-
lucis brevis tendon.
LOCALIZED PAIN IN THE FOREFOOT
Whereas metatarsalgia involves the entire forefoot, Freiberg’s disease (osteochondritis;
localized pain and tenderness is related to a specific
osteochondrosis)
anatomical site in the forefoot and could be due to a
variety of bone or soft tissue disorders: ‘sesamoiditis’, Osteochondritis (or osteochondrosis) of a metatarsal
osteochondritis of a metatarsal head (Freiberg’s dis- head is probably a type of traumatic osteonecrosis of
ease), a metatarsal stress fracture or digital nerve the subarticular bone in a bulbous epiphysis (akin to
entrapment (Morton’s disease). osteochondritis dissecans of the knee). It usually
affects the second metatarsal head (rarely the third) in
young adults, mostly women.
Sesamoiditis The patient complains of pain at the MTP joint. A
Pain and tenderness directly under the first metatarsal bony lump (the enlarged head) is palpable and tender
head, typically aggravated by walking or passive dorsi- and the MTP joint is irritable. X-rays show the head
(a) (b) (c) (d)
21.40 Pain in the forefoot (a) Long-standing deformities such as dropped anterior arches, hallux valgus, hammer-toe,
curly toes and overlapping toes (all of which are present in this patient) can cause metatarsalgia. Localized pain and
tenderness suggest a more specific cause. (b,c) Stages in the development of Freiberg’s disease. (d) Periosteal new-bone
620 formation along the shaft of the second metatarsal, the classic sign of a healing stress fracture.
to be flattened and wide, the neck thick and the joint Surgical intervention is often successful; the nerve 21
space apparently increased. should be released by dividing the tight transverse
If discomfort is marked, a walking plaster or moulded intermetatarsal ligament; this can be done through
sandal will help to reduce pressure on the metatarsal either a dorsal longitudinal or a plantar incision; most
head. If pain and stiffness persist, operative synovec- surgeons will also excise the thickened portion of the
tomy, debridement and trimming of the metatarsal nerve. This is successful in about 90 per cent of
The ankle and foot

head should be considered. Pain relief is usually good patients; the remaining 10 per cent will continue to
and the range of dorsiflexion is improved. experience varying degrees of discomfort.
Stress fracture
TARSAL TUNNEL SYNDROME
Stress fracture, usually of the second or third
metatarsal, occurs in young adults after unaccustomed Pain and sensory disturbance in the medial part of the
activity or in women with postmenopausal osteoporo- forefoot, unrelated to weightbearing, may be due to
sis. The dorsum of the foot may be slightly oedema- compression of the posterior tibial nerve behind and
tous and the affected shaft feels thick and tender. The below the medial malleolus. Sometimes this is due to
x-ray appearance is at first normal, but later shows a space-occupying lesion, e.g. a ganglion, haeman-
fusiform callus around a fine transverse fracture. Long gioma or varicosity. The pain is often worse at night
before x-ray signs appear, a radioisotope scan will and the patient may seek relief by walking around or
show increased activity. Treatment is either unneces- stamping the foot. Paraesthesia and numbness may
sary or consists simply of rest and reassurance. follow the characteristic sensory distribution, but
these symptoms are not as well defined as in other
entrapment syndromes. The diagnosis is difficult to
Interdigital nerve compression (Morton’s establish but nerve conduction studies may show
metatarsalgia) slowing of motor or sensory conduction.
Morton’s metatarsalgia is a common problem, with Treatment To decompress the nerve it is exposed
neuralgia affecting a single distal metatarsal inter- behind the medial malleolus and followed into the sole;
space, usually the third (affecting the third and fourth sometimes it is trapped by the belly of adductor hallucis
toes), sometimes the second (affecting the second and arising more proximally than usual.
third toes), rarely others. The patient typically com-
plains of pain on walking, with the sensation of walk-
ing on a pebble in the shoe, or of the sock being
rucked-up under the ball of the foot. The pain is SKIN DISORDERS
worse in tight footwear and often has to be relieved by
removing the footwear and massaging the foot. Activ-
Painful skin lesions are important for two reasons:
ities that load the forefoot (running, jumping, danc-
(1) they demand attention in their own right; (2) pos-
ing) exacerbate the condition, which often consists of
tural adjustments to relieve pressure may give rise to
severe forefoot pain and then a reluctance to weight-
secondary problems and metatarsalgia.
bear. In Morton’s metatarsalgia the pain is typically
reproduced by laterally compressing the forefoot
whilst also compressing the affected interspace – this Corns and calluses
produces the pathognomic Mulder’s click as the ‘neu-
These are hyperkeratotic lesions that develop as a
roma’ displaces between the metatarsal heads.
reaction to localized pressure or friction. Corns are
This is essentially an entrapment or compression
fairly small and situated at ‘high spots’ in contact with
syndrome affecting one of the digital nerves, but sec-
the shoe upper: the dorsal knuckle of a claw toe or
ondary thickening of the nerve creates the impression
hammer toe, or the tip of the toe if it impinges against
of a ‘neuroma’. The lesion, and an associated bursa,
the shoe. Soft corns also appear on adjacent surfaces
occupy a restricted space between the distal
of toes that rub against each other. Treatment consists
metatarsals, and are pinched, especially if footwear
of paring the hyperkeratotic skin, applying felt pads
also laterally compresses the available space.
that will prevent shoe or toe pressure, correcting any
Treatment A step-wise treatment programme is advis- significant deformity (if necessary by operation) and
able. Simple offloading of the metatarsal heads by us- attending to footwear.
ing a metatarsal dome insole and wider fitting shoes Calluses are more diffuse keratotic plaques on the
may help. If symptoms do not improve with these soles – either under prominent metatarsal heads or
measures then a steroid injection into the interspace will under the heel. They are seen mainly in people with
bring about lasting relief in about 50 per cent of cases. ‘dropped’ metatarsal arches and claw toes, or varus or 621
21 21.41 Skin lesions (a) Corns.
(b) Callosities in a patient with claw toes
and a ‘dropped’ anterior metatarsal arch.
(c) A typical pressure ulcer in a patient with
longstanding diabetic neuropathy.
(d) Keratoderma blenorrhagica, a
complication of Reiter’s disease.
(a)
(b)
(c) (d)
valgus heels. Treatment is much the same as for corns; pressure area. X-rays may help to detect the foreign
it is important to redistribute foot pressure by altering body. Treatment consists of removing the object; the
the shoes, fitting pressure-relieving orthoses and reactive lesion heals quickly.
ensuring that the shoes can accommodate the mal-
shaped feet. Surgical treatment for claw toes may be
needed.
TOE-NAIL DISORDERS
Plantar warts The toe-nail of the hallux may be ingrown, overgrown
Plantar warts resemble calluses but they tend to be or undergrown.
more painful and tender, especially if squeezed. They
Ingrown toe-nails The nail burrows into the nail
can be distinguished from calluses by paring down the
groove; this ulcerates and its wall grows over the nail,
hyperkeratotic skin to expose the characteristic papil-
so the term ‘embedded toe-nail’ would be better. The
lomatous ‘core’, which is seen to be dotted with fine
patient is taught to cut the nail square, to insert
blood vessels. These are viral lesions but it is usually
pledgets of wool under the ingrowing edges and to
local pressure that renders them painful.
keep the feet clean and dry at all times.
Treatment is frustrating as they are difficult to erad-
If these measures fail, the portion of germinal
icate. Salicylic acid plasters are applied at regular inter-
matrix that is responsible for the ‘ingrow’ should be
vals, and smaller lesions may respond to cryosurgery.
ablated, either by operative excision or by chemical
Surgical excision is avoided as this usually leaves a
ablation with phenol. The phenol is applied to the
painful scar at the pressure site.
exposed matrix with a cotton bud for 3 minutes and
then washed off with alcohol, which neutralizes the
Foreign body ‘granuloma’ caustic effect. Rarely is it necessary to remove the
entire nail or completely ablate the nail bed.
The sole is particularly at risk of penetration by small
foreign bodies (usually a thorn, a splinter or a piece of Overgrown toe-nails (onychogryposis) The nail is hard,
glass), which may give rise to a painful lump resem- thick and curved. A chiropodist can usually make the
bling a wart or callus. This diagnosis should always be patient comfortable, but occasionally the nail may need
622 considered if the ‘callosity’ is situated in a non- excision.
21.42 Toe-nail disorders 21
(a) Ingrown toe-nails.
(b) Overgrown toe-nail
(onychogryposis). (c,d) Exostosis
from the distal phalanx, pushing
the toe-nail up.
The ankle and foot

(a) (b)
(c) (d)
Undergrown toe-nails A subungual exostosis grows on FOOT

the dorsum of the terminal phalanx and pushes the nail
upwards. The exostosis should be removed. The footprint gives some idea of the arched structure
of the foot. This derives from the tripodial bony frame-
work between the calcaneum posteriorly and the first
and fifth metatarsal heads. The medial arch is high, with
NOTES ON APPLIED ANATOMY the navicular as its keystone; the lateral arch is flatter.
The anterior arch formed by the metatarsal bones
The ankle and foot function as an integrated unit, and thrusts maximally upon the first and fifth metatarsal
together provide stable support, proprioception, heads and flattens out (spreading the foot) during
balance and mobility. weightbearing; it can be pulled up by contraction of the
intrinsic muscles, which flex the MTP joints.
ANKLE
MOVEMENTS
The ankle fits together like a tenon and mortise; the
tibial and fibular parts of the mortise are bound The ankle allows movement in the sagittal plane only
together by the inferior tibiofibular ligament, and sta- (plantarflexion and dorsiflexion). Adduction and
bility is augmented by the collateral ligaments. The abduction (turning the toes towards or away from the
medial ligament fans out from the tibial malleolus to midline) are produced by rotation of the entire leg
the talus, the superficial fibres forming the deltoid lig- below the knee; if either is forced at the ankle, the
ament. The lateral ligament has three thickened mortise fractures. Pronation and supination occur at
bands: the anterior and posterior talofibular ligaments the intertarsal and tarsometatarsal joints; the foot
and, between them, the calcaneofibular ligament. rotates about an axis running through the second
Tears of these ligaments may cause tilting of the talus metatarsal, the sole turning laterally (pronation) or
in its mortise. Forced abduction or adduction may medially (supination) – movements analogous to
disrupt the mortise altogether by (1) forcing the tibia those of the forearm. The combination of plantarflex-
and fibula apart (diastasis of the tibiofibular joint); ion, adduction and supination is called inversion; the
(2) tearing the collateral ligaments; (3) fracturing the opposite movement of dorsiflexion, abduction and
malleoli. pronation is eversion. 623
21 GW (Ed.) The Clubfoot. Springer-Verlag, New York,
1994, pp 246–52.
Cholmeley JA. Elmslie’s operation for the calcaneus foot.
J Bone Joint Surg 1953; 35B: 46–9.
Coleman WC, Brand PW, Birke JA. The total contact cast.
A therapy for plantar ulceration on insensitive feet. J Am
Podiatry Med Assoc 1984; 74: 548–52.

Coughlin MJ, Shurnas PS. Hallux rigidus: demographics,
etiology, and radiographic assessment. Foot Ankle Int
2003; 24: 731–43.
Crawford A, Marxen J, Osterfeld D. The Cincinatti inci-
sion: A comprehensive approach for surgical procedures
of the foot and ankle in childhood. J Bone Joint Surg
1982; 64A: 1355–8.
(a) (b) (c) Delanois RE, Mont MA, Yoon TR et al. Atraumatic
21.43 Footprints (a) The normal foot, (b) flat-foot (the osteonecrosis of the talus. J Bone Joint Surg 1998; 80A:
medial arch touches the ground), and (c) cavus foot (even 529–36.
the lateral arch barely makes contact). Duncan RD, Fixsen JA. Congenital convex pes valgus.
J Bone Joint Surg 1999; 81B: 250–4.
Evans D. Relapsed clubfoot. J Bone Joint Surg 1961; 43B:
Inversion and eversion are necessary for walking on 722–33.
rough ground or across a slope. If the joints at which Gage JR. Gait Analysis in Cerebral Palsy. MacKeith Press,
they occur are arthrodesed in childhood, a compensa- New York, 1991.
tory change may occur at the ankle so that it becomes Herzenberg JE, Carroll NC, Christofersen MR, Lee EH,
a ball-and-socket joint. White S, Munroe R. Clubfoot analysis with three-
dimensional computer modeling. J Paediatr Orthop
1988; 8: 257–62.
FOOT POSITIONS AND DEFORMITIES Lapidus PW. Transplantation of the extensor tendon for
correction of the overlapping fifth toe. J Bone Joint Surg
A downward-pointing foot is said to be in equinus; 1942; 24: 555–9.
the opposite is calcaneus. If only the forefoot points Piggott H. The natural history of hallux valgus in adoles-
downwards the term ‘plantaris’ is used. Supination cence and early adult life. J Bone Joint Surg 1960; 42B:
with adduction produces a varus deformity; pronation 749–60.
with abduction causes pes valgus. An unusually high Ponsetti IV. Treatment of congenital club foot. J Bone Joint
arch is called pes cavus. Many of these terms are used Surg 1992; 74A: 448–54.
as if they were definitive diagnoses when, in fact, they Rang M. High arches. In: Wenger DR, Rang M (Eds) The
are nothing more than Latin translations of descrip- Art and Practice of Children’s Orthopaedics. Raven Press,
tive anatomy. New York, 1993, pp 168–79.
Robinson AHN, Limbers JP. Modern concepts in the treat-
ment of hallux valgus. J Bone Joint Surg 2005; 87B:
REFERENCES AND FURTHER READING 1038–45.
Rose GK, Welton EA, Marshall T. The diagnosis of flat
Apley AG. Open sessamoid. Proc R Soc Med 1966; 59: 120. foot in the child. J Bone Joint Surg 1985; 67B: 71–8.
Banta J, Sutherland DH, Wyatt M. Anterior tibialis trans- Samilson RL. Proscentic osteotomy of the os calcis for cal-
fer to os calcis with Achilles tenodesis for calcaneal defor- caneocavus feet. In: Bateman JE (Ed.) Foot Science. WB
mity in myelomeningocoele. J Paediatr Orthop 1981; 1: Saunders, Philadelphia, 1976, p. 18.
125–30. Turco V. Surgical correction of the resistant clubfoot. One
Caroll NC. Technique of plantar fascia release and calca- stage posteromedial release with internal fixation; a pre-
neocuboid joint release in clubfoot surgery. In: Simons liminary report. J Bone Joint Surg 1971; 53A: 477–97.
624
Section 3
Fractures and
Joint Injuries
22 The management of major injuries 627
23 Principles of fractures 687
24 Injuries of the shoulder, upper arm and elbow 733
25 Injuries of the forearm and wrist 767
26 Hand injuries 787
27 Injuries of the spine 805
28 Injuries of the pelvis 829
29 Injuries of the hip and femur 843
30 Injuries of the knee and leg 875
31 Injuries of the ankle and foot 907
The management of
major injuries 22
David Sutton, Max Jonas
100%
INTRODUCTION Other
Aetiology of major trauma

80% Bus or coach
Trauma is the commonest cause of death in people
from 1–44 years of age throughout the developed
world. The largest proportion of deaths (1.2 million per Car occupant
year) result from road accidents. The World Health Or- 60%
ganization (WHO) predicts that by 2020 road traffic
injuries will rank third in the causes of premature death Motorcyclist
and loss of health from disability (Peden et al., 2004). 40%
In the UK vehicular accidents causing death or serious
injury are usually car related (Figs 22.1 and 22.2).
Pedal cyclist
20%
Global Percentage of Deaths due to Injury (1999) Pedestrian
5.0% 11.0% 0%
5.0%
All
4.0% 22.2 Proportion of casualties by road user type (UK
2007 Department of Transport data).
14.0%
For every death from trauma, three victims suffer

23.0% permanent disability. As well as causing personal
tragedy, this represents an enormous drain on a
nation’s healthcare economy; timely and effective
17.0% management of major injuries can reduce both mor-
0.3% bidity and mortality.
18.0%
Mode of death
War Homicide and Self-Inflicted
Mortality subsequent to major trauma is dependent
Violence
on a number of factors, of which the economic level
Other Landmines Road Traffic of a nation is a major determinant. The 2004 WHO
Unintentional report (Mock et al., 2004) cites mortality rates for
Injuries
seriously injured adults, i.e. those with an injury
Poisoning Falls Fire severity score (ISS) of 9 or higher. ISS will be described
Source: WHO in greater detail in a subsequent section. The overall
22.1 Global percentage of deaths due to injury mortality rate, including pre-hospital and in-hospital
(1999) (World Health Organization, Department of Vio- deaths, is 35 per cent in high-income nations, but
lence and Injury Prevention). rises to 55 per cent in middle-income economies and
22 63 per cent in low-income economies. More seriously
injured patients (ISS 15–24) reaching hospital show a
six-fold increase in mortality in low-income
economies.
Road traffic deaths and serious injuries show a peak
Death rate
incidence in young people between the ages of 17
FRACTURES AND JOINT INJURIES
(age of learning to drive) and 23.

There is a stark contrast between major trauma
mortality in a high-income country hospital (6 per
cent) and in a rural area of a low-income country (36
per cent). These statistics demonstrate the impact that
a high-income economy with a developed emergency
medical system can have on the outcomes of major
trauma. 0 1 2 3 0 1 2 3 4 5 6
Deaths as a result of trauma classically follow a tri- Hours Weeks
modal pattern, with three waves following the injury.
Some 50 per cent of fatally injured casualties die from 22.4 Death following trauma The trimodal pattern of
mortality following severe trauma.
non-survivable injuries immediately, or within min-
utes after the accidents; 30 per cent survive the initial
trauma, but die within 1–3 hours; the remaining 20
per cent die from complications at a late stage during secondary brain injury caused by the hypoxia and
the 6 weeks after injury. This trimodality represents hypercarbia associated with airway obstruction and
civilian trauma deaths; combat deaths in a war fit a respiratory dysfunction.
bimodal distribution, with merging of the second and The second peak of deaths during the first few
third peaks due to the penetrative nature of the hours after injury is most often due to hypoxia and
injuries and the extended timelines of advanced med- hypovolaemic shock. A significant proportion of these
ical care (Clasper and Rew, 2003). deaths can be avoided with an effective emergency
The initial mortality peak is usually due to non-sur- medical service (EMS); hence, this period has been
vivable central nervous system or cardiovascular dis- called ‘the golden hour’. One-third of all deaths
ruption. The severe nature of the injuries, the occurring after major injury may be preventable in
immediate nature of the deaths and the usual location hospitals with appropriate resources (Commission on
in the pre-hospital environment means that very few the Provision of Surgical Services, 1988).
of these casualties can be saved. However, a small pro- The third peak in the cumulative mortality rate
portion die as a result of early airway obstruction and within the 6 weeks following injury is largely due to
external haemorrhage, and these deaths can be pre- multisystem failure and sepsis. These complications of
vented by immediate first-aid measures. A significant trauma need a high level of intensive care, but can be
proportion of head-injured casualties who die on the reduced by early and effective treatment during the
scene succumb not to the primary brain injury but to preceding phases of casualty management.
1200
Car passenger
1000 Car driver
Motorcycle rider/passenger
800 Pedal cyclist
Pedestrian
600
400
200
0
0 5 10 15 20 25 30 35 40 45 50 55 60 65 70 75 80 85 90⫹
628 22.3 Deaths and serious injuries by road user type and age (UK 2007 Department of Transport data).
Sequence of management 22
In developed healthcare systems, an effective EMS is
available to initiate management at the scene of the
injury and transfer the casualty rapidly to hospital.
Immediate first-aid manoeuvres such as opening the
The management of major injuries

airway and controlling external haemorrhage with
direct pressure are life-saving interventions that
require minimal equipment and training.
More complex treatment requires specialist equip-
ment and expert training not always available at the
scene, and rapid transfer to a medical centre is manda-
tory. However, medical teams can deliver advanced
management to entrapped casualties. Such treatment 22.5 Acid burns Patient with acid burns to his ear and
is difficult to deliver in vehicles and aircraft, and a bal- chest from spilt battery acid during a car accident.
ance has to be drawn between delaying to give treat-
ment on scene and transferring an unstable casualty. The most integrated system is probably the French
In sophisticated healthcare systems, casualties are Services de l’Aide Medical Urgente (SAMU): all
taken to the nearest hospital offering comprehensive emergencies are triaged by a control room team,
Emergency Department management. Treatment is which includes a doctor, and an appropriate response
centred on evaluation, resuscitation and stabilization. is mounted. For major cases, intervention is provided
This phase merges into definitive care in the operating by Services Mobile d’Urgence et de Reanimation
theatre, with control of airway, ventilation and surgi- (SMUR) teams – hospital-based medical teams with
cal management of haemorrhage. Musculoskeletal sophisticated equipment and access to a range of
injuries are initially stabilized, followed by definitive transport including helicopters. SMUR teams can
treatment. deliver an advanced level of care on scene with rapid
Level 2 or 3 critical care may be required to mini- transfer to an appropriate hospital, and European
mize complications and prevent third-phase deaths, experience (Frankema et al., 2004) is that a doctor-led
and prolonged rehabilitation may be necessary to pre-hospital service leads to a 2.8-fold improvement
address the needs of casualties with brain injuries and in mortality for seriously injured patents. However,
complex musculoskeletal damage. the service is very expensive and demands a high num-
ber of experienced medical staff (Earlam, 1997).
Safety on scene and personal protective

PRE-HOSPITAL MANAGEMENT equipment
Hospital doctors in acute specialties may be required
Essential elements include:
to form part of a medical team to manage trauma
1. Organization. cases on scene. Although surgery on entrapped
2. Safety on scene.
3. Immediate actions and triage.
4. Assessment and initial management.
5. Extrication and immobilization.
6. Transfer to hospital.
7. Air ambulances.
Organization
Provision of a pre-hospital EMS depends on eco-
nomic resources, and varies from no provision in
rural, low-income countries to sophisticated services
linked to hospital care in developed economies. The
EMS in most countries is based on ambulances
crewed by medical technicians or paramedics. Medical
support is variable, ranging from volunteer doctors in (a) (b)
the UK by the British Association for Immediate Care 22.6 Medical personal protective equipment (PPE)
(BASICS) to hospital-based teams in North America. (a) Inadequate PPE. (b) Correct PPE. 629
22 casualties is a rare event, surgeons may be sent out for Priority 1 Immediate
serious or major incidents, and so some knowledge of Priority 2 Urgent
pre-hospital care is important. Priority 3 Delayed
The scene of a traumatic incident is invariably haz- Priority 4 Dead
ardous, and the immediate priority for a doctor on
In the event of an overwhelming number of casual-
scene is personal safety; if this is neglected, the doctor
ties, an expectant category can be used. This identifies

can become a casualty rather than a rescuer. Some
casualties whose injuries suggest that survival is
hazards are obvious, such as unstable wreckage,
unlikely, enabling medical resources to be deployed to
jagged metal debris and fire. However, there are con-
those more likely to survive. In the event of improved
cealed hazards that can injure the unwary. Unde-
resources, expectant casualties are re-categorized as
ployed airbags can be triggered, and a variety of toxic
Priority 1.
chemicals can be released, such as battery acid.
The category of a casualty does not necessarily dic-
All members of pre-hospital medical teams should
tate the order of evacuation or treatment; for
therefore be equipped with personal protective equip-
example, the ‘walking wounded’ and uninjured (Pri-
ment (PPE) and clothing appropriate to the incident,
ority 3) may be evacuated first (‘reverse triage’).
and this should be deployed before the scene is
entered (Calland, 2000). The safety of the immediate
scene will normally be the responsibility of the fire
service, with police controlling the incident overall.
Assessment and initial management
Nations’ differing EMS will have their own specific Once safety, command, communications and priori-
regulations covering the specification of PPE for doc- ties have been established, patients can be given indi-
tors working in the pre-hospital environment. As a vidual attention. This calls for an organized approach
rule, PPE must protect the head, eyes, hands, feet, involving awareness, recognition and management
limbs and body to an appropriate extent against phys- (ARM).
ical, chemical, thermal and acoustic risks. Full chemi-
cal, biological, radiological and nuclear protection is a AWARENESS
specialist requirement rarely applicable to doctors Awareness of the environment, pattern of damage to
outside a military setting. a vehicle and the nature of the incident can help the
attending doctor predict the likely injuries and facili-
tate their early recognition. For example, ejection
Immediate actions and triage from a vehicle or death of an occupant increases the
The initial action of a doctor arriving on scene is to likelihood of serious injury. Particular impaction pat-
establish safety – personal safety, scene safety and casu- terns and intrusion of wreckage into the passenger
alty safety. Contact should be made with the officers compartment can suggest specific injuries; a bulls-eye
commanding medical, fire and police emergency serv- fracture of a windscreen from inside a car indicates
ices for a situation report and direction to casualties impaction of the passenger’s head against the wind-
on a priority basis. Communications should be estab- screen and likely head, maxillofacial and neck injuries.
lished. In the event of multiple casualties, priorities Entrapment in a fire is associated with smoke inhala-
are established by triage. tion and possible inhalational burns.
Triage is a system of medical sorting originating
from the Napoleonic battlefields to identify casualties RECOGNITION
in an order of priority for evacuation and treatment. Recognition of injuries is based on a rapid and sys-
In trauma management, triage is used when the num- tematic questioning and examination of the casualty.
ber of casualties is greater than can be managed simul- An immediate assessment is made of the airway,
taneously by the medical personnel available. There breathing and circulation – the ‘ABC’ of trauma
are two stages applicable in the pre-hospital environ- assessment. An instant assessment can be made by
ment: a triage sieve and a triage sort (Hodgetts and questioning the patient and eliciting a verbal
Porter, 2002). response; the ability to speak means that the brain is
The triage sieve is a quick and uncomplicated sys- being perfused with oxygenated blood and hence the
tem based on simple clinical observation of a casu- patient has a patent airway, is breathing and has an
alty’s ability to walk, breathe and maintain a pulse. It adequate circulation. Head injury leading to loss of
can be performed by trained but non-clinical person- consciousness is the most common cause of airway
nel. The triage sort requires a degree of clinical train- obstruction and consequent hypoxia and hypercarbia;
ing and uses physiological measurements to score lack of response to command or painful stimulus indi-
casualties and place them into priority groups. Both cates a significant level of coma. Access to an
triage systems place casualties into four colour-coded, entrapped casualty may be extremely limited, but an
630 priority categories: assessment can usually be made of the airway and
breathing, presence of peripheral pulses and periph- cricothyroidotomy is very difficult in entrapped casu- 22
eral perfusion, head, chest, abdomen, pelvis and alties. Without the use of anaesthetic drugs and mus-
limbs. This initial assessment guides immediate man- cle relaxants, casualties can only be intubated when
agement and the urgency of extrication and transfer jaw tone and protective reflexes have disappeared
to hospital. immediately prior to cardiac arrest. The survival rates
of intubation in this situation are, not surprisingly,

very poor, however intubation with a rapid sequence
MANAGEMENT
of anaesthetic induction remains the gold standard of
Management of injuries is prioritized on treating the
airway securement for trauma casualties, as it offers
most immediately life-threatening injuries first, tradi-
reliable protection from airway leaks and aspiration.
tionally following the ABCDE sequence. The excep-
Prolonged attempts at intubation should not be made
tion to this is the casualty suffering external,
without effective oxygenation and ventilation; casual-
peripheral haemorrhage. Military experience has
ties do not die from not being intubated, they die
shown that bleeding from limb wounds is a leading
from hypoxia and hypercarbia. Accumulating evi-
cause of combat casualty deaths, a significant propor-
dence suggests that only practitioners with an appro-
tion of which are avoidable. This has led to the devel-
priate level of anaesthetic training should be
opment of a CABC sequence, where C stands for
attempting rapid-sequence induction and intubation.
catastrophic haemorrhage (Hodgetts et al., 2006).
Life-threatening, external bleeding is controlled, and
Breathing Once the airway is opened and secure, an
then the usual ABC sequence is followed.
assessment of the casualty’s breathing is made. If
As casualties with airway obstruction succumb
breathing is clearly adequate, oxygen is administered
within minutes, securing a patent airway is always a
from a high flow, non-re-breathing reservoir mask.
priority. Once the airway is open, the casualty must be
With a flow rate of 15 L/minute, approximately 85 per
oxygenated and ventilated if breathing is not ade-
cent oxygen is delivered; there is no place for lower
quate. Further circulatory compromise is addressed
concentrations of oxygen in this situation. If there is
primarily by control of external haemorrhage; an
any doubt that breathing is adequate, then ventilation
intravenous cannula should be placed, but fluids must
must be supported with a bag–valve–mask (BVM)
be administered cautiously (see later).
assembly. This should have a reservoir attached with
During this immediate management phase, the
oxygen flows of 15 L/minute. BVM ventilation is a
assumption is always made that damage to the cervi-
difficult skill even in ideal situations, but chances of
cal and thoraco-lumbar spine may have occurred. The
success can be improved with a two-person technique;
stability of the cervical spine must be protected at all
one person holds the mask in place over the face with
times until the neck can be cleared from the risk of
both hands and pulls the jaw up into the mask to open
injury. Stabilization is achieved by two methods:
up the airway, whilst the second squeezes the bag.
manual immobilization, or securement with head
Adequacy of oxygenation should be judged by clin-
blocks, head straps and a rigid cervical collar. The
ical assessment of lip colour to detect cyanosis, or use
thoraco-lumbar spine is protected by immobilization
of a pulse oximeter. Adequacy of ventilation can be
with straps on a long spinal board or other extrication
judged by clinical assessment of chest expansion and
device.
breath sounds, or use of a chemical or electronic end-
Airway The airway is opened initially with the ‘bare tidal carbon dioxide (EtCO2) monitor, if a supra-
hands’ manoeuvres of chin lift and jaw thrust; the head glottic airway device or tracheal tube is in place.
should not be extended and should be kept in a neutral Absence of breath sounds indicates a pneumotho-
position. If blood, saliva or vomit are present in the rax or haemothorax, and when associated with devia-
airway, suction should be used. If ‘bare hands’ tion of the trachea and hyper-resonance, a tension
techniques are not adequate, an oropharyngeal airway pneumothorax. A tension pneumothorax is an imme-
or nasopharyngeal (NP) airway should be carefully diately life-threatening injury, and is treated in the
placed to prevent the posterior aspect of the tongue first instance by decompression with a large-bore (14-
obstructing the pharynx. NP airways are particularly gauge) intravenous cannula through the second inter-
useful in casualties with obstructing airways who have costal space in the mid-clavicular line. This converts
retained enough of a gag reflex to resist oropharyngeal the tension pneumothorax into a simple pneumotho-
airways, however they should be used cautiously in rax; definitive treatment of a simple pneumothorax in
casualties with clinically apparent basal skull fractures. a spontaneously breathing casualty is to insert a wide-
If these manoeuvres are unsuccessful, there is a range bore chest drain in the 5th intercostal space, anterior
of supraglottic devices such as the laryngeal mask to the mid-axillary line, with the drain being con-
airway (LMA), which can be inserted in difficult nected to a Heimlich-type valve. However, if the casu-
situations. alty is breathing and stable with a simple
Definitive airway securement with intubation or pneumothorax, rapid transfer to hospital is preferable. 631
22 Open or sucking pneumothoraces should be covered raise the blood pressure and bleeding can resume that
with an occlusive dressing secured on three sides – the has previously stopped due to low pressure. The blood
open fourth side prevents a tension pneumothorax pressure drops again, and more fluid administration
developing. causes increasing anaemia. Large volumes of intra-
Positive-pressure ventilation is likely to accelerate venous fluid administered to casualties with haemor-
the conversion of a simple pneumothorax into a ten- rhage have been shown to increase mortality, and cur-
sion pneumothorax. If the casualty is intubated and rent guidance in the UK (National Institute for Clinical
ventilated, and a pneumothorax suspected, a simple Excellence, 2004) is to titrate fluids against the pres-
thoracostomy is made in the 5th intercostal space, ence of a radial pulse in 250 mL boluses, with a crys-
anterior to the mid-clavicular line. This allows a ten- talloid solution such as Ringer’s lactate or Hartmann’s
sion pneumothorax to decompress; however, the lung compound sodium lactate being the preferred fluid
can still be inflated as the casualty is being ventilated. (large, infused volumes of sodium chloride 0.9 per
A thoracostomy is made by making a 3 cm horizontal cent can be associated with the development of a hy-
incision immediately above the 6th rib, just anterior perchloraemic acidosis and should be avoided).
to the mid-axillary line, dissecting the subcutaneous Severe, unresponsive shock is likely to be the result
tissues with large, straight Spencer Wells forceps until of uncontrollable bleeding externally or into the
the chest cavity is entered. A finger is used to open up chest, abdomen, pelvis and multiple long bones
the thoracostomy and ensure no vital structures are (embodied in the aperçu ‘onto the floor and four
felt. more’). Loss of cardiac output can also be due to ten-
sion pneumothorax or cardiac tamponade. Cardiac
Circulation External haemorrhage is controlled tamponade is most commonly associated with pene-
primarily by direct pressure with a dressing, and limb trating trauma of the chest within the nipple lines
elevation if possible. Other methods used are wound anteriorly or scapulae posteriorly.
packing, the windlass technique, indirect pressure and Severe shock leading to pulseless electrical activity
use of a tourniquet; haemostatic dressings can also be (PEA) or asystolic cardiac arrest is an indication for
used at any stage (Lee et al., 2007). bilateral thoracostomies and/or clam-shell opening of
The windlass technique involves the application of the chest and incision of the pericardium. These
a dressing directly over the wound, which is then held manoeuvres will treat the reversible causes of trauma
in place with an appropriate bandage, knotted over cardiac arrest – hypoxia, hypovolaemia, tension pneu-
the wound. A pen or similar object is placed under the mothorax and cardiac tamponade, and may precede
knot, rotated to exert direct pressure over the site of intubation, ventilation and intravenous cannulation in
the haemorrhage, and then secured. this dire, pre-mortem situation.
Tourniquets have been discouraged in contempo-
Disability The casualty is quickly assessed for neuro-
rary, civilian, pre-hospital care, due to the significant
logical disability using the Glasgow Coma Scale (GCS)
risk of serious complications. Inappropriately applied
and assessment for pupillary size and inequality.
tourniquets can increase bleeding (from a venous
tourniquet effect), result in distal limb ischaemia, and
cause direct pressure damage to skin, muscle and
nerves. However, with limb injuries resulting in cata-
Extrication and immobilization
strophic haemorrhage, judicious use of tourniquets More complex management is often impractical in an
can be life saving. Civilian indications include (Hod- entrapped casualty, and so extrication becomes a
getts et al., 2006): priority. This should be done with regard to spinal
protection, usually using spinal boards or other rigid
• life-threatening limb haemorrhage due to shooting,
immobilization devices. Fractured limbs should be
stabbing and industrial or farming accidents;
splinted in an anatomical position to preserve neuro-
• haemorrhagic, traumatic amputation;
vascular function. Analgesia may be necessary to extri-
• limb haemorrhage not controllable with direct
cate an injured casualty, and this can be achieved with
pressure, or where direct pressure cannot be applied
inhalational or intravenous agents.
due to inaccessibility of wound from entrapment;
The initial manoeuvre in the extrication process is
• multiple casualties with lack of manpower to apply
manual immobilization of the cervical spine. This can
direct pressure.
be done from behind the casualty (typically in seated
If possible, a wide-bore cannula should be sited in a casualties entrapped in vehicles with a rescuer in the
large vein, or intraosseus access achieved with a place- rear of the vehicle), or from the front and side if access
ment device such as the EZ-IO®, FAST1™ or BIG is limited. A stiff cervical collar is sized and fitted at
Bone Injection Gun. Administration of intravenous the earliest opportunity, but manual immobilization is
fluids should be judicious in the pre-hospital environ- still mandatory until the casualty can be placed on a
632 ment; rapid infusion of large volumes of fluids can spinal board.
Further immobilization and extrication may be intravenously, and a general anaesthetic in doses of 2– 22
impossible until wreckage has been cleared enough to 4 mg/kg. The advantage of ketamine is that it does
enable an extrication device to be positioned under not cause respiratory depression, and the casualty’s
the casualty. Managing wreckage is a specialist skill airway is more predictably maintained. Doses and
that is the province of the Fire and Rescue crews; administration times of all drugs given should be
however, the pre-hospital doctor should be familiar noted.

with the techniques used to advise how extrication
can be managed without causing additional injury to
Transfer to hospital
the casualty. Common manoeuvres in road vehicle
wreckage are removal of glass and doors, a dashboard Delayed or prolonged transfer to hospital is associated
roll to lift the dashboard off trapped limbs, and with poor outcomes, and every effort should be made
removal of the roof by cutting through the A, B and to minimize the on-scene times for injured casualties.
C pillars. The seat can then be carefully flattened, and There is a balance between ‘scoop and run’ and ‘stay
a long spinal board slid under the casualty from the and play’ management. The airway must be secured,
rear of the vehicle, minimizing movement of the and life-threatening chest injuries (e.g. tension pneu-
spinal column. If a casualty is deteriorating fast, the mothorax) and catastrophic, external haemorrhage
rescue crews should be advised and a rapid extrication dealt with before transfer commences. Prolonged
carried out. attempts at complex management on scene are disad-
Limb fractures and dislocations should be reduced vantageous, and should be limited to life-saving inter-
and the limb returned, if possible, to its anatomical ventions where possible.
position with gentle traction and straightening. This The appropriate method of transport should be
may require analgesia. Note that some injuries such as chosen, with helicopters offering some advantage for
posterior hip dislocations may prevent an anatomical long-distance transfers or rescue from remote and
alignment, and the limb must not be forced. The limb rough terrain. Police escorts can be used to aid ambu-
should then be splinted with traction, gutter or vac- lance progress, and a balance sought between speed of
uum splints as appropriate. This reduces pain and transfer and violent movement of the casualty and
haemorrhage, and minimizes neurovascular damage. attendants.
Femoral traction splints such as the Thomas are effec- The appropriate destination hospital should be cho-
tive for mid-shaft femur fractures, providing the pelvic sen for the casualty’s likely injuries, and this may mean
ring is intact. The traction reduces the fracture, and bypassing a small unit that does not have the appro-
the fusiform compression of the fracture haematoma priate facilities. Wherever possible, the receiving med-
reduces further bleeding. A unilateral, closed, femoral ical team should be directly advised of the estimated
fracture can cause a 1.5 L blood loss – 30 per cent of time of arrival (ETA) and the identified injuries,
the adult blood volume and enough to cause signifi- enabling an appropriate trauma team to be standing
cant shock without other injury. by.
Open-book pelvic fractures cause uncontrollable During the transfer, the casualty’s vital signs should
retroperitoneal bleeding. Blood loss can be minimized be monitored clinically and with available equipment.
by stabilizing and reducing the fracture using special- Conscious casualties should be constantly assessed by
ist, pelvic compression devices or a rolled sheet speaking to them, and a decrease in conscious level
around the pelvis and twisted above. detected early. ECG and pulse should be continuously
Analgesia may be necessary to extricate an injured monitored, blood pressure measured with a non-inva-
casualty. This can be administered by inhalation with sive blood pressure (NIBP) monitor, and oxygen sat-
Entonox, a 50:50 mixture of nitrous oxide and oxy- urations measured if peripheral perfusion allows.
gen, delivered via a breath-actuated regulator valve EtCO2 monitors are useful for gauging adequacy of
and mask or mouthpiece. Parenteral analgesics should ventilation in intubated and ventilated casualties.
only be given intravenously, and titrated cautiously The casualty’s airway must be maintained at all
against effect. Other routes of administration are very times, and oxygenation and ventilation maintained.
unpredictable, especially in shocked casualties. Pure Oxygen saturations should be maintained above 95
opioid agonists such as morphine, diamorphine and per cent if possible, and ventilated casualties have their
fentanyl are most effective, but it should be noted that EtCO2 maintained at a low normal level (4.0–4.5
there is a wide variation in response between individ- kPa). Haemorrhage is controlled with direct pressure,
uals, and care should be taken not to cause respiratory and Hartmann’s solution titrated intravenously to
depression by overdosage. Partial opioid agonists such maintain a palpable radial pulse.
as nalbuphine are used, but have a degree of narcotic If the patient deteriorates en route, the medical
antagonism that can make further administration of attendant must decide whether to attempt resuscita-
opioids unpredictable. Ketamine is a very useful drug tion whilst on the move, stop and resuscitate or make
that is a powerful analgesic in doses of 0.5 mg/kg a run for the nearest hospital. This decision will 633
22 depend on the nature of the intervention required Cramped cabin space and poor patient access in
and the ETA at the hospital. these helicopters greatly restrict the patient interven-
Contemporaneous records are almost impossible to tions possible during flight. The aircraft are noisy and
maintain during a transfer, but electronic equipment vibration considerable, so monitoring the patient’s
can usually download a paper or electronic record. If condition is difficult. These factors make it essential
not, notes should be made as soon as possible after that the patient is stabilized and immobilized prior to
arrival at the hospital. On arrival, the medical atten- transfer; the airway must be secured and protected,
dant should remain part of the resuscitation team ventilation maintained, haemorrhage controlled and
until an effective handover can be made. intravenous access for fluid administration preserved.
Monitoring should be reliable, and the ECG, blood
pressure, oxygen saturations and end-tidal carbon
Helicopters and air ambulances
dioxide observed.
A helicopter emergency medical service (HEMS) is Safety is paramount for doctors working with heli-
ideal, but is expensive to run. HEMS (London) data copters, and all personnel should be trained and famil-
show that the primary life-saving benefit is the rapid iar with safety guidelines. The helicopter should not
delivery of advanced resuscitation skills to the scene. be exited until directed by the crew. If asked to dis-
The most essential life-saving skill is advanced airway embark whilst the rotor blades are revolving, person-
management, and this requires an anaesthetically nel must keep their heads down and be aware that the
trained doctor who can perform a rapid sequence rotor disc droops as it slows and may come below
anaesthetic induction and manage tracheal intubation head height, especially uphill if landing on an incline.
in difficult circumstances. International data show
that, as a result of these interventions, there is a
reduction of 15 per cent in death from head injuries,
and a reduction of between 5 and 7 days in intensive
care stays. HOSPITAL MANAGEMENT
However, the availability of appropriately trained
doctors is variable; many HEMS are crewed by para- Upon reaching hospital, the following are important
medics only, and this reduces the effectiveness of the in hospital management:
service to less advanced life support and rapid delivery
1. Organization.
and evacuation of casualties to an appropriate facility.
2. Trauma teams.
A common standard for response times in the UK and
3. Assessment and management. The ATLS concept.
Europe is 12 minutes from call-out to arrival. This
4. Initial management.
ability to transport casualties quickly over large dis-
5. Systemic management.
tances also means that smaller, less well-equipped and
well-staffed hospitals can be bypassed in favour of
large, specialist centres. Organization
A wide variety of helicopters are used internation-
The aim of any integrated EMS is to “get the right
ally for HEMS work, ranging from large aircraft such
patient to the right hospital in the right amount of
as the Sikorsky S61-N to smaller craft like the Bolkow
time” (Trunkey). Regional services were set up in the
105-DBS. A feature common to all HEMSs is that the
USA in 1973, with three levels of hospital designated
helicopter is twin-engined for safety and flexibility of
as able to manage trauma to differing levels:
flight paths. As costs rise dramatically with increased
size of the helicopter, HEMS aircraft are a compro- Level III centres: capable of treating most trauma
mise. With the exception of military and Coastguard victims, and stabilizing critically ill patients prior
craft, the size is usually restricted. to transfer.
22.7 HEMS helicopter interior

(a) Interior of Bolkow 105-DBS showing
medical attendant seat (facing) and
restricted patient access (stretcher on
right). (b) Rear clam-shell doors for
patient loading.
634 (a) (b)

Level II centres: capable of managing almost all arrive at the hospital. Team members would normally 22
critically ill patients, but not offering all include the following personnel:
subspecialties.
• First-tier response:
Level I centres: able to manage all trauma patients
Emergency department physician
with all specialist needs provided on site.
Physician anaesthetist
However, the development and integration of this Emergency department nurses

system was patchy, and the expense of such a system Radiographer
prevents full development in many countries. There
• First- or second-tier response:
are also arguments as to whether such a system, which
Surgeon from appropriate specialty
may be effective in a society with a high level of pen-
Intensive care specialists
etrating trauma, is appropriate for all environments.
Specific specialists, e.g. paediatric, obstetric, ear,
In the UK, an experimental trauma centre and
nose and throat (ENT), maxillofacial etc.
regional trauma system was set up in the Northwest
Midlands in 1991–1992, and examined over the first The development of emergency medicine, and the
4 years. The assessment found little evidence of an increasing availability of experienced and senior emer-
integrated trauma system having developed, and there gency medicine doctors with sophisticated trauma
was no reliable evidence that survival rates from major imaging availability on a ‘round the clock’ basis, has
trauma in the region had improved (Nicholl and enabled a two-tier call-out for trauma teams. Initial
Turner, 1997). However, after another 5 years, sig- assessment and resuscitation rarely requires immediate
nificant improvements in survival were noted (Oakley specialist surgical skills; once the initial assessment and
et al., 1998). This suggests that regional trauma sys- imaging has been completed, the appropriate special-
tems take some time to develop to maximum effec- ist surgeon can be called in or stood by in the operat-
tiveness, but do demonstrate reductions in mortality. ing theatre for definitive surgical management of
These findings are backed up by a meta-analysis of US specific injuries.
and Canadian trauma centres. Trauma teams should function in an appropriate
Regionalized trauma systems are now operational environment, and most hospitals will have a resuscita-
in many countries, including the USA, Canada, Aus- tion room with all required equipment immediately
tralia, and across Europe. In the UK, a nationally available. Personal protective equipment to include
funded enquiry in 2007 advocated regionalization of gowns, gloves and eye protection must be available. A
trauma care and the establishment of Level 1 trauma sophisticated resuscitation room will have anaesthetic
centres (Findlay et al., 2007). However, in many or delivery systems, equipment and drugs for airway man-
most health care economies, the majority of available agement, intravenous fluid and rapid administration
hospitals will not have all the specialist staff and facil- systems for shock management, and a variety of surgi-
ities to adequately manage major injuries. Each hospi- cal packs for specific interventions such as chest drain in-
tal must therefore have standard operating procedures sertion etc. Patient trolleys should be compatible with
(SOPs) for assessing, managing and if indicated, trans- the taking of x-rays, and the x-ray equipment can be
ferring trauma casualties, depending on the facilities built onto an overhead gantry. Ultrasound imaging
available. equipment should be available for central venous can-
nulation and Focussed Assessment Sonography in
Trauma (FAST). Both the environment and intravenous
Trauma teams fluids should be warmed to minimize hypothermia.
Casualties who have survived their initial trauma and
reached hospital alive need rapid assessment and
The ATLS concept
appropriate resuscitation to avoid their dying during
the ‘golden hour’. Crucial to the effective manage- Major musculoskeletal injuries can be dramatic and
ment of seriously injured casualties is the immediate distracting, but it is rare for them to be immediately
availability of appropriately trained and experienced life-threatening in the absence of catastrophic haem-
doctors and healthcare professionals, and this need orrhage. The classic mistake when treating trauma is to
has led to the development of the trauma team con- focus on the attention-grabbing compound fracture,
cept. and miss the obstructing airway, which is far more
The team is led by a senior doctor with advanced likely to cause a ‘golden hour’ death. Hence the most
trauma skills, whose base specialty is less important immediately life-threatening injuries should always be
than his or her training and experience. The trauma treated first. However, although this principle has been
team is preferably activated by the pre-hospital practi- known for generations, in the stress of the moment a
tioners according to a set of standard criteria, and logical sequence may not be followed unless the treat-
should therefore be awaiting the casualty as they ing doctor is trained and practised. To meet this need, 635
22 a number of training systems have been developed Initial assessment and management
over the years, of which the best known is the Advanced
Trauma Life Support Program for Doctors (ATLS®) The initial assessment and management is part of a
(American College of Surgeons Committee on Trauma, sequence leading to the transfer and definitive care of
2005), developed by the American College of Sur- a casualty. During the primary and secondary surveys,
geons Committee on Trauma. The 2004 7th edition a number of monitoring and investigative adjuncts are
has been revised with updates from international ATLS used alongside clinical examination as given in Figure
subcommittees to reflect trauma developments across 22.8 and the accompanying Box.
the world (Kortbeek et al., 2008).
ATLS originates from 1976, when James Styner, an THE ABCs
orthopaedic surgeon, crashed his light aircraft in rural The underlying principle of ATLS is to identify the
Nebraska with his wife and four children on board. most immediately life-threatening injuries first and start
His wife was killed instantly and three of his four chil- resuscitation. As a general rule, airway obstruction kills
dren sustained critical injuries. Having arrived at the in a matter of minutes, followed by respiratory failure,
nearest hospital, Styner found that the care delivered circulatory failure and expanding intracranial mass le-
to his family was inadequate and inappropriate, and sions. This likely sequence of deterioration has led to
this stimulated him to initiate a trauma care training the development of the trauma ‘ABCs’, a planned se-
programme that became ATLS. The course has since quence of management predicated on treating the most
become an internationally recognized standard and is lethal injuries first. Throughout this sequence, the as-
currently taught in over 40 countries worldwide. sumption is made (until proved otherwise) that there
The ATLS course is based on validated teaching may be an unrecognized and unstable cervical spine in-
techniques, and uses a system of core content lectures jury. Hence, the sequence is:
and practical skill stations to develop skills that are
practised and finally tested in simulated trauma scenar-
ios. The system taught is based on a three-stage ap- Injury Definitive care
proach:
1. Primary survey and simultaneous resuscitation – a Primary survey Transfer
rapid assessment and treatment of life-threatening Adjuncts
injuries.
2. Secondary survey – a detailed, head-to-toe
evaluation to identify all other injuries. Resuscitation Re-evaluation
3. Definitive care – specialist treatment of identified
injuries. Re-evaluation Secondary survey
The primary and secondary surveys constitute the Adjuncts
initial assessment and management, which leads to the
definitive care of the casualty following transfer if 22.8 Algorithm of ATLS initial assessment and
required. management
The intention of ATLS is to train doctors who do
not manage major trauma on a regular basis, but it is
applicable to any trauma situation as an underlying ADJUNCTS TO PRIMARY SURVEY
system on which to base management of an injured
Vital signs
casualty. The sequence is taught assuming one non-
specialist doctor supported by one nurse, working on ECG
a single casualty, but the various components can be
Pulse oximetry
performed simultaneously if a team is available. The
training is didactic, but the use of specialist skills (e.g. End-tidal carbon dioxide
anaesthetic) should not be excluded. Although the
Arterial blood gases
course is updated on a 4-yearly basis, there is an
inevitable time lag, and fast-developing areas such as Urinary output
imaging may introduce changes to local trauma man-
Urethral catheter (unless contra-indicated)
agement not found in current ATLS courses. There
are also national and local variations in practice that Naso-gastric tube (unless contra-indicated)
need to be taken into account, and these are discussed
Chest x-ray
later in this chapter; however ATLS has stood the test
of time and remains the most widely recognized basis Pelvic x-ray
636 for trauma management internationally.
A Airway with cervical spine protection. Awareness – a head injury is the most likely cause of 22
B Breathing. unconsciousness and obstructed airway in trauma
C Circulation with haemorrhage control. casualties.
D Disability or neurological status. Recognition – an obstructed airway is recognized by
E Exposure and Environment – remove clothing, looking, listening and feeling for the diagnostic
keep warm. signs.

Management – the airway is established with simple
As previously described, catastrophic haemorrhage
‘bare hands’ manoeuvres, airway adjuncts,
may be controlled before the airway, designated by
advanced airway interventions or surgical airway
the ABC sequence; however, death is ultimately caused
techniques.
by cerebral anoxia, regardless of whether the anoxia is
a result of airway obstruction, respiratory failure, shock As each stage in the ABCs is completed, the casu-
or old age. Hence, the goal of resuscitation is to pre- alty is re-evaluated for deterioration or improvement;
serve the perfusion of the brain with oxygenated blood. on completion of the breathing assessment, the airway
is re-examined and the airway and breathing re-
TRIAGE assessed before moving onto the circulation etc.
Triage, as described in the pre-hospital section of this
chapter, is medical sorting to prioritize multiple casu- A – Airway and cervical spine control The cervical spine
alties for resuscitation, and is used when the number is stabilized immediately on the basis that an unstable
of casualties outstrips the available resources. The ini- injury cannot initially be ruled out. There are two
tial two phases of triage, usually pre-hospital, are the techniques for this:
sieve and the sort, to group casualties into the four pri- • manual, in-line immobilization
ority groups of immediate, urgent, delayed or dead. • cervical collar, head supports and strapping.
Within the ATLS® system, multiple casualties are
triaged by rapidly assessing each patient’s ABCs. Simultaneously, the airway is examined for obstruc-
Those with the most immediately life-threatening tion by looking, listening and feeling for signs such as
injuries are treated first; these are injuries of the: respiratory distress, use of auxiliary muscles of respira-
tion, decreased conscious level and lack of detectable
Airway: Actual or impending Priority 1 breath on hand or cheek. The airway is supported ini-
obstruction tially by lifting the chin or thrusting the jaw forward
Breathing: Hypoxia or ventilatory Priority 2 from under the angles of the mandible. Secretions and
failure blood are carefully suctioned, and oropharyngeal or
Circulation: External haemorrhage Priority 3 NP airways used to hold the tongue forward. If these
or shock simple manoeuvres are unsuccessful, the options are
supraglottic airway devices (e.g. the laryngeal mask
PRIMARY SURVEY AND RESUSCITATION airway), tracheal intubation or surgical airway. All
During the primary survey, life-threatening condi- these techniques can be performed without extending
tions are identified and resuscitation started simulta- the neck.
neously, again following the ABCDE sequence.
The Awareness Recognition Management system B – Breathing A clear airway does not mean the
enables the treating doctor to focus rapidly on the casualty is breathing adequately enough to enable
likely problems; for example: peripheral tissue oxygenation. As soon as the airway is
22.9 Triage priorities

(a) Priority 1 – Airway: severe face
and neck wounds. (b) Priority 2 –
Breathing: severe chest wounds;
(c) Priority 3 – Circulation: severe
bleeding and shock.
(a) (b) (c) 637

22 secured, the chest must be exposed and examined by Table 22.1 Glasgow Coma Score
looking, listening and feeling. Adequate and Response Score
symmetrical excursion, bruising, open wounds and Eye opening:
tachypnoea are looked for, and the chest is auscultated Spontaneous 4
for abnormal or absent breath sounds, which indicate On command 3
a pneumothorax or haemothorax. The trachea is On pain 2
palpated in the supra-sternal notch to detect the Nil 1

deviation caused by a tension pneumothorax, and the Best motor response:
chest is percussed for the hyper-resonance of a tension Obeys 6
Localizes pain 5
pneumothorax or dullness of a haemothorax.
Normal flexor 4
A tension pneumothorax must be treated immedi- Abnormal flexor 3
ately if the diagnostic signs of absent breath sounds, Extensor 2
hyper-resonance and deviated trachea are found. Initial Nil 1
management is decompression with a 14-gauge can- Verbal response:
nula placed in the second intercostal space in the mid- Orientated 5
clavicular line, followed by chest drain placement. If Confused 4
there is any doubt as to the adequacy of the casualty’s Words 3
Sounds 2
breathing and oxygenation, ventilation should be Nil 1
started with a reservoir BVM assembly using high-
flow oxygen. Any trauma casualty who has required
intubation must be ventilated.
E – Exposure and environment The patient should have
C – Circulation with haemorrhage control The circulation
all clothing removed to enable a full examination of
is assessed by looking for external bleeding and the
the entire body surface area to take place. This will
visible signs of shock such as pallor, prolonged capillary
require log rolling to examine the posterior aspects,
refill and decreased conscious level. The heart is
and allow removal of any glass or debris. The casualty
auscultated to detect the muffled sounds of cardiac
should be kept warm to maintain body temperature as
tamponade, and poor perfusion assessed by feeling for
close to 37ºC as possible, and all fluids and ventilated
clammy and cool skin. The peripheral and central
gases warmed. Although patient cooling is used in
pulses are palpated to detect tachycardia and
some specialist situations, this is not indicated in the
diminished or absent pulse pressure.
initial resuscitation. A hypothermic patient becomes
External bleeding is controlled by pressure, and
peripherally shut down and acidotic, and if shivering,
two 14-gauge cannulae sited for administration of in
has greatly increased oxygen demands.
fluids and blood. Blood samples can be drawn from
the cannulae for baseline diagnostic tests and transfu-
ADJUNCTS TO PRIMARY SURVEY
sion cross-matching. As blood is available quickly in a
A number of monitoring and diagnostic adjuncts are
hospital setting, warmed, crystalloid intravenous
used to supplement the primary survey and resuscita-
fluids can be given in an initial volume of 2 L to main-
tion, in addition to vital signs monitoring and haema-
tain cardiac output.
tological assays:
D – Disability The key element of assessing a patient’s • Electrocardiographic (ECG) monitoring – used to
neurological status is the Glasgow Coma Score (GCS) monitor heart rate and detect arrhythmias and
(Table 22.1). This score records eye opening, the best ischaemic changes.
motor response and the verbal response, giving a score • Pulse oximetry – measures arterial oxygen saturations
of between 15 for normal responses, and 3 for no (SaO2) and monitors peripheral tissue perfusion (this
responses. Repeat GCS scoring can track deterioration is unreliable in low-output states, hypothermia and
in the conscious level, and indicate the need for elective with motion artefact).
intubation and ventilation. It is much more precise • End-tidal carbon dioxide monitoring (EtCO2) –
than the AVPU score (Aware, Verbally responsive, gives an estimation of arterial carbon dioxide partial
Pain responsive and Unresponsive). The classic pitfall pressure in intubated and ventilated patients, allow-
of intoxication should be considered, but a lowered ing optimization of lung ventilation. It also con-
GCS is assumed to be secondary to a cerebral injury firms tracheal intubation and alerts the practitioner
until proved otherwise. to a drop in cardiac output.
The pupils are examined for any difference in size • Arterial blood gases (ABGs) – allows quantification
indicating raised intra-cerebral pressure, and unre- of arterial oxygen and carbon dioxide partial pres-
sponsive pupils, fixed at mid-point, which can indicate sures with acid–base balance. This will also give the
638 serious brain damage. haemoglobin, sodium and potassium levels.
• Urethral catheter – allows measurement of hourly log-roll to ensure that all the body surfaces are 22
urine output (unless contraindicated, e.g. in the examined. The guiding injunctions are look, listen and
case of a ruptured urethra). feel.
• Nasogastric tube – decompresses the stomach and The head is examined for contusions, lacerations
helps prevent aspiration (unless contraindicated, and clinically detectable fractures. The eyes and ears
e.g. because of a basal skull fracture). are examined for local damage, and examined inter-

• Chest x-ray – for diagnosis of life-threatening chest nally with an ophthalmoscope/otoscope for signs of
injuries such as pneumothorax, which will require bleeding etc. Bleeding from the ears can indicate a
early treatment. basal skull fracture. The GCS should be repeated.
• Pelvic x-rays – enable a fractured pelvis to be diag- The face is examined for signs of fractures with a
nosed, which will alert to the likelihood of consequent risk of airway obstruction – contusion,
retroperitoneal bleeding. laceration, deformity, malocclusion of teeth and crepi-
tus. Cerebrospinal fluid issuing from the nose (rhine-
(NOTE: lateral cervical spine x-rays do not exclude
orrhoea) is indicative of a basal skull fracture.
fractures or unstable necks and so do not alter man-
All aspects of the neck are examined for contusions,
agement; although important, they can be left until
lacerations, swelling, tenderness, and a step in the cer-
the secondary survey.)
vical spine indicative of fracture/dislocation. Minor-
looking contusions over the anterior neck can be
SECONDARY SURVEY
indicative of underlying damage to the laryngeal and
The secondary survey is a detailed, head-to-toe evalu-
tracheal structures, which are associated with airway
ation to identify all injuries not recognized in the pri-
obstruction. A lateral cervical spine x-ray is taken at
mary survey. It takes place after the primary survey has
this stage.
been completed, if the patient is stable enough and
The chest is inspected for deformity, contusions such
not in immediate need of definitive care; it may, in
as the classic ‘seat belt’ sign and open, possibly pene-
fact, take place after surgery, or on the intensive care
trating, wounds. A stethoscope is used to auscultate
unit (ICU). The importance of the secondary survey
the lungs, comparing left and right apices and bases to
is that relatively minor injuries can be missed during
identify the loss of breath sounds, indicating a pneu-
the primary survey and resuscitation, but cause long-
mothorax. Feel for tenderness and crepitus due to
term morbidity if overlooked, for example small joint
fractured ribs and sternum, which may also be associ-
dislocations.
ated with underlying lung and heart contusions. Per-
The components of the secondary survey are:
cussion can reveal the hyper-resonance of a tension
• history pneumothorax, and the dullness of a haemothorax.
• physical examination The abdomen is inspected for contusions and
• ‘tubes and fingers in every orifice’ wounds, and auscultated for the absence of bowel
• neurological examination sounds indicative of visceral damage. Palpation prima-
• further diagnostic tests rily detects rigidity and tenderness in the conscious
• re-evaluation. patient, and percussion can identify gastric distension,
but these are unreliable in many trauma casualties.
The history The patient’s ongoing experience of his or
The early use of specialist imaging such as ultrasound
her injuries, as well as details of events immediately
and computed tomography (CT) is indicated. Dis-
before, during and after the injury should be recorded.
crete areas such as the perineum, rectum and vagina
Particularly important is to establish whether the
should not be forgotten, and must be examined for
trauma was subsequent to a medical collapse: did the
bleeding, contusions, lacerations etc.
patient suffer a myocardial infarct causing a car crash,
The key indicators for pelvic fracture are unequal leg
or was the infarct a result of hypovolaemia? With the
length and pain or crepitus on palpation or gentle
increasing proportion of the elderly in developed
compression of the pelvis. If these signs are positive, a
societies, more patients are receiving chronic treatment
pelvic fracture is indicated, with the risk of profound
for hypertension etc., which can have a profound effect
haemorrhage. The examination should not be
on their response to hypovolaemia. An example of this
repeated.
is a combination of beta-blockers and angiotensin-
All four limbs are examined for contusions, defor-
converting enzyme (ACE) inhibitors, which cause a
mity and pallor. Pain and crepitus on palpation are
profound drop in blood pressure if the patient’s cardiac
indicative of underlying fracture or dislocation, and
output is minimally compromised. A useful mnemonic
this examination should not be repeated if positive.
is AMPLE: allergies; medications; past illnesses; last
Distal pallor and absence of pulses suggest a vascular
meal; events and environment.
injury, and sensory loss, neurological damage. X-rays
Examination Examination follows a logical sequence that include the joint above and below the injury site
from the head down to the extremities, including a are indicated. 639
22 Table 22.2 Palpable pulses at different blood
pressures
and the risks of sedation and respiratory depression.
However, in expert hands, there are various tech-
Pulses palpable Likely systolic blood pressure niques that can be used in the hospital setting.
Intravenous analgesia – This is the most commonly
Carotid, femoral, radial > 80 mmHg
preferred technique, with morphine being the usual
Carotid, femoral > 70 mmHg drug. Morphine is a pure agonist opioid and should be
Carotid > 60 mmHg diluted and titrated against patient response as there is
No pulse < 60 mmHg a wide variation in effect between individuals. It also
provides a degree of mental detachment and euphoria
useful in the trauma patient, but has the side effects
associated with opioids of respiratory depression,
sedation, hypotension, nausea and dysphoria. Being a
A rapid neurological assessment is carried out to
pure agonist, its effects can be reversed with naloxone.
detect lateralizing signs, loss of sensation and motor
Respiratory depression can be reversed whilst preserv-
power, and abnormality of reflexes. Levels of sensory
ing analgesia with the respiratory stimulant doxapram.
loss should be carefully documented to enable deteri-
Partial agonists such as buprenorphine should be
oration or improvement to be quantified. X-rays and
avoided as they are not fully reversed by naloxone. An
CT may be indicated to detect spinal fractures.
anti-emetic such as cyclizine or ondansetron should be
Imaging Imaging techniques are developing rapidly, given with morphine to minimize nausea.
and changing practice. The use of chest and pelvis x-rays Inhalational analgesia – Nitrous oxide/oxygen
is still standard in the primary survey, but false-negative 50:50 mix (Entonox) is useful for short-term analge-
results with cervical spine radiographs limits their use. sia when moving patients or aligning fractures. How-
The incidence of spinal cord injury without radio- ever, nitrous oxide diffuses into air-filled closed
graphic abnormality (SCIWORA) is around 10 per cavities such as a pneumothorax, and will expand the
cent of all spinal injuries, and is more common in volume by a factor of four, potentially causing an
children. undrained pneumothorax to tension.
CT scans have in the past had the drawback that Nerve blocks – Nerve blocks can be used with great
sending an unstable casualty for a lengthy procedure effect in some limb injuries, but should only be
in a remote radiology department is too dangerous. administered after discussion with an orthopaedic sur-
However, modern spiral CT scanners are fast, and if geon due to the risk of masking a compartment syn-
located adjacent to the Emergency Department, a drome. Femoral nerve blocks are technically
whole-body trauma CT can be completed in minutes. straightforward and can be used for mid-shaft femur,
The risk of patient instability may therefore be out- anterior thigh and knee injuries.
weighed by the benefit of a CT scan in enabling accu-
rate diagnosis, and this technique is becoming a gold INTRA-HOSPITAL AND INTER-HOSPITAL TRANSFER
standard. Few hospitals enjoy the luxury of having the Emer-
Magnetic resonance imaging (MRI) is not usually gency Department, radiology, operating theatres and
available as an emergency procedure, and is not safe ICUs all in the same location, and so transfer of seri-
with an unstable casualty. However, its ability to ously injured casualties is inevitable at some point.
identify soft-tissue injuries is of use in diagnosing Transfer is indicated when the patient’s needs exceed
SCIWORA; removal of spinal precautions may not be what can be delivered with the resources immediately
safe until an MRI has excluded unstable spinal available. The transfer may be between units within
injuries. the same hospital, from a small hospital to a larger
Ultrasound scanning is often helpful, particularly facility (e.g. a Level I trauma centre), or to a special-
for diagnosing intra-abdominal bleeding. In many ist unit (e.g. burns, neurosurgical or cardiothoracic).
departments focussed assessment with sonography in Even the shortest transfer within a hospital is fraught
trauma (FAST) has largely supplanted diagnostic with hazard as monitoring and resuscitation are diffi-
peritoneal lavage; however, its usefulness is limited to cult on the move, and so must be carefully planned. A
detecting fluid in the peritoneum, and it will not reli- number of questions should be answered before the
ably enable diagnosis of specific visceral injuries. transfer is initiated: When? Where? Who? What way?
Though it remains a quick and useful Emergency With?
Department adjunct, it does not provide the diagnos- When to transfer is determined by the condition of
tic information of CT. the casualty and the urgency of definitive care. Patient
outcome is directly related to time from injury to
PAIN MANAGEMENT definitive care, so delays should be minimized. How-
Pain management has in the past been underempha- ever, transferring partially assessed and unstable
640 sized, due to concerns about masking surgical signs patients is dangerous, and so transfer is not usually
contemplated until the primary survey and resuscita- spinal injury cannot be excluded. This may require 22
tion have been completed. Ideally, the patient should immobilization on a spinal board with a cervical col-
be stable when transferred, but this may not be possi- lar and head restraints; bear in mind that closely fitting
ble if bleeding is severe. Definitive care may be so cervical collars can raise intracerebral pressure, and
urgent that intervention is required before the sec- prolonged restraint on a spinal board results in pres-
ondary survey is reached, e.g. for evacuation of an sure injuries. The casualty should be transferred on an

expanding intracerebral bleed. Transfer should not be appropriate trolley, and a medical kit with equipment
delayed for investigations such as cervical spine x-ray, for ABC interventions must be carried. Full monitor-
which will not change management. However, it is ing to include ECG, NIBP/intra-arterial BP, SaO2
crucial that the ABCs are addressed; the airway should and EtCO2 should be available. For transfers between
be secured and protected, the patient must be oxy- hospitals, an appropriate form of transport must be
genated and ventilated optimally, and shock should be available.
addressed. With the casualty should go a full set of paperwork
Where to transfer the casualty to is determined by to include patient identity and documentation of the
the definitive care required and the best facility avail- full initial assessment; it is particularly important to
able that can offer that care. Multiply-injured patients note whether the secondary survey has been carried
may have injuries requiring input from differing sur- out, with any injuries duly noted. If the urgency of the
gical specialties such as neurosurgery and general sur- transfer has taken precedence over the secondary sur-
gery; in this situation, the definitive care surgeons vey, then this should be highlighted so the survey can
must decide on the priorities, having assessed the be completed after the initial, life-saving, definitive
patient. The back of the head should always be exam- care. Results of all blood tests and investigations such
ined as injuries at the back of the head may sometimes as x-rays must accompany the patient.
be missed (Fig. 22.10). In life-threatening circum-
stances (e.g. with expanding intracerebral and intra- DEFINITIVE CARE
abdominal bleeds), the patient may require Definitive care describes the specialist care required to
simultaneous management of both injuries. manage the injuries identified during the initial assess-
Who conducts the transfer is determined by the staff ment and subsequent investigations. This may be
available. The transferring physician should have an specialist surgery to address a particular problem (e.g.
appropriate set of critical care competencies including neurosurgical evacuation of an intracerebral bleed), or
advanced airway skills – this is not a job for the near- critical care management on an ICU to provide sys-
est junior doctor. Transfer should be authorized by temic support (e.g. oxygenation and ventilation of
the senior doctor with responsibility for the patient, patients with severe lung contusions).
and an appropriate team of nurses, technicians and
paramedics should accompany the patient. The refer-
ring doctor should have direct communication with SYSTEMIC MANAGEMENT
the receiving doctor, who should be briefed on the
patient’s condition, destination and ETA. Accurate and effective management of a casualty with
In which way the transfer is achieved depends on multiple injuries depends on a logical progression of
factors such as whether the transfer is between hospi- examination, moving through the systems in a
tals or within units of the same facility. The casualty sequence most likely to identify the most immediately
must be secured and full spinal stabilization in place if life-threatening injuries first. Using the ARM system
described earlier helps structure the approach:
Awareness – use the history and accident mechanism
to predict likely injuries and anticipate problems.
Recognition – examine the patient logically using the
look – listen – feel sequence to identify the physical
signs of injury.
Management – having identified injuries, implement
the most effective and life-saving interventions
first.
Systemic management may progress simultaneously
in a hospital location with a trauma team; in the
absence of a team, work through the systems follow-
ing the ABCDE format. The exception to this would
22.10 The head Failure to examine the back of the head be control of catastrophic haemorrhage preceding air-
may result in missed injuries!! way management. 641
22 A – Airway and cervical spine
Management of the airway in all forms can be imple-
mented whilst protecting the cervical spine. Until the
airway is both secured and protected, this is best done
by in-line immobilization, as use of a stiff cervical col-
lar makes intubation difficult. Conventionally, in-line

immobilization is performed with the practitioner
standing at the head of the casualty, holding the head
on both sides with the hands and maintaining it in a
(a) (b)
neutral position, in line with the neck and torso. This
can make airway management difficult, with the in- 22.11 Mandibular fracture (a,b) Patient with a
line immobilizer squatting awkwardly to one side. An mandibular fracture showing the characteristic position to
maintain the airway.
alternative and more effective stance is for the immo-
bilizer to stand to one side of the casualty’s shoulder
and immobilize the head from below.
An additional technique is to stand at the casualty’s structures, leading to immediate or incipient airway
head and support the head between the forearms obstruction. Signs can be subtle; contusion over the
whilst linking the hands behind the neck. This effec- larynx with a hoarse voice, coughing of bright red
tively immobilizes the cervical spine, but makes blood and surgical emphysema should alert the
examination of the posterior neck difficult, and is practitioner to the likelihood of sudden airway
uncomfortable for a tall practitioner. obstruction.
Once the airway is secured and protected, the trin- Inhalational burns Inhaling super-heated air burns the
ity of stiff collar, head blocks and tape should be airway and can result in rapid development of swelling
implemented. Whatever techniques are used, the cer- and airway obstruction. Signs such as facial burns,
vical spine should be immobilized at all times until an smoke staining and singed nasal hair suggest an
unstable injury is excluded – this may require CT or inhalational burn, requiring early and expert
MRI scanning, and be after definitive care. intubation.
AIRWAY – RECOGNITION
AIRWAY – AWARENESS
Airway obstruction and respiratory failure may be
Head injury This is by far the most common cause of
obvious (to an experienced clinician), but early signs
airway compromise in the trauma patient. As the level
can sometimes be subtle and need systematic exami-
of consciousness decreases, so does muscle tone, and
nation to detect:
the pharynx collapses around the glottis, obstructing
the airway. In the supine position, the tongue drops Look
backwards, plugging the glottis anteriorly. Airway Agitation, aggression, anxiety – suggest hypoxia.
obstruction can be sudden or insidious, and partial or Obtunded conscious level – suggests hypercarbia.
complete, but will result in damaging hypoxia and Cyanosis – blue discoloration of nail beds and lips
hypercarbia, which are particularly dangerous in a caused by hypoxaemia due to inadequate
head-injured casualty. oxygenation.
Sweating – increased autonomic activity.
Maxillofacial trauma Disruption of the facial bones
Use of accessory muscles of ventilation; casualty
allows the face to fall back, compressing and
classically sitting forward splinting chest, and
obstructing the pharynx. This is associated with soft
using neck and shoulder muscles to aid breathing.
tissue swelling and bleeding, which further obtund the
May also display flared nostrils.
airway. Typically, these patients need to sit up to allow
Tracheal tug and intercostal retraction – caused by
the face to fall away from the pharynx and open up the
exaggerated intrathoracic pressure swings.
airway.
Listen
Neck trauma Penetrating or blunt-force trauma results
Noisy breathing – collapsing pharyngeal muscles
in haemorrhage and swelling, which compresses,
obstruct airway leading to snoring sounds.
distorts and obstructs the upper airway. This can
Stridor – air flow through an obstructing upper
progress rapidly and make tracheal intubation
airway changes from laminar to turbulent,
impossible and surgical airway difficult.
resulting in the typical hoarse wheeze of stridor – a
Laryngeal trauma Blunt force trauma from impact to sinister sign, as even minimal further reduction in
the anterior neck (on a car steering wheel, for example) the airway lumen can result in critical airway
642 can disrupt the larynx and fracture the cartilaginous obstruction.
22

(b) (c)
22.12 Pharyngeal airways preventing the tongue from falling back
across the glottis (a) Open airway. (b) Obstructed airway. Collapse of phar-
ynx and tongue across glottis. (c) Airway secured with oropharyngeal airway.
(a)
Hoarse voice (dysphonia) – functional damage to Chin lift The chin is lifted forwards with the
larynx. practitioner positioned at the casualty’s head or side,
Absence of noise – may indicate complete airway using one hand. This pulls the jaw and pharyngeal
obstruction or apnoea. structures forward off the posterior pharyngeal wall
and glottis, and opens up the airway.
Feel
Feel for passage of air through mouth and nose with Jaw thrust This is a more assertive manoeuvre that is
palm of hand; very sensitive for detecting air flow. effective in patients with small jaws or thick necks, or
Palpation of the trachea in supra-sternal notch will who are edentulous. From the casualty’s head, the
detect the deviation associated with a tension thenar eminences are rested on the casualty’s maxillae
pneumothorax. (assuming no obvious fracture), and the four fingers
positioned under the angles of the mandible. Using the
AIRWAY – MANAGEMENT thenar eminences to provide a counterpoint on the
A range of manoeuvres is available to secure a patent maxillae, the mandible is lifted up and forwards to
airway, ranging from ‘bare hands’ techniques to a sur- open up the airway as with chin lift. Considerable
gical airway. All these techniques can be performed pressure can be exerted without displacing the head on
without extending the head and compromising an the neck, and the manoeuvre can be combined with
unstable cervical spine. The anaesthetic ‘sniffing the application of a BVM assembly for ventilation of the
early morning air’ position (head extended and neck lungs.
flexed) should not be used in the trauma patient. Bare
hands techniques and the use of pharyngeal airways
are used together to pull the pharyngeal tissues and
tongue off the posterior pharyngeal wall and away
from the glottis, opening up the airway.
Supra-glottic airway devices (e.g. the laryngeal
mask airway) provide more reliable airway mainte-
nance, but only intubation and the surgical airway will
provide a definitive airway that is both secured and
protected.
All the non-surgical airway manoeuvres described
are applicable to children, but require some modifica-
tion in technique to accommodate their anatomical
and physiological differences. Surgical cricothyroido-
tomy is not recommended in children under 12 years
of age, as the cricoid cartilage can be damaged, lead-
ing to tracheal collapse. 22.13 Chin lift 643
22
22.14 Jaw thrust 22.15 Jaw thrust with O2 mask
Release of chin lift and jaw thrust almost inevitably A correctly sized OP airway should neither project up
results in loss of the airway, and progression to airway beyond the teeth, nor disappear into the buccal cavity.
adjuncts will be required to free up the practitioner. Use of the OP airway may need to be combined
with chin lift or jaw thrust to maintain a patent airway,
Oropharyngeal (OP) airway The oropharyngeal, or
as they should only be used in obtunded patients with
Guedel, airway is a curved and flattened, hard, plastic
absent gag reflexes.
tube with a proximal flange, which is shaped and sized
to hold the tongue and pharynx off the posterior Nasopharyngeal (NP) airway The NP airway is a soft,
pharyngeal wall. They are available in a range of sizes plastic tube with a smooth, distal bevel and a proximal
from neonate to large adult; selection of the correct flange. Some makes have a safety pin to insert through
size is important, as the pharyngeal tissues will collapse the flange to prevent the NP airway disappearing into
across the end of too small a device, whilst one too the nose. It is supplied in a number of internal diameter
large will risk impinging on the glottis. The correct size sizes, and should be selected according to the
is selected by lining up the OP airway alongside the approximate size of the casualty’s little finger. The NP
patient’s jaw; the flange to tip length of the OP airway airway is lubricated with aqueous jelly, and inserted
should match the distance from the corner of the along the floor of the nasal cavity into the nasopharynx.
patient’s mouth to the external auditory canal. The NP airway should not be inserted up the nose as
The OP airway is inserted above the tongue, ini- this risks haemorrhage from the mucosa and
tially with the concave aspect upwards. As the tip turbinates, further compromising the airway, and also
passes over the tongue, the OP airway is rotated so introduces the possibility of entering the cranial cavity
the concave aspect slides over the tongue, and slipped through a basal skull fracture.
into the pharynx until the flange rests on the incisors. NP airways are particularly useful as they can be tol-
644 22.16 OP airway (Guedel) 22.17 OP airway – correct position

22

22.18 NP airway 22.19 NP airway – correct position
erated by responsive casualties with obstructing air- advantages that it is more effective than other airway
ways. They also provide access to suction the devices, but does not require the skill and training
nasopharynx with a soft suction catheter. required for successful tracheal intubation.
Mounting international evidence suggests that
Oropharyngeal suction Secretions and blood should be
intubation performed by practitioners without anaes-
cleared with a specialist pharyngeal sucker such as the
thetic training can be detrimental to patient survival,
Yankauer. Care should be taken not to damage the soft
and in the UK the ambulance service regulatory body
tissues, and as a general rule, the sucker should not be
(Joint Royal Colleges Ambulance Service Liaison
passed further than can be seen. Suction of the oro-
Committee, 2008) has removed tracheal intubation as
nasopharynx with a Yankauer sucker, under direct
a core paramedic skill, and recommends the use of
vision using a laryngoscope, is effective in the
supra-glottic airway devices.
obtunded patient.
The LMA is available in a range of sizes from
Supra-glottic airway devices These are devices that neonatal to large adult; for adult use, a size 3 will fit
function between an OP airway and a tracheal tube, small women, size 4 larger women and smaller men,
and include multi-lumen oesophageal airway devices and size 5, larger men. The device consists of a cuffed
(e.g. Combitube), the laryngeal tube airway, and the distal portion shaped to fit into the oropharynx over
laryngeal mask airway. The most commonly used the glottis. The cuff is inflated with air to fit snugly
device is the laryngeal mask airway (LMA). The LMA against the pharynx, but does not seal as does a tra-
was developed by Dr Archie Brain and introduced cheal tube cuff, and hence does not reliably protect
initially in the UK for anaesthetic use in the late 1980s. the airway. The LMA is lubricated and inserted over
Since then it has found an international role for the tongue with the open end of the cuffed distal
resuscitation and trauma airway management, with the portion positioned inferiorly. The device is slipped
around the oropharynx until it is snugly positioned
over the glottis, and the cuff inflated according to the
size of the device (#3 20 mL, #4 30 mL, #5 40 mL).
As the laryngeal mask, in common with other
supra-glottic airway devices, does not provide a defin-
itive and protected airway, consideration should be
given to its being replaced with a tracheal tube at the
earliest opportunity.
Tracheal intubation Oro-tracheal intubation is the
preferred method for securing and protecting the
compromised airway in the trauma patient. However,
it is a difficult procedure with minimal survival rates in
un-anaesthetized, trauma casualties; un-anaesthetized
casualties can normally only be intubated when
protective reflexes are absent, allowing a view of the
vocal cords on laryngoscopy. Lack of reflexes to this
22.20 Supraglottic airways degree is associated with terminally deep levels of 645
22 coma, when casualties are at the point of death. and slide the tracheal tube over the bougie into
Casualties requiring a definitive airway should the trachea, then remove the bougie.
therefore be identified early, and expert assistance 11. Connect the self-inflating resuscitation bag to the
sought from an anaesthetist or critical care physician. tracheal tube directly or with a catheter mount,
The indications for oro-tracheal intubation are: via a heat/moisture exchanger (HME) filter.
12. Inflate the cuff until no air leak is audible during
• apnoea
ventilation.
• inability to maintain airway by other means.
13. Secure the tracheal tube with ties or tapes.
• need to protect airway from aspiration of blood and
14. Confirm intubation with chest auscultation and
stomach contents
EtCO2 detection, and ventilate the patient with
• impending airway obstruction, e.g. inhalational
100 per cent oxygen to normal EtCO2 levels.
burn, expanding neck haematoma, facial fractures
• closed head injury with GCS below 8 All intubated, trauma patients should be ventilated,
• inability to maintain adequate oxygenation and as it is unlikely that they would be able to maintain
ventilation with face mask or BVM assembly. adequate oxygenation and ventilation spontaneously.
Nasotracheal intubation is indicated only in a spon- Needle cricothyroidotomy Needle cricothyroidotomy is
taneously breathing patient, and has a poor success the insertion of a needle through the cricothyroid
rate with a high incidence of complications such as membrane into the trachea to allow jet insufflation of
nasal haemorrhage. the lungs with oxygen. It is used in emergency ‘can’t
Trauma tracheal intubation should be performed intubate, can’t ventilate’ situations to buy time whilst
with a rapid sequence induction (RSI) anaesthetic; expert assistance is sought, or a definitive surgical
after pre-oxygenation, anaesthesia is rapidly induced airway prepared. Oxygenation is achievable, but
with an intravenous agent, cricoid pressure applied to ventilation limited, so carbon dioxide accumulates and
hold the oesophagus closed and prevent passive reflux the EtCO2 rises. Specialist equipment is available (e.g.
of stomach contents, the patient paralyzed with sux- ventilation with a Sanders injector driven from a high-
amethonium and a tracheal tube placed under direct pressure oxygen source, via a curved cricothyroid
vision with use of a laryngoscope. The tracheal tube needle). However, a system can be rapidly assembled
cuff is inflated until no leak is detected, and the from routinely available components. The following
cricoid pressure not released until the anaesthetist sequence should be followed:
confirms the tracheal tube is secure.
1. Prepare a 12- or 14-gauge, preferably unported,
This procedure should not be performed by any
intravenous cannula, and attach it to a 10 mL
practitioner without the necessary training and expe-
syringe.
rience in anaesthetic techniques, as injudicious use of
2. Prepare a length of oxygen tubing with a distal Y
muscle relaxants can lead to immediate loss of the air-
connector, three-way tap or cut side-hole, and
way and a ‘can’t intubate, can’t ventilate’ scenario.
attach it to a cylinder or wall oxygen source with
If a non-anaesthetically trained, trauma practitioner
a flow rate set at 15 L/minute.
has to attempt intubation in extremis, the following
3. Prepare skin with 2 per cent chlorhexidine in 70
sequence should be followed:
per cent isopropyl alcohol, and insert the cannula
1. Select appropriately sized tracheal tube; size 8 through the patient’s cricothyroid membrane in
(internal diameter) will be appropriate for most the midline, angled caudally at 45 degrees, aspi-
men and most women. rating air as the trachea is entered.
2. Leave tube uncut but ensure proximal connector 4. Slide the cannula fully into the trachea over the
is securely attached. trochar and secure manually or with tape.
3. Have a smaller diameter tube available as back up. 5. Attach the Y connector end of the oxygen tubing
4. Lubricate the cuff and test inflate, then deflate, to the cannula.
to detect cuff leakage. 6. Occlude the Y connector for 1 second to allow
5. Have two functioning laryngoscopes available lung insufflation.
with bright lights. 7. Allow a 4-second pause with the Y connector
6. Have intubating bougie or catheter available. un-occluded to allow lung deflation.
7. Maintain head and neck immobilized in neutral, 8. Continue 1:4 cycles of insufflations until a
in-line position. definitive airway is secured.
8. Pre-oxygenate the patient, if possible, with a
Complications of needle cricothyroidotomy and jet
BVM assembly.
insufflation are commonly misplacement, surgical
9. Use a laryngoscope in the left hand to visualize
emphysema and barotrauma. It should only be
the vocal cords.
attempted if intubation and other airway maintenance
10. Insert, intubating the bougie through the cords
646 techniques have failed.
Surgical cricothyroidotomy Surgical cricothyroidotomy Take-home message Whatever the means of airway 22
is the insertion of a tracheal or tracheostomy tube management used, the goal is to secure and protect the
through an incision in the cricothyroid membrane into airway. The focus should be on oxygenation and
the trachea. It is used in emergency situations when ventilation, not intubation. Casualties die from hypoxia
oro-tracheal intubation has been attempted, and failed, and hypercarbia, not failure of intubation.
and will both secure and protect the airway. Adequate

ventilation is just as achievable as with oro-tracheal
intubation, and 100 per cent oxygen can be delivered. B – Breathing and chest injuries
The following sequence should be followed:
Of severely injured patients admitted to hospital in
the UK, 20 per cent have chest injuries (Joint Royal
1. Prepare skin over cricothyroid membrane with 2
Colleges Ambulance Service Liaison Committee
per cent chlorhexidine in 70 per cent isopropyl
(JRCALC), 2008), and thoracic trauma is a significant
alcohol, and infiltrate with local anaesthetic if the
cause of mortality (Findlay et al., 2007). However,
patient is aware.
the majority of chest injuries are not fatal and do not
2. Prepare an appropriate tracheal tube; a 6 mm
require specialist, surgical intervention.
internal diameter, reinforced/armoured tracheal
tube is optimal, as this allows use of an
BREATHING/CHEST INJURY – AWARENESS
intubating bougie and will not kink and
The proportion of penetrating to blunt chest injuries
obstruct. Alternatively, a tracheostomy tube with
varies between countries, and between rural and
obturator can be used.
urban environments. Only 10 per cent of blunt chest
3. Prepare a scalpel, ideally with a curved No. 10
injuries and 20 per cent of penetrating injuries require
blade.
thoracotomy (Findlay et al., 2007; Joint Royal Col-
4. Prepare an intubating bougie or catheter, e.g.
leges Ambulance Service Liaison Committee
Cook Medical Frova intubation catheter.
(JRCALC), 2008). Non-surgical management centres
5. Identify the cricothyroid membrane; place a
on supportive treatment of contused lungs and the
finger on the thyroid cartilage prominence and
insertion of chest drains. However with blunt trauma,
roll it down onto a notch of cricothyroid
the force of impact and energy transfer to the lung
membrane.
parenchyma should alert the clinician to the likeli-
6. Tension skin over the cricothyroid membrane
hood of severe intrathoracic damage and the potential
with the thumb and fore-finger on either side.
for progressive cardiopulmonary problems.
7. Make a single, 1–2 cm transverse incision
Early recognition and management of immediately
through the skin and cricothyroid membrane
life-threatening injuries in the primary survey is
into the trachea.
imperative, with early imaging repeated as necessary.
8. Without releasing skin tension, insert the
Potentially life-threatening injuries are sought during
intubation catheter through the incision and pass
the secondary survey, and sophisticated imaging modal-
it inferiorly down the trachea.
ities such as CT and MRI may be indicated. Major
9. Slide the tracheal tube over the intubation
chest injuries will require urgent referral to a specialist
catheter into the trachea until the cuff is in the
thoracic or cardiothoracic surgeon, and a surgeon
lumen of the trachea.
capable of immediate thoracotomy must be available in
10. Inflate the cuff until the leak is sealed on
hospitals designated as receiving major trauma cases.
ventilation.
11. Ventilate with a self-inflating bag and high-flow
BREATHING/CHEST INJURY – RECOGNITION
oxygen.
The patient’s chest, neck and abdomen must be fully
12. Secure the tracheal tube with ties or tape.
exposed to allow assessment of the chest. Examination
13. Confirm that both lungs are ventilated; if
should be systematic:
one-lung ventilation is detected (usually on the
right), deflate the cuff, pull back the tracheal Look
tube and re-inflate the cuff. • Respiratory rate – tachypnoea is indicative of hypoxia.
• Shallow, gasping or laboured breathing – suggests
Surgical cricothyroidotomy can be a difficult proce- respiratory failure.
dure in casualties with challenging anatomy, and com- • Cyanosis – indicates hypoxia.
plications can be serious; this procedure should only • Plethora and petechiae – suggest asphyxia and chest
be used if oro-tracheal intubation has been attempted crushing.
and failed. Complications include haemorrhage, dam- • Paradoxical respiration; ‘pendulum’ breathing with
age to laryngeal structures, false passage formation, asynchronization between chest and abdomen,
misplacement of the tracheal tube, surgical emphy- resulting in a seesaw motion – indicates respiratory
sema and barotrauma. failure or structural damage. 647
22 • Unequal chest inflation – suggestive of pneumoth-
IMMEDIATELY LIFE-THREATENING CHEST
orax or flail chest.
• Bruising and contusions – indicate significant energy INJURIES (PRIMARY SURVEY)
transfer and consequent underlying lung contusion
1. Tension pneumothorax
and potential hypoxia (e.g. ‘seat belt’ sign).
• Penetrating chest injuries – potential for pneu- 2. Open pneumothorax (sucking chest wound)
mothorax and open, sucking pneumothorax.

3. Massive haemothorax
• Distended neck veins – increased venous pressure
secondary to a tension pneumothorax or cardiac 4. Cardiac tamponade
tamponade.
5. Flail chest
Listen
6. Disruption of tracheal–bronchial tree
• Absent breath sounds – indicate apnoea or tension
pneumothorax.
• Noisy breathing/crepitations/stridor/wheeze –
ventilation–perfusion mismatch leads to hypoxia.
suggest a partially obstructed airway, blood and
However, the life-threatening, terminal event is a shift
secretions in airways, tracheal or bronchial damage.
of the mediastinum away from the affected side, kink-
• Reduced air entry unilaterally – indicate a pneu-
ing the great vessels and obstructing venous return to
mothorax, haemothorax or haemo-pneumothorax,
the heart. This results in a deadly combination of
and flail chest.
hypoxia and loss of cardiac output, with a pulseless
Feel electrical activity (PEA) cardiac arrest.
• Tracheal deviation – indicative of tension pneu- Diagnosis should usually be clinical, not radiological,
mothorax, shifting the mediastinum (Note: the tra- and the clinician should look specifically for the three
chea is felt inferiorly in the suprasternal notch; do cardinal signs:
not confuse it with the larynx, which is extra-tho-
• absent breath sounds – on the side of the pneu-
racic and hence does not shift.)
mothorax
• Tenderness – suggests significant chest wall contu-
• deviated trachea – away from the side of the tension
sion and/or fractured ribs
pneumothorax
• Crepitus/instability – underlying fractured ribs
• hyper-resonance – on the side of the pneumo-
• Surgical emphysema (classic ‘bubble wrap’ feel to
thorax.
subcutaneous tissues on palpation, due to presence
of air forced into tissues under pressure) – tension The neck veins may be distended, as venous return
pneumothorax, ruptured bronchi or trachea, and is obstructed; however, this may not be readily visible,
fractured larynx. and is unreliable with concurrent hypovolaemia.
There is an argument for radiological diagnosis if this
BREATHING/CHEST INJURY – MANAGEMENT is immediately available in the resuscitation room, and
Immediate management is to stabilize the cervical the patient is not exhibiting cardiovascular compro-
spine, control catastrophic limb haemorrhage, secure mise; a tension pneumothorax can be mimicked by
the airway, administer oxygen at high flow and venti- other conditions such as endo-bronchial intubation
late the lungs if breathing is absent or inadequate. It with distal lung collapse.
is vital to rapidly identify and manage immediately The immediate management is decompression
life-threatening chest injuries during the primary sur- (needle thoracocentesis) of the tensioning pneumoth-
vey, as positive-pressure ventilation of the lungs can orax by insertion of a 14-gauge cannula into the pleu-
cause a rapid deterioration; a simple pneumothorax ral cavity through the second intercostal space, in the
can be converted to a tension pneumothorax, and a mid-clavicular line.
tension pneumothorax will increase in pressure, lead- Diagnostically, a hiss is heard as air under pressure
ing to sudden collapse and cardiac arrest. Hence, if a escapes. However, this is unreliable, and the relatively
patient is intubated and ventilated, signs of a pneu- short 50 mm intravenous cannulae commonly used
mothorax must immediately be sought and, if present, may not penetrate a thick chest wall in muscular or
decompressed and drained. Potentially life-threaten- obese casualties. Presence of the cannula within the
ing injuries can then be identified during the second- pleura is likely if air can be aspirated with a syringe,
ary survey. and use of the longer 140 mm cannulae will make cor-
rect placement more likely. Once sited, the cannula
TENSION PNEUMOTHORAX should be left open to reduce the risk of re-tension-
A tension pneumothorax is the build-up of air under ing.
pressure in the pleural cavity, leading to compression Needle decompression should not be performed if
648 and collapse of the underlying lung. The resultant the only sign elicited is reduced or absent breath
ineffective in practice, and an occlusive dressing with 22
immediate chest drain may be more reliable. The
patient may need intubating and ventilating.
MASSIVE HAEMOTHORAX
The chest cavity presents an enormous potential space

in which blood can accumulate following both blunt
and penetrating chest injury (one of the four of
‘bleeding onto the floor and four more’). 1500 mL or
one-third of the patient’s blood volume can rapidly
accumulate, leading to a combination of hypoxia and
shock. Smaller haemothoraces are usually due to lung
parenchymal tears, fractured ribs and minor venous
injuries and are self-limiting. Massive bleeds are usu-
ally due to arterial damage, which is more likely to
require surgical repair and pulmonary lobectomy.
Diagnosis is based on the presence of hypoxia,
reduced chest expansion, absent breath sounds
and/or dullness to chest percussion, and hypo-
volaemic shock. Supine chest percussion may not
demonstrate dullness, and supine x-rays may not
reveal moderate haemothoraces. Specific management
is by the insertion of a chest drain, correction of hypo-
volaemia and blood transfusion. If the total volume of
22.21 Left-sided tension pneumothorax blood initially drained is greater than 1500 mL, or the
bleeding continues at 200 mL/hour, or the patient
remains haemodynamically unstable, surgical referral
sounds, as there are associated complications such as and thoracotomy is indicated.
misplacement and damage to the underlying lung.
Insertion of a needle into the pleural cavity will con- CARDIAC TAMPONADE
vert a tension pneumothorax into a simple pneu- Cardiac tamponade is the accumulation of blood
mothorax, which will in turn need draining. In an within the pericardium, restricting the ability of the
intubated and ventilated patient, immediate thoracos- heart to fill, and resulting in a progressive loss of car-
tomies can be performed prior to formal chest drain diac output leading to PEA cardiac arrest. It is more
insertion; the positive-pressure ventilation of the commonly associated with penetrating rather than
lungs will enable the lungs to be satisfactorily inflated. blunt trauma, especially stab wounds between the nip-
If immediately available, a controlled chest drain ple lines or scapulae, and gunshot wounds.
insertion is preferable to a blind needle decompres- Clinical diagnosis can be difficult, as the signs can
sion. be subtle and difficult to elicit in the trauma room.
The three classic diagnostic criteria constitute Beck’s
OPEN PNEUMOTHORAX (SUCKING CHEST WOUND) Triad:
An open wound in the chest wall will immediately
1. Distended neck veins due to elevated venous pres-
result in a simple pneumothorax as intrathoracic pres-
sure.
sure equilibrates with atmospheric pressure. If the
2. Muffled heart sounds.
defect is greater than some two-thirds of the diameter
3. Fall in arterial blood pressure.
of the trachea (which has a lateral diameter of 20–25
mm), air is preferentially drawn into the pleural cavity If an arterial line is present, a fall in systolic blood
rather than into the lungs via the trachea. This causes pressure may be seen on inspiration (pulsus para-
paradoxical respiration, where the lung deflates on doxus). If a central venous pressure (CVP) line is in
inspiration, with resulting hypoventilation and situ, a rise in CVP may be seen on inspiration, in
hypoxia. If a flap valve effect occurs, the intra-pleural contrast to its normal fall on inspiration (Kussmaul’s
pressure will rise with each breath, leading to a ten- sign).
sion pneumothorax. Reliable diagnosis may require sophisticated imag-
Specific, immediate management is the application ing. No change is seen on standard chest x-rays, but
of an occlusive dressing, sealed on three sides, but CT scanning, MRI scanning, FAST ultrasound and
leaving the third side open to allow any build up of trans-oesophageal echo-cardiogram (TOE) can all be
positive intra-pleural pressure to vent. This can be used to confirm the diagnosis. 649
22 Management has two components; relieving the DISRUPTION OF TRACHEOBRONCHIAL TREE
pressure within the pericardium by draining the accu- Major disruption of the tracheobronchial tree can
mulated blood, and stopping the source of the bleed- result in a broncho-pleural fistula; the disrupted tra-
ing to prevent re-accumulation. Since the bleeding is chea or bronchus allows an air leak into the pleura
likely to come from the heart, immediate surgical which, if large enough, will not allow inflation of the
repair to the myocardium may be required, and surgi- lung, even with a large-bore chest drain in situ. Diag-
cal assistance should be sought early. nosis is made by the presence of a persistent pneu-
Classically, aspiration of blood from the peri- mothorax, pneumomediastinum, pneumopericardium
cardium is achieved by needle peri-cardiocentesis, or air below the deep fascia of the neck, often in
which should be viewed as a diagnostic procedure patients who have suffered a deceleration injury.
rather than curative. The ECG is monitored, and a Immediate management with tracheal intubation
long cannula (16–14 gauge, 14 cm as above) is may not be successful, as the air leak may prevent
attached to a syringe. The skin is prepared, pierced inflation of either lung. In this situation, endo-
with the cannula to the left of the xiphisternum, and bronchial intubation of the opposite lung or use of a
the cannula directed towards the pericardium in the bronchial blocker may be required before adequate
direction of the left scapula tip. As the pericardium is lung ventilation can be achieved, and this may need
entered, blood is aspirated. The needle can then be the services of a thoracic anaesthetist.
removed from the cannula, and a three-way tap
attached to the cannula to allow further aspirations. SIMPLE PNEUMOTHORAX
Advancement too far will cause the tip of the cannula A simple pneumothorax results from air entering the
needle to enter the myocardium, which will be seen pleural cavity, causing collapse of the lung with a
on the ECG as ventricular ectopics, widening QRS resulting ventilation–perfusion mismatch and
complexes or ST-T wave changes. Pericardiocentesis hypoxia. As the air is at atmospheric pressure, and
can be performed under ultrasound guidance. there is no one-way valve effect, no mediastinal shift
Alternative and more definitive procedures are sub- develops, and cardiac output is maintained. The cause
xiphoid pericardial window, or emergency thoraco- is usually a lung laceration, which can follow both
tomy and pericardiotomy. These are optimally blunt and penetrating chest trauma or thoracic spine
performed in the operating theatre if the patient’s fracture–dislocations.
condition allows. The diagnosis is made during the primary or sec-
ondary survey, primarily by the absence or reduction
FLAIL CHEST of breath sounds. Hyper-resonance may not be obvi-
Massive impact to the chest wall can result in multiple ous, and a chest x-ray may be required to confirm the
rib fractures, and this is more common in older peo- pneumothorax. If the pneumothorax is stable, defini-
ple who have less flexible rib cages. The multiple frac- tive treatment with a chest drain can be deferred to
tures, particularly if anterior and posterior, can result the secondary survey. However, a simple pneumotho-
in a loss of the structural integrity of the chest wall, rax can develop into a tension pneumothorax at any
and a segment can ‘float’; as the patient inspires, the time, and so a high index of suspicion should be main-
flail segment is sucked in and the lung cannot inflate tained.
(paradoxical respiration). This results in hypoxia and
ventilatory compromise. However, the force required
to cause this injury inevitably causes a severe, under- POTENTIALLY LIFE-THREATENING CHEST
lying lung contusion, and this is the more significant INJURIES (SECONDARY SURVEY)
cause of the hypoxia. The associated, severe pain fur-
ther compromises the respiratory function, and respi- 1. Simple pneumothorax
ratory failure can ensue.
2. Haemothorax
Diagnosis is by clinical examination, chest x-rays to
reveal the fractures and lung contusion, and arterial 3. Pulmonary contusion
blood gases to quantify the hypoxia.
4. Tracheobronchial tree injury
Management is initially supportive with administra-
tion of oxygen and analgesia. Advanced pain relieving 5. Blunt cardiac injury
methods such as epidurals may be required. Profound
6. Traumatic aortic disruption
hypoxia may require that patients are intubated and
ventilated until the contusion has adequately resolved, 7. Traumatic diaphragmatic injury
and pain can be controlled. Intravenous fluids may
8. Mediastinal traversing wounds
need to be restricted to avoid overload and worsening
hypoxia. Very rarely, fractured ribs or costo-chondral 9. Simple pneumothorax
650 disruption may require surgical stabilization.
Intubation and ventilation in the presence of a 22
pneumothorax predisposes to the development of a
tension pneumothorax, and so chest drains should
immediately be placed. Anaesthesia with a nitrous
oxide-based anaesthetic will increase the air space by a
factor of four, and can therefore cause rapid tension-

ing, as can air transport at altitude. In these situations,
chest drains should be placed prophylactically, and it
is good practice to insert chest drains in casualties (a) (b)
prior to transfer in case a tension pneumothorax
develops en route.
Chest drain insertion is a procedure with the poten-
tially dangerous complication of visceral damage, and
the classic chest drain technique using a pointed trochar
should not be used. The appropriate technique is:
1. Confirm the correct side on the chest x-ray.
2. Identify the fifth intercostal space, just anterior
to the mid-axillary line on the affected side. (c) (d)
3. Prepare the skin with 2 per cent chlorhexidine in
70 per cent isopropyl alcohol or alcoholic iodine.
4. Infiltrate the skin and subcutaneous tissues with
lignocaine if the patient is aware.
5. Make a 2–3 cm, horizontal incision through the
skin, just above the sixth rib (to avoid the
intercostals vessels below the fifth rib).
6. Bluntly dissect through the subcutaneous tissues
with a straight forceps, and puncture the parietal (e)
pleura with the tips.
7. Insert your gloved little finger through the
incision into the chest cavity and sweep the finger
around to ensure the cavity is empty and your
incision is above the diaphragm (no viscus is felt). (f)
8. Grasp the tip of an appropriately sized 22.22 Chest drain insertion sequence (a) Chest x-ray
thoracostomy tube between the tips of the to confirm correct side. (b) Identify the fifth intercostal
forceps and introduce through the incision into space, just anterior to the mid-axillary line on affected
the chest cavity; unclamp the forceps and slide side. (c) Insert gloved little finger through the incision into
the chest cavity and finger sweep to ensure cavity is empty
the tube posteriorly along the inside of the chest and the incision is above the diaphragm (no viscus is felt).
wall. (d) Grasp the tip of an appropriately sized thoracostomy
9. Attach the tube to an underwater drain or tube between tips of forceps and introduce through
Heimlich valve and observe for tube fogging and incision into chest cavity. Unclamp forceps and slide tube
underwater bubbling. posteriorly along inside of chest wall. (e) Attach tube to
underwater drain or Heimlich valve and observe for tube
10. Suture the chest drain in place and apply a fogging and underwater bubbling. (f) Check lung
dressing. reinflation with chest x-ray.
11. Check lung reinflation with a chest x-ray.
The important steps are illustrated in Figure 22.22. erect film, 400–500 mL of blood are required to
obliterate the costo-phrenic angle. The diagnosis may
Haemothorax Haemothoraces are primarily caused by require the use of FAST or CT scanning.
lung lacerations or damage to intercostals and internal An acute haemothorax visible on chest x-ray is treated
mammary vessels. Thoracic spine fracture dislocations with a large calibre chest drain, inserted using the
can also result in haemothoraces. They are normally technique described earlier. If more than 1500 mL are
self-limiting, and rarely require operative intervention. drained initially, or drainage continues at 200 mL/hour
Diagnosis can be difficult in the supine patient as or faster, thoracotomy should be considered.
breath sounds will remain present. Dullness to per-
cussion will be posterior and not reliable. Supine chest PULMONARY CONTUSION
x-rays will not reveal moderate amounts of blood, Pulmonary contusion is the commonest potentially
although erect films are more sensitive; even with an life-threatening chest injury, occurring in 20 per cent 651
22 of casualties with an injury severity score (ISS) of > 15. Treatment is initially with one or more, large chest
Mortality ranges from 15–20 per cent and 40–60 per drains that may need a high-volume/low-pressure
cent of patients will require ventilating. Blunt force pump to allow lung re-inflation. Persistent bron-
trauma to the chest wall, or crushing injury, will con- chopleural fistulae may require operative intervention.
tuse the underlying lung, which then becomes oede- Major tracheobronchial injuries are immediately life-
matous and haemorrhagic, with subsequent collapse threatening, and management is described earlier.
and consolidation. This causes a ventilation–perfusion

mismatch and hypoxia, dependant on the extent of BLUNT CARDIAC INJURY
the contusion and limitation of the patient’s ventilation Blunt cardiac injury follows a direct blow to the ante-
by pain. About half of these patients will develop rior chest, and is associated with a fractured sternum.
bilateral acute respiratory distress syndrome (ARDS), a This can result in myocardial contusion, or more
systemic inflammatory response to the injury. rarely, chamber rupture and valvular disruption. The
Pulmonary contusion may not be associated with myocardial damage can result in hypotension due to
obvious rib fractures, particularly in children and myocardial dysfunction, conduction abnormalities,
teenagers with pliable rib cages. The initial chest x-ray and dysrhythmias. Sudden onset of dysrhythmias can
may not reveal the extent of the contusion, which can result in death from ventricular fibrillation.
develop over the following 48 hours. The diagnosis Management is supportive, and the patient should
should be made taking into account the mechanism of be monitored closely for a minimum of 24 hours, fol-
injury and the degree of hypoxia revealed by oximeter lowing which the risk of sudden dysrhythmias dimin-
saturation readings and arterial blood gas estimations. ishes substantially.
Treatment is with supportive measures and oxygen
administration. Patients with severe hypoxia despite TRAUMATIC AORTIC DISRUPTION
inspired oxygen (e.g. PaO2 < 8.5 kPa or SaO2 < 90 per Blunt aortic injury is a deceleration injury commonly
cent) should be considered for elective ventilation. following high-speed road traffic crashes (RTCs) and
Pre-existing pulmonary disease should be taken into falls from a height. Up to 15 per cent of deaths from
account. road vehicle collisions are a result of damage to the
thoracic aorta (Williams et al., 1994). Most injuries
TRACHEOBRONCHIAL TREE INJURY occur in the proximal thoracic aorta, where the rela-
Tracheobronchial tree injuries are rare, but can easily tively mobile aortic arch can move against the fixed
be overlooked as signs can be subtle. Some 3 per cent descending aorta near the ligamentum arteriosum.
of chest-crushing injuries are associated with upper Complete transection or rupture is immediately fatal,
airway injuries, but most trachea-bronchial tree but the haematoma can be contained by the adventi-
injuries are within 1 inch of the carina. Patients fre- tial layer of the aortic wall, enabling the patient to sur-
quently present with haemoptysis, surgical emphy- vive to reach hospital.
sema and a simple or tension pneumothorax. The Specific clinical signs and symptoms are often
pneumothorax may be resistant to re-inflation with a absent, and the mechanism of injury should provoke
chest drain, and a post-drain and persistent air leak a high index of suspicion. Diagnosis is aided by chest
suggests the presence of a bronchopleural fistula. CT x-ray findings, classically of a widened mediastinum
and MRI imaging may confirm the diagnosis, but (note that an anteroposterior (AP) film will magnify a
bronchoscopy may be required. normal width mediastinum), loss of the aortic knuckle
22.23 Ruptured aorta

(a) Angiogram showing a
rupture of the arch of the
aorta. (b) CT scan showing
the haematoma around
the rupture.
652 (a) (b)

and deviation of the trachea to the right. Whilst nal structures (the heart, great vessels, tracheo- 22
angiography has been the gold standard diagnostic bronchial tree and oesophagus). The diagnosis is
tool, the advent of multidetector helical CT scanners made by careful examination of the chest, backed up
has supplanted the more invasive technique. Modern by chest x-ray and trauma CT imaging. The signifi-
CT scanning has an accuracy approaching 100 per cant clinical finding is an entrance wound in one
cent, and is highly specific for detecting the injury. hemithorax and an exit wound or radiologically visible

Initial management is supportive, but a contained missile in the other. Bullets and shrapnel can tumble,
haematoma may rupture if the patient is hypertensive. so the trajectory is unpredictable. The presence of
Blood pressure should therefore be controlled in fragments adjacent to the mediastinum on x-ray
patients with suspected blunt aortic injury until CT should raise suspicion of a traversing injury.
scanning has excluded the injury. Once the injury is Patients with symptomatic, haemodynamically
confirmed, the blood pressure must be controlled unstable mediastinal traversing wounds should be
until the patient can be taken to the operating theatre assumed to have an ongoing haemothorax, tension
for definitive cardiothoracic repair. Endovascular pneumothorax or cardiac tamponade.
repair is possible for some blunt aortic injuries. Initial management is ABC resuscitation with bilat-
eral chest drains, prior to definitive surgical manage-
TRAUMATIC DIAPHRAGMATIC INJURY ment. Stable patients should undergo extensive
Traumatic rupture of the diaphragm is associated with investigation with ultrasound, trauma CT, angiogra-
blunt and penetrating trauma to the abdomen. Blunt phy, oesophagoscopy and bronchoscopy as indicated,
trauma is usually the result of a lateral or frontal vehic- and on early consultation with a cardiothoracic sur-
ular collision, with distortion of the chest wall, shear- geon. Stable patients should be continually re-evalu-
ing of the diaphragm and compressive rise in ated as they can suddenly deteriorate and require
intra-abdominal pressure. Rupture is more common urgent surgical intervention; 50 per cent of patients
(in survivors) on the left side, probably because the with mediastinal traversing wounds are haemodynam-
severity of injury required to cause a right-sided rup- ically unstable on presentation, with a doubled mor-
ture above the protective liver is more usually fatal. tality of 40 per cent over those who are stable (Findlay
The injury is rarely found in isolation, and is associ- et al., 2007).
ated with other chest, abdominal and pelvic injuries.
Diaphragmatic ruptures associated with penetrating TAKE HOME MESSAGE
trauma are usually due to gunshot and stab injuries, The primary goal in management of traumatic chest
and result in a smaller tear with less visceral tissue pro- injuries is to rapidly identify and manage the six
truding through the diaphragm. immediately life-threatening injuries within the pri-
Signs and symptoms can be subtle, and the injury mary survey. The eight potentially life-threatening
missed, only becoming apparent years later as the injuries should be sought within the primary and sec-
herniation develops. The standard chest x-ray only ondary surveys, and may require sophisticated imag-
may show an elevated but indistinct hemidiaphragm; ing to diagnose. Only 15 per cent of chest injuries
however, the appearance of bowel gas or a nasogastric require operative intervention.
tube within the chest will help confirm the diagnosis.
Contrast studies via a nasogastric tube, CT and MRI
scanning are all useful adjuncts. Diaphragmatic
C – Circulation and shock
rupture and visceral herniation may be mistaken for a For the healthcare professional ‘shock’ is not the com-
haemothorax on the plain chest x-ray; however, the monly reported emotional condition in someone
insertion of a finger into the chest during chest drain witnessing a disturbing incident. It can be broadly
insertion may reveal the presence of stomach or bowel defined as circulatory failure, or inadequate perfusion
loops (hence the avoidance of sharp trochars to pre- of the tissues and organs with oxygenated blood.
vent visceral injury). Untreated, or inadequately treated, shock leads to
Initial management is supportive with careful assess- organ damage and ultimately death from multi-organ
ment and management of the ABCs. Careful chest drain failure. Recognition of shock, diagnosis of the cause
insertion is advisable prior to transfer or anaesthesia. and subsequent management are therefore important
Definitive treatment is surgical – the diaphragmatic steps in the resuscitation and care of the seriously ill or
rupture can be repaired during a trauma laparotomy, traumatized patient. The C for circulation follows the
but may require a thoracotomy or thoraco-abdominal A for airway and B for breathing, but in the presence
approach. of catastrophic, external bleeding from limb wounds,
control of the bleeding takes precedence. This is the
MEDIASTINAL TRAVERSING WOUNDS ABC sequence, and holds true in a hospital environ-
Penetrating objects that cross the mediastinum may ment if the airway and catastrophic limb bleed cannot
cause damage to the lungs and to the major mediasti- be managed simultaneously by the trauma team. 653
22 CIRCULATION AND SHOCK – AWARENESS within the bowel secondary to bowel damage caused
There are five main types of shock that can be by ischaemia. The profound vasodilatation that results
grouped into two pathogenic groups: dramatically reduces afterload; even with a normal
circulating blood volume and raised cardiac output, the
1. Vasoconstrictive: hypovolaemic and cardiogenic
patient’s blood pressure falls and the pulse pressure
shock.
widens, e.g. 110/70 Æ 90/30. Oxygen consumption
2. Vasodilative: septic, neurogenic and anaphylactic

increases, and despite the high cardiac output, tissue
shock.
perfusion and oxygenation are reduced, and organ
The majority of patients presenting with shock fol- damage results. The toxins can also damage the
lowing a major injury will be suffering from hypo- myocardium and cause capillary leakage, complicating
volaemic shock; however, any patient can present with the presentation with elements of cardiogenic and
a combination of types of shock. hypovolaemic shock.
Hypovolaemic shock Hypovolaemic shock results from Neurogenic shock Neurogenic shock is produced by
a loss of volume within the circulation; it may be due high spinal cord injury, which disrupts the sympathetic
to whole blood loss from haemorrhage, or plasma and nerves controlling vasoconstriction. The peripheral
fluid loss from burns or severe medical conditions. As vasculature relaxes and becomes profoundly dilated,
the circulating blood volume decreases, compensatory reducing pre-load and afterload. Even with a raised
mechanisms are triggered to preserve blood pressure cardiac output, the patient cannot maintain an
and vital organ perfusion. These mechanisms can adequate blood pressure, and shock ensues.
maintain systolic blood pressure up to around 30 per Neurogenic shock is not caused by an isolated head
cent blood loss in a fit patient. Above this, injury, and is different from ‘spinal shock’, which is a
compensation increasingly fails until unconsciousness, temporary flaccidity following spinal damage. Since
followed by death at around 50 per cent blood loss. neurogenic shock is always related to traumatic spinal
Early compensatory mechanisms are tachycardia cord damage, it is likely to co-exist with a degree of
and peripheral vasoconstriction with a narrowed pulse hypovolaemia from associated trauma.
pressure [vasoconstriction raises the diastolic blood Anaphylactic shock This is a type of allergic reaction.
pressure, bringing it closer to the systolic, e.g. Exposure to an antigen to which an individual has pre-
120/60 Æ 120/90]. Further compensations include viously been sensitized triggers off a cascade reaction.
tachypnoea, shift of fluid from tissues into circulation The mast cells degranulate and release large quantities
and reduced urine output. of histamine into the bloodstream. Other vasoactive
Some injuries mimic hypovolaemic shock, classically substances are released, and profound vasodilatation is
tension pneumothorax and cardiac tamponade; the caused. Massive capillary leakage results in sudden
low-output state follows obstruction to the venous oedema, which with loss of fluid into the bowel causes
return and cardiac output, respectively. Peripheral hypovolaemia [1 mm depth of oedema across the body
vasoconstriction is not a feature of these conditions in surface equates to a 1.5 L fluid loss]. This picture is
the absence of hypovolaemia, unlike cardiogenic complicated by other effects such as bronchospasm.
shock, and the veins remain full. Anaphylaxis can be triggered by many common
Cardiogenic shock Cardiogenic shock results from a antigens such as shellfish or peanuts. Of particular sig-
decrease in myocardial contractility, and hence a nificance to the hospital practitioner are allergies to
reduction in stroke volume and cardiac output. This drugs and latex.
classically follows myocardial infarction or severe
ischaemia, but can follow trauma damage to the CIRCULATION AND SHOCK – RECOGNITION
myocardium from blunt or penetrating injury, e.g. Recognition of shock is relatively easy in the late stages
fracture of the sternum. The disproportionate when signs of underperfusion are obvious. Earlier
vasoconstriction is due not to hypovolaemia, but an stages of shock present with more subtle signs that
outpouring of catecholamines and the profound require careful patient examination to elucidate; for
autonomic stimulus, which can put further strain on example, the systolic blood pressure may not drop
the heart by causing vasoconstriction and increasing significantly until 30 per cent of the patient’s blood
afterload. Trauma patients may present with volume has been lost. Hypovolaemic shock passes
cardiogenic shock if the cardiac event precedes, and through a number of clinical stages as blood loss
indeed causes, the traumatic event. increases, and these have been grouped into four classes
of shock, with increasingly apparent signs [adult blood
Septic shock This results from the entry of toxins into volume is approximately 7 per cent of ideal body
the circulation, which poison the vasoconstrictive weight, or 5 L for a non-obese man weighing 70 kg].
mechanisms within the blood vessels. These toxins It should be remembered, however, that the develop-
654 usually come from infection, or are released from ment and progression of shock is a continuum.
Blood loss of greater than 50 per cent (> 2500 mL) loss, but they deteriorate very rapidly when they 22
results in loss of consciousness, pulse and blood pres- decompensate. The pulse rate is a good indicator of
sure, and finally respiration, causing a hypovolaemic shock level, as is the respiratory rate; tables showing
PEA cardiac arrest. normal parameters for children at different ages are
The values shown in Table 22.1 relate to adults and available.
children above the age of 12. Younger children com- A reasonable approximation of blood pressure can

pensate more effectively to a greater degree of blood be gained from palpating pulses. However, practition-
ers tend to overestimate the blood pressure if pulses
are palpable, although there is wide variation (Deakin
CLASSES OF SHOCK and Low, 2000).
Recognition of shock therefore depends on a rapid
Class 1 – < 15 per cent loss blood volume (< 750 mL clinical assessment of the patient, with measurement
in a male weighing 70 kg) of the appropriate vital signs. The look, listen, feel
(no change in BP, pulse pressure, respiratory rate or sequence should be applied to identify the signs of
capillary refill) hypovolaemic shock; blood pressure and pulse alone
are not adequate.
• minimal tachycardia < 100 bpm
• skin pallor possible Look and listen
• peripheral/central cyanosis and pallor
Class 2 – 15–30 per cent loss blood volume • sweating
(750–1500 mL) • tachypnoea and respiratory distress
• change in mental status – anxiety, fear, aggression,
(no change in systolic blood pressure)
agitation
• Ø peripheral perfusion with cool, pale, clammy • depressed level of consciousness or unconsciousness
skin
• ≠ capillary refill > 2 seconds Feel
• tachycardia > 100 bpm • Peripheral perfusion poor – cool, clammy, shut
• Ø pulse pressure as diastolic BP rises down
• increased respiratory rate (tachypnoea) of 20–30 • Capillary refill time > 2 seconds (this is unreliable in
bpm cold and frightened patients)
• subtle mental status changes: anxiety, fear, • Pulse rate and character – tachycardia and thready
aggression pulse
• Loss of pulses – radials, then femorals, then carotids
Class 3 – 30–40 per cent loss blood volume (1500– as severity of shock increases
2000 mL) • Blood pressure – initially a raised diastolic with nar-
rowed pulse pressure, then drop in systolic and
marked tachycardia > 120 bpm diastolic, and finally an unrecordable blood pres-
• measurable fall in systolic blood pressure from sure.
patient’s normal, e.g. < 100 mmHg Observation of these factors will usually enable an
• thready peripheral pulses assessment to be made of the presence and level of
• flat/empty veins shock, and the likely degree of blood loss. This will act
• marked tachypnoea > 30 bpm as a guide to whether volume replacement is indi-
• significant mental status changes: agitated ++ cated, and if so how much.
• dropping urine output Hypovolaemic shock that remains unresponsive to
treatment is likely to be due to bleeding into the body
Class 4 – > 40 per cent loss blood volume cavities or potential spaces, and evidence of this
(> 2000 mL) should be sought. Diagnosis may be helped by trauma
• severe tachycardia > 140 bpm imaging such as FAST or CT. A useful reminder of
• moribund, decreased conscious level where to look is the catchy slogan: bleeding onto the
• significant drop in systolic blood pressure, e.g. floor and four more (i.e. external bleeding and chest,
< 70 mmHg abdomen, pelvis/retroperitoneum, long bones). Bear
• impalpable peripheral pulses, weak central in mind, though, that there are other forms of shock
pulses that need to be excluded.
• respiratory distress Cardiogenic shock can mimic many of the signs of
• central and peripheral cyanosis hypovolaemic shock. The history will give a good
• minimal urine output indication of the likely cause. The veins tend to be full
in cardiogenic shock, and cyanosis more profound. 655
22 There may be other diagnostic signs present such as Haemostatic dressings are useful for emergency
pulmonary oedema. control of arterial and venous haemorrhage from
Septic, neurogenic and anaphylactic shock are charac- proximal sites where tourniquets cannot be applied
terized by vasodilatation as opposed to vasoconstric- (Mahoney et al., 2005). Quikclot™ (granular zeolite,
tion. The veins tend to be full, and the peripheral derived from volcanic rock) can effectively control
pulses easily palpable and bounding. Peripheral perfu- devastating haemorrhage from large vessels, but gen-
sion may be good, with warm and flushed peripheries, erates tissue temperatures up to 570ºC, potentially
but the skin may be mottled or cyanosed with sepsis. causing tissue necrosis. HemCon™ (chitosan, derived
from crushed shellfish) is an alternative, which has the
advantage of not producing an exothermic reaction.
CIRCULATION AND SHOCK – MANAGEMENT
Clamping of bleeding points is difficult and can
Control of the airway (with cervical spine control), op-
damage vessels; this should remain the province of the
timal oxygenation and ventilation are prerequisites to
experienced surgeon.
shock management. Immediate management of haem-
Fracture of the pelvis can result in devastating
orrhagic shock depends on control of the bleeding
retroperitoneal haemorrhage; this can be reduced by
and administration of intravenous fluids and blood to
compressing the pelvis to approximate the bleeding
restore intravascular volume and haematocrit.
fracture sites. Compression can be achieved manually,
Control of haemorrhage This is achieved by direct with a towel or blanket passed under the patient and
pressure on the bleeding wounds with appropriate tightened from both sides above the pelvis, or with
dressings, and elevation where practicable. Continuing specialized devices such as the SAM Sling™. This is a
developments from military experience have led to the ratchet system compression belt for applying circum-
introduction of additional measures to control external ferential pressure around the pelvis. MAST trousers
and limb bleeding. Wounds can be packed with a are impracticable and now rarely used.
dressing, and a circumferential bandage applied around
Peripheral venous cannulation Intravenous access must
and over the packed wound. The bandage can then be
be secured at the earliest opportunity; this can be very
twisted in a windlass technique to press the pack down
difficult in later stages of shock. The size of the cannula
into the wound. Specialist bandages have been
is important because of its effect on flow, which is
designed for this purpose, such as the Oales™ Modular
directly proportional to the fourth power of the radius
Bandage. This incorporates a gauze bandage for
of the cannula (Poiseuille’s Law). As an example,
wound packing, with a plastic cup to compress into the
halving the radius of a cannula reduces the flow rate by
packed wound beneath a circumferential, elastic
a factor of 16. Flow is also reduced as the cannula
bandage.
lengthens.
Tourniquets have been developed for controlling
Clearly it is difficult, if not impossible, to keep up
peripheral limb haemorrhage, with devices such as the
with major haemorrhage without a minimum of two
Combat Application Tourniquet (C-A-T™). The C-A-
short, large-bore cannulae. Hence, the ATLS® guide-
T™ is a single-handed device that uses a windlass sys-
line for in-hospital trauma cannulation is insertion of
tem with a free moving internal band to provide
two cannulae, minimum size 16-gauge, but preferably
circumferential pressure around the extremity. Once
14-gauge, into large peripheral veins, typically in the
tightened and bleeding stopped, the windlass is
antecubital fossae.
locked in place. A Velcro® strap is then applied for
further securing of the windlass during casualty evac- Central venous cannulation This is an option reserved
uation. for those with appropriate expertise; it can be very
Once in place and controlling the bleeding, the difficult and carries a significant risk of life-threatening
tourniquet should not be loosened or removed until a complications (pneumothorax and arterial damage
surgeon is available to definitively repair the injury. most commonly). In the UK, the use of two-
Windlass Self-adhering
22.24 The C-A-T™ tourniquet
strap band (a) Tourniquet in use. (b) Tourniquet
components.
Windlass Windlass
rod clip
656 (a) (b)

22

(a)
22.25 SAM Sling™ ratcheted compression belt in use
dimensional (2D) ultrasound imaging is strongly

recommended in the routine siting of the CVP line.
Access to the internal jugular can be difficult in a
trauma patient, especially if he or she is immobilized
with a stiff cervical collar and head blocks in place. The
subclavian approach has the highest incidence of
complications; femoral cannulation is a safer option
than either central approach and a long cannula can
often be sited in the femoral vein, medial to the
femoral artery.
(b)
Intraosseus cannulation Intraosseous cannulation has
previously been reserved for young children up to the 22.27 Intraosseous cannulation. (a) The Cook
age of about 5 years, where intravenous cannulation is paediatric intraosseus needle. (b) Intraosseous needle in
not possible. The bone cortex is thin and relatively soft place in the medial proximal tibia.
in children, and the marrow plentiful and vascular. A
specialized 16-gauge intraosseus needle can be pushed
or screwed into the bone of the tibia, below and medial of the tibia. The FAST1® is designed to manually push
to the knee joint. Response time to drug a cannula into the manubrium. The more recent
administration is close to IV administration, and entire EZ-IO® system consists of a hand-held electric drill to
resuscitations can be performed through intraosseus ‘drill’ a cannula through the cortex of the tibia or
cannulae, including all anaesthetic drugs and fluids. humeral head.
Intraosseus cannulation for adults has been vali- Fluid administration Fluid administration has for long
dated, and specialist equipment is available for siting been a controversial issue. The traditional ATLS
the cannulae through the thick and tough adult bone approach for trauma circulation resuscitation, based on
cortex. The Bone Injection Gun (BIG) is a spring- military experience, is to site two large-bore
loaded device that fires a cannula through the cortex intravenous cannulae and administer an initial bolus of
2 L of warmed Ringer’s lactate or Hartmann’s
solution. This is certainly successful in improving
perfusion in bleeding patients, but is now not
recommended for pre-hospital use where haemorrhage
cannot be surgically controlled and blood is not
available for transfusion. Casualties bleeding to a level
3 or 4 shock can reach a steady state as the blood
pressure drops to a point where active bleeding may
cease. Restoring vascular volume with crystalloids or
colloids can restore the blood pressure to a point where
22.26 Cannulas A 16-gauge cannula (grey tap) has a bleeding resumes; further administration of clear fluids
20 per cent smaller diameter but 40 per cent less flow repeats the cycle until the haemoglobin level drops
than a 14-gauge cannula (orange tap). below a point where adequate oxygen can be carried. 657
22 Cardiac arrest and death then result from anaemic and CVP are all used to assess response. Serial meas-
hypoxia. urement of metabolic acidosis parameters such as
In the UK, NICE guidance on Pre-hospital Initia- bicarbonate, base deficit and lactate levels can be used
tion of Fluid Replacement Therapy in Trauma to gauge adequate response to fluid therapy. More
(National Institute for Clinical Excellence, 2004), sophisticated methods such as oesophageal Doppler
relating to traumatized casualties with likely haemor- and arterial waveform analysis are also used in the crit-
rhage, is to titrate intravenous crystalloid fluids in ical care setting.

250 mL boluses against the radial pulse. If a radial The use of hypertonic saline has been successfully
pulse cannot be felt, the fluids are administered until demonstrated, and may have some benefits over the
the pulse returns, then withheld. NICE emphasizes current use of isotonic fluids. Research with 7.5 per
the importance of not delaying transfer to hospital, cent saline and dextran (as opposed to isotonic 0.9 per
and suggest fluids are administered if necessary en cent) suggests that mean arterial blood pressure and
route. In penetrating chest wounds, fluids are titrated oxygen delivery are improved. Capillary damage is
against a palpable central pulse. This strategy is known lessened, and organ perfusion improved, with a much
as permissive hypotension. Assuming O Rhesus-nega- larger increase in the intravascular volume. Short-term
tive blood is immediately available in the Emergency survival is improved, but the role of hypertonic solu-
Department, the blood pressure can be brought up tions has yet to be determined.
with crystalloids pending rapid transfusion. The ultimate goal of synthetic, oxygen-carrying flu-
In UK practice, non-albumin colloid solutions are ids has been researched for decades, but as yet noth-
commonly used as plasma expanders (gelatine and ing has effectively replaced the supremely efficient red
starch formulations). These have a theoretical advan- blood cell. Blood transfusion should be given early if
tage in that they stay within an undamaged circulation haemorrhagic shock is demonstrated, with O Rhesus-
for longer than crystalloids (saline and Hartmann’s). negative, type-specific or cross-matched blood. Trans-
However, there is little robust evidence that there is a fusion should be titrated against the haematocrit, and
practical advantage, particularly as any shocked blood products such as fresh-frozen plasma, platelet
patient will develop leaky capillaries and nullify the concentrates and clotting factors given during massive
benefit of colloids. There is a risk of allergic reactions transfusions on the advice of the haematologists.
to these colloids, and NICE guidelines recommend The information given earlier refers to resuscitation
the use of crystalloids only. Large volumes (> 2 L) of of hypovolaemic patients only. Most other forms of
normal saline 0.9 per cent can cause a hyperchlo- shock will respond initially to IV fluids pending accu-
raemic acidosis, and a lactated, balanced electrolyte rate assessment and diagnosis. However, shock in eld-
solution such as Ringer’s lactate or Hartmann’s is erly casualties without evidence of major trauma should
preferable. raise a high index of suspicion for cardiogenic shock.
The dynamic response to a fluid challenge will give Infusion of even small volumes of fluid can overload the
information as to whether bleeding is continuous or circulation and cause collapse and cardiac arrest. Elderly
controlled. A 2 L volume of warmed Hartmann’s is patients may also be on medication for hypertension
initially given (20 mL/kg in children), and the etc., which can severely limit their ability to maintain an
response in vital signs recorded: adequate blood pressure and cardiac output. A drug
history should be obtained as soon as possible; patients
Rapid responders – respond rapidly and remain
on vasodilator drugs such as ACE inhibitors and sartans
haemodynamically normal, having lost < 20 per
may need inotropes to support the circulation, even if
cent blood volume. No further fluid is required
the patient is hypovolaemic.
and surgical intervention may be required.
Transient responders – respond to the initial bolus, Take home message In patients suffering from
then deteriorate, having lost 20–40 per cent blood haemorrhagic, hypovolaemic shock the source of the
volume. These patients will need further fluid bleeding must be identified and surgically or radiolog-
administration and blood transfusion, with ically controlled. The priorities for restoring and main-
probable surgical intervention. taining adequate circulation are:
Non-responders – show minimal or no response to
• control external bleeding
the initial bolus. These patients are likely to
• restore intravascular volume
require immediate transfusion and surgery to stop
• transfuse blood
exsanguinating haemorrhage. There may be other
• turn off the tap – call a surgeon early.
causes such as tension pneumothorax, cardiac
tamponade or non-haemorrhagic shock.
Fluids should be titrated against response, with
D – Disability – head injury
optimum organ and peripheral tissue perfusion the The immediate management of the seriously head-
658 goal. Blood pressure, pulse rate, peripheral perfusion injured patient is designed to prevent secondary
injury and to provide the neurosurgeon with a live 1. Dura mater – a tough, fibrous layer, firmly 22
patient who has some hope of recovery. A significant adherent to the inner skull.
number of fatalities from head injury are caused by 2. Arachnoid mater – a thin, transparent layer, not
the secondary and not the primary injury; prevention adherent to the overlying dura and so presenting
of this secondary brain injury is facilitated by follow- a potential space. Cerebrospinal fluid (CSF) is
ing the ABC principles set out in ATLS®. contained and circulates within this space.

3. Pia mater – a thin, transparent layer, firmly
adherent to the underlying surface of the brain.
HEAD INJURIES – AWARENESS
In the UK, severe head injuries account for more than The brain itself is divided into three main struc-
50 per cent of trauma-related deaths, and these usu- tures:
ally follow road traffic crashes, assaults and falls (Flan-
1. Cerebrum – composed of right and left
nery and Buxton, 2001). Injury patterns differ
hemispheres, divided into:
between countries; in the UK patients experience pre-
• frontal lobes – emotions, motor function,
dominantly closed injuries, with a peak incidence in
speech
males between the ages of 16 and 25 years. A second
• parietal lobes – sensory function, special
peak occurs in the elderly, with a high incidence of
orientation
chronic subdural haematomas.
• temporal lobes – some memory and speech
Only 10 per cent of head-injured patients present-
functions
ing at Emergency Departments have a severe injury.
• occipital lobes – vision
The injuries can be classified into three groups based
on the GCS (American College of Surgeons Commit- 2. Cerebellum – coordination and balance
tee on Trauma, 2004):
3. Brainstem – composed of three main structures:
Mild (80 per cent) GCS 13–15 • midbrain – reticular activating system (alertness)
Moderate (10 per cent) GCS 9–12 • pons – relays sensory information between cere-
Severe (10 per cent) GCS 3–8 brum and cerebellum
• medulla – vital cardiorespiratory centres.
Investigation, management and outcomes depend
The midbrain passes through a large opening in the
on the severity of the injury; however, this is a con-
tentorium, a fibrous membrane that divides the mid-
tinuum, and the classification given earlier is only a
dle and posterior fossae. The third cranial nerve,
guideline. Even mild head injuries can be associated
which controls pupillary constriction, also runs
with prolonged morbidity in the form of headaches
through this opening, and is vulnerable to pressure
and memory problems; only 45 per cent are fully
damage if the cerebral hemispheres swell. This results
recovered 1 year later. With moderate head injuries,
in pupillary dilatation, an early sign of a significant rise
63 per cent of patients remain disabled 1 year after the
in intracerebral pressure.
trauma, and this rises to 85 per cent with severe
injuries (Royal College of Surgeons of England, Pathophysiology The skull is in effect an enclosed,
1999). bony box containing the brain, blood vessels and the
A knowledge of anatomy and pathophysiology is CSF. The intracerebral pressure (ICP) is normally
needed to understand and anticipate the development maintained at approximately 10 mmHg, and is a
of a head injury. balance of brain, intravascular and CSF volumes.
The scalp comprises five layers of tissue, with the Traumatic damage to the brain can cause swelling of
mnemonic SCALP: skin, connective tissue, aponeuro- the brain tissue itself, and bleeds from arteries and veins
sis, loose areolar tissue, and periosteum. It has a into the extradural space, subdural space or brain
generous blood supply and serious scalp lacerations substance (intracerebral bleed) increase the
can result in major blood loss and shock if bleeding is intracerebral volume and raise the ICP. If the ICP is
not controlled. sustained at above 20 mmHg, permanent brain
The skull is composed of the cranial vault and the damage can result, with poor outcomes; this is the
base. The vault has an inner and outer table of bone, secondary brain injury. There is only limited,
and is particularly thin in the temporal regions, intracranial compensation for rising ICP, and this is
although protected by the temporalis muscle. The largely achieved by a reduction in CSF volume
base of the skull is irregular, which may contribute to (Monroe-Kelly doctrine). Once pressure compensation
accelerative injuries. The floor of the cranial cavity has has reached its limits, the ICP rises rapidly in a
three distinct regions: the anterior, middle and poste- breakaway exponential.
rior fossae: As the pressure rises, the conscious level decreases
The meninges cover the brain and consist of three and the GCS falls. The medial part of the temporal
layers: lobe (the uncus) herniates through the tentorial 659
22
(a)
(b) (c)
(d) (e) (f)
22.28 Fractured skull – imaging (a) X-ray showing a depressed fracture of the skull. (b–f) CT scans showing various
injuries: (b) a fracture; (c) an extradural haematoma; (d) a subdural haematoma and compression of the left ventricle;
(e) an intracerebral haematoma; (f) diffuse brain injury with loss of both ventricles.
notch, compressing the third cranial nerve and the brain injury as the resultant pressure wave moves across
midbrain pyramidal tracts. This usually results in the brain. The secondary brain injury is pressure
pupillary dilatation on the side of the injury, and related, and is caused by swelling within the brain,
hemiplegia on the opposite side. Pressure changes in causing a rise in ICP as described earlier. This is
the medulla cause a sympathetic discharge, with a rise compounded by hypoxia, hypercarbia and
in blood pressure and reflex bradycardia. With further hypotension.
pressure rise, cerebral blood flow is compromised, and
ceases terminally when the ICP rises above the mean Severity of brain injury The GCS is a well-tested and
arterial pressure (MAP). Ultimately, the cerebellar objective score for assessing the severity of brain injury:
tonsil is forced into the foramen magnum, resulting in 13–15 is mild; 9–13 is moderate; 8 or less is severe.
a loss of vital cardiorespiratory function; this is known
Morphology of brain injury Skull fractures are seen in
as brain stem or brain death, and is a terminal event.
the cranial vault or skull base; they may be linear or
Mechanism of brain injury Brain injury can be blunt or stellate, and open or closed. The significance of a skull
penetrating. The primary brain injury occurs at the fracture is in the energy transfer to the brain tissue as a
time of the trauma, and results from sudden distortion result of the considerable force required to fracture the
and shearing of brain tissue within the rigid skull. The bone. Open skull fractures may tear the underlying
damage sustained may be focal, typically resulting from dura, resulting in a direct communication between the
a localized blow or penetrating injury, or diffuse, scalp laceration and the cerebral surface, which may be
typically resulting from a high-momentum impact. extruded as ICP rises.
Sudden acceleration or deceleration can cause a contra- Basal skull fractures are caused by a blow to the
coup injury, as the brain impacts on the side of the skull back of the head, or rapid deceleration of the torso
away from the impact. High-velocity missile with the head unrestrained, as in high-speed vehicular
660 penetrating injuries will also cause a diffuse and severe crashes. Fractures are rare, occurring in 4 per cent of
severe head injuries, but can cause severe damage, and made that the neck is unstable until proved otherwise. 22
are a cause of death in front-end collisions and motor As the cervical spine x-ray does not rule out a fracture,
sport crashes. There are key physical signs pathog- full immobilization should remain in place until the
nomic of basal skull fracture: neck is cleared clinically or with further imaging such
as CT.
• peri-orbital ecchymosis (bruising – ‘raccoon’ or
A more thorough assessment of the neurological
‘panda’ eyes)

status takes place during the secondary survey. The
• retro-auricular ecchymosis (Battle sign – bruising
GCS and pupils are re-evaluated, lateralizing signs are
behind ears)
looked for, and the upper and lower limb motor and
• oto-rhinorrhea (CSF leakage from nose and ears)
sensory function evaluated. If the patient is stable, fur-
• VIIth and VIIIth cranial nerve dysfunction (facial
ther imaging may be indicated, and a number of
paralysis and hearing loss)
guidelines exist to aid the decision.
Basal skull fractures are not always visible on x-ray CT scanning is the primary examination of choice
or CT, but blood in the sinus cavities and the clinical for patients with a clinically important brain injury
signs should suggest their presence. (National Institute for Health and Clinical Excellence,
Diffuse brain injury is due to axonal disruption of 2007). Modern, fast, spiral CT scanners are increas-
the neurones and varies from minor, resulting in mild ingly available adjacent to Emergency Departments,
concussion, to severe, resulting in an ultimately fatal enabling rapid trauma CTs in the course of minutes.
hypoxic and ischaemic insult to the brain. All patients suffering a severe head injury require an
Extradural (epidural) haematomas are relatively urgent CT scan. Specific indications for a head CT are
uncommon, occurring in 0.5 per cent of all brain- (Royal College of Surgeons of England, 1999):
injured patients, and 9 per cent of those who are com-
• GCS < 13 on first Emergency department assess-
atose (Findlay et al., 2007). The haematoma is
ment
contained outside the dura but within the skull, and is
• GCS < 15 2 hours after initial assessment
typically biconvex or lenticular in shape. They are
• suspected open or depressed skull fracture
commonly located in the temporal or temporoparietal
• clinical basal skull fracture
region, and usually result from a middle meningeal
• post-traumatic seizure
artery caused by a fracture.
• focal neurological deficit
Subdural haematomas are more common, and con-
• > 1 episode of vomiting
stitute 30 per cent of severe brain injuries (Findlay et
• amnesia of events > 30 minutes before impact
al., 2007). They usually result from tearing of cortical
• post-injury amnesia if:
surface vessels, and normally cover the entire surface
age > 65 years
of the hemisphere. Underlying brain damage is usu-
associated with coagulopathy
ally much more severe due to the greater energy trans-
due to a dangerous mechanism of injury
fer.
(pedestrian versus motor vehicle, ejection from
Contusions and intracerebral haematomas are fairly
motor vehicle, fall from height > 1 m).
common (20–30 per cent of severe brain injuries).
The majority occur in the frontal and temporal lobes.
HEAD INJURIES – MANAGEMENT
Inoperative contusions can evolve into haematomas
The management of head injuries depends on the
requiring surgical evacuation over a period of hours or
severity, as assessed by the clinical examination, GCS
days, and repeat CT scanning within 24 hours may be
and CT scan. Patients with a mild head injury should
indicated.
be admitted and monitored, with frequent neurolog-
ical observations. Should there be any deterioration,
HEAD INJURIES – RECOGNITION
CT scanning is indicated, and referral to the local neu-
Initial recognition of a head injury takes place in the
rosurgical unit is necessary. Discharge is when a com-
primary survey as part of the ABCDE sequence. The
plete neurological recovery has been made and
airway, cervical spine, breathing and circulation must
provided the patient can be supervised at home by a
all be assessed and resuscitation commenced before
responsible adult.
the brief neurological assessment takes place, as these
Patients sustaining moderate head injuries will need
measures will prevent the development of a secondary
CT scanning and discussion with a neurosurgeon to
brain injury. The AVPU score is an instant and useful
decide on the need for transfer and definitive care.
assessment but the level of consciousness should be
Other indications for neurosurgical referral, regardless
assessed accurately at this point, using the GCS. The
of imaging findings, include:
pupils are assessed for equality, diameter and response
to light. • persistent coma after initial resuscitation (GCS < 8)
As there is a 5–10 per cent association of cervical • unexplained confusion > 4 hours
spine fracture with head injury, the assumption is • post-admission deterioration in GCS 661
22 • progressive, focal neurological signs E – Abdominal injuries
• seizure without full recovery
• definite or suspected penetrating injury The abdomen is difficult to assess in the multiply
• CSF leak. injured trauma patient, especially when the patient is
unconscious. The immediately life-threatening injury
Patients with severe head injuries will require is bleeding into the abdominal cavity, and this is one
immediate resuscitation as described previously. The of the ‘onto the floor and four more’ areas into which
cervical spine must be immobilized whilst the airway lethal volumes of blood may be sequestered. The
is secured; this will require a competent, rapid abdomen is therefore examined in the primary survey
sequence induction (RSI) of anaesthesia, and an as part of the circulation assessment.
anaesthetist must be involved early. Once the airway is
secured and protected with a tracheal tube, the oxy- ABDOMINAL INJURIES – AWARENESS
genation and ventilation must be optimized. Hypoxia Abdominal injuries may be blunt or penetrating.
and hypercarbia must be avoided, but overventilation Unrecognized abdominal injury is a cause of avoid-
is equally damaging, as cerebral blood flow is com- able death after blunt trauma and may be difficult to
promised. Ventilation must be monitored with end- detect. A direct blow from wreckage intrusion or
tidal carbon dioxide analysis, and the minute volume crushing from restraints can compress and distort hol-
adjusted to maintain a low-normal EtCO2 (4.5 kPa). low viscera, causing rupture and bleeding. Decelera-
Oxygen saturation levels should be maintained above tion causes differential movement of organs, and the
95 per cent, and sequential arterial blood gas estima- spleen and liver are frequently lacerated at the site of
tions made to ensure the oxygen partial pressure is supporting ligaments. In patients requiring laparo-
maintained in the normal range (> 13 kPa) as far as is tomy following blunt trauma, the organs most com-
possible. monly injured are (Findlay et al., 2007):
The circulation should be monitored to maintain • spleen (40–55 per cent)
intravascular filling within an appropriate range. Over- • liver (35–45 per cent
filling will worsen cerebral oedema, but hypovolaemia • small bowel (5–10 per cent)
will result in persistent shock. Central venous pressure • retroperitoneum (15 per cent).
should be monitored, and arterial pressures kept
within a normal range for that patient, with reference The mechanism of injury should lead to a high
to the ICP. This requires expert critical care skills, and index of suspicion, e.g. flexion lap-belt injuries in car
patients with a severe brain injury must be managed crashes can rupture the duodenum, with retroperi-
in an appropriate critical care unit. toneal leakage and subtle signs. Early imaging and
The rapid administration of intravenous mannitol at exploratory laparotomy may be required.
a dose of 0.5 mg/kg may be indicated to reduce ICP, Penetrating injuries between the nipples and the
and this should be given following discussion with the perineum may cause intra-abdominal injury, with
referral neurosurgeon. It can be a useful holding unpredictable and widespread damage resulting from
measure if signs of rising ICP (e.g. a dilated pupil) tumbling and fragmenting bullet fragments. High-
develop prior to or during transfer to a specialist velocity rounds transfer significant kinetic energy to
centre. the abdominal viscera, causing cavitation and tissue
Patients with significant head injuries in units with- destruction. Gunshot wounds most commonly
out neurosurgical capability will require transfer, on involve the:
discussion with the neurosurgeons. An expanding
intracerebral haematoma will need to be evacuated
within 4 hours of injury to prevent serious and per-
manent secondary brain injury.
TAKE HOME MESSAGE

Head-injured patients require early assessment and
recognition of their brain injury. With severe head
injuries, it should be remembered that:
1. A blow to the head causes a primary brain injury.

2. Hypoxia and hypercarbia cause cerebral swelling
and a secondary brain injury.
3. Secondary brain injury should be minimized by
optimal oxygenation, ventilation and blood 22.29 Abdominal injury Ruptured duodenum following
662 pressure management. flexion lap belt injury.
• small bowel (50 per cent) may not be possible with an unstable patient. Other 22
• colon (40 per cent) indications for laparotomy include:
• liver (30 per cent)
• unexplained shock
• abdominal vasculature (25 per cent).
• rigid silent abdomen
Stab wounds injure adjacent abdominal structures. • evisceration
Small wounds may result from thin-bladed knives that • radiological evidence of intraperitoneal gas

have penetrated deep and damaged several structures, • radiological evidence of ruptured diaphragm
with the most common injuries being: • gunshot wounds.
• liver (40 per cent) A naso- or oro-gastric tube should be passed in all
• small bowel (30 per cent) multiple trauma patients; this should be passed orally
• diaphragm (20 per cent) in the presence of facial and basal skull fractures. A
• colon (15 per cent). urinary catheter should be passed unless urethral
bleeding or other signs of urethral injury such as gen-
ital bruising or a high-riding prostate are present.
ABDOMINAL INJURIES – RECOGNITION
Laparotomy is the definitive management and the
The abdomen is initially examined during the primary
province of the surgeon; general principles at initial
survey to determine if shock is due to an abdominal
operation are to:
injury. A history from the patient, bystanders and
paramedics is important, as the mechanism of injury • control haemorrhage with ligation of vessels and
can be identified and injuries predicted. packing
Examination of the abdomen follows the ‘look, lis- • remove dead tissue
ten, feel’ format. The patient must be fully exposed, • control contamination with clamps, suturing and
and the anterior abdomen inspected for wounds, stapling devices
abrasions and contusions. • lavage the abdominal cavity
The flanks and posterior abdomen and back should • close the abdomen without tension.
be examined, and this may require log rolling to both
Initial surgery may be for damage limitation rather
sides. Auscultation is difficult in a noisy resuscitation
than definitive treatment, and a second-look laparo-
room, but may reveal absence of bowel sounds caused
tomy at 24–48 hours may be indicated to allow:
by free intraperitoneal blood or gastrointestinal fluid.
Percussion and palpitation may reveal tenderness or • removal of packs
peritonism. The genitalia and perineum should be • removal of dead tissue
examined, and a rectal examination performed during • definitive treatment of injuries
the log roll. • restoration of intestinal continuity
Early imaging is indicated (a FAST examination will • closure of musculofacial layers of the abdominal wall.
reveal the presence of intraperitoneal fluid) and can be
The patient will require supportive critical care, and
performed in the resuscitation room; however, the
may require ventilation on an ICU until after the sec-
technique has a high specificity but low sensitivity.
ond-look laparotomy.
Presence of fluid is an indication for laparotomy. CT
scanning requires the patient to be stable, but is a
TAKE HOME MESSAGE
much more effective diagnostic tool. Diagnostic peri-
Abdominal injuries are difficult to assess in the multi-
toneal lavage is a technique largely supplanted by
ply injured patient. The immediate threat to life is
FAST and CT, but if these are unavailable it may still
bleeding into the peritoneal cavity, and early imaging
be used. It should be performed by the surgeon who
with FAST and CT should be considered. Shock
would take the patient to the operating theatre.
should be treated, and early consultation with a sur-
geon facilitated. Diagnostic or definitive treatment
ABDOMINAL INJURIES – MANAGEMENT laparotomy may be required.
Initial management of an abdominal injury is to man-
age shock as described in circulation management.
External bleeding is controlled with direct pressure,
F – Musculoskeletal injuries
wound packing or haemostatic dressings. Intravenous In the absence of catastrophic bleeding, musculo-
access is established with two large-bore cannulae, and skeletal injuries are not immediately life-threatening.
2 L of warmed Hartmann’s or Ringer’s lactate infused They are, however, limb threatening and potentially
at speed. If the shock remains unresponsive, further life-threatening. Definitive management is detailed
fluid is administered, and blood transfused. Confirma- elsewhere in this book, so this section will merely put
tion of bleeding into the abdomen is an indication for these injuries into the context of the overall manage-
immediate laparotomy, and imaging other than FAST ment of a severely injured casualty. 663
22 PELVIC FRACTURES multiple injuries. A missed spinal injury can have dev-
Awareness The pelvis and retroperitoneum constitute astating consequences. Immediate management
one of the ‘onto the floor and four more’ spaces into therefore focuses on immobilization, recognition and
which blood can be sequestered to a level resulting in referral for definitive care.
non-responsive shock. A haemorrhaging fracture of the
Awareness Spinal injuries can be stable or unstable, an
pelvis therefore becomes a life-threatening emergency,
unstable injury being one where there is a significant

and should be considered in every patient with a
risk of fracture displacement and neurological sequelae.
serious abdominal or lower limb injury. Potential
The mechanisms of injury are traction (avulsion),
causes are road accidents, falls from a height or crush
direct injury and indirect injury. Direct injuries are
injuries.
penetrating wounds usually associated with firearms
Recognition The pelvis is examined in the primary and knives. Indirect injuries are the most common, and
survey as part of the C – circulation assessment, once are typically the result of falls from a height or vehicular
the airway and breathing have been assessed, and the accidents where there is violent free movement of the
cervical spine immobilized. Significant signs are neck or trunk. There is an association of cervical spinal
swelling and bruising of the lower abdomen, thighs, damage with injuries above the clavicles, and some 5
perineum, scrotum or vulva, and blood at the urethral per cent of head-injured patients have an associated
meatus. The pelvic ring should be gently palpated for spinal injury; 10 per cent of those with a cervical spine
tenderness side to side and front to back; however, if fracture have a second, non-contiguous spinal fracture.
clinical suspicion is high, the pelvis should not be Regional occurrences of spinal injuries are
compressed for crepitus, as this can dislodge a clot approximately:
from the fracture site and provoke further bleeding. If
• cervical (55 per cent)
tenderness and crepitus are elicited, the examination
• thoracic (15 per cent)
should not be repeated.
• thoracolumbar junction (15 per cent)
An AP x-ray should be obtained during the primary
• lumbosacral (15 per cent).
survey, and in most cases will enable a preliminary
diagnosis of pelvic fracture to be made. If the patient Spinal fractures with spinal cord transection also
is stable, a trauma CT scan will give more detailed disrupt the sympathetic nerve supply and cause distal
information, and also provide information on intra- vasodilatation. A high spinal transection will therefore
abdominal and retroperitoneal bleeding. cause neurogenic shock – this is vasodilatory shock
and is characterized by hypotension, a low diastolic
Management The immediate management of a pelvic
blood pressure, widened pulse pressure, warm and
fracture resulting in shock is to control the bleeding
well perfused peripheries and bradycardia. However,
and restore volume as described previously. There are
neurogenic shock can be complicated by hypo-
a number of proprietary devices available to wrap
volaemic shock in multiply injured patients.
around the pelvis and apply compression to
approximate the bleeding fracture sites and allow clot Recognition The spinal column and neurological
formation. If these are not available, manual function are examined in the secondary survey, with
approximation can be used; this can be facilitated with immobilization maintained throughout. Whilst the
a sheet wrapped around the pelvis and twisted head is immobilized manually, and the patient log-
anteriorly. rolled, the cervical spine and vertebral column from
Once in place, the pelvic compression devices neck to sacrum are examined for:
should not be removed until surgical interventions
• bruising, contusions and ecchymosis
such as external fixation are available. Developments
• penetrating injury
in interventional radiology and angiography have
• swelling or ‘bogginess’
enabled embolization to be used to control haemor-
• tenderness on palpation
rhage from a fractured pelvis.
• step or misalignment between vertebrae.
Take home message Pelvic fractures can result in life-
A rectal examination is performed to assess anal
threatening haemorrhage and should be recognized
tone. A neurological examination is carried out to
and managed as part of the circulation assessment
identify loss of sensory and motor function.
during the primary survey. Pelvic compression devices
If the casualty is conscious, has no neck pain, has no
should be used to minimize bleeding, and a rapid,
distracting painful injury, is not intoxicated and has
surgical referral made for definitive management.
not received any analgesia, the cervical spine can be
examined and a fracture clinically excluded. Head
SPINAL INJURIES blocks, cervical collar and tape are removed, and the
Vertebral column injury, with or without neurological patient taken through a full range of active move-
664 damage, must be considered in all patients with ments (i.e. patient’s voluntary movement). If there is
neither pain nor neurological symptoms on move- to the ABCs. Immobilization is crucial throughout, 22
ment, the cervical spine can be cleared. and ventilatory and circulatory failure must be
X-rays are of limited use in the resuscitation phase recognized and managed. Injuries should be excluded
as they do not reliably exclude unstable fracture-dislo- clinically, or with CT and MRI, as soon as possible.
cations. Hence, they do not alter initial management.
Plain x-rays of the spinal column are therefore taken
LONG-BONE INJURIES

during the secondary survey. Since cervical fractures
Long bone injuries can be spectacular, but should not
cannot be radiologically excluded in patients who do
distract from the injuries compromising the airway,
not meet the criteria for clinical cervical spine clear-
breathing or circulation. They are limb threatening,
ance as above, CT or MRI may be required.
but not immediately life-threatening, and in the
Management Initial management follows the ATLS® absence of catastrophic bleeding can be addressed in
ABCDE sequence. The cervical spine must be the secondary survey.
immobilized at all times; deterioration of neurological
Awareness Musculoskeletal injuries occur in 85 per
function of even one myotome can cause a devastating
cent of patients sustaining blunt trauma (Findlay et al.,
loss of motor function, with absence of any useful
2007). Major injuries signify significant force applied
function. However, only 5 per cent of multiply injured
to the body, and so are associated with an increased
patients have cervical spine injuries, in contrast to the
incidence of chest, abdomen and pelvis damage.
high percentage of patients with compromised airways;
Although not immediately-life threatening, they
this is particularly significant with head injuries. In high
present a potential threat to life and prejudice the
spinal transections, the patient’s respiratory function
integrity and survival of the limb. Crush injuries can
may be compromised, leading to ventilatory failure.
lead to compartment syndrome, and myoglobin release
The airway must be maintained without causing neck
with the risk of renal failure. These injuries must
flexion or extension, and secured and protected with
therefore be addressed as soon as the resuscitation
careful anaesthetic induction and intubation. This can
priorities have been addressed.
be successfully done with specialist laryngoscopes such
as the McCoy (lever activated, flexing tip to lift the Recognition The casualty must be fully exposed, log-
epiglottis), in conjunction with an intubating catheter. rolled and examined from head to toe in all planes. The
The procedure should be carried out by an experienced limbs are examined visually for:
anaesthetist.
• colour and perfusion
Oxygenation and ventilation is optimized, monitor-
• wounds
ing SaO2 and EtCO2. The neurogenic shock will
• deformity (angulation and shortening)
require judicious use of intravenous fluids, and may
• swelling
need circulatory support with vasoconstrictors and
• discoloration and bruising.
chronotropes.
The spinal fracture and neurological deficits are The extremities are then palpated to detect tender-
managed by immobilization and referral to a spinal ness, swelling and deformity, indicating underlying
surgeon. fractures and dislocations. Crepitus may be felt, but
should not be specifically elicited. Peripheral circula-
Take home message Spinal injuries should be identified
tion is assessed with palpation of pulses and capillary
during the secondary survey and managed according
refill. Doppler ultrasound examination may be needed
to confirm the presence of pulses – however, the pres-
ence of a pulse does not exclude compartment syn-
drome. X-rays should be obtained as indicated as soon
as the patient is stable.
Management The immediate management is to ensure
the airway and ventilation are optimized, and then
control limb haemorrhage with direct pressure,
tourniquets, wound packing and haemostatic dressings
as described previously. Large tissue deficits may need
ongoing fluid and blood replacement as immediate
haemorrhage control can be difficult.
Fractures and dislocations are splinted in the anatom-
ical position where possible, to minimize neurovas-
cular compromise, and significant analgesia may be
required to facilitate this (e.g. Entonox, morphine or
22.30 McCoy flexing tip laryngoscope ketamine 0.5 mg/kg intravenously). The anatomical 665
22 THERMAL BURNS – AWARENESS
Major burns can present a threat to life through com-
promise of the airway, breathing and circulation. In
addition, those burned may suffer other traumatic
harm due to explosions etc. and can present with any
of the systemic injuries described previously. Circum-
ferential burns around the neck can cause tissue

(a) (b) swelling and airway obstruction, and burns around
22.31 (a) Traumatic the chest may cause restrictive respiratory failure.
amputation, (b) blast Large burns result in significant fluid shifts, and
dressing and (c) blast resultant shock. In combination with coma from toxin
dressing in situ inhalation, burns present a potent mix of assaults on a
casualty’s life.
Cell damage occurs at a temperature greater than
45°C (113°F) owing to denaturation of cellular pro-
tein; a burn’s size and depth are functions of the burn-
(c) ing agent, its temperature and the duration of exposure.
Thermal injury to the skin damages the skin’s ability to
function as a semi-permeable barrier to evaporative
position should not be forced if resistance is felt, e.g. water loss, resulting in free water loss in moderate to
posterior hip dislocation. large burns. Other functions such as protection from
Tetanus toxoid should be given, and the patient the environment, control of body temperature, sensa-
referred urgently to an orthopaedic surgeon for defin- tion and excretion can also be harmed. Systemic effects
itive management. Significant fractures, compound include hormonal alterations, changes in tissue acid–
fractures and dislocations may need operative inter- base balance, haemodynamic changes and haemato-
vention whilst life-saving abdominal or neurological logical derangement. Massive thermal injury results in
surgery is taking place. an increase in haematocrit with increased blood vis-
cosity during the early phase, followed by anaemia
Take home message Limb injuries are not immediately from erythrocyte extravasation and destruction. Va-
life-threatening in the absence of catastrophic soactive substances are released and a systemic inflam-
haemorrhage. They should be recognized and initially matory reaction can result.
managed in the secondary survey. Splinting and
immobilization are instituted before prompt surgical Inhalational burns Inhalation of super-heated gases and
consultation. inhalation of toxic smoke in entrapment result in
Traumatic amputations, de-gloving injuries and inhalational burns and smoke inhalation. Inhalational
blast injuries can be initially managed with specialist injury is now the main cause of mortality in the burns
blast dressings. patient, and half of all fire-related deaths are due to
smoke inhalation. Direct thermal injury is usually
G – Burns (thermal, chemical, electrical, limited to the upper airway above the vocal cords, and
can result in rapid development of airway obstruction
cold injury)
due to mucosal oedema. Smoke has two noxious
A burn is a broad term that encompasses not only components: particulate matter and toxic inhalants.
thermal injury to tissues from heat, but injury from The particles are due to incomplete combustion, are
electric shock, chemicals and cold. In the UK, some usually less than 0.5 μm in size and can reach the
250 000 burn victims attend hospital each year, of terminal bronchioles, where they initiate an
whom 16 000 are admitted; in the USA, about 1.25 inflammatory reaction, leading to bronchospasm,
million burns occur annually, with 51 000 patients oedema and respiratory failure.
hospitalized. The risk is highest in the 18–35 year age Toxic inhalants are divided into three main groups:
group, with a male to female ratio of 2:1 for both (1) tissue asphyxiants; (2) pulmonary irritants; (3) sys-
injury and death, and serious burns occur most fre- temic toxins. The two major tissue asphyxiants are
quently in children under 5 years of age. There are carbon monoxide and hydrogen cyanide. Carbon
some 4500 burns deaths each year in the USA, and monoxide poisoning is a well-known consequence of
the death rate is much higher in those over the age of smoke inhalation injury. Severe carbon monoxide poi-
65. The last two decades have seen much improve- soning will produce brain hypoxia and coma, with loss
ment in burns care, and the mortality rate is now 4 of airway protective mechanisms, resulting in aspira-
per cent in those treated in specialist burns centres tion that exacerbates the pulmonary injury from
666 (Schwartz and Balakrishnan, 2004). smoke inhalation. The tight binding of the carbon
monoxide to the haemoglobin, forming carboxy- airway, breathing and circulation and conscious level. 22
haemoglobin, is resistant to displacement by oxygen, The likelihood of coincidental traumatic injuries
and so hypoxia is persistent. Hydrogen cyanide is should be remembered.
formed when nitrogen-containing polymers such as The patient is examined following the look, listen,
wool, silk, polyurethane, or vinyl are burned. Cyanide feel format. Diagnosis of an inhalational burn is made
binds to and disrupts mitochondrial oxidative phos- from the history of a fire in an enclosed space and

phorylation, leading to profound tissue hypoxia. physical signs that include facial burns, singed nasal
hair, soot in the mouth or nose, hoarseness, carbona-
Depth of burns The depth of a burn is classified
ceous sputum, and expiratory wheezing. There is no
according to the degree and extent of tissue damage:
single method capable of demonstrating the extent of
First degree burns involve only the epidermis, and
inhalation injury. Stridor is a particularly sinister find-
cause reddening and pain without blistering. They heal
ing, as it indicates an imminent loss of the airway.
within 7 days and require only symptomatic treatment.
Carboxyhaemoglobin levels for carbon monoxide
Second degree burns extend into the dermis, and can
poisoning are useful to document prolonged exposure
be subdivided into superficial partial-thickness and
within an enclosed space with incomplete combus-
deep partial-thickness burns.
tion, as the cherry red skin colour is rare.
In superficial partial-thickness burns, the epidermis
and the superficial dermis are injured. The deeper layers Table 22.3 Diagnosis of carbon monoxide poisoning
of the dermis, hair follicles, and sweat and sebaceous
Carbon monoxide level Physical symptoms
glands are spared. A common cause is hot water scald-
ing. There is blistering of the skin and the exposed der- < 20 per cent No physical symptoms
mis is red and moist at the blister’s base. These burns 20–30 per cent Headache and nausea
are very painful to touch. There is good perfusion of 30–40 per cent Confusion
the dermis with intact capillary refill. Superficial partial-
40–60 per cent Coma
thickness burns heal in 14–21 days, scarring is usually
minimal, and there is full return of function. > 60 per cent Death
Deep partial-thickness burns extend into the deep
dermis. There is damage to hair follicles as well as
The chest x-ray may be normal initially; bron-
sweat and sebaceous glands, but their deeper portions
choscopy and radionuclide scanning are useful in
usually survive. Hot liquids, steam, grease, or flame
determining the full extent of injury. Arterial blood
usually cause deep partial-thickness burns. The skin
gas analysis will track hypoxia, ventilatory failure and
may be blistered and the exposed dermis is pale white
the development of metabolic acidosis. Signs of shock
to yellow. The burned area does not blanch, has no cap-
are looked for, as detailed previously, and the GCS
illary refill and no pain sensation. Deep partial-thickness
and pupillary response assessed. The patient is fully
burns may be difficult to distinguish from full-thickness
exposed to allow evaluation of the whole-body surface
burns. Healing takes 3 weeks to 2 months. Scarring is
area.
common and is related to the depth of the injury. Sur-
The burnt areas are assessed for depth of burn, as
gical debridement and skin grafting may be necessary
described earlier. This is a subjective clinical assess-
to obtain maximum function.
ment. The extent of the burn is assessed and expressed
Third-degree or full-thickness burns involve the
as a percentage of body surface area (BSA). This can
entire thickness of the skin, and all epidermal and der-
be done using the ‘rule of nines’, or with aids such as
mal structures are destroyed. They are usually caused
the Lund and Browder charts. The rule of nines is an
by flame, hot oil, steam, or contact with hot objects.
approximate tool, and tends to overestimate the
The skin is charred, pale, painless, and leathery. These
extent of a burn.
injuries will not heal spontaneously, as all dermal ele-
For irregular burns, the palmar surface of the
ments are destroyed. Surgical repair and skin grafting
patient’s hand, including the fingers, represents
are necessary, and there will be significant scarring.
approximately 1 per cent of the patient’s body surface
Fourth-degree burns are those that extend through
area.
the skin to the subcutaneous fat, muscle, and even
Body surface areas are different in infants; they have
bone. These are devastating, life-threatening injuries.
a disproportionately larger head surface area and
Amputation or extensive reconstruction is sometimes
smaller lower limb surface area.
required.
THERMAL BURNS – MANAGEMENT
THERMAL BURNS – RECOGNITION The airway is secured as described previously. Inhala-
The initial assessment of burns takes place during the tional burns can cause pharyngeal oedema and
primary survey, and is designed to recognize imme- swelling, which can make tracheal intubation difficult
diately life-threatening injuries compromising the if not impossible, leaving a surgical airway as the only 667
22 Head and neck (9%)
Head and
neck (21%)
9%
Each arm
(10%)
Each arm (4.5%)
Back
9% (13%) Abdomen
(13%)
Hand (1%) Each leg

(13.5%)
Buttocks
(5%)
Genital area
Each leg (9%) (1%)
22.33 Burns in infants Surface areas differ markedly

from those in adults.
experienced anaesthetist, with a range of difficult intu-

bation equipment available. Needle cricothyroido-
tomy and surgical airway sets should be immediately
accessible.
Breathing should be supported with high-flow oxy-
gen administered via a non-rebreathing, reservoir
9% mask that delivers 85 per cent at a flow rate of 15
L/min. The ventilation may need support using a
BVM assembly with a reservoir and high-flow oxygen.
9% Stridor can be eased, as a holding measure pending
Each arm (4.5%) airway securement, by administering high-flow
helium and oxygen, as this gas mixture has a low den-
sity that increases flow through the obstructing air-
way. However, heliox is only 21 per cent oxygen and
will not address hypoxia and carbon monoxide poi-
soning. Once the airway has been secured by tracheal
intubation, the inspired oxygen concentration and
ventilation should be adjusted to give optimum SaO2
levels (> 95 per cent) and low normal EtCO2 (4.5
Each leg (9%) kPa).
The presence of an inhalational burn and pulmonary
oedema may hinder oxygenation and ventilation, and
a critical care physician should be involved early. Sig-
nificant carbon monoxide levels may indicate the need
for ventilation with 100 per cent oxygen and hyperbaric
therapy, and an early referral should be made to a hy-
22.32 Burns. Rule of nines for assessment of extent of
perbaric unit; these are often found located in diving
burns in adults.
and naval centres. Circumferential neck and chest burns
may need to be incised to allow effective breathing and
recourse. The airway may need fibre-optic assessment, ventilation.
and warning signs such as stridor and respiratory dis- The circulation should be supported in any burn
tress indicate the need for early intubation. This patient with signs of shock or a burn less than 20 per
668 should be performed under general anaesthesia by an cent BSA. Two large-bore intravenous cannulae are
sited, preferably, although not necessarily, through palpable. If there is compromise to the circulation, 22
unburned skin. If intravenous cannulation or central surgical escharotomy will be needed. The eschar
venous cannulation are not possible, intraosseus or should be incised on the midlateral side of the limb,
intravenous cut-down techniques should be used, as allowing the fat to bulge through. This may be
shock will develop rapidly in patients with large and extended to the hand and fingers. Escharotomy may
deep burns. cause substantial soft tissue bleeding.

Warmed Hartmann’s or Ringer’s lactate is the fluid Analgesia will be required for partial-thickness
of choice; large volumes of normal saline 0.9 per cent burns, which are most painful. Cooling and dressing
can cause a hyperchloraemic acidosis. Colloids and will help, but opioids may be required. These should
hypertonic saline have no proven beneficial role. If be administered intravenously, and can be given by
shock is present, 2 L should be administered as in the infusion or patient-controlled analgesia (PCA) sys-
ATLS® guidelines for shock management. If haemor- tems.
rhagic shock is excluded, the volume and rate of fluid Consultation is important. A burns specialist should
administration is calculated according to the Parkland be involved from the outset for all patients with severe
formula as given later. This regimen applies to partial- or unusual burns. Transfer will be required for these
and full-thickness burns only; superficial burns do not patients as outcomes are improved in specialist cen-
require intravenous fluids. The administration time is tres. Indications for transfer are:
calculated from the time of the burn, not from the
• partial-thickness burns > 20 per cent BSA
time of admission or time of assessment. Deeper
• partial-thickness burns > 10 per cent BSA in ages
burns are likely to cause more tissue damage and con-
10–50 years
sequent fluid shifts. The Parkland formula is a guide
• full-thickness burns > 5 per cent any age
only, and fluid administration should be titrated
• partial- and full-thickness burns involving: face,
against response. Blood pressure, central venous pres-
eyes, ears, hands, feet, genitals, perineum, skin over
sure, pulse, peripheral perfusion and urine outputs are
major joints
used, but more sophisticated techniques such as
• significant electrical burns (and lightning)
oesophageal Doppler and arterial waveform analysis
• significant chemical burns
may aid optimization. Fluid overload should be
• inhalational burns
avoided in patients with inhalational burns and sys-
• burns in patients with complicating illness, trauma,
temic inflammatory reactions. Documented anaemia
and long-term rehabilitation needs
may indicate the need for blood transfusion.
• children.
Wound care starts in the pre-hospital environment
with the removal of burnt clothing and the cooling
CHEMICAL BURNS
and dressing of wounds. Rings, jewellery, watches and
Awareness Most chemical burns result from exposure
belts are removed as they retain heat and can cause
of the skin to strong alkalis and acids, and phosphorus,
compression as tissues swell. Wounds can initially be
phenol and petroleum products can also damage tissue.
dressed with loose, clean, dry dressings. Alternatives
However, 25 000 products are capable of causing
are plastic sandwich wrap (known as cling film in the
chemical burns, and they account for 5–10 per cent of
UK, plastic wrap in USA and cling wrap in Australia),
US burns centre admissions. Full development of
specialized gel burns dressings or saline-moistened
chemical burns is slower than thermal injury, so the
dressings. Cooling eases pain, but hypothermia
true extent of the burn can be underestimated on
should be avoided.
initial evaluation. Alkali burns tend to be more serious
Patients with circumferential deep burns of the
and deeper, as the alkalis soften and penetrate tissue,
limbs may develop eschars (thick, black, dry and
whereas acids tend to form a protective eschar.
necrotic tissue that constricts) with compromise of
the distal circulation. Distal pulses need to be moni- Recognition Definitive diagnosis depends on the
tored closely, with a Doppler probe if not easily history, and both the chemical involved and its
Table 22.4 Intravenous fluid requirements in partial- and full-thickness burn patients (Parkland formula)
Adults Children
Hartmann’s or Ringer’s lactate: Hartmann’s or Ringer’s lactate:
4 mL ¥ weight (kg) ¥ per cent BSA over initial 24 hours 3 mL ¥ weight (kg) ¥ per cent BSA over initial 24 hours plus
maintenance
Half over first 8 hours from the time of burn Half over first 8 hours from the time of burn
(other half over subsequent 16 hours) (other half over subsequent 16 hours)
(Example: an adult weighing 70 kg with 40 per cent second- and third-degree burns would require 4 mL ¥ 70 kg ¥ 40 = 11 200 mL over 24 hours). 669
22 domestic, low-voltage shocks are not associated with
skin burns even though they may cause death from
ventricular fibrillation. Alternating current (AC) shocks
produce tetanic muscle spasm, which can cause the
victim’s hand to clutch onto the electrical source, and
the respiratory muscles can be paralyzed, resulting in
respiratory arrest. Electrical muscle damage can result

in rhabdomyolysis and renal failure.
Recognition The assessment of an electrical shock
victim should follow the ABC principles of ATLS®.
The airway may be obstructed if the victim is
unconscious, and prolonged apnoea may follow
paralysis of the respiratory muscles. The heart may be
Right ear Left ear arrested in ventricular fibrillation or asystole depending
on the nature of the shock. Of high voltage electrical
22.34 Chemical burns Sulphuric acid burn to left ear
from car battery acid in roll-over traffic accident. shock victims, 50 per cent will have a neurological
injury with coma, and spinal injuries can result from
violent muscle spasms. The entry and exit points
should be examined for burns that may be full
concentration should be determined if possible. Alkali thickness, and the true extent of underlying muscle
burns are frequently full-thickness injuries, appear pale, damage may not be apparent. There may be
and feel leathery and slippery. Acid burns are often musculoskeletal injuries from associated trauma or
partial-thickness injuries and are accompanied by muscle spasm, and all long bones should be examined
erythema and erosion. Skin is stained black by and x-rayed when indicated.
hydrochloric acid, yellow by nitric acid, and brown by
Management The immediate priority is to avoid
sulphuric acid.
personal injury if the casualty is in contact with or even
Management The goal of treatment is to minimize any adjacent to a high-voltage electrical source. Initial
area of irreversible damage, and maximize salvage in management is to secure the airway, protect the
the zone of reversible damage. If dry powder is cervical spine and oxygenate and ventilate the casualty.
present, it should be brushed off before irrigation with Intravenous access is secured, and fluids administered
water, which is the mainstay of treatment. Irrigation if the casualty is shocked. If in cardiac arrest, advanced
should be commenced immediately when the injury is life support should be instituted, following the
recognized, with copious amounts of tap water. appropriate Advanced Life Support algorithms for
Neutralizing agents (e.g. an acid to treat an alkali burn) VF/VT and non-shockable arrests as indicated.
should not be used, as there is a risk that heat The heart should be monitored for arrhythmias,
generated by the neutralizing reaction will cause which can occur in 30 per cent of high-voltage shock
further thermal injury. victims. Tissue damage may need surgical debride-
After copious water irrigation, some specific treatment, and compartment syndrome may develop,
ments are possible, e.g. calcium gluconate for hydro- requiring fasciotomies. A urinary catheter is sited, and
fluoric acid burns and polyethylene glycol for phenol. the urine observed for the brown discoloration indica-
An urgent referral to a burns surgeon should be made; tive of development of myoglobinuria; this is treated
eschar formation may make irrigation ineffective and by giving intravenous fluids to promote a diuresis, and
require emergency surgical excision. administration of mannitol. Myoglobinuria should be
considered present if a urine dipstick test registers
ELECTRICAL BURNS positive for haemoglobin, but the freshly spun urine
Awareness Electrical burns are caused when an sediment shows no red blood cells.
individual makes contact between an electrical source As ongoing treatment will be complex in severe elec-
and the earth, and severe, non-lethal electrical injuries trical injuries and burns, early consultation should be
constitute 3–5 per cent of admissions to US burns made with a burns surgeon and critical care specialist.
units. Current flows through the skin and variably Management on a critical care unit will be required.
through different tissues from the point of electrical
contact to the ground contact, causing burns and COLD INJURY BURNS
necrosis. The physiological effects of an electric shock Awareness Cold injury can be systemic, leading to
are related to the amount, duration, type (AC or DC), hypothermia, or localized, leading to localized tissue
and path of current flow. Severe electrical skin burns damage to varying degrees dependent on the degree
670 are associated with high-voltage shocks, whereas most of freezing.
Hypothermia is defined as a core body temperature 40–42°C moving water (re-warming rate: ~1– 22
of below 35oC (95oF). The systemic effects depend on 2°C/hour) The warming gradient should not be
the severity of the drop in core temperature: greater than this to avoid thermal injury. Re-warm-
ing should be slow to minimize peripheral dilation,
Mild hypothermia 35–32oC (95–89.6oF)
which can cause hypovolaemic shock.
Moderate hypothermia 32–30oC (89.6–86oF)
Severe hypothermia < 30oC (< 86oF) Severe hypothermia and hypothermic cardiac arrest

require active internal (core) rewarming:
As core temperature drops, the conscious level
deteriorates, and the airway can obstruct as coma • extracorporeal blood rewarming (cardiopulmonary,
develops. Respiratory and cardiac functions deterio- venovenous, or arteriovenous femorofemoral
rate until respiratory and cardiac arrest result. bypass) is the treatment of choice, especially with
Localized cold injury is seen in three forms: cardiac arrest
• without equipment for extracorporeal re-warming,
1. Frostnip – the mildest form, which is reversible on
left-sided thoracotomy followed by pericardial cav-
warming.
ity irrigation with warmed saline and cardiac mas-
2. Frostbite – due to freezing of tissue and resultant sage is effective in systemic hypothermia < 28°C
damage from intracellular ice crystals and • thoracic lavage or haemodialysis is also effective
microvascular occlusion. There are four degrees of • repeated peritoneal dialysis with 2 L of warm
frostbite: (43°C) potassium-free dialysate solution exchanged
• First degree – hyperaemia and oedema without skin every 10–12 minutes until core temperature is
necrosis. raised to ~35°C
• Second degree – vesicle formation with partial-thick- • parenteral fluids warmed to 43°C
ness skin necrosis. • administer humidified air heated to 42°C through a
• Third degree – full-thickness and subcutaneous tis- face mask or tracheal tube
sue necrosis, with haemorrhagic vesicle formation. • (NOTE: warm colonic and gastrointestinal [GI]
• Fourth degree – full-thickness necrosis, including irrigations are of less value.)
muscle and bone gangrene.
Localized cold injury is initially managed in the
3. Non-freezing injury – trench foot or immersion field. The hypothermia and dehydration associated
foot, with microvascular endothelial damage, sta- with frostbite should be addressed. Wet and constric-
sis and vascular occlusion. tive clothing should be removed, the involved extrem-
ities should be elevated and wrapped carefully in dry
Recognition Systemic cold injury is recognized in the
sterile gauze, and affected fingers and toes separated.
primary survey as the airway, breathing and circulation
Further cold injury should be avoided. Rapid re-
and neurological function are assessed. The patient is
warming is the single most effective therapy for frost-
cold to the touch, and looks gray and peripherally
bite. As soon as possible, the injured extremity should
cyanosed. Strikingly, the expired breath can feel deathly
be placed in gently circulating water at a temperature
cold on the hand. A low reading rectal or oesophageal
of 40–42°C (104–107.6°F) for approximately 10–30
temperature probe will be needed to accurately gauge
minutes, until the distal extremity is pliable and ery-
the degree of hypothermia.
thematous. The current consensus is that clear blisters
Local injuries are assessed during the secondary sur-
are aspirated or debrided and dressed. Early surgical
vey and the musculoskeletal survey. The affected part
intervention in the form of tissue debridement and
of the body initially appears hard, cold, white and
amputation is not indicated; full demarcation of dead
anaesthetic, but the appearance changes frequently
tissue can take 3–4 weeks to fully demarcate, and
during treatment.
debridement at this point will avoid unnecessary
Management Hypothermia is treated by securing the tissue loss (Rabold, 2004).
airway, oxygenating and ventilating the patient to
Take home message Thermal burns are assessed by
normal parameters, gaining intravenous access and
depth and extent, and managed by addressing the
treating shock with warmed intravenous fluids. In
airway, breathing and circulation. Huge volumes of
addition, the patient is re-warmed depending on the
intravenous fluids may be required to maintain
degree of hypothermia.
homeostasis. Chemical burns are treated primarily by
Mild and moderate hypothermia is treated by active
copious irrigation with water. Electrical burns may be
external re-warming:
associated with severe tissue damage and systemic
• heated blankets, warm baths, forced hot air. It is disturbance, and need treatment for the local burns
easier to monitor and perform diagnostic and ther- and systemic cardiac, respiratory and renal
apeutic procedures using heated blankets complications. Cold injury can be systemic
• warm bath re-warming is best done in a bath of hypothermia, which is treated by active external and 671
22 internal re-warming, depending on severity, or
ml/min/M2
mean increase in VO2 - 44%
localized tissue damage. Localized tissue damage is
200 n ⫽ 200
treated by rapid re-warming and delayed surgical
180
debridement. 160
140
120
100
INITIAL RESPONSE TO TRAUMA 80
60
40
The physiological effects of trauma are both wide- 20
spread and predictable, invoking a range of hormonal 0
and cellular mechanisms that have evolved to maxi- colorectal abdominal abdominal mean
aortic
mize the chances of survival following serious injury. aneurysm
These adaptations for survival can be considered as a
pre-op post-op
whole body, fluid conservation and repair strategy.
Following injury the first survival offensive is a plan 22.35 Oxygen consumption before and after surgery
to prevent blood loss. Direct injury to blood vessels (Older and Smith, 1988).
should induce an arterial vasospasm to reduce blood
loss followed by the formation of a ‘vascular patch’ con-
and generating a lactic acidosis as a consequence. This
sisting of a fibrin-reinforced, aggregation of platelets.
is clearly unsustainable and clinical studies show that an
If despite this strategy significant blood loss still
inability to mount a sustained cardiovascular response
occurs, some preservation of intravascular volume
is directly proportional to an increase in morbidity and
occurs by fluid redistribution between the vascular,
mortality. Survival and outcome relies on the speed of
cellular and interstitial fluid compartments. The
repayment of this oxygen debt. The slower the payback,
resulting change in compartmental volumes will stim-
the greater the ensuing complications.
ulate an endocrine response with the release of a num-
As a synopsis trauma and major surgery can be con-
ber of renal, adrenal and pituitary hormones (renin,
sidered to be like running a marathon. To survive, car-
aldosterone, cortisol and antidiuretic hormone
diorespiratory function and cellular physiology have
[ADH]). This hormonal response not only represents
to remain intact. Systemic failure, for whatever reason,
a secondary fluid conservation project but also heralds
to maintain tissue perfusion leads to shock, which is
another survival strategy.
one of the most frequently misused and misunder-
Serious injury, which in evolutionary terms would
stood terms in medicine and the media. Correctly
have limited the ability to hunt and feed, produces a
used it implies tissue hypoperfusion leading to cellular
metabolic re-conditioning. Under endocrine guid-
hypoxia and describes a medical emergency with a
ance, cellular metabolic priorities, and the type of sub-
high mortality rate from multiple organ failure.
strate used, change with a falling basal metabolic rate.
From an intensive care perspective, the recognition
These marked changes in metabolism represent an
and appreciation of the type of shock is essential as
approach to energy conservation, allowing a chan-
other reasons for hypoperfusion may coexist.
nelling of reserves to damage control and repair whilst
still keeping the brain fuelled.
Ultimately a successful outcome following trauma
(or major surgery) depends on the integration of
these strategies and the maintenance of whole-body
physiology. The integrity of the cardiorespiratory sys-
tem is pivotal. Failure to maintain cellular (organ) per-
fusion, oxygenation and ATP regeneration will lead to
cell apoptosis and death. Co-morbidities such as pre-
existing lung disease or cardiac failure will increase
complications and the chance of dying.
The normal physiological response to the increased
metabolic demands of trauma, illness and surgery is to
increase oxygen delivery in response to an increase in
tissue oxygen consumption.
Failure to respond to this demand will generate an
oxygen debt with metabolic consequences. This 22.36 Hypoperfusion This 70-year-old man with severe
limitation of oxygen availability will favour anaerobic sepsis developed hypoperfusion of the lower limbs. Note
672 metabolism over aerobic, reducing metabolic efficiency the typical marbling of the skin.
mechanisms are unable to maintain adequate tissue 22
SHOCK flow, leading to critical hypoperfusion.
In health, cardiac output and the delivery of oxygen Obstructive shock ‘Obstruction’ arises when venous
(global arterial blood flow multiplied by the blood return is compromised by raised intrathoracic or
oxygen content) and local tissue perfusion are closely pericardial pressure (pneumothorax and cardiac
tamponade), or if right ventricular ejection is blocked

matched to metabolic requirements. Shock follows a
mismatch of metabolic demand to oxygen delivery at by a massive pulmonary embolus, resulting in right
tissue level, leading to cellular hypoxia and (if uncor- ventricular overload and impaired left heart filling.
rected) to tissue and organ failure. The causes of Plain x-rays may not show changes and CT
circulatory shock can be classified as abnormalities of angiography is the initial investigation of choice.
cardiac output, of systemic vascular resistance, or a
combination of both.
Reduced systemic vascular resistance
Neurogenic shock This occurs when spinal cord injury
Reduced cardiac output
– usually at a cervical or high thoracic level – leads to
Impaired performance Cardiogenic shock is an intrinsic loss of sympathetic tone and hence peripheral
failure of cardiac function despite adequate circulating vasodilatation, venous pooling and reduced venous
volume and venous return, most commonly as a result return. This is aggravated by the absence of direct
of acute myocardial infarction. Cardiogenic shock may sympathetic nervous system connection into the heart,
occur following an apparently minor insult to a heart and hence impaired compensatory responses.
with any pre-existing functional impairment.
Anaphylactic shock A drug or parenteral fluid may be
Impaired venous return Hypovolaemic shock exists when the trigger that provokes an immunological response
a fall in circulating volume of sufficient magnitude with histamine release, resulting in cardiovascular
occurs such that compensatory physiological instability and (potentially) respiratory distress.
Septic shock This condition is defined as severe sepsis
with associated hypotension, evidence of tissue hypo-
AETIOLOGY OF CIRCULATORY SHOCK perfusion that is unresponsive to fluid resuscitation.
1. Reduction in cardiac output
Various mechanisms are responsible for the vasodilata-
tory response and catecholamine resistance, which are
a. HYPOVOLAEMIC SHOCK: characteristic of septic shock. It is becoming clearer that
Reduced circulating volume causing a reduc- this host response does not appear to be determined by
tion in venous return and cardiac output (e.g. the infecting organism and there is a suggestion of ge-
haemorrhage) netic susceptibility being a contributory factor in dic-
b. OBSTRUCTIVE SHOCK:
tating the severity of subsequent illness.
Mechanical obstruction to normal venous
return or cardiac output, e.g. tension pneu- Diagnosis of shock
mothorax, cardiac tamponade or massive pul-
monary embolism Early recognition, immediate resuscitation and treat-
ment of the underlying cause are the cornerstones of
c. CARDIOGENIC SHOCK: successful therapy.
Failure of cardiac pump to maintain cardiac There may be an easily identifiable cause of shock,
output, e.g. post myocardial infarction. but often the aetiology is difficult to establish. Follow-
2. Reduction in peripheral resistance ing massive trauma, shock may be hypovolaemic (blood
loss), obstructive (tamponade or tension pneumotho-
a. DISTRIBUTIVE SHOCK: rax), cardiogenic (cardiac contusion), neurogenic
A drop in peripheral resistance due to vasodi- (spinal cord injury) or anaphylactic (drug reaction).
latation, which is often associated with an Careful examination should clarify the aetiology in
increase in cardiac output but not sufficient most cases, and will aid in determining severity by
to maintain blood pressure, e.g. anaphylaxis, identifying end-organ effects. Examination should be
neurogenic shock, SIRS, septic shock thorough and structured to avoid missing useful signs.
b. ENDOCRINE SHOCK: Tests should include a full blood count and estima-
In the intensive care setting hypothyroidism, tion of electrolytes as well as assessment of renal func-
hyperthyroidism and adrenal insufficiency can tion, liver function, clotting and blood group/
all lead to reduced tissue perfusion. cross-match, serum glucose, blood cultures and
inflammatory markers (e.g. C-reactive protein, procal- 673
22 CLINICAL EXAMINATION IN SHOCK
heart to rest within a branch of the pulmonary artery.
Inflation of the distal balloon permits measurement of
Cardiovascular system
the pulmonary artery occlusion pressure (PAOP),
• Pulse (rate/rhythm), blood pressure, JVP (or CVP
which allows an estimate of left atrial pressure and
if central line in situ), heart sounds (muffling/
hence (it is assumed) left ventricular preload. Many
murmurs), peripheral perfusion (capillary refill
errors may, however, confound this measurement.
time/skin colour)
The PAFC also allows measurement of cardiac output
by way of thermodilution (either by cold injectate or
Respiratory system by proximal heating coil, allowing semi-continuous
• Respiratory rate, work of breathing, tracheal data to be recorded). This is calculated from the area
deviation, air entry, added sounds, oxygen under a curve of distal temperature (recorded by a
saturations (relative to inspired oxygen) thermistor at the catheter tip) plotted against time.
Abdomen
Cardiac output is inversely proportional to this area.
• Pain, distension, peritonitis, localizing signs,
PAFC use has declined in popularity recently due to
urine output
concern regarding the complications of what is a
highly invasive modality, failure to show outcome
Central nervous system benefit in studies of patients monitored by PAFC, and
• Level of consciousness, peripheral neurological the increasing availability of alternative, less invasive
signs (e.g. power, reflexes) monitors that generate similar data.
Other systems
• Temperature, skin signs (e.g. rashes), limbs (bony
CARDIAC OUTPUT FROM ANALYSIS OF ARTERIAL
integrity/perfusion)
WAVEFORM
Pulse contour analysis The PiCCO® cardiac output
monitor employs a mathematical analysis of the shape
of the arterial waveform using a dedicated femoral
citonin). Arterial blood gas analysis provides rapid arterial cannula to derive cardiac output data. It is
results, and the newer analyzers often measure a calibrated by a transpulmonary thermodilution
serum lactate level. This is a non-specific marker, but technique, following injection of cold saline into a
may indicate hypoperfusion if elevated. central line.
X-ray examination, ultrasound scanning (e.g. a
The Lithium Dilution Cardiac
Pulse power analysis
FAST scan) or CT may identify sources of blood loss
Output (LiDCO®) monitor also employs the arterial
and identify likely foci in the case of severe sepsis. An
ECG and urgent echocardiography are obligatory if a
cardiogenic cause of shock is suspected.
Careful and regularly repeated recording of vital Monitoring History
signs (heart rate, respiratory rate, blood pressure, oxy- (non Invasive) Observation
gen saturation) and indicators of end-organ perfusion Clinical Examination
ECG/BP
(consciousness level, urine output) are crucial. The
Pulse Oximetry
initial severity of illness at assessment, and subsequent
response to initial resuscitative and treatment meas- Early Recognition History
ures will dictate the need for more advanced and inva- Clinical Examination
sive monitoring tools. Continuous invasive blood Education
pressure and central venous pressure monitoring are Early Warning Scoring
generally required, and are essential if vasoactive
drugs are required, both to enable safe drug delivery Early Resuscitation Improvement
and to allow titration of dosing. and Treatment
Advanced monitoring systems Deterioration
Invasive techniques that allow an estimation of cardiac

output – and thereby tissue oxygen delivery – are used Monitoring Arterial monitoring
in the sickest patients, both as an aid to diagnosis and (Invasive) Bloods eg lactate
CVP
a guide to therapy.
CO
Oxygen flux
PULMONARY ARTERY FLOTATION CATHETERIZATION
Continuing Resuscitation and Treatment
In pulmonary artery flotation catheterization (PAFC),
674 a catheter is passed via a central vein through the right 22.37 Investigation and monitoring shock
waveform to derive haemodynamic data but using a 22
power algorithm that can be used in any artery, and TREATMENT OF UNDERLYING CAUSE
thus does not require insertion of a proprietary arterial OF SHOCK
line. The monitor is calibrated using either the lithium
dilution technique (LiDCO plus) or using a Hypovolaemic
nomogram of patient demographics with the LiDCO • Control of haemorrhage (may require surgery)

Rapid monitor. As with pulse contour analysis,
peripheral resistance and data indicating likely fluid • Restoration of circulating volume (fluid and
responsiveness are calculated beat-to-beat. It does also blood products)
have, unlike many other devices, positive outcome data Obstructive
in high-risk patients.
• Needle decompression of tension pneumothorax
Management of shock • Pericardiocentesis (tamponade)
Initial approach Initially attention should be focussed • Thrombolysis or surgical removal of pulmonary
on rapid assessment, with airway, breathing and embolus
circulation (ABC) addressed in the first instance. High- Cardiogenic
flow oxygen (FIO2 0.6 or greater) should be
administered via a patent airway, and intravenous • Inotropes
access obtained. Definitive treatment of the underlying • Anti-arrhythmics
cause of shock should be commenced alongside
resuscitative measures. The aim should be to support • Revascularization
the circulation to allow adequate tissue oxygen • Aortic balloon counterpulsation
delivery, whilst mitigating or reversing the effects of
the initial insult. This may be rapidly successful, for • Surgical repair of valve lesions
example in decompression of a tension pneumothorax; Distributive
in other cases it may prove impossible to correct the
underlying pathology (e.g. cardiogenic shock due to • Early treatment of infection (source control, e.g.
extensive myocardial infarction). drainage, early antibiotic administration)
Fluid therapy Often large volumes are needed, guided

by clinical response and monitored indicators of filling
cardiogenic shock. Combinations may be required,
(e.g. central venous pressure). The response of these
guided by haemodynamic data from monitoring equip-
variables to a fluid challenge, and trends, are
ment and clinical response. Significant doses of either
considerably more useful than ‘snapshot’ values.
inotropes or vasopressors should be mandatory. Cardiac
Indeed targeting a particular value of CVP or MAP is
output monitoring is much better than making deci-
physiologically unsound and may be to the patient’s
sions based on the arterial blood pressure.
detriment. It is always preferable to use fluid boluses
or ‘challenge techniques’ to interpret volaemic status. Endocrine support There is recent evidence that
In ventilated patients, changes in intrathoracic pres- treatment with ‘physiological’ doses of corticosteroid
sure generate cyclical changes in systolic pressure and in cases where adrenal response is inadequate may not
using the LiDCO or PiCCO monitors generates a improve outcomes as had previously been hoped.
stroke volume variation that is related to volaemic sta- There is considered to be some benefit from the use of
tus under certain conditions. These variations in steroids with septic shock with an improvement in
stroke volume may be more useful indicators of likely haemodynamic response but this is still the subject of
fluid responsiveness than other methods. considerable debate and there is a lack of cogent
The choice of fluid is dictated by the underlying outcome data. The use of vasopressin has traditionally
cause of the shock and local policies. There is an opti- been reserved for patients with catecholamine-resistant
mum amount of fluid to target resuscitation and it septic shock but new evidence suggests that there may
should be recognized that overenthusiastic transfu- be some benefit for those requiring lower doses of
sion, as with fluid restriction, is also associated with noradrenaline.
increased complications. Tight control of blood glucose levels has also been
shown to lead to improved outcomes in the sickest
Inotropes/vasopressors This treatment should be insti-
patients in intensive care.
tuted if the patient remains hypotensive despite ade-
quate fluid resuscitation. Again, choice is determined by Systemic support Shock leads to multiple organ
aetiology: vasopressor (e.g. norepinephrine) for dis- impairment or failure. Support of other organ systems
tributive shock and inotrope (e.g. dobutamine) for may well be required during treatment. 675
22 Outcome Mortality is determined both by aetiology criteria used and the case-mix of the population of
of circulatory shock and the response to treatment. ICU patients studied. The outcome data is remark-
Early recognition and prompt therapy are the most ably consistent between the studies, with mortality
important factors. linked to the number of organs failed.
The appearance of MODS broadly follows two clin-
ical courses, differing in onset relative to the initial
event, time course and sequence of organ failure. The

first pattern usually follows a direct pulmonary insult,
MULTIPLE ORGAN FAILURE such as trauma or aspiration. In this form the overall
course of the disease may be relatively short and
Multiple organ failure or dysfunction syndrome MODS occurs as a pre-terminal event, becoming evi-
(MODS) is the clinical appearance of a seemingly dent just prior to death. The second type is the more
poorly controlled severe systemic inflammatory reac- classical form, as found in severe sepsis, with pul-
tion, following a triggering event such as infection, monary manifestations of acute respiratory distress
inflammation or trauma. It represents the net result of syndrome (ARDS). MODS is present early in the
altered host defence and deregulation of the inflam- course of the illness but does not become progressive
matory response and the immune system. The condi- until after a 7–10-day delay, with manifestations of
tion has emerged with medical advances as a result of hepatic and subsequently renal failure becoming
increasing availability of intensive care facilities. Rec- apparent.
ognized as a syndrome in the early 1970s, progress in The initiating events for MODS are many and
the management of critically ill patients has unmasked diverse but by far the most common association is
this frequently lethal cocktail of sequential pulmonary, with severe sepsis and ARDS. The likelihood of occur-
hepatic and renal failure. rence and the progression of disease is related not
This pattern of progressive organ impairment and only to the severity of the initiating event but also to
failure complicates illnesses with diverse aetiologies the premorbid physiological reserve of the patient, i.e.
and, despite progress in understanding the underlying old age and pre-existing disease such as cardiac failure,
mechanisms involved, it carries a mortality rate that cirrhosis, drug abuse etc.
remains depressingly high. MODS has now become
the commonest cause of stays in surgical ITUs of
more than 5 days and (among these patients) the
most frequent cause of death. INITIATING EVENTS FOR MODS
It is essential to differentiate MODS from postop-
Severe sepsis Surgery
erative or traumatic, isolated organ dysfunction,
• Peritonitis • Vascular
which has a different pathogenesis and markedly dif-
• Abdominal
ferent survival outcomes.
Trauma Medical
• Chest injury • Pancreatitis
Epidemiology • Burns • Aspiration
Definitions of organ failure use two types of criteria
Shock Other
based on either measures of physiological derange-
• Cardiogenic • Massive transfusion
ment (e.g. hypotension, acidosis, serum creatinine
• Haemorrhagic
concentration) or on the treatment methods (e.g.
dialysis, ventilation, etc.).
The degrees of organ dysfunction, from covert
physiological impairment to overt failure, coupled
with the difficulties of monitoring the function of all
Pathogenesis
the organs involved has led to controversies about the MODS is now recognized as a systemic disorder
definition of organ failure and the clinical entities resulting in widespread microvascular injury. Most of
involved. This has hampered epidemiological surveys the initiating events can be characterized as infective,
and the assessment of treatment outcomes. Confusion traumatic or ischaemic and mechanistically it is unrav-
over the exact incidence of MODS stems from an elling as a disorder of the host defence system, with an
absence of universal diagnostic criteria; many of the unregulated and exaggerated immune response,
published studies have used differing clinical and tem- resulting in an excessive release of inflammatory medi-
poral definitions of organ failure. ators. It is these mediators that produce the wide-
Review of the published studies suggests that spread microvascular damage leading to organ failure.
MODS develops in 5–15 per cent of patients requir- As a syndrome, the classical form of MODS appears
676 ing ICU admission, depending on the diagnostic to progress through four clinical phases:
1. Shock (hypoperfusion). MEDIATORS OF THE SIRS\SEPSIS RESPONSE AND 22
2. Period of active resuscitation. MODS
3. Stable hypermetabolism (systemic inflammatory The metabolic and physiological alterations found in
response). the hyperdynamic\hypermetabolic phase and the sub-
4. Organ failure. sequent cellular damage are caused by complex inter-
actions of endogenous and exogenous mediators.
Shock Common to all the initiating events associated

These substances are mainly released from the host
with MODS are periods of relative or total ischaemia
endothelial and reticulo-endothelial cells, principally
relating to regional or global perfusion deficits, which
macrophages, in response to provocation by a variety
may go clinically unrecognized, i.e. cellular
of stimuli including ischaemia, sepsis and cytokines.
hypoperfusion as discussed earlier. The severity of these
Experimental administration of endogenously pro-
deficits, the passage of time to adequate resuscitation
duced mediators such as tumour necrosis factor
and the reserve functional capacity of the organs
(TNF), interleukins IL1, IL2 and IL6 and platelet-
concerned, appear to provide the key to the path of
activating factor and exogenously produced mediators
organ dysfunction and eventual failure.
such as bacterial endotoxin produce not only similar
Active resuscitation If resuscitation is rapid and effective physiological effects to those found in the SIRS\sepsis
the sequence of events precipitating MODS may be syndrome, but also organ dysfunction similar to that
aborted. However, in many cases, despite apparently found in patients with MODS.
adequate management the syndrome progresses, The wide variety of substances with vastly differing
suggesting a genetic component. molecular structures implicated in the pathogenesis of
the SIRS\sepsis syndrome, all producing the same
Systemic inflammatory response If resuscitation fails to
characteristic physiological response, suggests a ‘pre-
prevent further progression of the disease, the presence
programmed’ or stereotyped host reaction. The effec-
of widespread cellular damage manifests after several
tor systems involved in the translation of triggering
days with a picture of panendothelial dysfunction. This
injury to pathogenesis of MODS are additive and syn-
endothelial damage is manifest by increased
ergistic, and involve not only the endocrine and cen-
microvascular permeability with the formation of
tral nervous systems, but also the cellular and humoral
protein-rich oedema fluid. This period of
components of the inflammatory responses. Follow-
hypermetabolism has characteristic features that are a
ing injury a local inflammatory response occurs result-
consequence of the host response. This has been
ing from the products of the damaged endothelium
referred to as the systemic inflammatory response
and platelets. Leucocytes and macrophages are pre-
(SIRS) in the absence of proven sepsis and the sepsis
sumably attracted to the area as a result of these prod-
syndrome when associated with an identifiable
ucts and secondary activation of complement,
invading pathogen. Once this phase is entered the
coagulation and other components of the inflamma-
mortality rises to the 25–40 per cent range.
tory system occurs. If the injury is severe or persistent
Organ failure Failure adequately to control the inciting enough, this localized reaction may spill over into the
event and the inexorable progression of the disease is systemic circulation, producing the systemic inflam-
marked in this final stage by increasing organ matory response, or if identified with infection the
dysfunction, failure and death. The appearance of sepsis syndrome. MODS may subsequently develop.
clinically overt organ failure is a significant prognostic In health, cytokine production is strongly repressed
event signalling another leap in the mortality rate from since they are produced by immune cells following
the 25–40 per cent range to 40–60 per cent in the early activation by foreign particles, e.g. bacteria. Cytokine
stages and 90–100 per cent as the disease progresses induction and production is then closely regulated so
with increasing hepatic and renal dysfunction. as to benefit the host by localizing and destroying the
foreign organisms. However in certain situations, this
control system appears inadequate and cytokine pro-
duction becomes both inappropriate and excessive,
CLINICAL FEATURES OF SIR leading to destruction of normal cells with a general-
ized inflammatory response.
Fever A decade of studies has underlined the importance
of the immune system and these mediators in the
Tachycardia
sequence of events ultimately producing MODS.
Hyperdynamic circulation Interleukin-1 is the most extensively investigated
cytokine; produced by macrophages, this polypeptide
Tachypnoea
(as well as interleukin-6) can induce fever, hyperme-
Oliguria tabolism, muscle breakdown and hepatic acute phase
protein synthesis. The interleukins, however, appear 677
22 relatively late in the sequence of events as compared
to TNF.
TNF appears early in the systemic circulation dur-
ing critical infective illness, mediating directly or indi-
rectly many of the major features of sepsis. It is
probably one of the pivotal mediators with multiple
effects, producing endothelial membrane permeability

changes and cell death. Many of these effects appear
to be secondarily mediated by prostaglandins and
TNF-induced release of other cytokines; the full
extent of its actions are poorly understood.
SPECIFIC ORGAN INVOLVEMENT IN

MODS 22.38 ARDS – x-ray Chest radiograph of a patient with
ARDS following pulmonary contusion. Infiltrates and
patchy consolidation are typical features. Note the
Respiratory system pulmonary artery catheter in situ.
In the majority of critically ill patients who develop

MODS the lungs are the first organ to fail, the other leucocyte aggregation and oxygen free radical forma-
organs following in a sequential fashion. The lung tion. Platelet clumping and intravascular coagulation
appears to be a pivotal organ in the development of have also been implicated. Pathologically in ARDS
MODS, appearing either to generate inflammatory pulmonary capillary endothelial damage causes fluid
mediators that aggravate peripheral endothelial dys- leakage and surfactant abnormalities resulting in alve-
function or allow the persistence of mediators in the olar and interstitial oedema and fibrosis. This damage
circulation following its decreased capacity to clear to pulmonary architecture causes a reduction in func-
and metabolize inflammatory substances. tional residual capacity, increased ventilation\perfu-
As with other organs, a spectrum of dysfunction sion mismatching and a predilection for secondary
exists ranging from minor demonstrable pathology, infection. The net result is failure of gaseous exchange
designated acute lung injury (ALI), to massive alter- with hypoxia, hypercarbia and therefore an aggrava-
ations in pulmonary pathophysiology – the so-called tion of the peripheral tissue hypoxia.
adult respiratory distress syndrome (ARDS).
ARDS has been defined as a condition character-
ized by severe hypoxia despite high concentrations of
Cardiovascular system
supplemental oxygen, with a radiographic appearance Under normal physiological conditions, tissue oxygen
demonstrating diffuse infiltrates in the absence of utilization is closely matched by its delivery to the
infection or any other explanation for the respiratory tissues. Oxygen uptake by cells is normally dictated by
distress. Included in this definition are clinical values need. Cardiac output, minute ventilation and regional
reflecting the derangement of respiratory function. blood flow in the microcirculation are regulated to
prevent cellular ischaemia. If stressed in this situation,
cells cope with increasing metabolic demands by
FEATURES DEFINING ARDS increasing oxygen extraction. However, under the
Hypoxia (PaO2/FiO2< 300 mmHg) pathological conditions found in patients with SIRS
who are developing MODS, the tissues appear unable
Bilateral infiltrates on chest x-ray to extract oxygen efficiently from the blood, thus
Pulmonary capillary wedge pressure < 18 mmHg or resulting in cellular oxygenation having to rely on
no clinical evidence of increased left atrial pressure increased oxygen delivery rather than extraction – the
so-called pathological oxygen, supply or flow, depend-
ency.
ARDS is considered to be a more severe form of There may be a number of reasons for this.
ALI, in which the same criteria apply except that the Microvascular inflammatory injury with endothelial
hypoxia is more severe [PaO2/FiO2 < 200 mmHg and interstitial oedema hinders the diffusion of oxy-
regardless of positive end-expiratory pressure gen, and furthermore altered membrane characteris-
(PEEP)]. The pathogenesis of this lung injury has in tics of the erythrocytes render them less deformable
part been suggested to be endothelial damage initi- and therefore less accessible to transit within the
678 ated by complement activation with subsequent microcirculation.
Altered
wall to prevent seepage of these contents into the cir- 22
concious culation. This epithelial barrier is, however, also
level
involved in the systemic disease process, especially as
preferential redistribution of the blood from the
Tachycardia
splanchnic circulation to muscle predisposes the gut
Tachypnoea
Hypotension mucosa to ischaemia and membrane reperfusion

Hypoxia
Acidosis
injury. The epithelial barrier is then likely to fail,
Jaundice
allowing translocation of pathogenic bacteria, or
Enzymes endotoxins into the portal circulation. Under normal
Albumin Oliguria
PT Anuria circumstances overspill of gut luminal toxic products
Creatinine into the portal circulation would be cleared by hepatic
Failure to absorb reticulo-endothelial system. In the presence of MODS
Diarrhoea the hepatic clearance of these substances is greatly
GI bleeding Platelets
PT/APTT reduced and spillage of toxins will be washed into the
Protein C pulmonary microcapillary network. The appearance of
D-dimer
endotoxin and bacteria in the lung will activate pul-
22.39 Physiological effects of MODS monary alveolar macrophages with local damage
occurring from macrophage-derived mediator release,
adding to the destruction of pulmonary architecture
In the hypermetabolic SIRS phase, the response to already occurring in ARDS.
increased metabolic demands coupled with less effec-
tive utilization of oxygen must be met by an increased
Kidney
cardiac output. This increase, in conjunction with
mediator-induced systemic vasodilation, gives rise to The involvement of renal dysfunction and failure as
the hyperdynamic state characteristic of the SIRS– part of classical MODS heralds a large increase in
sepsis syndrome. Failure to meet this increased mortality. The explanation for this excess mortality is
oxygen demand heralds a diminished likelihood of unknown; perhaps the failing kidneys act as a further
survival. source of inflammatory mediators ‘fuelling’ the sys-
Poor cardiac performance may also contribute to temic disease process further. The loss of intravascular
the oxygen supply–utilization disequilibrium. It is volume control may exacerbate ARDS and heart fail-
well documented in sepsis that certain circulating fac- ure with the potential for volume overload. In addi-
tors adversely affect ventricular compliance and con- tion, institution of methods of renal support will have
tractility. Furthermore, if pre-existing coronary artery the potential for further activation of the reticulo-
disease co-exists with this hyperdynamic state, endothelial cells caused by bio-incompatibility prob-
myocardial ischaemia and failure may progressively lems of the extracorporeal circuit and haemofilter/
ensue. The effects of this may not only cause a dialyzer.
decrease in organ perfusion but may also aggravate
existing pulmonary dysfunction with raised left atrial
Haematological system
pressures and the generation of pulmonary oedema,
further aggravating oxygen delivery. Coagulopathy is common after major trauma. Initially
this may just reflect massive fluid replacement and
transfusion. Massive transfusion, the replacement of
Gastrointestinal tract greater than one circulating blood volume (approxi-
The gastrointestinal tract is particularly vulnerable to mately 10 u of blood) in less than 24 hours, may
the processes occurring in MODS. There is a growing result in diffuse microvascular bleeding from surgical
body of evidence to suggest that the persistence of the wounds, intravenous catheter sites and areas of minor
SIRS–sepsis syndrome may be driven by abnormal trauma. The source of the coagulopathy, ignoring the
colonization of the normally sterile upper gastroin- presumed continuing consumption, is the dilution of
testinal tract with pathogenic enteric bacteria. Some coagulation factors through the infusion of products
investigators believe that the development of MODS deficient in these factors (e.g. packed red blood cells,
in the absence of a recognized focus of infection is crystalloids and colloids). Laboratory tests demon-
caused by gut failure with translocation of bacteria strate thrombocytopaenia, hypofibrinogenemia and
and toxins from the gut eventually into the systemic prolongation of the prothrobin times.
circulation. This abnormal colonization of the gut, An insidious complication of severe injury and blood
coupled with potentially toxic gut luminal contents, loss is a widespread disorder of coagulation and
forms a deadly reservoir of pathogenic substances. haemostasis. This is due, at least in part, to the release
The body relies on the epithelial integrity of the gut of tissue thromboplastins into the circulation, en- 679
22 dothelial damage and platelet activation. The result is The treatment of ALI/ARDS remains mainly sup-
a complex mixture of intravascular coagulation, deple- portive and includes the management of precipitating
tion of clotting factors, fibrinolysis and thrombocy- causes. A large prospective study, supported by the
topaenia. Microvascular occlusion causes haemorrhagic National Heart Lung and Blood Institute in the USA
infarctions and tissue necrosis, while deficient has shown that the use of low tidal volume ventilatory
haemostasis leads to abnormal bleeding. This resulting strategies (6 mL/kg) and limited plateau pressure
coagulopathy is termed disseminated intravascular co- (< 30 cm H2O) was effective in reducing the mortal-
agulation (DIC). The pathophysiology results from ity rate from 40 per cent to 31 per cent. Other meas-
the generation of excessive amounts of thrombin. ures to improve oxygenation – e.g. prone positioning,
Thrombin generation in florid DIC is sufficiently high-frequency ventilation, nitrous oxide inhalation
intense that anticoagulant mechanisms such as anti- and extracorporeal life support – have limited success
thrombin and activated protein C systems become in improving overall outcome.
ineffective. Fibrin deposition in the microvasculature Renal and haematological management strategies
undergoes fibrinolysis and promotes the consumption are also largely supportive with renal replacement
of clotting factors (especially fibrinogen, platelet factors therapy and blood products frequently requiring
V and VIII). This in turn leads to a consumptive expert involvement.
coagulopathy characterized by thrombocytopaenia, Malnutrition is a common and major contributing
hypofibrinogenaemia and ongoing thrombolysis. factor to MODS. Nutritional starvation combined
The consequences of DIC are variable but include ex- with hypermetabolism leads to structural catabolism.
cessive bleeding due to consumption of haemostatic fac- Unlike starvation the substrates metabolized are
tors and secondary fibrinolysis, organ dysfunction, skin mixed, with a significant increase in amino-acid oxi-
infarction, haemolysis, and disseminated thrombosis. dation. With the temporal progression of MODS,
The clinical features are those of diffuse microvascular direct amino-acid oxidation increasingly becomes
thrombosis: restlessness, confusion, neurological dys- prevalent with rapid dissolution of skeletal muscle.
function, skin infarcts, oliguria and renal failure. Ab- Metabolic support in terms of providing adequate
normal haemostasis causes excessive bleeding at opera- calories and maintaining nitrogen balance is essential
tion, oozing drip sites and wounds, spontaneous if lean body mass is to be preserved and ‘auto-
bruising, gastrointestinal bleeding and haematuria. The cannabilism’ slowed. This has led to recommenda-
diagnosis is confirmed by finding a low haemoglobin tions for early parenteral feeding (this is still
concentration, prolonged prothrombin and thrombin controversial). Providing a calorie source for these
times, thrombocytopaenia, hypofibrinogenaemia and patients requires care and a balance of substrates has
raised levels of fibrinogen degradation products. to be given to prevent adding iatrogenic problems to
the metabolic mayhem already occurring. Whilst it is
known that glucose has a protein-sparing effect,
Management of MODS excessive amounts confers no additive advantages and
Once the clinical syndrome of MODS is established, may cause complications such as fatty liver, hyperos-
despite major advances in ITU technology and man- molarity, hyperglycaemia, and increased CO2 produc-
agement strategies, the chances of survival dwindle. tion, increasing the excretory load of the lungs and
The best treatment for MODS remains prevention. further exacerbating respiratory failure. The glucose
This entails early aggressive resuscitation following in- load should not therefore exceed 4–5
sult, avoidance of hypotensive episodes and removal of mg/kg/minute, with a non-protein calorific load of
risk factors, e.g. by early excision of necrotic tissue, early 25–30 kcal/kg/day and 0.5–1.0 g/kg/day of lipids.
fracture stabilization and ambulation, and appropriate Protein requirements run at 1–2 g/kg/day with
antibiotic usage following drainage of sources of sepsis. modified amino acid preparations as these appear to
Early circulatory resuscitation is of paramount be the most efficient protein source, producing less
importance and this should be guided by invasive urea and better nitrogen retention.
monitoring. Oxygen delivery should be maximized to Rigorous attention to these details has brought
a point where oxygen consumption no longer rises or improvements in prevention and outcome in MODS.
to the level where markers of anaerobic metabolism Other newer treatment strategies are still largely
such as serum lactate fall. It appears that the use of less unproven in terms of outcome. Selective decontami-
invasive clinical markers for the adequacy of the circu- nation of the digestive tract (SDD) by administration
lation, such as mean arterial pressure, temperature of non-absorbable antimicrobial agents may reduce
gradients and urine output, may not entirely reflect the incidence of nosocomial pneumonia by re-steriliz-
the success of microcirculatory resuscitation. Once ing the upper gastrointestinal tract. Trials of SDD
the sequence of MODS is established, early appro- have shown some benefit but large-scale effects on
priate institution of organ support, (e.g. endotracheal antibiotic resistance from widespread use of antio-
680 intubation and ventilation) is essential. biotics are awaited. The use of aggressive early enteral
feeding in patients without an ileus may not only μm in diameter occur in most adults after closed frac- 22
reduce the effects of catabolism but also prevent tures of long bones and histological traces of fat can
upper gut colonization by bacteria and hence nosoco- be found in the lungs and other internal organs. A
mial pneumonia by stimulation of bactericidal gastric small percentage of these patients develop clinical fea-
acid secretion. Recent studies appear to suggest that tures similar to those of ARDS; this was recognized as
this may have a positive effect on outcome. the fat embolism syndrome long before ARDS entered

Probably the most recent advances in treatment of the medical literature. Whether the fat embolism syn-
MODS have been in relation to modulation of the drome is an expression of the same condition or
hypermetabolic inflammatory response by use of spe- whether it is an entirely separate entity is still uncer-
cific agents. These include monoclonal antibodies tain.
against endotoxin and TNF inhibitors of nitric oxide The source of the fat emboli is probably the bone
synthase and receptor antagonists for interleukin-1. marrow, and the condition is more common in
Unfortunately interim reports of the therapeutic patients with multiple fractures.
effectiveness are conflicting and it would appear as yet
that the ‘magic bullet’ remains elusive.
Clinical features
Again it must be emphasized that prevention is
better than attempting cure for MODS, the major Early warning signs of fat embolism (usually within 72
killer of critically ill patients in intensive care. hours of injury) are a slight rise of temperature and
pulse rate. In more pronounced cases there is breath-
lessness and mild mental confusion or restlessness.
Pathognomonic signs are petechiae on the trunk, axil-
TETANUS lae and in the conjunctival folds and retinae. In more
severe cases there may be respiratory distress and
The tetanus organism Clostridium tetani flourishes coma, due both to brain emboli and hypoxia from
only in dead tissue. The exotoxin released passes to involvement of the lungs. The features at this stage
the central nervous system via the blood and the per- are essentially those of ARDS.
ineural lymphatics from the infected region. The toxin There is no infallible test for fat embolism; how-
is fixed in the anterior horn cells and therefore cannot ever, urinalysis may show fat globules in the urine and
be neutralized by antitoxin. the blood PO2 should always be monitored; values
Established tetanus is characterized by tonic, and below 8 kPa (60 mmHg or less) within the first 72
later clonic, contractions, especially of the muscles of hours of any major injury must be regarded as suspi-
the jaw and face (trismus, risus sardonicus), those near cious. A chest x-ray may show classical changes in the
the wound itself, and later of the neck and trunk. Ulti- lungs.
mately, the diaphragm and intercostal muscles may be
‘locked’ by spasm resulting in asphyxia.
Management
TREATMENT Management of severe fat embolism is supportive.
With established tetanus, intravenous antitoxin Symptoms of the syndrome can be reduced with the
(human for choice) is advisable. Heavy sedation and use of supplemental high inspired oxygen concentra-
muscle relaxant drugs may help; tracheal intubation tions immediately after injury and the incidence
and ventilation are the only options to treat respira- appears to be reduced by the prompt stabilization of
tory muscle involvement. long-bone fractures. Intramedullary nailing is not
Prophylaxis against tetanus by active immunization thought to increase the risk of developing the syn-
with tetanus toxoid vaccine is a valuable goal. If the drome. Fixation of fractures also allows the patient to
patient has been immunized, booster doses of toxoid be nursed in the sitting position, which optimizes the
are given after all but trivial skin wounds. In the non- ventilation–perfusion match in the lungs.
immunized patient prompt and thorough wound toi-
let together with antibiotics may be adequate, but if
the wound is contaminated, and particularly with a
delay before operation, antitoxin is advisable. CRUSH SYNDROME
This is seen when a limb is compressed for extended

periods, e.g. following entrapment in a vehicle or rub-
FAT EMBOLISM SYNDROME ble, but also after prolonged use of a pneumatic anti-
shock garment.
Fat embolism is a common phenomenon following The crushed limb is underperfused and myonecro-
limb fractures. Circulating fat globules larger than 10 sis follows, leading to the release of toxic metabolites 681
22 when the limb is freed and so generating a reperfusion Logical regression analysis, a multivariate statistical
injury. Reactive oxygen metabolites create further tis- procedure, is used to convert a score to a predicted
sue injury. Membrane damage and capillary fluid re- probability of the outcome measured, usually morbid-
absorption failure result in swelling that may lead to a ity or mortality, using a large patient database suitable
compartment syndrome, thus creating more tissue to the scoring system being developed. Finally the
damage from escalating ischaemia. Tissue necrosis scoring system has to be validated on a population of
also causes systemic problems such as renal failure patients independent from those used to develop the
from free myoglobin, which is precipitated in the scoring system.
renal glomeruli. Myonecrosis may cause a metabolic Patients form a heterogeneous population and dif-
acidosis with hyperkalaemia and hypocalcaemia. fer in many respects including age, previous health
status, reason for admission and severity of illness.
Clinical features and treatment When comparing patients on intensive care for the
purpose of research or audit, it is often difficult to
The compromised limb is pulseless and becomes red, standardize for all physiological variables due to the
swollen and blistered; sensation and muscle power diversity of patients and their conditions. Scoring sys-
may be lost. The most important measure is preven- tems are therefore used to standardize for the physio-
tion. From an intensive care perspective a high urine logical variables, age and reason for admission,
flow is encouraged with alkalization of the urine with allowing comparisons to be made between patients
sodium bicarbonate, which prevents myoglobin pre- with different severity of illness.
cipitating in the renal tubules. If oliguria or renal fail- In the majority of scoring systems a high score reflects
ure occurs then renal haemofiltration will be needed. a patient who is more sick than one with a lower score
If a compartment syndrome develops, and is con- (with the notable exception of the Glasgow Coma
firmed by pressure measurements, then a fasciotomy is Score), but the score does not always follow a linear
indicated. Excision of dead muscle must be radical to scale. Therefore a patient with a score of 20 is neither
avoid sepsis. Similarly, if there is an open wound then necessarily twice as sick nor has double the chance of dy-
this should be managed aggressively. If there is no ing than a patient with a score of 10. However, using
open wound and the compartment pressures are not logical regression it is possible to derive from the score
high, then the risk of infection is probably lower if a probability of morbidity, or mortality in hospital.
early surgery is avoided.
Audit
The most common use for scoring systems is for audit.
INTENSIVE CARE UNIT SCORING This allows ICUs to assess their performance in com-
SYSTEMS parison to other units and also their own performance
from year to year. If an ICU admitted patients who
The role of scoring systems in medicine has expanded were not very sick, then their actual mortality on that
since the 1950s. There are now many scoring systems unit would be lower than on a unit that admitted ex-
catering for most organ dysfunction, disease states, tremely sick patients and therefore it would be difficult
trauma and critical illness. New scoring systems are to compare the performance between those units. This
regularly being developed and older systems refined. has led to the comparisons of actual mortality to a pre-
This widespread use relates to their role in communi- dicted mortality. The ratio of the actual to predicted
cation, audit and research as well as the clinical man- mortality gives a figure for the standardized mortality
agement of patients. ratio (SMR). Therefore an ICU with an SMR of less
Scoring systems can theoretically be created from than 1 is theoretically performing better than expected
many types of variables. However, to be clinically use- and a unit with an SMR of more than 1 is performing
ful, scoring systems must have predictive properties, worse than expected. The SMR can then be used to
and the information has to be unambiguous, reliable compare performance between units. Also if the sever-
and easy to determine and collect. Ideally the variables ity of illness of patients varies, or if different types of
should be frequently recorded or measured. Variables patients are admitted from year to year, the SMR can
can be selected using clinical judgement and recog- be used to assess the performance of a unit over time.
nized physiological associations, or by using comput- Statistical significance of different SMRs can be evalu-
erized searching of data collected from patient ated using confidence intervals.
databases and relating it to outcome. The variables are
then assigned a weighting in relation to their impor-
tance to the predictive power of the scoring system,
Research
again either by clinical relevance or from computer- The diversity of patients and different pathologies on
682 ized databases. the ICU makes comparisons between treatments or
procedures difficult. Scoring systems can be used to (APACHE) model in 1981 and revised it to APACHE 22
adjust for the differences in case-mix in patients II in 1985. APACHE III was presented in 1991 but
recruited for trials, so if an intervention is used on all as the regression analysis modelling is not in the pub-
patients, the scoring systems can standardize for any lic domain its uptake has been slow.
heterogenicity between the groups prior to the inter- APACHE II is made up of four basic components:
vention being initiated. Stratification of the risk of (1) acute physiology score; (2) chronic health evalua-

death can also be inferred from the scoring systems, tion; (3) age; (4) urgency of admission to critical care.
allowing for investigation in different subgroups of The acute physiology score is composed of 12 vari-
patients in the ITU, and allowing researchers to assess ables, with the most deranged measurement during
response to interventions in patients at different risk the first 24 hours of admission to critical care being
of mortality. used to calculate the score. The original data collec-
tion for APACHE II occurred between 1979 and
1982 from ICUs in North America, and the popula-
Clinical management tion studied included relatively few surgical and
As well as quantifying the degree of physiological de- trauma patients. Also, there have been many advances
rangement or clinical intervention, and promoting bet- in patient care since the1980s, which have made
ter communication between clinicians, scoring systems APACHE II dated, despite its continued popularity.
can also be used to guide patient management. Some
scoring systems lend themselves to sequential reassess- Simplified acute physiology score
ment and thus can be used to monitor a patient’s
progress over time. Also, as most research conducted in The Simplified Acute Physiology Score (SAPS) initially
ICUs use scoring systems, the recommendations from used 14 variables, and did not provide any probability
research can sometimes be applied to subsets of patients of survival. In 1993 it was revised to SAPS II with the
with a severity of illness score within a certain range. data originating from European and North American
This allows therapies to be directed sensibly at patients ICUs. The score includes 12 physiological variables
with an appropriate severity of illness. As most ITU (the worst value within the first 24 hours), age, type of
scoring systems are an assessment of risk of mortality admission and three underlying disease variables (ac-
they have also been used to trigger admission to high- quired immune deficiency syndrome (AIDS), metasta-
dependency or intensive care. tic cancer, and haematological malignancy). Using lo-
gistic regression, SAPS II can also be used to estimate
the probability of survival. It is a simpler scoring system
Scoring systems on the ICU than APACHE and is also in the public domain, re-
sulting in its widespread use, particularly in Europe. It
Scoring systems are often classified into three subsets:
suffers similar disadvantages to APACHE with regards
(1) anatomical (e.g. the injury severity score); (2)
to the timing of data collection, but is based on more
physiological (e.g. the GCS) and therapeutic (e.g.
recent and international data.
therapeutic intervention scoring systems). Most
intensive care scoring systems are based on physiolog-
ical variables; however other data are also included in Mortality prediction model
the score, making simple classification very difficult.
An ideal scoring system would be simple to use and The original mortality prediction model (MPM) was
be applicable to all intensive care patients irrespective derived in the late 1980s with data from a single hos-
of age, diagnosis and urgency of admission. It should pital, and differed from many of the scoring systems
also not be dependent upon treatment given prior to by not depending on physiological data but on the
and on admission to ICU. The outcome prediction presence or absence of pathology. Therefore there was
modelling should have a high sensitivity and speci- less of an impact by treatment on the physiology prior
ficity. The intensive care scoring systems are devel- to and on admission to intensive care. In 1993 the
oped from large databases incorporating data from MPM was revised to MPM II based on the same data
many ICUs. The data include physiological variables, set as SAPS II but with the inclusion of six extra
co-morbidities, age, diagnoses, urgency of admission, ICUs. Initially the model was constructed of two time
and outcome at discharge from hospital. points: within 1 hour (MPM II0) and the first 24
hours (MPM II24) of admission. Now it can be used
for 48- and 72-hour points as well, giving a prediction
Acute physiology and chronic health of mortality at those time points. Its variables include
physiological parameters, age, acute diagnoses,
evaluation
chronic diseases, type of admission, as well as others.
Knaus et al (1981) introduced the first the Acute The MPM II0 is useful as it is minimally affected by
Physiology and Chronic Health Evaluation the treatment given in an ICU. 683
22 Therapeutic intervention scoring system Clasper J, Rew D. Trauma life support in conflict. Br Med
J 2003; 327: 1178–9.
The original therapeutic intervention scoring system Commission on the Provision of Surgical Services. The
(TISS) was devised in 1976, consisting of 76 thera- Management of Patients with Major Injuries. The Royal
peutic activities and was used initially to stratify the College of Surgeons of England, 1988.
severity of illness. Its use for this purpose has largely Deakin CD, Low JL. Do Advanced Trauma Life Support
been superseded by the newer scoring systems, but it guidelines accurately predict systolic blood pressure by
is still commonly used to assess nursing workload and palpation of carotid, femoral and radial pulses? An obser-
in resource management, for which it was not vational study. Br Med J 2000; 321: 674–5.
designed. A simplified TISS was developed in 1996, Earlam R. Trauma Care. Helicopter Emergency Medical
which included only 28 therapeutic activities. Service (HEMS), London, 1997.
Findlay G et al. Compilers. Trauma: Who cares? A report of
Limitations the National Confidential Enquiry into Patient Outcome
and Death (2007). NCEPOD 2007.
Overall there is very little to choose between the Flannery T, Buxton N. Modern management of head
third-generation scoring systems (APACHE III, SAPS injuries. J R Coll Surg Edinb 2001; 46: 150–3.
II, MPM II) in terms of their predictive power. Frankema SP, Ringburg AN, Steyerberg EW et al. Bene-
Despite this, APACHE II continues to dominate the ficial effect of helicopter emergency medical services on
literature and continues to be the most widely used survival of severely injured patients. Br J Surg 2004; 91:
score to date. 1520–6.
The APACHE II/III and SAPS I/II scoring sys- Hodgetts T, Mahoney P, Russell M, Byers M. ABC to
tems measure physiological variables during the first ABC: redefining the military trauma paradigm. Emer-
24 hours of ITU admission and there has been con- gency Med J 2006; 23: 745–6.
cern that this can lead to bias. If a patient is treated Hodgetts T, Porter C. Major Incident Management System.
prior to admission to ITU, their physiological vari- BMJ Books, London, 2002.
ables will have been improved and the patients will Joint Royal Colleges Ambulance Service Liaison Com-
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dealing with sicker patients. Lastly, if a patient dies Injured. The Royal College of Surgeons of England.
within 24 hours their scores before death will be very London, 2000.
high, and therefore skew the SMR of a unit to suggest Knaus WA, Zimmerman JE, Wagner DP. APACHE:
that it is admitting very sick patients. MPM II meas- Acute Physiology and Chronic Health Evaluation, a
ures variables during the first hour and within the first physiologically based classification system. Crit Care Med
24 hours, thereby reducing the bias that may occur in 1981; 16: 470–8.
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685
Principles of fractures
23
Selvadurai Nayagam
Fractures result from: (1) injury; (2) repetitive stress;

INTRODUCTION or (3) abnormal weakening of the bone (a ‘patholog-
ical’ fracture).
A fracture is a break in the structural continuity of
bone. It may be no more than a crack, a crumpling or
a splintering of the cortex; more often the break is FRACTURES DUE TO INJURY
complete and the bone fragments are displaced. If the
overlying skin remains intact it is a closed (or simple) Most fractures are caused by sudden and excessive
fracture; if the skin or one of the body cavities is force, which may be direct or indirect.
breached it is an open (or compound) fracture, liable to With a direct force the bone breaks at the point of
contamination and infection. impact; the soft tissues also are damaged. A direct
blow usually splits the bone transversely or may bend
it over a fulcrum so as to create a break with a ‘but-
terfly’ fragment. Damage to the overlying skin is com-
HOW FRACTURES HAPPEN mon; if crushing occurs, the fracture pattern will be
comminuted with extensive soft-tissue damage.
Bone is relatively brittle, yet it has sufficient strength With an indirect force the bone breaks at a distance
and resilience to withstand considerable stress. from where the force is applied; soft-tissue damage at
(a) (b) (c) (d)
23.1 Mechanism of injury Some fracture patterns suggest the causal mechanism: (a) spiral pattern (twisting); (b) short
oblique pattern (compression); (c) triangular ‘butterfly’ fragment (bending) and (d) transverse pattern (tension). Spiral and
some (long) oblique patterns are usually due to low-energy indirect injuries; bending and transverse patterns are caused by
high-energy direct trauma.
23 the fracture site is not inevitable. Although most PATHOLOGICAL FRACTURES
fractures are due to a combination of forces (twisting,
bending, compressing or tension), the x-ray pattern Fractures may occur even with normal stresses if the
reveals the dominant mechanism: bone has been weakened by a change in its structure
(e.g. in osteoporosis, osteogenesis imperfecta or
• Twisting causes a spiral fracture;
Paget’s disease) or through a lytic lesion (e.g. a bone
• Compression causes a short oblique fracture.

cyst or a metastasis).
• Bending results in fracture with a triangular ‘but-
terfly’ fragment;
• Tension tends to break the bone transversely; in
some situations it may simply avulse a small frag-
ment of bone at the points of ligament or tendon TYPES OF FRACTURE
insertion.
Fractures are variable in appearance but for practical
NOTE: The above description applies mainly to the reasons they are divided into a few well-defined
long bones. A cancellous bone, such as a vertebra or groups.
the calcaneum, when subjected to sufficient force, will
split or be crushed into an abnormal shape.
COMPLETE FRACTURES
The bone is split into two or more fragments. The
FATIGUE OR STRESS FRACTURES fracture pattern on x-ray can help predict behaviour
These fractures occur in normal bone which is subject after reduction: in a transverse fracture the fragments
to repeated heavy loading, typically in athletes, usually remain in place after reduction; if it is oblique
dancers or military personnel who have gruelling or spiral, they tend to shorten and re-displace even if
exercise programmes. These high loads create minute the bone is splinted. In an impacted fracture the frag-
deformations that initiate the normal process of ments are jammed tightly together and the fracture
remodelling – a combination of bone resorption and line is indistinct. A comminuted fracture is one in
new bone formation in accordance with Wolff’s law. which there are more than two fragments; because
When exposure to stress and deformation is repeated there is poor interlocking of the fracture surfaces,
and prolonged, resorption occurs faster than replace- these are often unstable.
ment and leaves the area liable to fracture. A similar
problem occurs in individuals who are on medication INCOMPLETE FRACTURES
that alters the normal balance of bone resorption and
replacement; stress fractures are increasingly seen in Here the bone is incompletely divided and the perios-
patients with chronic inflammatory diseases who are teum remains in continuity. In a greenstick fracture
on treatment with steroids or methotrexate. the bone is buckled or bent (like snapping a green
(a) (b) (c) (d) (e) (f)
23.2 Varieties of fracture Complete fractures: (a) transverse; (b) segmental and (c) spiral. Incomplete fractures:
688 (d) buckle or torus and (e,f) greenstick.
twig); this is seen in children, whose bones are more Marsh et al., 2007; Slongo and Audige 2007). Whilst 23
springy than those of adults. Children can also sustain it has yet to be fully validated for reliability and repro-
injuries where the bone is plastically deformed (mis- ducibility, it fulfils the objective of being comprehen-
shapen) without there being any crack visible on the sive. In this system, the first digit specifies the bone
x-ray. In contrast, compression fractures occur when (1 = humerus, 2 = radius/ulna, 3 = femur,
cancellous bone is crumpled. This happens in adults 4 = tibia/fibula) and the second the segment
and typically where this type of bone structure is pres- (1 = proximal, 2 = diaphyseal, 3 = distal, 4 = malleo-
ent, e.g. in the vertebral bodies, calcaneum and tibial lar). A letter specifies the fracture pattern (for the dia-
plateau. physis: A = simple, B = wedge, C = complex; for the
metaphysis: A = extra-articular, B = partial articular,
C = complete articular). Two further numbers specify
the detailed morphology of the fracture (Fig. 23.3).
CLASSIFICATION OF FRACTURES
Sorting fractures into those with similar features

brings advantages: it allows any information about a HOW FRACTURES ARE DISPLACED
fracture to be applied to others in the group (whether
this concerns treatment or prognosis) and it facilitates After a complete fracture the fragments usually become
a common dialogue between surgeons and others displaced, partly by the force of the injury, partly by
involved in the care of such injuries. gravity and partly by the pull of muscles attached to
Traditional classifications, which often bear the them. Displacement is usually described in terms of
originator’s name, are hampered by being applicable translation, alignment, rotation and altered length:
to that type of injury only; even then the term is often • Translation (shift) – The fragments may be shifted
inaccurately applied, famously in the case of Pott’s sideways, backward or forward in relation to each
fracture, which is often applied to any fracture around other, such that the fracture surfaces lose contact.
the ankle though that is not what Sir Percival Pott The fracture will usually unite as long as sufficient
implied when he described the injury in 1765. contact between surfaces is achieved; this may occur
A universal, anatomically based system facilitates even if reduction is imperfect, or indeed even if the
communication and the sharing of data from a variety fracture ends are off-ended but the bone segments
of countries and populations, thus contributing to come to lie side by side.
advances in research and treatment. An alphanumeric • Angulation (tilt) – The fragments may be tilted or
classification developed by Müller and colleagues has angulated in relation to each other. Malalignment,
now been adapted and revised (Muller et al., 1990; if uncorrected, may lead to deformity of the limb.
• Rotation (twist) – One of the fragments may be
twisted on its longitudinal axis; the bone looks
straight but the limb ends up with a rotational
deformity.
• Length – The fragments may be distracted and sep-
arated, or they may overlap, due to muscle spasm,
causing shortening of the bone.
(b) (c) (d)

HOW FRACTURES HEAL
It is commonly supposed that, in order to unite, a

fracture must be immobilized. This cannot be so
since, with few exceptions, fractures unite whether
they are splinted or not; indeed, without a built-in
mechanism for bone union, land animals could
(a) (e) (f) (g)
scarcely have evolved. It is, however, naive to suppose
23.3 Müller’s classification (a) Each long bone has three that union would occur if a fracture were kept moving
segments – proximal, diaphyseal and distal; the proximal indefinitely; the bone ends must, at some stage, be
and distal segments are each defined by a square based on brought to rest relative to one another. But it is not
the widest part of the bone. (b,c,d) Diaphyseal fractures
may be simple, wedge or complex. mandatory for the surgeon to impose this immobility
(e,f,g) Proximal and distal fractures may be extra-articular, artificially – nature can do it with callus, and callus
partial articular of complete articular. forms in response to movement, not to splintage. 689
23 Most fractures are splinted, not to ensure union but 5. Remodelling – The fracture has been bridged by a
to: (1) alleviate pain; (2) ensure that union takes place cuff of solid bone. Over a period of months, or
in good position and (3) permit early movement of even years, this crude ‘weld’ is reshaped by a
the limb and a return of function. continuous process of alternating bone resorption
The process of fracture repair varies according to and formation. Thicker lamellae are laid down
the type of bone involved and the amount of move- where the stresses are high, unwanted buttresses are
ment at the fracture site. carved away and the medullary cavity is reformed.
Eventually, and especially in children, the bone
reassumes something like its normal shape.
HEALING BY CALLUS
This is the ‘natural’ form of healing in tubular bones; HEALING BY DIRECT UNION
in the absence of rigid fixation, it proceeds in five Clinical and experimental studies have shown that cal-
stages: lus is the response to movement at the fracture site
1. Tissue destruction and haematoma formation – (McKibbin, 1978). It serves to stabilize the fragments
Vessels are torn and a haematoma forms around as rapidly as possible – a necessary precondition for
and within the fracture. Bone at the fracture bridging by bone. If the fracture site is absolutely
surfaces, deprived of a blood supply, dies back for immobile – for example, an impacted fracture in can-
a millimetre or two. cellous bone, or a fracture rigidly immobilized by a
2. Inflammation and cellular proliferation – Within 8 metal plate – there is no stimulus for callus (Sarmiento
hours of the fracture there is an acute et al., 1980). Instead, osteoblastic new bone forma-
inflammatory reaction with migration of tion occurs directly between the fragments. Gaps
inflammatory cells and the initiation of between the fracture surfaces are invaded by new cap-
proliferation and differentiation of mesenchymal illaries and osteoprogenitor cells growing in from the
stem cells from the periosteum, the breached edges, and new bone is laid down on the exposed sur-
medullary canal and the surrounding muscle. The face (gap healing). Where the crevices are very narrow
fragment ends are surrounded by cellular tissue, (less than 200 μm), osteogenesis produces lamellar
which creates a scaffold across the fracture site. A bone; wider gaps are filled first by woven bone, which
vast array of inflammatory mediators (cytokines is then remodelled to lamellar bone. By 3–4 weeks the
and various growth factors) is involved. The fracture is solid enough to allow penetration and
clotted haematoma is slowly absorbed and fine bridging of the area by bone remodelling units, i.e.
new capillaries grow into the area. osteoclastic ‘cutting cones’ followed by osteoblasts.
3. Callus formation – The differentiating stem cells Where the exposed fracture surfaces are in intimate
provide chrondrogenic and osteogenic cell contact and held rigidly from the outset, internal
populations; given the right conditions – and this is bridging may occasionally occur without any interme-
usually the local biological and biomechanical diate stages (contact healing).
environment – they will start forming bone and, in Healing by callus, though less direct (the term
some cases, also cartilage. The cell population now ‘indirect’ could be used) has distinct advantages: it
also includes osteoclasts (probably derived from ensures mechanical strength while the bone ends heal,
the new blood vessels), which begin to mop up and with increasing stress the callus grows stronger
dead bone. The thick cellular mass, with its islands and stronger (an example of Wolff’s law). With rigid
of immature bone and cartilage, forms the callus or metal fixation, on the other hand, the absence of cal-
splint on the periosteal and endosteal surfaces. As lus means that there is a long period during which the
the immature fibre bone (or ‘woven’ bone) bone depends entirely upon the metal implant for its
becomes more densely mineralized, movement at integrity. Moreover, the implant diverts stress away
the fracture site decreases progressively and at from the bone, which may become osteoporotic and
about 4 weeks after injury the fracture ‘unites’. not recover fully until the metal is removed.
4. Consolidation – With continuing osteoclastic and
osteoblastic activity the woven bone is transformed
into lamellar bone. The system is now rigid UNION, CONSOLIDATION AND
enough to allow osteoclasts to burrow through NON-UNION
the debris at the fracture line, and close behind
them. Osteoblasts fill in the remaining gaps Repair of a fracture is a continuous process: any stages
between the fragments with new bone. This is a into which it is divided are necessarily arbitrary. In this
slow process and it may be several months before book the terms ‘union’ and ‘consolidation’ are used,
690 the bone is strong enough to carry normal loads. and they are defined as follows:
23.4 Fracture healing Five stages of healing: (a) 23
Haematoma: there is tissue damage and bleeding
at the fracture site; the bone ends die back for a
few millimetres. (b) Inflammation: inflammatory
cells appear in the haematoma. (c) Callus: the cell
population changes to osteoblasts and osteoclasts;
dead bone is mopped up and woven bone appears
in the fracture callus. (d) Consolidation: woven
bone is replaced by lamellar bone and the fracture
is solidly united. (e) Remodelling: the newly formed
bone is remodelled to resemble the normal
(a) (b) (c) (d) (e)
structure.
(a) (b)
23.5 Fracture healing – histology Experimental fracture

healing: (a) by bridging callus and (b) by direct penetration (a) (b) (c)
of the fracture gap by a cutting cone. 23.6 Callus and movement Three patients with femoral
shaft fractures. (a) and (b) are both 6 weeks after fixation:
in (a) the Kuntscher nail fitted tightly, preventing
movement, and there is no callus; in (b) the nail fitted
loosely, permitting some movement, so there is callus.
(c) This patient had cerebral irritation and thrashed around
wildly; at 3 weeks callus is excessive.
23.7 Fracture repair (a) Fracture;

(b) union; (c) consolidation; (d) bone
remodelling. The fracture must be
protected until consolidated.
(a) (b) (c) (d) 691
23 23.8 Non-unions Aseptic non-unions are generally
divided into hypertrophic and atrophic types.
Hypertrophic non-unions often have florid streams of
callus around the fracture gap – the result of insufficient
stability. They are sometimes given colourful names, such
as: (a) elephant’s foot. In contrast, atrophic non-unions
usually arise from an impaired repair process; they are
classified according to the x-ray appearance as (b)

necrotic, (c) gap and (d) atrophic.
(a) (b) (c) (d)
• Union – Union is incomplete repair; the ensheath- and (5) infection. Of course surgical intervention, if
ing callus is calcified. Clinically the fracture site is ill-judged, is another cause!
still a little tender and, though the bone moves in
Non-unions are septic or aseptic. In the latter group,
one piece (and in that sense is united), attempted
they can be either stiff or mobile as judged by clinical
angulation is painful. X-Rays show the fracture line
examination. The mobile ones can be as free and pain-
still clearly visible, with fluffy callus around it.
less as to give the impression of a false joint
Repair is incomplete and it is not safe to subject the
(pseudoarthrosis). On x-ray, non-unions are typified by
unprotected bone to stress.
a lucent line still present between the bone fragments;
• Consolidation – Consolidation is complete repair;
sometimes there is exuberant callus trying – but fail-
the calcified callus is ossified. Clinically the fracture
ing – to bridge the gap (hypertrophic non-union) or at
site is not tender, no movement can be obtained
times none at all (atrophic non-union) with a sorry,
and attempted angulation is painless. X-rays show
withered appearance to the fracture ends.
the fracture line to be almost obliterated and
crossed by bone trabeculae, with well-defined callus
around it. Repair is complete and further protec-
tion is unnecessary.
• Timetable – How long does a fracture take to unite CLINICAL FEATURES
and to consolidate? No precise answer is possible
because age, constitution, blood supply, type of frac- HISTORY
ture and other factors all influence the time taken.
Approximate prediction is possible and Perkins’ There is usually a history of injury, followed by inabil-
timetable is delightfully simple. A spiral fracture in ity to use the injured limb – but beware! The fracture
the upper limb unites in 3 weeks; for consolidation is not always at the site of the injury: a blow to the
multiply by 2; for the lower limb multiply by 2 knee may fracture the patella, femoral condyles, shaft
again; for transverse fractures multiply again by 2. A of the femur or even acetabulum. The patient’s age
more sophisticated formula is as follows. A spiral and mechanism of injury are important. If a fracture
fracture in the upper limb takes 6–8 weeks to con- occurs with trivial trauma, suspect a pathological
solidate; the lower limb needs twice as long. Add lesion. Pain, bruising and swelling are common symp-
25% if the fracture is not spiral or if it involves the toms but they do not distinguish a fracture from a
femur. Children’s fractures, of course, join more soft-tissue injury. Deformity is much more suggestive.
quickly. These figures are only a rough guide; there Always enquire about symptoms of associated
must be clinical and radiological evidence of con- injuries: pain and swelling elsewhere (it is a common
solidation before full stress is permitted without mistake to get distracted by the main injury, particu-
splintage. larly if it is severe), numbness or loss of movement,
• Non-union – Sometimes the normal process of frac- skin pallor or cyanosis, blood in the urine, abdominal
ture repair is thwarted and the bone fails to unite. pain, difficulty with breathing or transient loss of con-
Causes of non-union are: (1) distraction and sepa- sciousness.
ration of the fragments, sometimes the result of Once the acute emergency has been dealt with, ask
interposition of soft tissues between the fragments; about previous injuries, or any other musculoskeletal
(2) excessive movement at the fracture line; (3) a abnormality that might cause confusion when the
severe injury that renders the local tissues non- x-ray is seen. Finally, a general medical history is im-
692 viable or nearly so; (4) a poor local blood supply portant, in preparation for anaesthesia or operation.
GENERAL SIGNS more important to ask if the patient can move the 23
joints distal to the injury.
Unless it is obvious from the history that the patient has
sustained a localized and fairly modest injury, priority
must be given to dealing with the general effects of X-RAY
trauma (see Chapter 22). Follow the ABCs: look for,
and if necessary attend to, Airway obstruction, Breath- X-ray examination is mandatory. Remember the rule
ing problems, Circulatory problems and Cervical spine of twos:
injury. During the secondary survey it will also be nec-
• Two views – A fracture or a dislocation may not be
essary to exclude other previously unsuspected injuries
seen on a single x-ray film, and at least two views
and to be alert to any possible predisposing cause (such
(anteroposterior and lateral) must be taken.
as Paget’s disease or a metastasis).
• Two joints – In the forearm or leg, one bone may be
fractured and angulated. Angulation, however, is
impossible unless the other bone is also broken, or
LOCAL SIGNS a joint dislocated. The joints above and below the
fracture must both be included on the x-ray films.
Injured tissues must be handled gently. To elicit crepi- • Two limbs – In children, the appearance of imma-
tus or abnormal movement is unnecessarily painful; x-ray ture epiphyses may confuse the diagnosis of a frac-
diagnosis is more reliable. Nevertheless the familiar head- ture; x-rays of the uninjured limb are needed for
ings of clinical examination should always be considered, comparison.
or damage to arteries, nerves and ligaments may be • Two injuries – Severe force often causes injuries at
overlooked. A systematic approach is always helpful: more than one level. Thus, with fractures of the cal-
caneum or femur it is important to also x-ray the
• Examine the most obviously injured part.
pelvis and spine.
• Test for artery and nerve damage.
• Two occasions – Some fractures are notoriously diffi-
• Look for associated injuries in the region.
cult to detect soon after injury, but another x-ray
• Look for associated injuries in distant parts.
examination a week or two later may show the
lesion. Common examples are undisplaced fractures
Look of the distal end of the clavicle, scaphoid, femoral
neck and lateral malleolus, and also stress fractures
Swelling, bruising and deformity may be obvious, but
and physeal injuries wherever they occur.
the important point is whether the skin is intact; if the
skin is broken and the wound communicates with the
fracture, the injury is ‘open’ (‘compound’). Note also
the posture of the distal extremity and the colour of SPECIAL IMAGING
the skin (for tell-tale signs of nerve or vessel damage).
Sometimes the fracture – or the full extent of the frac-
ture – is not apparent on the plain x-ray. Computed
Feel tomography may be helpful in lesions of the spine or
The injured part is gently palpated for localized ten- for complex joint fractures; indeed, these cross-
derness. Some fractures would be missed if not specifi- sectional images are essential for accurate visualization
cally looked for, e.g. the classical sign (indeed the only of fractures in ‘difficult’ sites such as the calcaneum or
clinical sign!) of a fractured scaphoid is tenderness on acetabulum. Magnetic resonance imaging may be the
pressure precisely in the anatomical snuff-box. The only way of showing whether a fractured vertebra is
common and characteristic associated injuries should threatening to compress the spinal cord. Radioisotope
also be felt for, even if the patient does not complain scanning is helpful in diagnosing a suspected stress
of them. For example, an isolated fracture of the prox- fracture or other undisplaced fractures.
imal fibula should always alert to the likelihood of an
associated fracture or ligament injury of the ankle, and
in high-energy injuries always examine the spine and DESCRIPTION
pelvis. Vascular and peripheral nerve abnormalities
should be tested for both before and after treatment. Diagnosing a fracture is not enough; the surgeon
should picture it (and describe it) with its properties:
(1) Is it open or closed? (2) Which bone is broken,
Move and where? (3) Has it involved a joint surface? (4)
Crepitus and abnormal movement may be present, What is the shape of the break? (5) Is it stable or
but why inflict pain when x-rays are available? It is unstable? (6) Is it a high-energy or a low-energy 693
23
(a) (b) (c) (d)
(e)
(g) (h)
(f)
23.9 X-ray examination must be ‘adequate’ (a,b) Two films of the same tibia: the fracture may be ‘invisible’ in one
view and perfectly plain in a view at right angles to that. (c,d) More than one occasion: A fractured scaphoid may not be
obvious on the day of injury, but clearly seen 2 weeks later. (e,f) Two joints: The first x-ray (e) did not include the elbow.
This was, in fact, a Monteggia fracture – the head of the radius is dislocated; (f) shows the dislocated radiohumeral joint.
(g,h) Two limbs: Sometimes the abnormality can be appreciated only by comparision with the normal side; in this case
there is a fracture of the lateral condyle on the left side (h).
injury? And last but not least (7) who is the person 1. Shift or translation – backwards, forwards,
with the injury? In short, the examiner must learn to sideways, or longitudinally with impaction or
recognize what has been aptly described as the ‘per- overlap.
sonality’ of the fracture. 2. Tilt or angulation – sideways, backwards or
forwards.
3. Twist or rotation – in any direction.
Shape of the fracture
A problem often arises in the description of angula-
A transverse fracture is slow to join because the area
tion. ‘Anterior angulation’ could mean that the apex
of contact is small; if the broken surfaces are accu-
of the angle points anteriorly or that the distal frag-
rately apposed, however, the fracture is stable on com-
ment is tilted anteriorly: in this text it is always the lat-
pression. A spiral fracture joins more rapidly (because
ter meaning that is intended (‘anterior tilt of the distal
the contact area is large) but is not stable on com-
fragment’ is probably clearer).
pression. Comminuted fractures are often slow to join
because: (1) they are associated with more severe soft-
tissue damage and (2) they are likely to be unstable.
SECONDARY INJURIES
Displacement Certain fractures are apt to cause secondary injuries
For every fracture, three components must be and these should always be assumed to have occurred
694 assessed: until proved otherwise:
• Thoracic injuries – Fractured ribs or sternum may Tscherne (Oestern and Tscherne, 1984) has 23
be associated with injury to the lungs or heart. It is devised a helpful classification of closed injuries:
essential to check cardiorespiratory function.
• Grade 0 – a simple fracture with little or no soft-
• Spinal cord injury – With any fracture of the spine,
tissue injury.
neurological examination is essential to: (1) estab-
• Grade 1 – a fracture with superficial abrasion or
lish whether the spinal cord or nerve roots have
bruising of the skin and subcutaneous tissue.
been damaged and (2) obtain a baseline for later
• Grade 2 – a more severe fracture with deep soft-
comparison if neurological signs should change.
tissue contusion and swelling.
• Pelvic and abdominal injuries – Fractures of the pelvis
• Grade 3 – a severe injury with marked soft-tissue
may be associated with visceral injury. It is especially
damage and a threatened compartment syndrome.
important to enquire about urinary function; if a
urethral or bladder injury is suspected, diagnostic The more severe grades of injury are more likely to
urethrograms or cystograms may be necessary. require some form of mechanical fixation; good skele-
• Pectoral girdle injuries – Fractures and dislocations tal stability aids soft-tissue recovery.
around the pectoral girdle may damage the brachial
plexus or the large vessels at the base of the neck.
Neurological and vascular examination is essential.
REDUCTION
Although general treatment and resuscitation must
TREATMENT OF CLOSED always take precedence, there should not be undue
delay in attending to the fracture; swelling of the soft
FRACTURES parts during the first 12 hours makes reduction
increasingly difficult. However, there are some situa-
General treatment is the first consideration: treat the tions in which reduction is unnecessary: (1) when
patient, not only the fracture. The principles are dis- there is little or no displacement; (2) when displace-
cussed in Chapter 22. ment does not matter initially (e.g. in fractures of the
Treatment of the fracture consists of manipulation clavicle) and (3) when reduction is unlikely to succeed
to improve the position of the fragments, followed by (e.g. with compression fractures of the vertebrae).
splintage to hold them together until they unite; Reduction should aim for adequate apposition and
meanwhile joint movement and function must be pre- normal alignment of the bone fragments. The greater
served. Fracture healing is promoted by physiological the contact surface area between fragments the more
loading of the bone, so muscle activity and early likely healing is to occur. A gap between the fragment
weightbearing are encouraged. These objectives are ends is a common cause of delayed union or non-
covered by three simple injunctions: union. On the other hand, so long as there is contact
and the fragments are properly aligned, some overlap
• Reduce.
at the fracture surfaces is permissible. The exception is
• Hold.
a fracture involving an articular surface; this should be
• Exercise.
reduced as near to perfection as possible because any
Two existential problems have to be overcome. The irregularity will cause abnormal load distribution
first is how to hold a fracture adequately and yet per- between the surfaces and predispose to degenerative
mit the patient to use the limb sufficiently; this is a changes in the articular cartilage.
conflict (Hold versus Move) that the surgeon seeks to There are two methods of reduction: closed and
resolve as rapidly as possible (e.g. by internal fixation). open.
However the surgeon also wants to avoid unnecessary
risks – here is a second conflict (Speed versus Safety).
This dual conflict epitomizes the four factors that CLOSED REDUCTION
dominate fracture management (the term ‘fracture
quartet’ seems appropriate). Under appropriate anaesthesia and muscle relaxation,
The fact that the fracture is closed (and not open) the fracture is reduced by a three-fold manoeuvre: (1)
is no cause for complacency. The most important the distal part of the limb is pulled in the line of the
factor in determining the natural tendency to heal is bone; (2) as the fragments disengage, they are reposi-
the state of the surrounding soft tissues and the local tioned (by reversing the original direction of force if
blood supply. Low-energy (or low-velocity) fractures this can be deduced) and (3) alignment is adjusted in
cause only moderate soft-tissue damage; high-energy each plane. This is most effective when the perios-
(velocity) fractures cause severe soft-tissue damage, teum and muscles on one side of the fracture remain
no matter whether the fracture is open or closed. intact; the soft-tissue strap prevents over-reduction 695
23 better alignment to be obtained; this practice is help-
ful for femoral and tibial shaft fractures and even
supracondylar humeral fractures in children.
In general, closed reduction is used for all mini-
mally displaced fractures, for most fractures in chil-
dren and for fractures that are not unstable after
reduction and can be held in some form of splint or

cast. Unstable fractures can also be reduced using
closed methods prior to stabilization with internal or
(a) external fixation. This avoids direct manipulation of
the fracture site by open reduction, which damages
the local blood supply and may lead to slower healing
times; increasingly, surgeons resort to reduction
manoeuvres that avoid fracture-site exposure, even
when the aim is some form of internal or external fix-
ation. Traction, which reduces fracture fragments
through ligamentotaxis (ligament pull), can usually be
applied by using a fracture table or bone distractor.
(b)
OPEN REDUCTION
Operative reduction of the fracture under direct vision
is indicated: (1) when closed reduction fails, either
because of difficulty in controlling the fragments or
because soft tissues are interposed between them; (2)
when there is a large articular fragment that needs
accurate positioning or (3) for traction (avulsion) frac-
tures in which the fragments are held apart. As a rule,
however, open reduction is merely the first step to
(c) internal fixation.
23.10 Closed reduction (a) Traction in the line of the
bone. (b) Disimpaction. (c) Pressing fragment into reduced
position.
HOLD REDUCTION
and stabilizes the fracture after it has been reduced The word ‘immobilization’ has been deliberately
(Charnley 1961). avoided because the objective is seldom complete
Some fractures are difficult to reduce by manipula- immobility; usually it is the prevention of displace-
tion because of powerful muscle pull and may need ment. Nevertheless, some restriction of movement is
prolonged traction. Skeletal or skin traction for several needed to promote soft-tissue healing and to allow
days allows for soft-tissue tension to decrease and a free movement of the unaffected parts.
23.11 Closed
reduction These two
ankle fractures look
somewhat similar but
are caused by different
forces. The causal force
must be reversed to
(b) (c) achieve reduction:
(a) requires internal
rotation (b); an
adduction force (c) is
needed for (d).
696 (a) (d)

Traction cannot hold a fracture still; it can pull a 23
long bone straight and hold it out to length but to
maintain accurate reduction is sometimes difficult.
Meanwhile the patient can move the joints and exer-
cise the muscles.
Traction is safe enough, provided it is not excessive
and care is taken when inserting the traction pin. The
23.12 Hold reduction Showing how, if the soft tissues problem is speed: not because the fracture unites
around a fracture are intact, traction will align the bony slowly (it does not) but because lower limb traction
fragments. keeps the patient in hospital. Consequently, as soon as
the fracture is ‘sticky’ (deformable but not displace-
able), traction should be replaced by bracing, if this
HOLD
method is feasible. Traction includes:
SPEED • Traction by gravity – This applies only to upper
SAFETY limb injuries. Thus, with a wrist sling the weight of
the arm provides continuous traction to the
MOVE humerus. For comfort and stability, especially with
a transverse fracture, a U-slab of plaster may be
bandaged on or, better, a removable plastic sleeve
23.13 Continuous traction ‘Speed’ is the weak member from the axilla to just above the elbow is held on
of the quartet. with Velcro.
• Skin traction – Skin traction will sustain a pull of no
The available methods of holding reduction are: more than 4 or 5 kg. Holland strapping or one-
way-stretch Elastoplast is stuck to the shaved skin
• Continuous traction. and held on with a bandage. The malleoli are pro-
• Cast splintage. tected by Gamgee tissue, and cords or tapes are
• Functional bracing. used for traction.
• Internal fixation. • Skeletal traction – A stiff wire or pin is inserted –
• External fixation. usually behind the tibial tubercle for hip, thigh and
In the modern technological age, ‘closed’ methods knee injuries, or through the calcaneum for tibial
are often scorned – an attitude arising from ignorance fractures – and cords tied to them for applying trac-
rather than experience. The muscles surrounding a tion. Whether by skin or skeletal traction, the frac-
fracture, if they are intact, act as a fluid compartment; ture is reduced and held in one of three ways: fixed
traction or compression creates a hydraulic effect that traction, balanced traction or a combination of the
is capable of splinting the fracture. Therefore closed two.
methods are most suitable for fractures with intact
soft tissues, and are liable to fail if they are used as the Fixed traction
primary method of treatment for fractures with severe
soft-tissue damage. Other contraindications to non- The pull is exerted against a fixed point. The usual
operative methods are inherently unstable fractures, method is to tie the traction cords to the distal end of
multiple fractures and fractures in confused or unco- a Thomas’ splint and pull the leg down until the prox-
operative patients. If these constraints are borne in imal, padded ring of the splint abuts firmly against the
mind, closed reduction can be sensibly considered in pelvis.
choosing the most suitable method of fracture splin-
tage. Remember, too, that the objective is to splint
Balanced traction
the fracture, not the entire limb!
Here the traction cords are guided over pulleys at the
foot of the bed and loaded with weights; counter-trac-
CONTINUOUS TRACTION tion is provided by the weight of the body when the
Traction is applied to the limb distal to the fracture, foot of the bed is raised.
so as to exert a continuous pull in the long axis of the
bone, with a counterforce in the opposite direction
(to prevent the patient being merely dragged along
Combined traction
the bed). This is particularly useful for shaft fractures If a Thomas’ splint is used, the tapes are tied to the
that are oblique or spiral and easily displaced by mus- end of the splint and the entire splint is then sus-
cle contraction. pended, as in balanced traction. 697
23 23.14 Methods of
traction (a) Traction by
gravity. (b,c,d) Skin traction:
(b) fixed; (c) balanced;
(d) Russell. (e) Skeletal
traction with a splint and a
knee-flexion piece.
(a) (b) (c)
(d) (e)
Complications of traction
HOLD
Circulatory embarrassment In children especially,
traction tapes and circular bandages may constrict the SPEED SAFETY
circulation; for this reason ‘gallows traction’, in which
the baby’s legs are suspended from an overhead beam, MOVE
should never be used for children over 12 kg in weight.

Nerve injury In older people, leg traction may
23.15 Casts ‘Move’ is the weakest member of the
predispose to peroneal nerve injury and cause a drop- quartet.
foot; the limb should be checked repeatedly to see that
it does not roll into external rotation during traction.
Pin sites must be kept clean and
Pin site infection cannot move and are liable to stiffen; stiffness, which
should be checked daily. has earned the sobriquet ‘fracture disease’, is the
problem with conventional casts. While the swelling
and haematoma resolve, adhesions may form that
CAST SPLINTAGE bind muscle fibres to each other and to the bone; with
articular fractures, plaster perpetuates surface irregu-
Plaster of Paris is still widely used as a splint, especially larities (closed reduction is seldom perfect) and lack of
for distal limb fractures and for most children’s frac- movement inhibits the healing of cartilage defects.
tures. It is safe enough, so long as the practitioner is Newer substitutes have some advantages over plaster
alert to the danger of a tight cast and provided pres- (they are impervious to water, and also lighter) but as
sure sores are prevented. The speed of union is neither long as they are used as full casts the basic drawback
greater nor less than with traction, but the patient can is the same.
go home sooner. Holding reduction is usually no Stiffness can be minimized by: (1) delayed splintage
problem and patients with tibial fractures can bear – that is, by using traction until movement has been
698 weight on the cast. However, joints encased in plaster regained, and only then applying plaster; or (2)
23
(a) (b) (c)
(d) (e) (f)
(g) (h) (i)
23.16 Plaster technique Applying a well-fitting and effective plaster needs experience and
attention to detail. (a) A well-equipped plaster trolley is invaluable. (b) Adequate anaesthesia and
careful study of the x-ray films are both indispensable. (c) For a below-knee plaster the thigh is
best supported on a padded block. (d) Stockinette is threaded smoothly onto the leg. (e) For a
padded plaster the wool is rolled on and it must be even. (f) Plaster is next applied smoothly,
taking a tuck with each turn, and (g) smoothing each layer firmly onto the one beneath. (h)
While still wet the cast is moulded away from the point points. (i) With a recent injury the plaster
is then split.
starting with a conventional cast but, after a few angle and the tarsus and forefoot neutral (this ‘planti-
weeks, when the limb can be handled without too grade’ position is essential for normal walking). In the
much discomfort, replacing the cast by a functional upper limb the position of the splinted joints varies
brace which permits joint movement. with the fracture. Splintage must not be discontinued
(though a functional brace may be substituted) until
the fracture is consolidated; if plaster changes are
Technique needed, check x-rays are essential.
After the fracture has been reduced, stockinette is
threaded over the limb and the bony points are pro-
tected with wool. Plaster is then applied. While it is Complications
setting the surgeon moulds it away from bony promi-
Plaster immobilization is safe, but only if care is taken
nences; with shaft fractures three-point pressure can
to prevent certain complications. These are tight cast,
be applied to keep the intact periosteal hinge under
pressure sores and abrasion or laceration of the skin.
tension and thereby maintain reduction.
If the fracture is recent, further swelling is likely; Tight cast The cast may be put on too tightly, or it may
the plaster and stockinette are therefore split from top become tight if the limb swells. The patient complains
to bottom, exposing the skin. Check x-rays are essen- of diffuse pain; only later – sometimes much later – do
tial and the plaster can be wedged if further correction the signs of vascular compression appear. The limb
of angulation is necessary. should be elevated, but if the pain persists, the only safe
With fractures of the shafts of long bones, rotation course is to split the cast and ease it open: (1)
is controlled only if the plaster includes the joints throughout its length and (2) through all the padding
above and below the fracture. In the lower limb, the down to skin. Whenever swelling is anticipated the cast
knee is usually held slightly flexed, the ankle at a right should be applied over thick padding and the plaster 699
23
23.17 Functional bracing (cast bracing) Despite plaster the patient has
excellent joint movement. (Courtesy of Dr John A Feagin).
should be split before it sets, so as to provide a firm Technique

but not absolutely rigid splint.
Considerable skill is needed to apply an effective
Pressure sores Even a well-fitting cast may press upon brace. First the fracture is ‘stabilized’: by a few days on
the skin over a bony prominence (the patella, heel, traction or in a conventional plaster for tibial frac-
elbow or head of the ulna). The patient complains of tures; and by a few weeks on traction for femoral frac-
localized pain precisely over the pressure spot. Such tures (until the fracture is sticky, i.e. deformable but
localized pain demands immediate inspection through not displaceable). Then a hinged cast or splint is
a window in the cast. applied, which holds the fracture snugly but permits
Skin abrasion or laceration This is really a complication joint movement; functional activity, including weight-
of removing plasters, especially if an electric saw is bearing, is encouraged. Unlike internal fixation, func-
used. Complaints of nipping or pinching during plas- tional bracing holds the fracture through compression
ter removal should never be ignored; a ripped forearm of the soft tissues; the small amount of movement that
is a good reason for litigation. occurs at the fracture site through using the limb
encourages vascular proliferation and callus forma-
Loose cast Once the swelling has subsided, the cast tion. Details of the rationale, technique and applica-
may no longer hold the fracture securely. If it is loose, tions are given by Sarmiento and Latta (Sarmiento
the cast should be replaced. and Latta 1999, 2006).
FUNCTIONAL BRACING INTERNAL FIXATION

Functional bracing, using either plaster of Paris or one Bone fragments may be fixed with screws, a metal
of the lighter thermoplastic materials, is one way of plate held by screws, a long intramedullary rod or nail
preventing joint stiffness while still permitting fracture (with or without locking screws), circumferential
splintage and loading. Segments of a cast are applied bands or a combination of these methods.
only over the shafts of the bones, leaving the joints Properly applied, internal fixation holds a fracture
free; the cast segments are connected by metal or plas- securely so that movement can begin at once; with
tic hinges that allow movement in one plane. The early movement the ‘fracture disease’ (stiffness and
splints are ‘functional’ in that joint movements are
much less restricted than with conventional casts.
Functional bracing is used most widely for fractures
of the femur or tibia, but since the brace is not very HOLD
rigid, it is usually applied only when the fracture is SAFETY
beginning to unite, i.e. after 3–6 weeks of traction or SPEED
conventional plaster. Used in this way, it comes out
well on all four of the basic requirements: the fracture MOVE
can be held reasonably well; the joints can be moved;
the fracture joins at normal speed (or perhaps slightly
quicker) without keeping the patient in hospital and 23.18 Internal fixation ‘Safety’ is the weak member of
700 the method is safe. the quartet.
23
COULD be fixed
P
SHOULD be fixed -U
K
C
A
B (a) (b)
MUST be fixed L
IL
K
S
23.19 Indications staircase The indications for fixation

are not immutable; thus, if the surgical skill or back-up
facilities (staff, sterility and equipment) are of a low order,
internal fixation is indicated only when the alternative is
unacceptable (e.g. with femoral neck fractures). With
average skill and facilities, fixation is indicated when
alternative methods are possible but very difficult or
unwise (e.g. multiple injuries). With the highest levels of
skill and facilities, fixation is reasonable if it saves time, (b) (d)
money or beds.
23.20 Indications for internal fixation (a) This patella
has been pulled apart and can be held together only be
internal fixation. (b) Fracture dislocation of the ankle is
oedema) is abolished. As far as speed is concerned, often unstable after reduction and usually requires fixation.
the patient can leave hospital as soon as the wound (c) This patient was considered to be too ill for operation;
is healed, but he must remember that, even though her femoral neck fracture has failed to unite without rigid
the bone moves in one piece, the fracture is not fixation. (d) Pathological fracture in Paget bone; without
fixation, union may not occur.
united – it is merely held by a metal bridge and
unprotected weightbearing is, for some time,
unsafe.
The greatest danger, however, is sepsis; if infection fractures). Also included are those fractures liable
supervenes, all the manifest advantages of internal fix- to be pulled apart by muscle action (e.g. transverse
ation (precise reduction, immediate stability and early fracture of the patella or olecranon).
movement) may be lost. The risk of infection depends 3. Fractures that unite poorly and slowly, principally
upon: (1) the patient – devitalized tissues, a dirty fractures of the femoral neck.
wound and an unfit patient are all dangerous; (2) the 4. Pathological fractures in which bone disease may
surgeon – thorough training, a high degree of surgi- prevent healing.
cal dexterity and adequate assistance are all essential 5. Multiple fractures where early fixation (by either
and (3) the facilities – a guaranteed aseptic routine, a internal or external fixation) reduces the risk of
full range of implants and staff familiar with their use general complications and late multisystem organ
are all indispensable. failure (Pape et al., 2005; Roberts et al., 2005).
6. Fractures in patients who present nursing
difficulties (paraplegics, those with multiple
Indications injuries and the very elderly).
Internal fixation is often the most desirable form of
treatment. The chief indications are:
Types of internal fixation
1. Fractures that cannot be reduced except by
operation. Interfragmentary screws Screws that are only partially
2. Fractures that are inherently unstable and prone to threaded (a similar effect is achieved by overdrilling the
re-displace after reduction (e.g. mid-shaft fractures ‘near’ cortex of bone) exert a compression or ‘lag’
of the forearm and some displaced ankle effect when inserted across two fragments. The 701
23 technique is useful for reducing single fragments onto 1. Neutralization – when used to bridge a
the main shaft of a tubular bone or fitting together fracture and supplement the effect of
fragments of a metaphyseal fracture. interfragmentary lag screws; the plate is to
resist torque and shortening.
Wires (transfixing, cerclage and tension-band) Transfixing
2. Compression – often used in metaphyseal
wires, often passed percutaneously, can hold major
fractures where healing across the cancellous
fracture fragments together. They are used in situations
fracture gap may occur directly, without

where fracture healing is predictably quick (e.g. in
periosteal callus. This technique is less
children or for distal radius fractures), and some form
appropriate for diaphyseal fractures and there
of external splintage (usually a cast) is applied as
has been a move towards the use of long plates
supplementary support.
that span the fracture, thus achieving some
Cerclage and tension-band wires are essentially
stability without totally sacrificing the
loops of wire passed around two bone fragments and
biological (and callus producing) effect of
then tightened to compress the fragments together.
movement.
When using cerclage wires, make sure that the wires
3. Buttressing – here the plate props up the
hug the bone and do not embrace any of the close-
‘overhang’ of the expanded metaphyses of long
lying nerves or vessels.
bones (e.g. in treating fractures of the proximal
Both techniques are used for patellar fractures: the
tibial plateau).
tension-band wire is placed such that the maximum
4. Tension-band – using a plate in this manner,
compressive force is over the tensile surface, which is
again on the tensile surface of the bone, allows
usually the convex side of the bone.
compression to be applied to the biomechanically
Plates and screws This form of fixation is useful for more advantageous side of the fracture.
treating metaphyseal fractures of long bones and 5. Anti-glide – by fixing a plate over the tip of a
diaphyseal fractures of the radius and ulna. Plates have spiral or oblique fracture line and then using
five different functions: the plate as a reduction aid, the anatomy is
23.21 Internal fixation The method used

must be appropriate to the situation:
(a) screws – interfragmentary compression;
(b) plate and screws – most suitable in the
forearm or around the metaphysis; (c) flexible
intramedullary nails – for long bones in
children, particularly forearm bones and the
femur; (d) interlocking nail and screws – ideal
for the femur and tibia; (e) dynamic
compression screw and plate – ideal for the
proximal and distal ends of the femur;
(f) simple K-wires – for fractures around the
elbow and wrist and (g) tension-band wiring
– for olecranon or fractures of the patella.
(a) (b) (c)
702 (d) (e) (f) (g)

23.22 Bad fixation (how not to do it) 23
(a) Too little. (b) Too much. (c) Too weak.
(a) (b) (c)
restored with minimal stripping of soft tissues. away from partial weightbearing for 6 weeks or longer,
The position of the plate acts to prevent until callus or other radiological sign of fracture healing
shortening and recurrent displacement of the is seen on x-ray. Pain at the fracture site is a danger sig-
fragments. nal and must be investigated.
Intramedullary nails These are suitable for long bones. Refracture It is important not to remove metal
A nail (or long rod) is inserted into the medullary canal implants too soon, or the bone may refracture. A year
to splint the fracture; rotational forces are resisted by is the minimum and 18 or 24 months safer; for several
introducing transverse interlocking screws that transfix weeks after removal the bone is weak, and care or pro-
the bone cortices and the nail proximal and distal to tection is needed.
the fracture. Nails are used with or without prior
reaming of the medullary canal; reamed nails achieve
an interference fit in addition to the added stability
from interlocking screws, but at the expense of EXTERNAL FIXATION
temporary loss of the intramedullary blood supply. A fracture may be held by transfixing screws or tensioned
wires that pass through the bone above and below the
fracture and are attached to an external frame. This is
Complications of internal fixation especially applicable to the tibia and pelvis, but the
method is also used for fractures of the femur, humerus,
Most of the complications of internal fixation are due
lower radius and even bones of the hand.
to poor technique, poor equipment or poor operating
conditions:
Indications
Infection Iatrogenic infection is now the most com-
External fixation is particularly useful for:
mon cause of chronic osteomyelitis; the metal does
not predispose to infection but the operation and 1. Fractures associated with severe soft-tissue damage
quality of the patient’s tissues do. (including open fractures) or those that are
contaminated, where internal fixation is risky and
Non-union If the bones have been fixed rigidly with a
repeated access is needed for wound inspection,
gap between the ends, the fracture may fail to unite.
dressing or plastic surgery.
This is more likely in the leg or the forearm if one
2. Fractures around joints that are potentially suitable
bone is fractured and the other remains intact. Other
for internal fixation but the soft tissues are too
causes of non-union are stripping of the soft tissues
swollen to allow safe surgery; here, a spanning
and damage to the blood supply in the course of oper-
external fixator provides stability until soft-tissue
ative fixation.
conditions improve.
Implant failure Metal is subject to fatigue and can fail 3. Patients with severe multiple injuries, especially if
unless some union of the fracture has occurred. Stress there are bilateral femoral fractures, pelvic
must therefore be avoided and a patient with a broken fractures with severe bleeding, and those with limb
tibia internally fixed should walk with crutches and stay and associated chest or head injuries. 703
23 23.23 External fixation
of fractures External
fixation is widely used for
‘damage control’
(a,b) temporary
stabilization of fractures in
order to allow the patient’s
general condition or the

state of soft tissues to
improve prior to definitive
surgery or
(c–f) reconstruction of
limbs using distraction
(a) (b) osteogenesis.
(c) A bone defect after
surgical resection with
gentamicin beads used to
fill the space temporarily.
(d) Bone transport from a
more proximal osteotomy.
(e) ‘Docking’ of the
transported segment and
(f) final union and
restoration of structural
integrity.
(c) (d) (e) (f)
4. Ununited fractures, which can be excised and as early as possible to ‘stimulate’ fracture healing.
compressed; sometimes this is combined with Some fixators incorporate a telescopic unit that allows
bone lengthening to replace the excised segment. ‘dynamization’; this will convert the forces of weight-
5. Infected fractures, for which internal fixation bearing into axial micromovement at the fracture site,
might not be suitable. thus promoting callus formation and accelerating
bone union (Kenwright et al., 1991).
Technique
Complications
The principle of external fixation is simple: the bone is
transfixed above and below the fracture with screws or Damage to soft-tissue structures Transfixing pins or
tensioned wires and these are then connected to each wires may injure nerves or vessels, or may tether
other by rigid bars. There are numerous types of ligaments and inhibit joint movement. The surgeon
external fixation devices; they vary in the technique of must be thoroughly familiar with the cross-sectional
application and each type can be constructed to pro- anatomy before operating.
vide varying degrees of rigidity and stability. Most of
OverdistractionIf there is no contact between the
them permit adjustment of length and alignment after
fragments, union is unlikely.
application on the limb.
The fractured bone can be thought of as broken into Pin-track infection This is less likely with good
segments – a simple fracture has two segments whereas operative technique. Nevertheless, meticulous pin-site
a two-level (segmental) fracture has three and so on. Each care is essential, and antibiotics should be administered
segment should be held securely, ideally with the half-pins immediately if infection occurs.
or tensioned wires straddling the length of that segment.
The wires and half-pins must be inserted with care.
Knowledge of ‘safe corridors’ is essential so as to avoid
injuring nerves or vessels; in addition, the entry sites EXERCISE
should be irrigated to prevent burning of the bone (a
temperature of only 50ºC can cause bone death). More correctly, restore function – not only to the
The fracture is then reduced by connecting the var- injured parts but also to the patient as a whole. The
ious groups of pins and wires by rods. objectives are to reduce oedema, preserve joint move-
Depending on the stability of fixation and the ment, restore muscle power and guide the patient
704 underlying fracture pattern, weightbearing is started back to normal activity:
23
(a) (b)
23.24 Some aspects of soft tissue
treatment Swelling is minimized by
improving venous drainage. This can be
accomplished by: (1) elevation and (2)
firm support. Stiffness is minimized by
exercise. (a,c) Intermittent venous plexus
pumps for use on the foot or palm to
help reduce swelling. (b) A made-to-
measure pressure garment that helps
reduce swelling and scarring after
treatment. (d) Coban wrap around a
limb to control swelling during
treatment.
(c) (d)
Prevention of oedema Swelling is almost inevitable after exercise the limb actively, but not to let it dangle.
a fracture and may cause skin stretching and blisters. When the plaster is finally removed, a similar routine of
Persistent oedema is an important cause of joint activity punctuated by elevation is practised until
stiffness, especially in the hand; it should be prevented circulatory control is fully restored.
if possible, and treated energetically if it is already Injuries of the upper limb also need elevation. A
present, by a combination of elevation and exercise. sling must not be a permanent passive arm-holder; the
Not every patient needs admission to hospital, and less limb must be elevated intermittently or, if need be,
severe injuries of the upper limb are successfully continuously.
managed by placing the arm in a sling; but it is then
essential to insist on active use, with movement of all
the joints that are free. As with most closed fractures,
in all open fractures and all fractures treated by internal
fixation it must be assumed that swelling will occur;
the limb should be elevated and active exercise begun
as soon as the patient will tolerate this. The essence of
soft-tissue care may be summed up thus: elevate and
exercise; never dangle, never force.
Elevation An injured limb usually needs to be elevated;
after reduction of a leg fracture the foot of the bed is
raised and exercises are begun. If the leg is in plaster
the limb must, at first, be dependent for only short
periods; between these periods, the leg is elevated on 23.25 Continuous passive motion The motorized frame
a chair. The patient is allowed, and encouraged, to provides continuous flexion and extension to pre-set limits. 705
23 Active exercise Active movement helps to pump away atre. The patient is given antibiotics, usually co-amox-
oedema fluid, stimulates the circulation, prevents soft- iclav or cefuroxime, but clindamycin if the patient is
tissue adhesion and promotes fracture healing. A limb allergic to penicillin. Tetanus prophylaxis is adminis-
encased in plaster is still capable of static muscle tered: toxoid for those previously immunized, human
contraction and the patient should be taught how to antiserum if not. The limb is then splinted until sur-
do this. When splintage is removed the joints are gery is undertaken.
mobilized and muscle-building exercises are steadily The limb circulation and distal neurological status
increased. Remember that the unaffected joints need will need checking repeatedly, particularly after any
exercising too; it is all too easy to neglect a stiffening fracture reduction manoeuvres. Compartment syn-
shoulder while caring for an injured wrist or hand. drome is not prevented by there being an open frac-
ture; vigilance for this complication is wise.
Assisted movement It has long been taught that passive
movement can be deleterious, especially with injuries
around the elbow, where there is a high risk of
developing myositis ossificans. Certainly forced CLASSIFYING THE INJURY
movements should never be permitted, but gentle
Treatment is determined by the type of fracture, the
assistance during active exercises may help to retain
nature of the soft-tissue injury (including the wound
function or regain movement after fractures involving
size) and the degree of contamination. Gustilo’s clas-
the articular surfaces. Nowadays this is done with
sification of open fractures is widely used (Gustilo et
machines that can be set to provide a specified range
al., 1984):
and rate of movement (‘continuous passive motion’).
Type 1 – The wound is usually a small, clean puncture
Functional activity As the patient’s mobility improves,
through which a bone spike has protruded. There is
an increasing amount of directed activity is included in
little soft-tissue damage with no crushing and the
the programme. He may need to be taught again how
fracture is not comminuted (i.e. a low-energy
to perform everyday tasks such as walking, getting in
fracture).
and out of bed, bathing, dressing or handling eating
Type II – The wound is more than 1 cm long, but
utensils. Experience is the best teacher and the patient
there is no skin flap. There is not much soft-tissue
is encouraged to use the injured limb as much as
damage and no more than moderate crushing or
possible. Those with very severe or extensive injuries
comminution of the fracture (also a low- to
may benefit from spending time in a special
moderate-energy fracture).
rehabilitation unit, but the best incentive to full
Type III – There is a large laceration, extensive
recovery is the promise of re-entry into family life,
damage to skin and underlying soft tissue and, in the
recreational pursuits and meaningful work.
most severe examples, vascular compromise. The
injury is caused by high-energy transfer to the bone
and soft tissues. Contamination can be significant.
There are three grades of severity. In type III A the
TREATMENT OF OPEN fractured bone can be adequately covered by soft tis-
sue despite the laceration. In type III B there is exten-
FRACTURES sive periosteal stripping and fracture cover is not
possible without use of local or distant flaps. The frac-
INITIAL MANAGEMENT ture is classified as type III C if there is an arterial
injury that needs to be repaired, regardless of the
Patients with open fractures may have multiple injuries; amount of other soft-tissue damage.
a rapid general assessment is the first step and any life- The incidence of wound infection correlates
threatening conditions are addressed (see Chapter 22). directly with the extent of soft-tissue damage, rising
The open fracture may draw attention away from from less than 2 per cent in type I to more than 10 per
other more important conditions and it is essential cent in type III fractures.
that the step-by-step approach in advanced trauma life
support not be forgotten.
When the fracture is ready to be dealt with, the
wound is first carefully inspected; any gross contami- PRINCIPLES OF TREATMENT
nation is removed, the wound is photographed with a
Polaroid or digital camera to record the injury and the All open fractures, no matter how trivial they may
area then covered with a saline-soaked dressing under seem, must be assumed to be contaminated; it is
an impervious seal to prevent desiccation. This is left important to try to prevent them from becoming
706 undisturbed until the patient is in the operating the- infected. The four essentials are:
• Antibiotic prophylaxis. and Pseudomonas, both of which are near the top of 23
• Urgent wound and fracture debridement. the league table of responsible bacteria. The total
• Stabilization of the fracture. period of antibiotic use for these fractures should not
• Early definitive wound cover. be greater than 72 hours (Table 23.1).
Sterility and antibiotic cover Debridement
The wound should be kept covered until the patient The operation aims to render the wound free of for-
reaches the operating theatre. In most cases co-amox- eign material and of dead tissue, leaving a clean surgi-
iclav or cefuroxime (or clindamycin if penicillin allergy cal field and tissues with a good blood supply
is an issue) is given as soon as possible, often in the throughout. Under general anaesthesia the patient’s
Accident and Emergency department. At the time of clothing is removed, while an assistant maintains trac-
debridement, gentamicin is added to a second dose of tion on the injured limb and holds it still. The dress-
the first antibiotic. Both antibiotics provide prophy- ing previously applied to the wound is replaced by a
laxis against the majority of Gram-positive and Gram- sterile pad and the surrounding skin is cleaned. The
negative bacteria that may have entered the wound at pad is then taken off and the wound is irrigated thor-
the time of injury. Only co-amoxiclav or cefuroxime oughly with copious amounts of physiological saline.
(or clindamycin) is continued thereafter; as wounds of The wound is covered again and the patient’s limb
Gustilo grade I fractures can be closed at the time of then prepped and draped for surgery.
debridement, antibiotic prophylaxis need not be for Many surgeons prefer to use a tourniquet as this
more than 24 hours. With Gustilo grade II and IIIA provides a bloodless field. However this induces
fractures, some surgeons prefer to delay closure after ischaemia in an already badly injured leg and can make
a ‘second look’ procedure. Delayed cover is also usu- it difficult to recognize which structures are devital-
ally practised in most cases of Grade IIIB and IIIC ized. A compromise is to apply the tourniquet but not
injuries. As the wounds have now been present in a to inflate it during the debridement unless absolutely
hospital environment for some time, and there are necessary.
data to indicate infections after such open fractures Because open fractures are often high-energy
are caused mostly by hospital-acquired bacteria and injuries with severe tissue damage, the operation
not seeded at the time of injury, gentamicin and van- should be performed by someone skilled in dealing
comycin (or teicoplanin) are given at the time of with both skeletal and soft tissues; ideally this will be
definitive wound cover. These antibiotics are effective a joint effort by orthopaedic and plastic surgeons. The
against methicillin-resistant Staphylococcus aureus following principles must be observed:
Table 23.1 Antibiotics for open fractures1
Grade I Grade II Grade IIIA Grade IIIB/IIIC
As soon as possible Co-amoxiclav2 Co-amoxiclav2 Co-amoxiclav2 Co-amoxiclav2

(within 3 hours of
injury)
At debridement Co-amoxiclav2 and Co-amoxiclav2 and Co-amoxiclav2 and Co-amoxiclav2 and

gentamicin gentamicin gentamicin gentamicin
At definitive fracture Wound cover is usually Wound cover is usually Wound cover is usually Gentamicin and
cover possible at debridement; possible at debridement. possible at debridement. vancomycin (or
delayed closure If delayed, gentamicin If delayed, gentamicin teicoplanin)
unnecessary and vancomycin (or and vancomycin (or
teicoplanin) at the time teicoplanin) at the time
of cover of cover
Continued prophylaxis Only co-amoxiclav2* Only co-amoxiclav2 Only co-amoxiclav2 Only co-amoxiclav2
continued after surgery continued between continued between continued between
procedures and after final procedures and after final procedures and after final
surgery surgery surgery
Maximum period 24 hours 72 hours 72 hours 72 hours

1
Based on the Standards for the Management of Open Fractures of the Lower Limb, British Orthopaedic Association and British
Association of Plastic, Reconstructive and Aesthetic Surgeons, 2009
2
Or cefuroxime (clindamycin for those with penicillin allergy). 707
23
(a) (b)
23.27 Delivering the fracture Debridement is only

possible if the fracture is adequately seen; for this, the
fracture ends have to be delivered from within.
which it was forced at the moment of injury; the frac-

ture surfaces will be exposed through the wound with-
out any additional damage to the soft tissues. Large
(c) (d)
bone levers and retractors should not be used.
23.26 Wound extensions for access in open fractures
of the tibia Wound incisions (extensions) for adequate Removal of devitalized tissue Devitalized tissue provides
access to an open tibial fracture are made along standard a nutrient medium for bacteria. Dead muscle can be
fasciotomy incisions: 1 cm behind the posteromedial recognized by its purplish colour, its mushy
border of the tibia and 2–3 cm lateral to the crest of the consistency, its failure to contract when stimulated and
tibia as shown in this example of a two-incision fasciotomy.
The dotted lines mark out the crest (C) and posteromedial
its failure to bleed when cut. All doubtfully viable
corner (PM) of the tibia (a). These incisions avoid injury to tissue, whether soft or bony, should be removed. The
the perforating branches that supply areas of skin that can fracture ends can be nibbled away until seen to bleed.
be used as flaps to cover the exposed fracture (b). This
clinical example shows how local skin necrosis around an Wound cleansing All foreign material and tissue debris
open fracture is excised and the wound extended is removed by excision or through a wash with copious
proximally along a fasciotomy incision (c,d). quantities of saline. A common mistake is to inject
syringefuls of fluid through a small aperture – this only
serves to push contaminants further in; 6–12 L of
Wound excision The wound margins are excised, but saline may be needed to irrigate and clean an open
only enough to leave healthy skin edges. fracture of a long bone. Adding antibiotics or
antiseptics to the solution has no added benefit.
Wound extension Thorough cleansing necessitates
adequate exposure; poking around in a small wound Nerves and tendons As a general rule it is best to leave
to remove debris can be dangerous. If extensions are cut nerves and tendons alone, though if the wound is
needed they should not jeopardize the creation of skin absolutely clean and no dissection is required – and pro-
flaps for wound cover if this should be needed. The vided the necessary expertise is available – they can be
safest extensions are to follow the line of fasciotomy sutured.
incisions; these avoid damaging important perforator
vessels that can be used to raise skin flaps for eventual Wound closure
fracture cover. A small, uncontaminated wound in a Grade I or II
Delivery of the fracture Examination of the fracture sur- fracture may (after debridement) be sutured, provided
faces cannot be adequately performed without extract- this can be done without tension. In the more severe
ing the bone from within the wound. The simplest (and grades of injury, immediate fracture stabilization and
708 gentlest) method is to bend the limb in the manner in wound cover using split-skin grafts, local or distant
23.28 Covering the 23
fracture The best fracture
cover is skin or muscle – with
the help of a plastic surgeon
(a–c). If none is available,
gentamicin beads can be
inserted and sealed with an
impervious dressing until the
second operation, where a
further debridement and,
ideally, definitive fracture
(a) (b) (c) cover is obtained (d,e).
(d) (e)
flaps is ideal, provided both orthopaedic and plastic the soft tissues. The method of fixation depends
surgeons are satisfied that a clean, viable wound has on the degree of contamination, length of time from
been achieved after debridement. In the absence of injury to operation and amount of soft-tissue damage.
this combined approach at the time of debridement, If there is no obvious contamination and definitive
the fracture is stabilized and the wound left open and wound cover can be achieved at the time of debride-
dressed with an impervious dressing. Adding gentam- ment, open fractures of all grades can be treated as for
icin beads under the dressing has been shown to help, a closed injury; internal or external fixation may be
as has the use of vacuum dressings. Return to surgery appropriate depending on the individual characteris-
for a ‘second look’ should have definitive fracture tics of the fracture and wound. This ideal scenario of
cover as an objective. It should be done by 48– judicious soft-tissue and bone debridement, wound
72 hours, and not later than 5 days. Open fractures do cleansing, immediate stabilization and cover is only
not fare well if left exposed for long and multiple possible if orthopaedic and plastic surgeons are pres-
debridement can be self-defeating. ent at the time of initial surgery.
If wound cover is delayed, then external fixation is
safer; however, the surgeon must take care to insert
Stabilizing the fracture
the fixator pins away from potential flaps needed by
Stabilizing the fracture is important in reducing the the plastic surgeon!
likelihood of infection and assisting recovery of The external fixator may be exchanged for internal
23.29 Stabilizing the limb

in open fractures Spanning
external fixation is a useful
method of holding the
fracture in the first instance
(a,b). When definitive fracture
cover is carried out, this can
be substituted with internal
fixation, provided the wound
is clean and the interval
between the two procedures
is less than 7 days.
(a) (b) 709

23
(a) (b) (c) (d)
23.30 Complications of fractures Fractures can become infected (a,b), fail to unite (c) or (d) unite in poor alignment.
fixation at the time of definitive wound cover as long Bone

as (1) the delay to wound cover is less than 7 days; (2)
wound contamination is not visible and (3) internal Infection involves the bone and any implants that may
fixation can control the fracture as well as the external have been used. Early infection may present as wound
fixator. This approach is less risky than introducing inflammation without discharge. Identifying the
internal fixation at the time of initial surgery and leav- causal organism without tissue samples is difficult but,
ing both metalwork and bone exposed until definitive at best guess, it is likely to be S. aureus (including
cover several days later. methicillin-resistant varieties) or Pseudomonas. Sup-
pression by appropriate antibiotics, as long as the fix-
ation remains stable, may allow the fracture to
Aftercare proceed to union, but further surgery is likely later,
In the ward, the limb is elevated and its circulation when the antibiotics are stopped.
carefully watched. Antibiotic cover is continued but Late presentation may be with a sinus and x-ray ev-
only for a maximum of 72 hours in the more severe idence of sequestra. The implants and all avascular
grades of injury. Wound cultures are seldom helpful as pieces of bone should be removed; robust soft tissue
osteomyelitis, if it were to ensue, is often caused by cover (ideally a flap) is needed. An external fixator can
hospital-derived organisms; this emphasizes the need be used to bridge the fracture. If the resulting defect is
for good debridement and early fracture cover. too large for bone grafting at a later stage, the patient
should be referred to a centre with the necessary
experience and facilities for limb reconstruction.
Joints
SEQUELS TO OPEN FRACTURES When an infected fracture communicates with a joint,
the principles of treatment are the same as with bone
infection, namely debridement and drainage, drugs and
Skin
splintage. On resolution of the infection, attention
If split-thickness skin grafts are used inappropriately, can be given to stabilizing the fracture so that joint
particularly where flap cover is more suited, there can movement can recommence. Permanent stiffness is a real
be areas of contracture or friable skin that breaks threat; where fracture stabilization cannot be achieved to
down intermittently. Reparative or reconstructive sur- allow movement, the joint should be splinted in the
710 gery by a plastic surgeon is desirable. optimum position for ankylosis, lest this should occur.
similar open fractures. If the injury is to soft tissues 23
GUNSHOT INJURIES only with minimal bone splinters, the wound may be
safely treated without surgery but with local wound
Missile wounds are looked upon as a special type of care and antibiotics.
open injury. Tissue damage is produced by: (1) direct High-velocity injuries demand thorough cleansing
injury in the immediate path of the missile; (2) con- of the wound and debridement, with excision of deep
tusion of muscles around the missile track and (3) damaged tissues and, if necessary, splitting of fascial
bruising and congestion of soft tissues at a greater dis- compartments to prevent ischaemia; the fracture is
tance from the primary track. The exit wound (if any) stabilized and the wound is treated as for a Gustilo
is usually larger than the entry wound. type III fracture. If there are comminuted fractures,
With high-velocity missiles (bullets, usually from these are best managed by external fixation. The
rifles, travelling at speeds above 600 m/s) there is method of wound closure will depend on the state of
marked cavitation and tissue destruction over a wide tissues after several days; in some cases delayed pri-
area. The splintering of bone resulting from the trans- mary suture is possible but, as with other open
fer of large quantities of energy creates secondary mis- injuries, close collaboration between plastic and
siles, causing greater damage. With low-velocity orthopaedic surgeons is needed (Dicpinigaitis et al.,
missiles (bullets from civilian hand-guns travelling at 2006).
speeds of 300–600 m/s) cavitation is much less, and Close-range shotgun injuries, although the missiles
with smaller weapons tissue damage may be virtually may be technically low velocity, are treated as high-
confined to the bullet track. However, with all gun- velocity wounds because the mass of shot transfers
shot injuries debris is sucked into the wound, which is large quantities of energy to the tissues.
therefore contaminated from the outset.
Emergency treatment
As always, the arrest of bleeding and general resusci- COMPLICATIONS OF
tation take priority. The wounds should each be FRACTURES
covered with a sterile dressing and the area examined
for artery or nerve damage. Antibiotics should be The general complications of fractures (blood loss,
given immediately, following the recommendations shock, fat embolism, cardiorespiratory failure etc.) are
for open fractures (see Table 23.1). dealt with in Chapter 22.
Local complications can be divided into early (those
Definitive treatment that arise during the first few weeks following injury)
and late.
Traditionally, all missile injuries were treated as severe
open injuries, by exploration of the missile track and
formal debridement. However, it has been shown that
low-velocity wounds with relatively clean entry and EARLY COMPLICATIONS
exit wounds can be treated as Gustilo type I injuries,
by superficial debridement, splintage of the limb and Early complications may present as part of the primary
antibiotic cover; the fracture is then treated as for injury or may appear only after a few days or weeks.
23.31 Gunshot
injuries (a) Close-
range shotgun blasts,
although technically
low velocity, transfer
large quantities of
destructive force to
the tissues due to the
mass of shot. They
should be treated like
high-energy open
fractures (b,c).
(a) (b) (c) 711

23 Table 23.2 Local complications of fractures either by the initial injury or subsequently by jagged
bone fragments. Even if its outward appearance is
Urgent Less urgent Late
normal, the intima may be detached and the vessel
Local visceral injury Fracture blisters Delayed union blocked by thrombus, or a segment of artery may be
Vascular injury Plaster sores Malunion in spasm. The effects vary from transient diminution
Nerve injury Pressure sores Non-union
Compartment Nerve entrapment Avascular
of blood flow to profound ischaemia, tissue death and
syndrome Myositis ossificans necrosis peripheral gangrene.

Haemarthrosis Ligament injury Muscle
Infection Tendon lesions contracture
Gas gangrene Joint stiffness Joint instability Clinical features
Algodystrophy Osteoarthritis
The patient may complain of paraesthesia or numb-
ness in the toes or the fingers. The injured limb is cold
and pale, or slightly cyanosed, and the pulse is weak or
VISCERAL INJURY absent. X-rays will probably show one of the ‘high-
Fractures around the trunk are often complicated by risk’ fractures listed above. If a vascular injury is sus-
injuries to underlying viscera, the most important pected an angiogram should be performed
being penetration of the lung with life-threatening immediately; if it is positive, emergency treatment
pneumothorax following rib fractures and rupture of must be started without further delay.
the bladder or urethra in pelvic fractures. These
injuries require emergency treatment. Treatment
All bandages and splints should be removed. The frac-
Table 23.3 Common vascular injuries ture is re-x-rayed and, if the position of the bones sug-
Injury Vessel gests that the artery is being compressed or kinked,
prompt reduction is necessary. The circulation is then re-
First rib fracture Subclavian
Shoulder dislocation Axillary
assessed repeatedly over the next half hour. If there is no
Humeral supracondylar fracture Brachial improvement, the vessels must be explored by operation
Elbow dislocation Brachial – preferably with the benefit of preoperative or perop-
Pelvic fracture Presacral and internal iliac erative angiography. A cut vessel can be sutured, or a seg-
Femoral supracondylar fracture Femoral ment may be replaced by a vein graft; if it is thrombosed,
Knee dislocation Popliteal
Proximal tibial Popliteal or its branches
endarterectomy may restore the blood flow. If vessel re-
pair is undertaken, stable fixation is a must and where it
is practicable, the fracture should be fixed internally.
VASCULAR INJURY
The fractures most often associated with damage to a NERVE INJURY
major artery are those around the knee and elbow,
and those of the humeral and femoral shafts. The Nerve injury is particularly common with fractures of
artery may be cut, torn, compressed or contused, the humerus or injuries around the elbow or the knee
23.32 Vascular injury This patient was

brought into hospital with a fractured femur
and early signs of vascular insufficiency. The
plain x-ray (a) looked as if the proximal bone
fragment might have speared the popliteal
artery. The angiogram (b) confirmed these
fears. Despite vein grafting the patient
developed peripheral gangrene (c).
712 (a) (b) (c)

Table 23.4 Common nerve injuries COMPARTMENT SYNDROME 23
Injury Nerve
Fractures of the arm or leg can give rise to severe
Shoulder dislocation Axillary ischaemia, even if there is no damage to a major ves-
Humeral shaft fracture Radial
sel. Bleeding, oedema or inflammation (infection)
Humeral supracondylar fracture Radial or median
Elbow medial condyle Ulnar may increase the pressure within one of the osseofas-
Monteggia fracture–dislocation Posterior-interosseous cial compartments; there is reduced capillary flow,
Hip dislocation Sciatic which results in muscle ischaemia, further oedema,
Knee dislocation Peroneal still greater pressure and yet more profound ischaemia
– a vicious circle that ends, after 12 hours or less, in
necrosis of nerve and muscle within the compartment.
Nerve is capable of regeneration but muscle, once
infarcted, can never recover and is replaced by inelas-
(see also Chapter 11). The telltale signs should be tic fibrous tissue (Volkmann’s ischaemic contracture).
looked for (and documented) during the initial exam- A similar cascade of events may be caused by swelling
ination and again after reduction of the fracture. of a limb inside a tight plaster cast.
Closed nerve injuries Clinical features

In closed injuries the nerve is seldom severed, and High-risk injuries are fractures of the elbow, forearm
spontaneous recovery should be awaited – it occurs in bones, proximal third of the tibia, and also multiple
90 per cent within 4 months. If recovery has not
occurred by the expected time, and if nerve conduc-
tion studies and EMG fail to show evidence of recov-
ery, the nerve should be explored.
Open nerve injuries

With open fractures the nerve injury is more likely to
be complete. In these cases the nerve should be
explored at the time of debridement and repaired at
the time or at wound closure.
Acute nerve compression

Nerve compression, as distinct from a direct injury,
sometimes occurs with fractures or dislocations
around the wrist. Complaints of numbness or paraes- (a) (b)
thesia in the distribution of the median or ulnar
nerves should be taken seriously and the patient mon-
itored closely; if there is no improvement within 48
hours of fracture reduction or splitting of bandages
around the splint, the nerve should be explored and
decompressed.
INDICATIONS FOR EARLY EXPLORATION

Nerve injury associated with open fracture
(c)
Nerve injury with fractures that need internal
fixation 23.33 Compartment syndrome (a) A fracture at this
level is always dangerous. This man was treated in plaster.
Presence of a concomitant vascular injury Pain became intense and when the plaster was split (which
should have been done immediately after its application),
Nerve damage diagnosed after manipulation of the the leg was swollen and blistered (b). Tibial compartment
fracture decompression (c) requires fasciotomies of all the
compartments in the leg. 713
23 fractures of the hand or foot, crush injuries and cir- (Ulmer, 2002). If the clinical signs are ‘soft’, the limb
cumferential burns. Other precipitating factors are should be examined at 30-minute intervals and if
operation (usually for internal fixation) or infection. there is no improvement within 2 hours of splitting
The classic features of ischaemia are the five Ps: the dressings, fasciotomy should be performed. Mus-
cle will be dead after 4–6 hours of total ischaemia –
• Pain
there is no time to lose!
• Paraesthesia
• Pallor
• Paralysis
• Pulselessness. HAEMARTHROSIS
However in compartment syndrome the ischaemia Fractures involving a joint may cause acute
occurs at the capillary level, so pulses may still be felt haemarthrosis. The joint is swollen and tense and the
and the skin may not be pale! The earliest of the ‘clas- patient resists any attempt at moving it. The blood
sic’ features are pain (or a ‘bursting’ sensation), should be aspirated before dealing with the fracture.
altered sensibility and paresis (or, more usually, weak-
ness in active muscle contraction). Skin sensation
should be carefully and repeatedly checked. INFECTION
Ischaemic muscle is highly sensitive to stretch. If
the limb is unduly painful, swollen or tense, the muscles Open fractures may become infected; closed fractures
(which may be tender) should be tested by stretching hardly ever do unless they are opened by operation.
them. When the toes or fingers are passively hyperex- Post-traumatic wound infection is now the most
tended, there is increased pain in the calf or forearm. common cause of chronic osteitis. The management
Confirmation of the diagnosis can be made by meas- of early and late infection is summarized under the
uring the intracompartmental pressures. So important section Sequels to open fractures (page 710).
is the need for early diagnosis that some surgeons ad-
vocate the use of continuous compartment pressure
monitoring for high-risk injuries (e.g. fractures of the GAS GANGRENE
tibia and fibula) and especially for forearm or leg frac-
tures in patients who are unconscious. A split catheter This terrifying condition is produced by clostridial
is introduced into the compartment and the pressure is infection (especially Clostridium welchii). These are
measured close to the level of the fracture. A differen- anaerobic organisms that can survive and multiply
tial pressure (ΔP) – the difference between diastolic only in tissues with low oxygen tension; the prime site
pressure and compartment pressure – of less than for infection, therefore, is a dirty wound with dead
30 mmHg (4.00 kilopascals) is an indication for im- muscle that has been closed without adequate
mediate compartment decompression. debridement. Toxins produced by the organisms
destroy the cell wall and rapidly lead to tissue necro-
sis, thus promoting the spread of the disease.
Treatment Clinical features appear within 24 hours of the
The threatened compartment (or compartments) injury: the patient complains of intense pain and
must be promptly decompressed. Casts, bandages and swelling around the wound and a brownish discharge
dressings must be completely removed – merely split- may be seen; gas formation is usually not very marked.
ting the plaster is utterly useless – and the limb should There is little or no pyrexia but the pulse rate is
be nursed flat (elevating the limb causes a further increased and a characteristic smell becomes evident
decrease in end capillary pressure and aggravates the (once experienced this is never forgotten). Rapidly the
muscle ischaemia). The ΔP should be carefully moni- patient becomes toxaemic and may lapse into coma
tored; if it falls below 30 mmHg, immediate open fas- and death.
ciotomy is performed. In the case of the leg, It is essential to distinguish gas gangrene, which is
‘fasciotomy’ means opening all four compartments characterized by myonecrosis, from anaerobic celluli-
through medial and lateral incisions. The wounds tis, in which superficial gas formation is abundant but
should be left open and inspected 2 days later: if there toxaemia usually slight. Failure to recognize the dif-
is muscle necrosis, debridement can be carried out; if ference may lead to unnecessary amputation for the
the tissues are healthy, the wounds can be sutured non-lethal cellulitis.
(without tension) or skin-grafted.
NOTE: If facilities for measuring compartmental
pressures are not available, the decision to operate will
Prevention
have to be made on clinical grounds. If three or more Deep, penetrating wounds in muscular tissue are dan-
714 signs are present, the diagnosis is almost certain gerous; they should be explored, all dead tissue
23
(b) (c)
23.34 Infection after fracture treatment Operative fixation is one of the commonest causes
of infection in closed fractures. Fatigue failure of implants is inevitable if infection hinders union
(a). Deep infection can lead to development of discharging sinuses (b,c).
(a)
should be completely excised and, if there is the FRACTURE BLISTERS

slightest doubt about tissue viability, the wound
should be left open. Unhappily there is no effective Two distinct blister types are sometimes seen after
antitoxin against C. welchii. fractures: clear fluid-filled vesicles and blood-stained
ones. Both occur during limb swelling and are due to
elevation of the epidermal layer of skin from the der-
Treatment
mis (Giordano et al., 1994). There is no advantage to
The key to life-saving treatment is early diagnosis. puncturing the blisters (it may even lead to increased
General measures, such as fluid replacement and intra- local infection) and surgical incisions through blisters,
venous antibiotics, are started immediately. Hyper- whilst generally safe, should be undertaken only when
baric oxygen has been used as a means of limiting the limb swelling has decreased.
spread of gangrene. However, the mainstay of treat-
ment is prompt decompression of the wound and
removal of all dead tissue. In advanced cases, amputa- PLASTER AND PRESSURE SORES
tion may be essential.
Plaster sores occur where skin presses directly onto
bone. They should be prevented by padding the bony
points and by moulding the wet plaster so that
pressure is distributed to the soft tissues around the
bony points. While a plaster sore is developing the
patient feels localized burning pain. A window must
(a) (b)
(a) (b)
23.36 Pressure sores Pressure sores are a sign of
23.35 Gas gangrene (a) Clinical picture of gas gangrene. carelessness. (a,b) Sores from poorly supervised treatment
(b) X-rays show diffuse gas in the muscles of the calf. in a Thomas splint. 715
23 immediately be cut in the plaster, or warning pain Infection Both biology and stability are hampered by
quickly abates and skin necrosis proceeds unnoticed. active infection: not only is there bone lysis, necrosis
Even traction on a Thomas splint requires skill in and pus formation, but implants which are used to
nursing care; careless selection of ring size, excessive hold the fracture tend to loosen.
fixed (as opposed to balanced) traction, and neglect can
lead to pressure sores around the groin and iliac crest. PATIENT RELATED
In a less than ideal world, there are patients who are:

• Immense
• Immoderate
LATE COMPLICATIONS • Immovable
• Impossible.
DELAYED UNION
These factors must be accommodated in an appro-
The timetable on page 692 is no more than a rough priate fashion.
guide to the period in which a fracture may be
expected to unite and consolidate. It must never be
relied upon in deciding when treatment may be dis- Clinical features
continued. If the time is unduly prolonged, the term Fracture tenderness persists and, if the bone is sub-
‘delayed union’ is used. jected to stress, pain may be acute.
On x-ray, the fracture line remains visible and there
Causes is very little or incomplete callus formation or
periosteal reaction. However, the bone ends are not
Factors causing delayed union can be summarized as:
sclerosed or atrophic. The appearances suggest that,
biological, biomechanical or patient-related.
although the fracture has not united, it eventually will.
BIOLOGICAL
Inadequate blood supply A badly displaced fracture of a Treatment
long bone will cause tearing of both the periosteum
and interruption of the intramedullary blood supply. CONSERVATIVE
The fracture edges will become necrotic and The two important principles are: (1) to eliminate any
dependent on the formation of an ensheathing callus possible cause of delayed union and (2) to promote
mass to bridge the break. If the zone of necrosis is healing by providing the most appropriate environ-
extensive, as might occur in highly comminuted ment. Immobilization (whether by cast or by internal
fractures, union may be hampered. fixation) should be sufficient to prevent shear at the
fracture site, but fracture loading is an important stim-
Severe soft tissue damage Severe damage to the soft ulus to union and can be enhanced by: (1) encourag-
tissues affects fracture healing by: (1) reducing the ing muscular exercise and (2) by weightbearing in the
effectiveness of muscle splintage; (2) damaging the cast or brace. The watchword is patience; however,
local blood supply and (3) diminishing or eliminating there comes a point with every fracture where the ill-
the osteogenic input from mesenchymal stem cells effects of prolonged immobilization outweigh the
within muscle. advantages of non-operative treatment, or where the
risk of implant breakage begins to loom.
Periosteal stripping Over-enthusiastic stripping of
periosteum during internal fixation is an avoidable
OPERATIVE
cause of delayed union.
Each case should be treated on its merits; however, if
union is delayed for more than 6 months and there is
BIOMECHANICAL
no sign of callus formation, internal fixation and bone
Imperfect splintage Excessive traction (creating a
grafting are indicated. The operation should be
fracture gap) or excessive movement at the fracture site
planned in such a way as to cause the least possible
will delay ossification in the callus. In the forearm and
damage to the soft tissues.
leg a single-bone fracture may be held apart by an
intact fellow bone.
Over-rigid fixation Contrary to popular belief, rigid fix- NON-UNION
ation delays rather than promotes fracture union. It is
only because the fixation device holds the fragments so In a minority of cases delayed union gradually turns
securely that the fracture seems to be ‘uniting’. Union into non-union – that is it becomes apparent that the
by primary bone healing is slow, but provided stability fracture will never unite without intervention. Move-
716 is maintained throughout, it does eventually occur. ment can be elicited at the fracture site and pain
23.37 Non-union 23
(a) This patient has an
obvious pseudarthrosis
of the humerus. The
x-ray (b) shows a typical
hypertrophic non-union.
(c,d) Examples of
atrophic non-union.
(a) (b) (c) (d)
diminishes; the fracture gap becomes a type of 2. Alignment – Was the fracture adequately aligned,
pseudoarthrosis. to reduce shear?
X-ray The fracture is clearly visible but the bone on 3. Stability – Was the fracture held with sufficient
either side of it may show either exuberant callus or stability?
atrophy. This contrasting appearance has led to non- 4. Stimulation – Was the fracture sufficiently ‘stimu-
union being divided into hypertrophic and atrophic lated’? (e.g. by encouraging weightbearing).
types. In hypertrophic non-union the bone ends are
There are, of course, also biological and patient-
enlarged, suggesting that osteogenesis is still active
related reasons that may lead to non-union: (1) poor
but not quite capable of bridging the gap. In atrophic
soft tissues (from either the injury or surgery); (2)
non-union, osteogenesis seems to have ceased. The
local infection; (3) associated drug abuse, anti-inflam-
bone ends are tapered or rounded with no suggestion
matory or cytotoxic immunosuppressant medication
of new bone formation.
and (4) non-compliance on the part of the patient.
Causes
When dealing with the problem of non-union, four Treatment
questions must be addressed. They have given rise to
CONSERVATIVE
the acronym CASS:
Non-union is occasionally symptomless, needing no
1. Contact – Was there sufficient contact between treatment or, at most, a removable splint. Even if
the fragments? symptoms are present, operation is not the only
23.38 Non-union –
treatment (a) This
patient with fractures of
the tibia and fibula was
initially treated by internal
fixation with a plate and
screws. The fracture failed
to heal, and developed the
typical features of
hypertrophic non-union.
(b) After a further
operation, using more
rigid fixation (and no bone
grafts), the fractures
healed solidly. (c,d) This
patient with atrophic non-
union needed both
internal fixation and bone
grafts to stimulate bone
formation and union (e).
(a) (b) (c) (d) (e) 717
23 23.39 Non-union –
treatment by the
Ilizarov technique
Hypertrophic non-unions
can be treated by gradual
distraction and
realignment in an external
fixator (a–d). Atrophic

non-unions will need
more surgery; the poor
tissue is excised (e,f) and
replaced through bone
transport (g,h).
(a) (b) (c) (d)
(e) (f) (g) (h)
answer; with hypertrophic non-union, functional fracture is said to be malunited. Causes are failure to
bracing may be sufficient to induce union, but splin- reduce a fracture adequately, failure to hold reduction
tage often needs to be prolonged. Pulsed electromag- while healing proceeds, or gradual collapse of com-
netic fields and low-frequency, pulsed ultrasound can minuted or osteoporotic bone.
also be used to stimulate union.
Clinical features
OPERATIVE
With hypertrophic non-union and in the absence of The deformity is usually obvious, but sometimes the
deformity, very rigid fixation alone (internal or exter- true extent of malunion is apparent only on x-ray.
nal) may lead to union. With atrophic non-union, fix- Rotational deformity of the femur, tibia, humerus or
ation alone is not enough. Fibrous tissue in the forearm may be missed unless the limb is compared
fracture gap, as well as the hard, sclerotic bone ends is with its opposite fellow. Rotational deformity of a
excised and bone grafts are packed around the frac- metacarpal fracture is detected by asking the patient
ture. If there is significant ‘die-back’, this will require to flatten the fingers onto the palm and seeing
more extensive excision and the gap is then dealt with whether the normal regular fan-shaped appearance is
by bone advancement using the Ilizarov technique. reproduced (Chapter 26).
X-rays are essential to check the position of the frac-
ture while it is uniting. This is particularly important
during the first 3 weeks, when the situation may
MALUNION change without warning. At this stage it is sometimes
difficult to decide what constitutes ‘malunion’;
When the fragments join in an unsatisfactory position acceptable norms differ from one site to another and
718 (unacceptable angulation, rotation or shortening) the these are discussed under the individual fractures.
23
(a) (b) (c) (d) (e)
(f) (g) (h) (i)
23.40 Malunion – treatment by internal fixation An osteotomy, correction of deformity and internal fixation can be
used to treat both intra-articular deformities (a–e) and those in the shaft of a long bone (f–i).
Treatment 2. In children, angular deformities near the bone

ends (and especially if the deformity is in the same
Incipient malunion may call for treatment even before plane as that of movement of the nearby joint) will
the fracture has fully united; the decision on the need usually remodel with time; rotational deformities
for re-manipulation or correction may be extremely will not.
difficult. A few guidelines are offered: 3. In the lower limb, shortening of more than 2.0 cm
1. In adults, fractures should be reduced as near to the is seldom acceptable to the patient and a limb
anatomical position as possible. Angulation of more length equalizing procedure may be indicated.
than 10–15 degrees in a long bone or a noticeable 4. The patient’s expectations (often prompted by
rotational deformity may need correction by re- cosmesis) may be quite different from the
manipulation, or by osteotomy and fixation. surgeon’s; they are not to be ignored.
(a) (b) (c)
23.41 Avascular necrosis (a) Displaced fractures of the femoral neck are at considerable risk of
developing avascular necrosis. Despite internal fixation within a few hours of the injury (b), the
head-fragment developed avascular necrosis. (c) X-ray after removal of the fixation screws. 719
23 5. Early discussion with the patient, and a guided GROWTH DISTURBANCE
view of the x-rays, will help in deciding the need
for treatment and may prevent later In children, damage to the physis may lead to abnor-
misunderstanding. mal or arrested growth. A transverse fracture through
6. Very little is known of the long-term effects of the growth plate is not always disastrous; the fracture
small angular deformities on joint function. runs through the hypertrophic and calcified layers and
However, it seems likely that malalignment of not through the germinal zone, so provided it is accu-
more than 15 degrees in any plane may cause rately reduced, there may not be any disturbance of
asymmetrical loading of the joint above or below growth. However fractures that split the epiphysis
and the late development of secondary inevitably traverse the growing portion of the physis,
osteoarthritis; this applies particularly to the large and so further growth may be asymmetrical and the
weightbearing joints. bone end characteristically angulated; if the entire
physis is damaged, there may be slowing or complete
cessation of growth. The subject is dealt with in more
detail on page 727.
AVASCULAR NECROSIS
Certain regions are notorious for their propensity to BED SORES
develop ischaemia and bone necrosis after injury (see
also Chapter 6). They are: (1) the head of the femur Bed sores occur in elderly or paralysed patients. The
(after fracture of the femoral neck or dislocation of skin over the sacrum and heels is especially vulnerable.
the hip); (2) the proximal part of the scaphoid (after Careful nursing and early activity can usually prevent
fracture through its waist); (3) the lunate (following bed sores; once they have developed, treatment is dif-
dislocation) and (4) the body of the talus (after frac- ficult – it may be necessary to excise the necrotic tis-
ture of its neck). sue and apply skin grafts. In recent years
Accurately speaking, this is an early complication of vacuum-assisted closure (a form of negative pressure
bone injury, because ischaemia occurs during the first dressing) has been used for sacral bed sores.
few hours following fracture or dislocation. However,
the clinical and radiological effects are not seen until
weeks or even months later. MYOSITIS OSSIFICANS
Heterotopic ossification in the muscles sometimes
occurs after an injury, particularly dislocation of the
Clinical features
elbow or a blow to the brachialis, deltoid or quadri-
There are no symptoms associated with avascular ceps. It is thought to be due to muscle damage, but it
necrosis, but if the fracture fails to unite or if the bone also occurs without a local injury in unconscious or
collapses the patient may complain of pain. X-ray paraplegic patients.
shows the characteristic increase in x-ray density,
which occurs as a consequence of two factors: disuse
osteoporosis in the surrounding parts gives the
Clinical features
impression of ‘increased density’ in the necrotic seg- Soon after the injury, the patient (usually a fit young
ment, and collapse of trabeculae compacts the bone man) complains of pain; there is local swelling and
and increases its density. Where normal bone meets
the necrotic segment a zone of increased radiographic
density may be produced by new bone formation.
Treatment
Treatment usually becomes necessary when joint
function is threatened. In old people with necrosis of
the femoral head an arthroplasty is the obvious
choice; in younger people, realignment osteotomy
(or, in some cases, arthrodesis) may be wiser. Avascu-
lar necrosis in the scaphoid or talus may need no more
than symptomatic treatment, but arthrodesis of the 23.42 Bed sores Bed sores in an elderly patient, which
720 wrist or ankle is sometimes needed. kept her in hospital for months.
leg in full external rotation. Radial palsy may follow 23
the faulty use of crutches. Both conditions are due to
lack of supervision.
Bone or joint deformity may result in local nerve
entrapment with typical features such as numbness or
paraesthesia, loss of power and muscle wasting in the
distribution of the affected nerve. Common sites are:
(1) the ulnar nerve, due to a valgus elbow following a
malunited lateral condyle or supracondylar fracture;
(2) the median nerve, following injuries around the
wrist and (3) the posterior tibial nerve, following frac-
tures around the ankle. Treatment is by early decom-
pression of the nerve; in the case of the ulnar nerve
23.43 Myositis ossificans This followed a fractured head this may require anterior transposition.
of the radius.
soft-tissue tenderness. X-ray is normal but a bone scan

MUSCLE CONTRACTURE
may show increased activity. Over the next 2–3 weeks Following arterial injury or compartment syndrome,
the pain gradually subsides, but joint movement is the patient may develop ischaemic contractures of the
limited; x-ray may show fluffy calcification in the soft affected muscles (Volkmann’s ischaemic contracture).
tissues. By 8 weeks the bony mass is easily palpable Nerves injured by ischaemia sometimes recover, at
and is clearly defined in the x-ray. least partially; thus the patient presents with deformity
and stiffness, but numbness is inconstant. The sites
most commonly affected are the forearm and hand,
Treatment
leg and foot.
The worst treatment is to attack an injured and In a severe case affecting the forearm, there will be
slightly stiff elbow with vigorous muscle-stretching wasting of the forearm and hand, and clawing of the
exercises; this is liable to precipitate or aggravate the fingers. If the wrist is passively flexed, the patient can
condition. The joint should be rested in the position extend the fingers, showing that the deformity is
of function until pain subsides; gentle active move- largely due to contracture of the forearm muscles.
ments are then begun. Detachment of the flexors at their origin and along
Months later, when the condition has stabilized, it the interosseous membrane in the forearm may
may be helpful to excise the bony mass. Indomethacin improve the deformity, but function is no better if
or radiotherapy should be given to help prevent a sensation and active movement are not restored. A
recurrence. pedicle nerve graft, using the proximal segments of
the median and ulnar nerves may restore protective
sensation in the hand, and tendon transfers (wrist
TENDON LESIONS extensors to finger and thumb flexors) will allow
active grasp. In less severe cases, median nerve sensi-
Tendinitis may affect the tibialis posterior tendon fol- bility may be quite good and, with appropriate tendon
lowing medial malleolar fractures. It should be pre- releases and transfers, the patient regains a consider-
vented by accurate reduction, if necessary at surgery. able degree of function.
Rupture of the extensor pollicis longus tendon may Ischaemia of the hand may follow forearm injuries,
occur 6–12 weeks after a fracture of the lower radius. or swelling of the fingers associated with a tight fore-
Direct suture is seldom possible and the resulting dis- arm bandage or plaster. The intrinsic hand muscles
ability is treated by transferring the extensor indicis fibrose and shorten, pulling the fingers into flexion at
proprius tendon to the distal stump of the ruptured the metacarpophalangeal joints, but the interpha-
thumb tendon. Late rupture of the long head of langeal joints remain straight. The thumb is adducted
biceps after a fractured neck of humerus usually across the palm (Bunnell’s ‘intrinsic-plus’ position).
requires no treatment. Ischaemia of the calf muscles may follow injuries or
operations involving the popliteal artery or its divi-
sions. This is more common than is usually supposed.
NERVE COMPRESSION The symptoms, signs and subsequent contracture are
similar to those following ischaemia of the forearm.
Nerve compression may damage the lateral popliteal One of the causes of late claw-toe deformity is an
nerve if an elderly or emaciated patient lies with the undiagnosed compartment syndrome. 721
23
(b) (d)
(a) (c) (e)
23.44 Volkmann’s ischaemia (a) Kinking of the main artery is an important cause, but intimal tears
may also lead to blockage from thrombosis. A delayed diagnosis of compartment syndrome carries the
same sorry fate. (b,c) Volkmann’s contracture of the forearm. The fingers can be straightened only
when the wrist is flexed (the constant length phenomenon). (d) Ischaemic contracture of the small
muscles of the hand. (e) Ischaemic contracture of the calf muscles with clawing of the toes.
JOINT INSTABILITY a haemarthrosis forms and leads to synovial adhesions.

More often the stiffness is due to oedema and fibrosis
Following injury a joint may give way. Causes include of the capsule, ligaments and muscles around the
the following: joint, or adhesions of the soft tissues to each other or
to the underlying bone. All these conditions are made
• Ligamentous laxity – especially at the knee, ankle
worse by prolonged immobilization; moreover, if the
and metacarpophalangeal joint of the thumb.
joint has been held in a position where the ligaments
• Muscle weakness – especially if splintage has been
are at their shortest, no amount of exercise will after-
excessive or prolonged, and exercises have been
wards succeed in stretching these tissues and restoring
inadequate (again the knee and ankle are most
the lost movement completely.
often affected).
In a small percentage of patients with fractures of the
• Bone loss – especially after a gunshot fracture or
forearm or leg, early post-traumatic swelling is accom-
severe compound injury, or from crushing of meta-
panied by tenderness and progressive stiffness of the
physeal bone in joint depression fractures.
distal joints. These patients are at great risk of devel-
Injury may also lead to recurrent dislocation. The oping a complex regional pain syndrome; whether this is
commonest sites are: (1) the shoulder – if the glenoid an entirely separate entity or merely an extension of the
labrum has been detached (a Bankart lesion) and (2) ‘normal’ post-traumatic soft-tissue reaction is uncertain.
the patella – if, after traumatic dislocation, the What is important is to recognize this type of ‘stiffness’
restraining patellofemoral ligament heals poorly. when it occurs and to insist on skilled physiotherapy un-
A more subtle form of instability is seen after frac- til normal function is restored.
tures around the wrist. Patients complaining of per-
sistent discomfort or weakness after wrist injury
Treatment
should be fully investigated for chronic carpal instabil-
ity (see Chapters 15 and 25). The best treatment is prevention – by exercises that keep
the joints mobile from the outset. If a joint has to be
splinted, make sure that it is held in the ‘position of
JOINT STIFFNESS safety’ (page 431).
Joints that are already stiff take time to mobilize, but
Joint stiffness after a fracture commonly occurs in the prolonged and patient physiotherapy can work wonders.
knee, elbow, shoulder and (worst of all) small joints of If the situation is due to intra-articular adhesions,
722 the hand. Sometimes the joint itself has been injured; arthroscopic-guided releases may free the joint suffi-
ciently to permit a more pliant response to further ex- The patient complains of continuous pain, often 23
ercise. Occasionally, adherent or contracted tissues need described as ‘burning’ in character. At first there is
to be released by operation (e.g. when knee flexion is local swelling, redness and warmth, as well as tender-
prevented by adhesions in and around the quadriceps). ness and moderate stiffness of the nearby joints. As
the weeks go by the skin becomes pale and atrophic,
movements are increasingly restricted and the patient
may develop fixed deformities. X-rays characteristi-
COMPLEX REGIONAL PAIN SYNDROME cally show patchy rarefaction of the bone.
(ALGODYSTROPHY) The earlier the condition is recognized and treat-
ment begun, the better the prognosis. Elevation and
Sudeck, in 1900, described a condition characterized active exercises are important after all injuries, but in
by painful osteoporosis of the hand. The same condi- CRPS they are essential. In the early stage of the con-
tion sometimes occurs after fractures of the extremi- dition anti-inflammatory drugs and adequate analge-
ties and for many years it was called Sudeck’s atrophy. sia are helpful. Involvement of a pain specialist who
It is now recognized that this advanced atrophic dis- has familiarity with desensitization methods, regional
order is the late stage of a post-traumatic reflex sym- anaesthesia, and use of drugs like amitriptyline, carba-
pathetic dystrophy (also known as algodystrophy), which mazepine and gabapentin may help; this, combined
is much more common than originally believed with prolonged and dedicated physiotherapy, is the
(Atkins, 2003) and that it may follow relatively trivial mainstay of treatment.
injury. Because of continuing uncertainty about its
nature, the term complex regional pain syndrome
(CRPS) has been introduced (see page 261). OSTEOARTHRITIS
Two types of CRPS are recognized:
A fracture involving a joint may severely damage the
• Type 1 –a reflex sympathetic dystrophy that devel- articular cartilage and give rise to post-traumatic
ops after an injurious or noxious event. osteoarthritis within a period of months. Even if the
• Type 2 – causalgia that develops after a nerve injury. cartilage heals, irregularity of the joint surface may
23.45 Complex regional pain

syndrome (a) Regional osteoporosis is
common after fractures of the extremities.
The radiolucent bands seen here are
typical. (b) In algodystrophy the picture is
exaggerated and the soft tissues are also
involved: here the right foot is somewhat
swollen and the skin has become dusky,
smooth and shiny. (c) In the full-blown
case, x-rays show a typical patchy
osteoporosis. (d) Similar changes may
occur in the wrist and hand; they are
always accompanied by (e) increased
activity in the radionuclide scan.
(a) (b) (c)
(d) (e) 723

23 cause localized stress and so predispose to secondary children, middle third in athletes and trainee para-
osteoarthritis years later. If the step-off in the articu- troopers, distal third in the elderly); distal shaft of the
lar surface involves a large fragment in a joint that is fibula (the ‘runner’s fracture’); calcaneum (adults);
readily accessible to surgery, intra-articular navicular (athletes) and metatarsals (especially the sec-
osteotomies and re-positioning of the fragment may ond).
help. Often though the problem arises from areas that
were previously comminuted and depressed – little

Clinical features
can be done once the fracture has united.
Malunion of a metaphyseal fracture may radically There may be a history of unaccustomed and repeti-
alter the mechanics of a nearby joint and this, too, can tive activity or one of a strenuous physical exercise
give rise to secondary osteoarthritis. It is often programme. A common sequence of events is: pain
asserted that malunion in the shaft of a long bone after exercise – pain during exercise – pain without
(e.g. the tibia) may act in a similar manner; however, exercise. Occasionally the patient presents only after
there is little evidence to show that residual angula- the fracture has healed and may then complain of a
tion of less than 15 degrees can cause proximal or dis- lump (the callus).
tal osteoarthritis. The patient is usually healthy. The affected site may
be swollen or red. It is sometimes warm and usually
tender; the callus may be palpable. ‘Springing’ the
bone (attempting to bend it) is often painful.
STRESS FRACTURES
Imaging
A stress or fatigue fracture is one occurring in the nor-
mal bone of a healthy patient, due not to any specific X-RAY
traumatic incident but to small repetitive stresses of Early on, the fracture is difficult to detect, but
two main types: bending and compression. radioscintigraphy will show increased activity at the
Bending stress causes deformation and bone painful spot. Plain x-rays taken a few weeks later may
responds by changing the pattern of remodelling. show a small transverse defect in the cortex and/or
With repeated stress, osteoclastic resorption exceeds localized periosteal new-bone formation. These
osteoblastic formation and a zone of relative weakness appearances have, at times, been mistaken for those of
develops – ultimately leading to a breach in the cor- an osteosarcoma, a horrifying trap for the unwary.
tex. This process affects young adults undertaking Compression stress fractures (especially of the femoral
strenuous physical routines and is probably due to neck and upper tibia) may show as a hazy transverse
muscular forces acting on bone. Athletes in training, band of sclerosis with (in the tibia) peripheral callus.
dancers and military recruits build up muscle power Another typical picture is that of a small osteoartic-
quickly but bone strength only slowly; this accounts ular fracture – most commonly of the dome of the
for the high incidence of stress fractures in these medial femoral condyle at the knee or the upper sur-
groups. face of the talus at the ankle. Later, ischaemic necro-
Compressive stresses act on soft cancellous bone; with sis of the detached fragment may render the lesion
frequent repetition an impacted fracture may result. even more obvious.
A combination of compression and shearing stresses
may account for the osteochondral fracures that char-
acterize some of the so-called osteochondritides.
‘Spontaneous fractures’ occur with even greater ease
in people with osteoporosis or osteomalacia and in
patients treated with drugs that affect bone remodel-
ling in a similar way (e.g. corticosteroids and
methotrexate). These are often referred to as insuffi-
ciency fractures.
Sites affected
Least rare are the following: shaft of humerus (ado-
(a) (b)
lescent cricketers); pars interarticularis of fifth lumbar
vertebra (causing spondylolysis); pubic rami (inferior 23.46 Stress fracture (a) The stress fracture in this tibia
is only just visible on x-ray, but it had already been
in children, both rami in adults); femoral neck (at any suspected 2 weeks earlier when the patient first com-
age); femoral shaft (chiefly lower third); patella (chil- plained of pain and a radioisotope scan revealed a ‘hot’
724 dren and young adults); tibial shaft (proximal third in area just above the ankle (b).
23.47 Stress fractures Stress 23
fractures are often missed or
wrongly diagnosed. (a) This tibial
fracture was at first thought to be
an osteosarcoma. (b) Stress
fractures of the pubic rami in
elderly women can be mistaken
for metastases.
(a) (b)
MRI Table 23.5 Causes of pathological fracture

The earliest changes, particularly in ‘spontaneous’
Generalized bone disease Primary malignant tumours
undisplaced osteoarticular fractures, are revealed by
MRI. This investigation should be requested in older 1. Osteogenesis imperfecta 1. Chondrosarcoma
patients (possibly with osteoporosis) complaining of 2. Postmenopausal 2. Osteosarcoma
osteoporosis 3. Ewing’s tumour
sudden onset of pain over the anteromedial part of the 3. Metabolic bone disease
knee. 4. Myelomatosis
5. Polyostotic fibrous dysplasia
6. Paget’s disease
Diagnosis
Local benign conditions Metastatic tumours
Many disorders, including osteomyelitis, scurvy and 1. Chronic infection Carcinoma in breast, lung,
battered baby syndrome, may be confused with stress 2. Solitary bone cyst kidney, thyroid, colon
fractures. The great danger, however, is a mistaken 3. Fibrous cortical defect and prostate
diagnosis of osteosarcoma; scanning shows increased 4. Chondromyxoid fibroma
uptake in both conditions and even biopsy may be 5. Aneurysmal bone cyst
6. Chondroma
misleading. 7. Monostotic fibrous dysplasia
Treatment
Most stress fractures need no treatment other than an
elastic bandage and avoidance of the painful activity
until the lesion heals; surprisingly, this can take many HISTORY
months and the forced inactivity is not easily accepted
by the hard-driving athlete or dancer. Bone that fractures spontaneously, or after trivial
An important exception is stress fracture of the injury, must be regarded as abnormal until proved
femoral neck. This should be suspected in all elderly otherwise. Older patients should always be asked
people who complain of pain in the hip for which no about previous illnesses or operations. A malignant
obvious cause can be found. If the diagnosis is con- tumour, no matter how long ago it occurred, may be
firmed by bone scan, the femoral neck should be the source of a late metastatic lesion; a history of gas-
internally fixed with screws as a prophylactic measure. trectomy, intestinal malabsorption, chronic alco-
holism or prolonged drug therapy should suggest a
metabolic bone disorder.
Symptoms such as loss of weight, pain, a lump,
PATHOLOGICAL FRACTURES cough or haematuria suggest that the fracture may be
through a secondary deposit.
When abnormal bone gives way this is referred to as a In younger patients, a history of several previous
pathological fracture. The causes are numerous and fractures may suggest a diagnosis of osteogenesis
varied; often the diagnosis is not made until a biopsy imperfecta, even if the patient does not show the clas-
is examined (Table 23.5). sic features of the disorder. 725
23
(a) (b) (c) (d) (e) (f)

23.48 Pathological fractures Six examples of pathological fractures, due to: (a) primary chondrosarcoma;
(b) postoperative bone infection at a screw-hole following plating of an intertrochanteric fracture; (c) Paget’s disease;
(d) vertebral metastases; (e) metastasis from carcinoma of the breast and (f) myelomatosis.
EXAMINATION tebral compression in a male younger than 75 years

should be regarded as ‘pathological’ until proven
Local signs of bone disease (an infected sinus, an old otherwise.
scar, swelling or deformity) should not be missed. The
site of the fracture may suggest the diagnosis: patients
with involutional osteoporosis develop fractures of the Additional investigations
vertebral bodies and corticocancellous junctions of Local radionuclide imaging may help elucidate the
long bones; a fracture through the shaft of the bone diagnosis, and whole-body scanning is important in
in an elderly patient, especially in the subtrochanteric revealing or excluding other deposits.
region, should be regarded as a pathological fracture X-ray of other bones, the lungs and the urogenital
until proved otherwise. tract may be necessary to exclude malignant disease.
General examination may be informative. Congen- Investigations should always include a full blood
ital dysplasias, fibrous dysplasia, Cushing’ syndrome count, ESR, protein electrophoresis, and tests for
and Paget’ disease all produce characteristic appear- syphilis and metabolic bone disorders.
ances. The patient may be wasted (possibly due to Urine examination may reveal blood from a
malignant disease). The lymph nodes or liver may be tumour, or Bence–Jones protein in myelomatosis.
enlarged. It should be noted whether there is a mass
in the abdomen or pelvis. Old scars should not be
overlooked and rectal and vaginal examinations are Biopsy
mandatory. Some lesions are so typical that a biopsy is unnecessary
Under the age of 20 the common causes of patho- (solitary cyst, fibrous cortical defect, Paget’s disease).
logical fracture are benign bone tumours and cysts. Others are more obscure and a biopsy is essential for
Over the age of 40 the common causes are multiple diagnosis. If open reduction of the fracture is indicated,
myeloma, secondary carcinoma and Paget’s disease. the biopsy can be carried out at the same time; other-
wise a definitive procedure should be arranged.
X-ray
Understandably, the fracture itself attracts most atten- Treatment
tion but the surrounding bone must also be exam-
The principles of fracture treatment remain the same:
ined, and features such as cyst formation, cortical
reduce, hold, exercise. However the choice of method
erosion, abnormal trabeculation and periosteal thick-
is influenced by the condition of the bone; and the
ening should be sought. The type of fracture, too, is
underlying pathological disorder may need treatment
important: vertebral compression fractures may be
in its own right (see Chapter 9).
due to severe osteoporosis or osteomalacia, but they
can also be caused by skeletal metastases or myeloma. Generalized bone disease In most of these conditions
Middle-aged men, unlike women, do not normally (including Paget’s disease) the bones fracture more
726 become osteoporotic: x-ray signs of bone loss and ver- easily, but they heal quite well provided the fracture is
23
(a) (b) (c) (d)
23.49 Pathological fractures – treatment (a,b) Paget’s disease of the femur increases the brittleness of bone, making
it more likely to fracture. Intramedullary fixation allows the entire femur to be supported. (c,d) A fracture through a solitary
metastasis from a previously excised renal cell carcinoma can be resected in order to achieve cure. In this case replacement
of the proximal femur with an endoprosthesis is needed.
properly immobilized. Internal fixation is therefore Preoperatively, imaging studies should be per-
advisable (and for Paget’s disease almost essential). formed to detect other bone lesions; these may be
Patients with osteomalacia, hyperparathyroidism, renal amenable to prophylactic fixation. Once the wound
osteodystrophy and Paget’s disease will need systemic has healed, local irradiation should be applied to
treatment as well. reduce the risk of progressive osteolysis.
Pathological compression fractures of the spine cause
Local benign conditions Fractures through benign cyst-
severe pain. This is due largely to spinal instability and
like lesions usually heal quite well and they should be
treatment should include operative stabilization. If
allowed to do so before tackling the local lesion.
there are either clinical or imaging features of actual
Treatment is therefore the same as for simple fractures
or threatened spinal cord or cauda equina compres-
in the same area, although in some cases it will be
sion, the segment should also be decompressed. Post-
necessary to take a biopsy before immobilizing the
operative irradiation is given as usual.
fracture. When the bone has healed, the tumour can
With all types of metastatic lesion, the primary
be dealt with by curettage or local excision.
tumour should be investigated and treated as well.
Primary malignant tumour The fracture may need
splinting but this is merely a prelude to definitive
treatment of the tumour, which by now will have
spread to the surrounding soft tissues. The prognosis INJURIES OF THE PHYSIS
is almost always very poor.
Metastatic tumours Metastasis is a frequent cause of In children over 10 per cent of fractures involve
pathological fracture in older people. Breast cancer is injury to the growth plate (or physis). Because the
the commonest source and the femur the commonest physis is a relatively weak part of the bone, joint
site. Nowadays cancer patients (even those with metas- strains that might cause ligament injuries in adults
tases) often live for several years and effective treatment are liable to result in separation of the physis in chil-
of the fracture will vastly improve their quality of life. dren. The fracture usually runs transversely through
Fracture of a long-bone shaft should be treated by the hypertrophic or the calcified layer of the growth
internal fixation; if necessary the site is also packed plate, often veering off into the metaphysis at one of
with acrylic cement. Bear in mind that the implant the edges to include a triangular lip of bone. This has
will function as a load-bearing and not a load-sharing little effect on longitudinal growth, which takes
device; intramedullary nails are more suitable than place in the germinal and proliferating layers of the
plates and screws. physis. However, if the fracture traverses the cellular
Fracture near a bone end can often be treated by ‘reproductive’ layers of the physis, it may result in
excision and prosthetic replacement; this is especially premature ossification of the injured part and serious
true of femoral neck fractures. disturbances of bone growth. 727
23 23.50 Battered baby
syndrome (a–c) The
fractures are not
pathological but the
family is. The
metaphyseal lesions in
each humerus are
characteristic.
(a) (b) (c)
Classification fractures are liable to displacement and a conse-

quent misfit between the separated parts of the
The most widely used classification of physeal injuries physis, resulting in asymmetrical growth.
is that of Salter and Harris (Salter and Harris, 1963), • Type 5 – A longitudinal compression injury of the ph-
which distinguishes five basic types of injury: ysis. There is no visible fracture but the growth plate
• Type 1 – A transverse fracture through the hyper- is crushed and this may result in growth arrest.
trophic or calcified zone of the plate. Even if the Rang (Rang, 1969) has added a Type 6, an injury to
fracture is quite alarmingly displaced, the growing the perichondrial ring (the peripheral zone of Ran-
zone of the physis is usually not injured and growth vier), which carries a significant risk of growth distur-
disturbance is uncommon. bance. The diagnosis is made usually in retrospect
• Type 2 – This is essentially similar to type 1, but after development of deformity.
towards the edge the fracture deviates away from
the physis and splits off a triangular metaphyseal
fragment of bone (sometimes referred to as the Mechanism of injury
Thurston– Holland fragment).
• Type 3 – A fracture that splits the epiphysis and then Physeal fractures usually result from falls or traction
veers off transversely to one or the other side, injuries. They occur mostly in road accidents and dur-
through the hypertrophic layer of the physis. ing sporting activities or playground tumbles.
Inevitably it damages the ‘reproductive’ layers of
the physis (as these layers are closer to the epiphysis
than the metaphysis) and may result in growth dis-
Clinical features
turbance. These fractures are more common in boys than in
• Type 4 – As with type 3, the fracture splits the epi- girls and are usually seen either in infancy or between
physis, but it extends into the metaphysis. These the ages of 10 and 12. Deformity is usually minimal,
1 2 3 4 5
23.51 Physeal injuries Type 1 – separation of the epiphysis – which usually occurs in infants but is also seen at puberty as
a slipped femoral epiphysis. Type 2 – fracture through the physis and metaphysis – is the commonest; it occurs in older
children and seldom results in abnormal growth. Type 3 – an intra-articular fracture of the epiphysis – needs accurate
reduction to restore the joint surface. Type 4 – splitting of the physis and epiphysis – damages the articular surface and may
also cause abnormal growth; if it is displaced it needs open reduction. Type 5 – crushing of the physis – may look benign
728 but ends in arrested growth.
but any injury in a child followed by pain and tender- 23
ness near the joint should arouse suspicion, and x-ray
examination is essential.
X-rays
The physis itself is radiolucent and the epiphysis may
be incompletely ossified; this makes it hard to tell
whether the bone end is damaged or deformed.
The younger the child, the smaller the ‘visible’ part of (a) (b)
the epiphysis and thus the more difficult it is to make
the diagnosis; comparison with the normal side is a
great help. Telltale features are widening of the phy-
seal ‘gap’, incongruity of the joint or tilting of the epi-
physeal axis. If there is marked displacement the
diagnosis is obvious, but even a type 4 fracture may at
first be so little displaced that the fracture line is hard
to see; if there is the faintest suspicion of a physeal
fracture, a repeat x-ray after 4 or 5 days is essential.
Types 5 and 6 injuries are usually diagnosed only in
retrospect.
(c) (d)
Treatment
Undisplaced fractures may be treated by splinting the
part in a cast or a close-fitting plaster slab for 2–4
weeks (depending on the site of injury and the age of
the child). However, with undisplaced types 3 and 4
fractures, a check x-ray after 4 days and again at about
10 days is mandatory in order not to miss late dis-
placement.
Displaced fractures should be reduced as soon as
possible. With types 1 and 2 this can usually be done (e) (f)
closed; the part is then splinted securely for 3–6 23.52 Physeal injuries (a) Type 2 injury. The fracture
weeks. Types 3 and 4 fractures demand perfect does not traverse the width of the physis; after reduction
anatomical reduction. An attempt can be made to (b) bone growth is not distorted. (c,d) This type 4 fracture
achieve this by gentle manipulation under general of the tibial physis was treated immediately by open
reduction and internal fixation and a good result was
anaesthesia; if this is successful, the limb is held in a obtained. (e,f) In this case accurate reduction was not
cast for 4–8 weeks (the longer periods for type 4 achieved and the physeal fragment remained displaced;
injuries). If a type 3 or 4 fracture cannot be reduced the end result was partial fusion of the physis and severe
accurately by closed manipulation, immediate open deformity of the ankle.
reduction and internal fixation with smooth K-wires is
essential. The limb is then splinted for 4–6 weeks, but
it takes that long again before the child is ready to diagnosis is missed and the fracture remains unre-
resume unrestricted activities. duced (e.g. fracture separation of the medial humeral
epicondyle).
Types 3 and 4 injuries may result in premature
Complications
fusion of part of the growth plate or asymmetrical
Types 1 and 2 injuries, if properly reduced, have an growth of the bone end. Types 5 and 6 fractures cause
excellent prognosis and bone growth is not adversely premature fusion and retardation of growth. The size
affected. Exceptions to this rule are injuries around and position of the bony bridge across the physis can
the knee involving the distal femoral or proximal tib- be assessed by tomography or magnetic resonance
ial physis; both growth plates are undulating in shape, imaging (MRI). If the bridge is relatively small (less
so a transverse fracture plane may actually pass than one-third the width of the physis) it can be
through more than just the hypertrophic zone but excised and replaced by a fat graft, with some prospect
also damage the proliferative zone. Complications of preventing or diminishing the growth disturbance
such as malunion or non-union may also occur if the (Langenskiold, 1975; 1981). However, if the bone 729
23
(a) (b) (c) (d) (e)
23.53 Langenskiold procedure for physeal arrest Small tethers across the physis can be mapped out by MRI (a,b),
then surgically removed by drilling out and curettage (c) and filling the defect with fat graft (d,e).
bridge is more extensive the operation is contraindi- ligament may be strained to the point of complete
cated as it can end up doing more harm than good. rupture.
Established deformity, whether from asymmetrical
growth or from malunion of a displaced fracture (e.g.
a valgus elbow due to proximal displacement of a lat- STRAINED LIGAMENT
eral humeral condylar fracture) should be treated by
corrective osteotomy. If further growth is abnormal, Only some of the fibres in the ligament are torn and
the osteotomy may have to be repeated. the joint remains stable. The injury is one in which
the joint is momentarily twisted or bent into an
abnormal position. The joint is painful and swollen
and the tissues may be bruised. Tenderness is localized
INJURIES TO JOINTS to the injured ligament and tensing the tissues on that
side causes a sharp increase in pain.
Joints are usually injured by twisting or tilting forces
that stretch the ligaments and capsule. If the force is
Treatment
great enough the ligaments may tear, or the bone to
which they are attached may be pulled apart. The The joint should be firmly strapped and rested until
articular cartilage, too, may be damaged if the joint the acute pain subsides. Thereafter, active movements
surfaces are compressed or if there is a fracture into are encouraged, and exercises practised to strengthen
the joint. the muscles.
As a general principle, forceful angulation will tear
the ligaments rather than crush the bone, but in older
people with porotic bone the ligaments may hold and RUPTURED LIGAMENT
the bone on the opposite side of the joint is crushed
instead, while in children there may be a fracture- The ligament is completely torn and the joint is unsta-
separation of the physis. ble. Sometimes the ligament holds and the bone to
which it is attached is avulsed; this is effectively the
same lesion but easier to deal with because the bone
Sprains, strains and ruptures fragment can be securely reattached.
There is much confusion about the use of the terms As with a strain, the joint is suddenly forced into an
‘sprain’, ‘strain’ and ‘rupture’. Strictly speaking, a abnormal position; sometimes the patient actually
sprain is any painful wrenching (twisting or pulling) hears a snap. The joints most likely to be affected are
movement of a joint, but the term is generally the ones that are insecure by virtue of their shape or
reserved for joint injuries less severe than actual tear- least well protected by surrounding muscles: the knee,
ing of the capsule or ligaments. Strain is a physical ankle and finger joints.
effect of stress, in this case tensile stress associated Pain is severe and there may be considerable bleed-
with some stretching of the ligaments; in colloquial ing under the skin; if the joint is swollen, this is prob-
usage, ‘strained ligament’ is often meant to denote ably due to a haemarthrosis. The patient is unlikely to
an injury somewhat more severe than a ‘sprain’, permit a searching examination, but under general
which possibly involves tearing of some fibres. If the anaesthesia the instability can be demonstrated; it is
730 stretching or twisting force is severe enough, the this that distinguishes the lesion from a strain. X-ray
23
(a) (b) (c) (d) (e)
23.54 Joint injuries Severe stress may cause various types of injury. (a) A ligament may rupture,
leaving the bone intact. If the soft tissues hold, the bone on the opposite side may be crushed (b), or
a fragment may be pulled off by the taut ligament (c). Subluxation (d) means the articular surfaces
are partially displaced; dislocation (e) refers to complete displacement of the joint.
may show a detached flake of bone where the liga- Clinical features
ment is inserted.
Following an injury the joint is painful and the patient
tries at all costs to avoid moving it. The shape of the
Treatment joint is abnormal and the bony landmarks may be dis-
Torn ligaments heal by fibrous scarring. Previously placed. The limb is often held in a characteristic
this was thought inevitable and the surgeon’s task was position; movement is painful and restricted. X-rays
to ensure that the torn ends were securely sutured so will usually clinch the diagnosis; they will also show
as to restore the ligament to its normal length. In whether there is an associated bony injury affecting
some injuries, e.g. rupture of the ulnar collateral liga- joint stability – i.e. a fracture-dislocation.
ment of the metacarpophalangeal joint of the thumb, Apprehension test If the dislocation is reduced by the
this approach is still valid. In others, however, it has time the patient is seen, the joint can be tested by
changed; thus, solitary medial collateral ligament rup- stressing it as if almost to reproduce the suspected
tures of the knee, even complete ruptures, are often dislocation: the patient develops a sense of impending
treated non-operatively in the first instance. The joint disaster and violently resists further manipulation.
is splinted and local measures are taken to reduce
swelling. After 1–2 weeks, the splint is exchanged for Recurrent dislocation If the ligaments and joint margins
a functional brace that allows joint movement but at are damaged, repeated dislocation may occur. This is
the same time prevents repeat injury to the ligament, seen especially in the shoulder and patellofemoral joint.
especially if some instability is also present. Physio- Some patients acquire
Habitual (voluntary) dislocation
therapy is applied to maintain muscle strength and the knack of dislocating (or subluxating) the joint by
later proprioceptive exercises are added. This non- voluntary muscle contraction. Ligamentous laxity may
operative approach has shown better results not only make this easier, but the habit often betrays a
in the strength of the healed ligament but also in the manipulative and neurotic personality. It is important
nature of healing – there is less fibrosis (Woo et al., to recognize this because such patients are seldom
2000). An exception to this non-operative approach is helped by operation.
when the ligament is avulsed with an attached frag-
ment of bone; reattachment of the fragment is indi-
cated if the piece is large enough. Occasionally Treatment
non-operative treatment may result in some residual The dislocation must be reduced as soon as possible;
instability that is clinically detectable; often this is not usually a general anaesthetic is required, and some-
symptomatic, but if it is then surgical reconstruction times a muscle relaxant as well. The joint is then
should be considered. rested or immobilized until soft-tissue swelling
reduces – usually after 2 weeks. Controlled move-
ments then begin in a functional brace; progress with
physiotherapy is monitored. Occasionally surgical
DISLOCATION AND SUBLUXATION reconstruction for residual instability is called for.
‘Dislocation’ means that the joint surfaces are com-

pletely displaced and are no longer in contact; ‘sub-
Complications
luxation’ implies a lesser degree of displacement, such Many of the complications of fractures are seen also after
that the articular surfaces are still partly apposed. dislocations: vascular injury, nerve injury, avascular 731
23 necrosis of bone, heterotopic ossification, joint stiffness Müller M., Nazarian S, Koch P, Schatzker J. The Compre-
and secondary osteoarthritis. The principles of diagno- hensive Classification of Fractures of Long Bones. Springer
sis and management of these conditions have been dis- Verlag, Berlin, Heidelberg, New York, 1990.
cussed earlier. Oestern H, Tscherne H. Pathophysiology and classification
of soft tissue injuries associated with fractures. In: H.
Tscherne and L. Gotzen (eds) Fractures with Soft Tissue
Injuries. Springer Verlag, Berlin, 1984.

Pape HC, Giannoudis PV, Kretteck C, Trentz O. Timing
of fixation of major fractures in blunt polytrauma: role of
conventional indicators in clinical decision making.
Atkins RM. Complex regional pain syndrome. J Bone Joint J Orthop Trauma 2005; 19: 551–62.
Surg 2003; 85B: 1100–6. Rang M. The growth plate and its disorders. Churchill Liv-
Charnley J. The Closed Treatment of Common Fractures. ingstone, Edinburgh, 1969.
Churchill Livingstone, Edinburgh, 1961. Roberts CS, Pape HC, Jones AL et al. Damage control
Dicpinigaitis PA, Koval KJ, Tejwani NC, Egol KA. Gun- orthopaedics. Evolving concepts in the treatment of
shot wounds to the extremities. Bull NYU Hosp Jt Dis patients who have sustained orthopaedic trauma. J Bone
2006; 64: 139–55. Joint Surg 2005; 87A: 434–49.
Giordano CP, Koval KJ, Zuckerman JD, Desai P. Fracture Salter RB, Harris WR. Injuries involving the epiphyseal
blisters. Clin Orthop 1994; 307: 214–21. plate. J Bone Joint Surg 1963; 45A: 587–622.
Gustilo RB, Mendoza RM, Williams DN. Problems in the Sarmiento A, Latta L. Functional fracture bracing. J Am
management of type III (severe) open fractures: a new Acad Orthop Surg 1999; 7: 66–75.
classification of type III open fractures. J Trauma 1984; Sarmiento A, Latta L. The evolution of functional bracing
24: 742–6. of fractures. J Bone Joint Surg 2006; 88B: 141–8.
Kenwright J, Richardson JB, Cunningham JL et al. Axial Sarmiento A, Mullis DL, Latta L et al. A quantitative com-
movement and tibial fractures. A controlled randomised parative analysis of fracture healing under the influence of
trial of treatment. J Bone Joint Surg 1991; 73B: 654–9. compression plating vs. closed weight-bearing treatment.
Langenskiold A. An operation for partial closure of an epi- Clin Orthop 1980; 149: 232–9.
physial plate in children, and its experimental basis. J Bone Slongo TF, Audige L. Fracture and dislocation classification
Joint Surg 1975; 57B: 325–30. compendium for children: the AO pediatric comprehen-
Langenskiold A. Surgical treatment of partial closure of the sive classification of long bone fractures (PCCF). J Orthop
growth plate. J Pediatr Orthop 1981; 1: 3–11. Trauma 2007; 21(Suppl): S135–60.
Marsh JL, Slongo TF, Agel J et al. Fracture and dislocation Ulmer T. The clinical diagnosis of compartment syndrome
classification compendium – 2007: Orthopaedic Trauma of the lower leg: Are clinical findings predictive of the dis-
Association classification, database and outcomes com- order? J Orthop Trauma 2002; 16: 572–577.
mittee. J Orthop Trauma 2007; 21(Suppl): S1–133. Woo SL, Vogrin TM, Abramowitch SD. Healing and
McKibbin B. The biology of fracture healing in long bone. repair of ligament injuries in the knee. J Am Acad Orthop
J Bone Joint Surg 1978; 60B: 150–62. Surg 2000; 8: 364–72.
732
Injuries of the
shoulder, upper arm 24
and elbow
Andrew Cole, Paul Pavlou, David Warwick
The great bugbear of upper limb injuries is stiffness – pulse and gently to palpate the root of the neck.
particularly of the shoulder but sometimes of the Outer third fractures are easily missed or mistaken for
elbow and hand as well. Two points should be con- acromioclavicular joint injuries.
stantly borne in mind:
• Whatever the injury, and however it is treated, all Imaging
the joints that are not actually immobilized – and Radiographic analysis requires at least an anteroposte-
especially the finger joints – should be exercised rior view and another taken with a 30 degree cephalic
from the start. tilt. The fracture is usually in the middle third of the
• In elderly patients it is sometimes best to disregard the bone, and the outer fragment usually lies below the
fracture and concentrate on regaining movement. inner. Fractures of the outer third may be missed, or
the degree of displacement underestimated, unless
additional views of the shoulder are obtained. With
medial third fractures it is also wise to obtain x-rays of
FRACTURES OF THE CLAVICLE the sterno-clavicular joint. In assessing clinical
progress, remember that ‘clinical’ union usually pre-
In children the clavicle fractures easily, but it almost cedes ‘radiological’ union by several weeks.
invariably unites rapidly and without complications. CT scanning with three-dimensional reconstruc-
In adults this can be a much more troublesome injury. tions may be needed to determine accurately the
In adults clavicle fractures are common, accounting degree of shortening or for diagnosing a sterno-
for 2.6–4 per cent of fractures and approximately 35 per clavicular fracture-dislocation, and also to establish
cent of all shoulder girdle injuries. Fractures of the mid- whether a fracture has united.
shaft account for 69–82 per cent, lateral fractures for
21–28 per cent and medial fractures for 2–3 per cent.
Classification
Mechanism of injury Clavicle fractures are usually classified on the basis of
their location: Group I (middle third fractures),
A fall on the shoulder or the outstretched hand may Group II (lateral third fractures) and Group III
break the clavicle. In the common mid-shaft fracture, (medial third fractures). Lateral third fractures can be
the outer fragment is pulled down by the weight of further sub-classified into (a) those with the coraco-
the arm and the inner half is held up by the sternoclavicular ligaments intact, (b) those where the cora-
mastoid muscle. In fractures of the outer end, if the coclavicular ligaments are torn or detached from the
ligaments are intact there is little displacement; but if medial segment but the trapezoid ligament remains
the coracoclavicular ligaments are torn, or if the frac- intact to the distal segment, and (c) factures which are
ture is just medial to these ligaments, displacement intra-articular. An even more detailed classification
may be severe and closed reduction impossible. proposed by Robinson (1998) is useful for managing
data and comparing clinical outcomes.
Clinical features
Treatment
The arm is clasped to the chest to prevent movement.
A subcutaneous lump may be obvious and occasion- MIDDLE THIRD FRACTURES
ally a sharp fragment threatens the skin. Though vas- There is general agreement that undisplaced fractures
cular complications are rare, it is prudent to feel the should be treated non- operatively. Most will go on to
24 is no evidence that the traditional figure-of-eight
bandage confers any advantage and it carries the risk
of increasing the incidence of pressures sores over the
fracture site and causing harm to neurological struc-
tures; it may even increase the risk of non-union.
There is less agreement about the management of
displaced middle third fractures. Treating those with

shortening of more than 2 cm by simple splintage is
now believed to incur a considerable risk of sympto-
(a) matic mal-union – mainly pain and lack of power dur-
ing shoulder movements (McKee et al., 2006) – and
an increased incidence of non-union. There is, there-
fore, a growing trend towards internal fixation of
acute clavicular fractures associated with severe dis-
placement. Methods include plating (specifically con-
toured locking plates are available) and intramedullary
fixation.
(b) LATERAL THIRD FRACTURES

24.1 Fracture of the clavicle (a) Displaced fracture of Most lateral clavicle fractures are minimally displaced
the middle third of the clavicle – the most common injury. and extra-articular. The fact that the coracoclavicular
(b) The fracture usually unites in this position, leaving a ligaments are intact prevents further displacement and
barely noticable ‘bump’. non-operative management is usually appropriate.
Treatment consists of a sling for 2–3 weeks until the
pain subsides, followed by mobilization within the
limits of pain.
unite uneventfully with a non-union rate below 5 per Displaced lateral third fractures are associated with
cent and a return to normal function. disruption of the coracoclavicular ligaments and are
Non-operative management consists of applying a therefore unstable injuries. A number of studies have
simple sling for comfort. It is discarded once the pain shown that these particular fractures have a higher
subsides (between 1–3 weeks) and the patient is then than usual rate of non-union if treated non-opera-
encouraged to mobilize the limb as pain allows. There tively. Surgery to stabilize the fracture is often recom-
(a)
(c)
(b)
24.2 Severely displaced fracture (a) A comminuted

fracture which united in this position (b) leaving an
unsightly deformity (c). This fracture would have been bet-
734 ter managed by (d) open reduction and internal fixation. (d)
LATE 24
Non-union In displaced fractures of the shaft non-
union occurs in 1–15 per cent. Risk factors include in-
creasing age, displacement, comminution and female
sex. However accurate prediction of those fractures
most likely to go on to non-union remains difficult.
Injuries of the shoulder, upper arm and elbow

Symptomatic non-unions are generally treated with
plate fixation and bone grafting if necessary. This
procedure usually produces a high rate of union and
satisfaction.
Lateral clavicle fractures have a higher rate of non-
(a)
union (11.5–40 per cent). Treatment options for symp-
tomatic non-unions are excision of the lateral part of
the clavicle (if the fragment is small and the coraco-
clavicular ligaments are intact) or open reduction, in-
ternal fixation and bone grafting if the fragment is
large. Locking plates and hooked plates are used.
Malunion All displaced fractures heal in a non-
anatomical position with some shortening and
angulation, however most do not produce symptoms.
Some may go on to develop periscapular pain and this
(b) is more likely with shortening of more than 1.5cm. In
24.3 Fracture of the outer (lateral) third (a) The shaft these circumstances the difficult operation of corrective
of the clavicle is elevated, suggesting that the medial part osteotomy and plating can be considered.
of the coracoclavicular ligament is ruptured. (b) This was
treated by open reduction and internal fixation, using a Stiffness of the shoulder This is common but
long screw to fix the clavicle to the coracoid process tem- temporary; it results from fear of moving the fracture.
porarily while the soft tissues healed. Unless the fingers are exercised, they also may become
stiff and take months to regain movement.
mended. However the converse argument is that

many of the fractures that develop non-union do not
cause any symptoms and surgery can therefore be FRACTURES OF THE SCAPULA
reserved for patients with symptomatic non-union.
Operations for these fractures have a high complica-
tion rate and no single procedure has been shown to
Mechanisms of injury
be better than the others. Techniques include the use The body of the scapula is fractured by a crushing
of a coracoclavicular screw, plate and hook plate fixa- force, which usually also fractures ribs and may dislo-
tion and suture and sling techniques with Dacron cate the sternoclavicular joint. The neck of the scapula
graft ligaments. may be fractured by a blow or by a fall on the shoul-
der; the attached long head of triceps may drag the
MEDIAL THIRD FRACTURES glenoid downwards and laterally. The coracoid
Most of these rare fractures are extra-articular. They process may fracture across its base or be avulsed at
are mainly managed non-operatively unless the frac- the tip. Fracture of the acromion is due to direct
ture displacement threatens the mediastinal struc- force. Fracture of the glenoid fossa usually suggests a
tures. Initial fixation is associated with significant medially directed force (impaction of the joint) but
complications, including migration of the implants may occur with dislocation of the shoulder.
into the mediastinum, particularly when K-wires are
used. Other methods of stabilization include suture
and graft techniques and the newer locking plates.
Clinical features
The arm is held immobile and there may be severe
bruising over the scapula or the chest wall. Because of
Complications
the energy required to damage the scapula, fractures
EARLY of the body of the scapula are often associated with
Despite the close proximity of the clavicle to vital severe injuries to the chest, brachial plexus, spine,
structures, a pneumothorax, damage to the subclavian abdomen and head. Careful neurological and vascular
vessels and brachial plexus injuries are all very rare. examinations are essential. 735
24 X-Ray
Scapular fractures can be difficult to define on plain x-
rays because of the surrounding soft tissues. The films
may reveal a comminuted fracture of the body of the
scapula, or a fractured scapular neck with the outer
fragment pulled downwards by the weight of the arm.

Occasionally a crack is seen in the acromion or the
coracoid process. CT is useful for demonstrating
glenoid fractures or body fractures.
Type I Type II
Classification
Fractures of the scapula are divided anatomically into
scapular body, glenoid neck, glenoid fossa, acromion
and coracoid processes. Scapular neck fractures are the
most common. Further subdivisions are shown in
Table 24.1.
Table 24.1
Fractures of the scapular body Type III Type IV
Fractures of the glenoid neck
Intra-articular glenoid fossa fractures (Ideberg modified

by Goss)
Type I Fractures of the glenoid rim
Type II Fractures through the glenoid fossa, inferior fragment
displaced with subluxed humeral head
Type III Oblique fracture through glenoid exiting superiorly (may
be associated with acromioclavicular dislocation or fracture)
Type IV Horizontal fracture exiting through the medial border of
Type V Type VI
the scapula
24.4 Fractures of the glenoid – classification Dia-
Type V Combination of Type IV and a fracture separating the
grams showing the main types of glenoid fracture.
inferior half of the glenoid
Type VI Severe comminution of the glenoid surface
Fractures of acromion process Intra-articular fractures Type I glenoid fractures, if

displaced, may result in instability of the shoulder. If
Type I Minimally displaced
the fragment involves more than a third of the glenoid
Type II Displaced but not reducing subacromial space surface and is displaced by more than 5 mm surgical
Type III Inferior displacement and reduced subacromial space fixation should be considered. Anterior rim fractures
are approached through a delto-pectoral incision and
Fractures of coracoid process
posterior rim fractures through the posterior approach.
Type I Proximal to attachment of the coracoclavicular ligaments Type II fractures are associated with inferior
and usually associated with acromioclavicular separation subluxation of the head of the humerus and require
Type II Distal to the coraco-acromial ligaments open reduction and internal fixation. Types III, IV, V
and VI fractures have poorly defined indications for
surgery. Generally speaking, if the head is centred on
Treatment the major portion of the glenoid and the shoulder is
stable a non-operative approach is adopted.
Body fractures Surgery is not necessary. The patient Comminuted fractures of the glenoid fossa are likely
wears a sling for comfort, and from the start practises to lead to osteoarthritis in the longer term.
active exercises to the shoulder, elbow and fingers.
Fractures of the acromion Undisplaced fractures are
The fracture is usually
Isolated glenoid neck fractures treated non-operatively. Only Type III acromial
impacted and the glenoid surface is intact. A sling is fractures, in which the subacromial space is reduced,
736 worn for comfort and early exercises are begun. require operative intervention to restore the anatomy.
24.5 Glenoid fracture – 24
imaging (a) Three-dimen-
tional CT of a Type II glenoid
fracture.
(b) X-ray after open reduction
and internal fixation.

(a) (b)
Fractures of the coracoid process Fractures distal to the Treatment

coracoacromial ligaments do not result in serious
anatomical displacement; those proximal to the The patient is resuscitated. The outcome for the
ligaments are usually associated with acromioclavicular upper limb is very poor. Neither vascular reconstruc-
separations and may need operative treatment. tion nor brachial plexus exploration and repair are
likely to give a functional limb.
Combined fractures Whereas an isolated fracture of
the glenoid neck is stable, if there is an associated
fracture of the clavicle or disruption of the acromio-
clavicular ligament the glenoid mass may become ACROMIOCLAVICULAR JOINT
markedly displaced giving rise to a ‘floating shoulder’ INJURIES
(Williams et al, 2001). Diagnosis can be difficult and
may require advanced imaging and three-dimensional Acute injury of the acromioclavicular joint is common
reconstructions. At least one of the injuries (and and usually follows direct trauma. Chronic sprains,
sometimes both) will need operative fixation before often associated with degenerative changes, are seen
the fragments are stabilized. in people engaged in athletic activities like weightlift-
ing or occupations such as working with jack-ham-
mers and other heavy vibrating tools.
SCAPULOTHORACIC DISSOCIATION
Mechanism of injury
This is a high energy injury. The scapula and arm are
wrenched away from the chest, rupturing the subcla- A fall on the shoulder with the arm adducted may strain
vian vessels and brachial plexus. Many patients die. or tear the acromioclavicular ligaments and upward
subluxation of the clavicle may occur; if the force is
severe enough, the coracoclavicular ligaments will also
Clinical features be torn, resulting in complete dislocation of the joint.
The limb is flail and ischaemic. The diagnosis is usu-
ally made on the chest x-ray. There is swelling above
the clavicle from an expanding haematoma. A distrac-
Pathological anatomy and classification
tion of more than 1 cm of a fractured clavicle should The injury is graded according to the type of ligament
give rise to suspicion of this injury. injury and the amount of displacement of the joint.
(a) (b) (c) (d)

24.6 Acromioclavicular joint injuries (a) Normal joint. (b) Sprained acromioclavicular joint; no displacement. (c) Torn
capsule and subluxation but coracoclavicular ligaments intact. (d) Dislocation with torn coracoclavicular ligaments. 737
24 ideal. There is no convincing evidence that surgery
provides a better functional result than conservative
treatment for a straightforward Type III injury. Oper-
ative repair should be considered only for patients
with extreme prominence of the clavicle, those with
posterior or inferior dislocation of the clavicle and
those who aim to resume strenuous overarm or over-

(a) (b)
head activities.
24.7 Acromioclavicular dislocation (a) Clinically one Whilst there is no consensus regarding the best sur-
sees a definite ‘step’ in the contour at the lateral end of
the clavicle. (b) The x-ray shows complete separation of
gical solution, there are a number of underlying prin-
the acromioclavicular joint. ciples to consider if surgery is contemplated. Accurate
reduction should be the goal. The ligamentous stabil-
ity can be recreated either by transferring existing lig-
Type I is an acute sprain of the acromioclavicular liga- aments (the coracoacromial or conjoined tendons), or
ments; the joint is undisplaced. In Type II the acromio- by using a free graft (e.g., autogenous semitendinosis
clavicular ligaments are torn and the joint is subluxated or a synthetic ligament). This reconstruction must
with slight elevation of the clavicle. In Type III the have sufficient stability to prevent re-dislocation dur-
acromioclavicular and coracoclavicular ligaments are ing recovery. Any rigid implants which cross the joint
torn and the joint is dislocated; the clavicle is elevated will need to be removed at a later date to prevent
(or the acromion depressed) creating a visible and loosening or fracture.
palpable ‘step’. Other types of displacement are less In the modified Weaver–Dunn procedure the lateral
common, but occasionally the clavicle is displaced pos- end of the clavicle is excised and the coracoacromial
teriorly (Type IV), very markedly upwards (Type V) or ligament is transferred to the outer end of the clavicle
inferiorly beneath the coracoid process (Type VI). and attached by trans-osseous sutures. Tension on the
repair can be reduced either by anchoring the clavicle
to the coracoid with a Bosworth coracoclavicular screw
Clinical features
(which has to be removed after 8 weeks) or by em-
The patient can usually point to the site of injury and ploying a Dacron sling – looped round the coracoid
the area may be bruised. If there is tenderness but no and the clavicle – for the same purpose. Great care is
deformity, the injury is probably a sprain or a sublux- needed to avoid entrapment or damage to a nerve or
ation. With dislocation the patient is in severe pain vessel. Elbow and forearm exercises are begun on the
and a prominent ‘step’ can be seen and felt. Shoulder day after operation and active-assisted shoulder move-
movements are limited. ments 2 weeks later, increasing gradually to active
movements at 4–6 weeks. Strenuous lifting movements
are avoided for 4–6 months.
X-ray
Recent advances in instrumentation have made it
The acromioclavicular joint is not always easily visual-
ized; anteroposterior, cephalic tilt and axillary views
are advisable. In addition, a stress view is sometimes
helpful in distinguishing between a Type II and Type
III injury: this is an anteroposterior x-ray including
both shoulders with the patient standing upright,
arms by the side and holding a 5 kg weight in each
hand. The distance between the coracoid process and
the inferior border of the clavicle is measured on each
side; a difference of more than 50 per cent is diagnos-
tic of acromioclavicular dislocation.
Treatment
Sprains and subluxations do not affect function and
do not require any special treatment; the arm is rested 24.8 Modified Weaver Dunn operation The lateral
in a sling until pain subsides (usually no more than a end of the clavicle is excised; the acromial end of the
coracoacromial ligament is detached and fastened to the
week) and shoulder exercises are then begun. lateral end of the clavicle. Tension on the ligament is
Dislocations are poorly controlled by padding and lessened by placing a ‘sling’ around the clavicle and the
bandaging, yet the role of surgery is controversial. coracoid process. (Dotted lines show former position of
738 The large number of operations suggests that none is coracoacromial ligament).
feasible to perform this type of reconstructive surgery lows a direct blow to the front of the joint. Anterior 24
arthroscopically (Snow and Funk, 2006). dislocation is much more common than posterior.
The joint can be sprained, subluxed or dislocated.
Complications
Clinical features
Rotator cuff syndrome An acute strain of the acromio-

clavicular joint is sometimes followed by supraspinatus Anterior dislocation is easily diagnosed; the dislocated
tendinitis. Whether this is directly due to the primary medial end of the clavicle forms a prominent bump
injury or whether it results from post-traumatic over the sternoclavicular joint. The condition is
oedema or inflammation of the overlying acromio- painful but there are usually no cardiothoracic com-
clavicular joint is unclear. Treatment with plications.
anti-inflammatory preparations may help. Posterior dislocation, though rare, is much more
serious. Discomfort is marked; there may be pressure
Unreduced dislocation An unreduced dislocation is ugly
on the trachea or large vessels, causing venous con-
and sometimes affects function. Simple excision of the
gestion of the neck and arm and circulation to the arm
distal clavicle will only make matters worse. An attempt
may be decreased.
should be made to reconstruct the coracoclavicular
ligament. The Weaver–Dunn procedure may be
suitable (See Figure 24.8). X-Ray
Ossification of the ligamentsThe more severe injuries Because of overlapping shadows, plain x-rays are diffi-
are quite often followed by ossification of the coraco- cult to interpret. Special oblique views are helpful and
clavicular ligaments. Bony spurs may predispose to CT is the ideal method.
later rotator cuff dysfunction, which may require
operative treatment.
Treatment
Secondary osteoarthritis A late complication of Type I
Sprains and subluxations do not require specific treat-
and II injuries is osteoarthritis of the acromioclavicular
ment.
joint. This can usually be managed conservatively, but
Anterior dislocation can usually be reduced by
if pain is marked the outer 2 cm of the clavicle can be
exerting pressure over the clavicle and pulling on the
excised. The patient will be aware of some weakness
arm with the shoulder abducted. However, the joint
during strenuous over-arm activities and pain is often
usually redislocates. Not that this matters much; full
not completely abolished.
function will be regained, though this may take sev-
eral months.
Internal fixation is unnecessary and very dangerous
STERNOCLAVICULAR DISLOCATIONS (because of the large vessels behind the sternum).
Posterior dislocation should be reduced as soon as
possible. This can usually be done closed (if necessary
Mechanism of injury under general anaesthesia) by lying the patient supine
This uncommon injury is usually caused by lateral with a sandbag between the scapulae and then pulling
compression of the shoulders; for example, when on the arm with the shoulder abducted and extended.
someone is pinned to the ground following a road The joint reduces with a snap and stays reduced. If
accident or an underground rock-fall. Rarely, it fol- this manoeuvre fails, the medial end of the clavicle is
grasped with bone forceps and pulled forwards. If this
too, fails (a very rare occurrence) open reduction is
justified, but great care must be taken not to damage
the mediastinal structures. After reduction, the shoul-
ders are braced back with a figure-of-eight bandage,
which is worn for 3 weeks.
DISLOCATION OF THE SHOULDER

(a) (b)
Of the large joints, the shoulder is the one that most
24.9 Sternoclavicular dislocation (a) The bump over
the sternoclavicular joint may be obvious, though this is commonly dislocates. This is due to a number of
difficult to demonstrate on plain x-ray. (b) Tomography (or, factors: the shallowness of the glenoid socket; the
better still, CT) will show the lesion. extraordinary range of movement; underlying condi- 739
24 tions such as ligamentous laxity or glenoid dysplasia; A lateral view aimed along the blade of the scapula
and the sheer vulnerability of the joint during stress- will show the humeral head out of line with the
ful activities of the upper limb. socket.
In this chapter, acute anterior and posterior dislo- If the joint has dislocated before, special views may
cations are described. Chronic instability is described show flattening or an excavation of the posterolateral
in Chapter 13. contour of the humeral head, where it has been
indented by the anterior edge of the glenoid socket,

the Hill–Sachs lesion.
ANTERIOR DISLOCATION
Treatment
Mechanism of injury
Various methods of reduction have been described,
Dislocation is usually caused by a fall on the hand. some of them now of no more than historical interest.
The head of the humerus is driven forward, tearing In a patient who has had previous dislocations, simple
the capsule and producing avulsion of the glenoid traction on the arm may be successful. Usually,
labrum (the Bankart lesion). Occasionally the pos- sedation and occasionally general anaesthesia is
terolateral part of the head is crushed. Rarely, the required.
acromion process levers the head downwards and the With Stimson’s technique, the patient is left prone
joint dislocates with the arm pointing upwards (luxa- with the arm hanging over the side of the bed. After
tio erecta); nearly always the arm then drops, bringing 15 or 20 minutes the shoulder may reduce.
the head to its subcoracoid position. In the Hippocratic method, gently increasing trac-
tion is applied to the arm with the shoulder in slight
Clinical features abduction, while an assistant applies firm counter-
traction to the body (a towel slung around the
Pain is severe. The patient supports the arm with the patient’s chest, under the axilla, is helpful).
opposite hand and is loathe to permit any kind of With Kocher’s method, the elbow is bent to 90°
examination. The lateral outline of the shoulder may and held close to the body; no traction should be
be flattened and, if the patient is not too muscular, a applied. The arm is slowly rotated 75 degrees laterally,
bulge may be felt just below the clavicle. The arm then the point of the elbow is lifted forwards, and
must always be examined for nerve and vessel injury finally the arm is rotated medially. This technique car-
before reduction is attempted. ries the risk of nerve, vessel and bone injury and is not
recommended.
Another technique has the patient sitting on a
X-Ray reduction chair and with gentle traction of the arm
The anteroposterior x-ray will show the overlapping over the back of the padded chair the dislocation is
shadows of the humeral head and glenoid fossa, with reduced.
the head usually lying below and medial to the socket. An x-ray is taken to confirm reduction and exclude
(c)
(a) (b) (d)
24.10 Anterior dislocation of the shoulder (a) The prominent acromion process and flattening of the contour over
740 the deltoid are typical signs. (b) X-ray confirms the diagnosis of anterior dislocation. (c,d) Two methods of reduction.
a fracture. When the patient is fully awake, active 24
abduction is gently tested to exclude an axillary nerve
injury and rotator cuff tear. The median, radial, ulnar
and musculocutaneous nerves are also tested and the
pulse is felt.
The arm is rested in a sling for about three weeks in

those under 30 years of age (who are most prone to
recurrence) and for only a week in those over 30 (who
are most prone to stiffness). Then movements are
begun, but combined abduction and lateral rotation (a) (b)
must be avoided for at least 3 weeks. Throughout this
period, elbow and finger movements are practised 24.11 Anterior fracture-discloation Anterior disloca-
tion of the shoulder may be complicated by fracture of (a)
every day.
the greater tuberosity or (b) the neck of the humerus –
There has been some interest in the use of external this often needs open reduction and internal fixation.
rotation splints, based on the theory that this would
reduce the Bankart lesion into a better position for
healing. However a recent Cochrane review has con- Vascular injury The axillary artery may be damaged,
cluded that there is insufficient evidence to inform on particularly in old patients with fragile vessels. This can
the choices for conservative treatment and that fur- occur either at the time of injury or during overzealous
ther trials are needed to compare different types and reduction. The limb should always be examined for
duration of immobilization. signs of ischaemia both before and after reduction.
Young athletes who dislocate their shoulder trau-
Fracture-dislocation If there is an associated fracture of
matically and who continue to pursue their sports
the proximal humerus, open reduction and internal
(particularly contact sports) are at a much higher risk
fixation may be necessary. The greater tuberosity may
of re-dislocation in the future. With increasing
be sheared off during dislocation. It usually falls into
advances and techniques of arthroscopy and arthro-
place during reduction, and no special treatment is
scopic anterior stabilization surgery, some are now
then required. If it remains displaced, surgical
advocating early surgery in this group of patients to
reattachment is recommended to avoid later
repair the Bankart lesion of the anterior labrum.
subacromial impingement.
However a consensus on early surgery has still not
been reached.
LATE
Shoulder stiffness Prolonged immobilization may lead
to stiffness of the shoulder, especially in patients over
Complications
the age of 40. There is loss of lateral rotation, which
EARLY automatically limits abduction. Active exercises will
Rotator cuff tear This commonly accompanies anterior usually loosen the joint. They are practised vigorously,
dislocation, particularly in older people. The patient bearing in mind that full abduction is not possible until
may have difficulty abducting the arm after reduction; lateral rotation has been regained. Manipulation under
palpable contraction of the deltoid muscle excludes an anaesthesia or arthroscopic capsular release is advised
axillary nerve palsy. Most do not require surgical only if progress has halted and at least 6 months have
attention, but young active individuals with large tears elapsed since injury.
will benefit from early repair.
Unreduced dislocation Surprisingly, a dislocation of the
Nerve injury The axillary nerve is most commonly shoulder sometimes remains undiagnosed. This is
injured; the patient is unable to contract the deltoid more likely if the patient is either unconscious or very
muscle and there may be a small patch of anaesthesia old. Closed reduction is worth attempting up to 6
over the muscle. The inability to abduct must be weeks after injury; manipulation later may fracture the
distinguished from a rotator cuff tear. The nerve lesion bone or tear vessels or nerves. Operative reduction is
is usually a neuropraxia which recovers spontaneously indicated after 6 weeks only in the young, because it is
after a few weeks; if it does not, then surgery should be difficult, dangerous and followed by prolonged
considered as the results of repair are less satisfactory if stiffness. An anterior approach is used, and the vessels
the delay is more than a few months. and nerves are carefully identified before the
Occasionally the radial nerve, musculocutaneous dislocation is reduced. ‘Active neglect’ summarizes the
nerve, median nerve or ulnar nerve can be injured. treatment of unreduced dislocation in the elderly. The
Rarely there is a complete infra-clavicular brachial dislocation is disregarded and gentle active movements
plexus palsy. This is somewhat alarming, but fortu- are encouraged. Moderately good function is often
nately it usually recovers with time. regained. 741
24 24.12 Recurrent dislocation
of the shoulder (a) The
classic x-ray sign is a depres-
sion in the posterosuperior part
of the humeral head (the Hill-
Sachs lesion). (b,c) MRI scans
showing both the Hill–Sachs
lesion and a Bankart lesion of

the glenoid rim (arrows).
(a) (b) (c)
Recurrent dislocation If an anterior dislocation tears a direct blow to the front of the shoulder or a fall on
the shoulder capsule, repair occurs spontaneously fol- the outstretched hand.
lowing reduction and the dislocation may not recur;
but if the glenoid labrum is detached, or the capsule
is stripped off the front of the neck of the glenoid,
Clinical features
repair is less likely and recurrence is more common. The diagnosis is frequently missed – partly because
Detachment of the labrum occurs particularly in reliance is placed on a single anteroposterior x-ray
young patients, and, if at injury a bony defect has (which may look almost normal) and partly because
been gouged out of the posterolateral aspect of the those attending to the patient fail to think of it. There
humeral head, recurrence is even more likely. In older are, in fact, several well-marked clinical features. The
patients, especially if there is a rotator cuff tear or arm is held in internal rotation and is locked in that
greater tuberosity fracture, recurrent dislocation is position. The front of the shoulder looks flat with a
unlikely. The period of post-operative immobilization prominent coracoid, but swelling may obscure this
makes no difference. deformity; seen from above, however, the posterior
The history is diagnostic. The patient complains displacement is usually apparent.
that the shoulder dislocates with relatively trivial
everyday actions. Often he can reduce the dislocation
himself. Any doubt as to diagnosis is quickly resolved
X-Ray
by the apprehension test: if the patient’s arm is pas- In the anteroposterior film the humeral head, because
sively placed behind the coronal plane in a position of it is medially rotated, looks abnormal in shape (like an
abduction and lateral rotation, his immediate resist-
ance and apprehension are pathognomonic. An
anteroposterior x-ray with the shoulder medially
rotated may show an indentation in the back of the
humeral head (the Hill–Sachs lesion).
Even more common, but less readily diagnosed, is
recurrent subluxation. The management of both types
of instability is dealt with in Chapter 13.
POSTERIOR DISLOCATION OF THE

SHOULDER
Posterior dislocation is rare, accounting for less than 2

per cent of all dislocations around the shoulder.
Mechanism of injury
Indirect force producing marked internal rotation and
adduction needs be very severe to cause a dislocation. 24.13 Posterior dislocation of the shoulder The
characteristic x-ray image. Because the head of the
This happens most commonly during a fit or convul- humerus is internally rotated, the anteroposterior x-ray
sion, or with an electric shock. Posterior dislocation shows a head-on projection giving the classic ‘electric
742 can also follow a fall on to the flexed, adducted arm, light-bulb’ appearance.
electric light bulb) and it stands away somewhat from 24
the glenoid fossa (the ‘empty glenoid’ sign). A lateral INFERIOR DISLOCATION OF THE
film and axillary view is essential; it shows posterior SHOULDER (LUXATIO ERECTA)
subluxation or dislocation and sometimes a deep
indentation on the anterior aspect of the humeral Inferior dislocation is rare but it demands early recog-
head. Posterior dislocation is sometimes complicated nition because the consequences are potentially very

by fractures of the humeral neck, posterior glenoid serious. Dislocation occurs with the arm in nearly full
rim or lesser tuberosity. Sometimes the patient is too abduction/elevation. The humeral head is levered out
uncomfortable to permit adequate imaging and in of its socket and pokes into the axilla; the arm remains
these difficult cases CT is essential to rule out poste- fixed in abduction.
rior dislocation of the shoulder.
Mechanism of injury and pathology

Treatment The injury is caused by a severe hyper-abduction
force. With the humerus as the lever and the
The acute dislocation is reduced (usually under gen- acromion as the fulcrum, the humeral head is lifted
eral anaesthesia) by pulling on the arm with the shoul- across the inferior rim of the glenoid socket; it
der in adduction; a few minutes are allowed for the remains in the subglenoid position, with the humeral
head of the humerus to disengage and the arm is then shaft pointing upwards. Soft-tissue injury may be
gently rotated laterally while the humeral head is severe and includes avulsion of the capsule and sur-
pushed forwards. If reduction feels stable the arm is rounding tendons, rupture of muscles, fractures of the
immobilized in a sling; otherwise the shoulder is held glenoid or proximal humerus and damage to the
widely abducted and laterally rotated in an airplane brachial plexus and axillary artery.
type splint for 3–6 weeks to allow the posterior
capsule to heal in the shortest position. Shoulder
movement is regained by active exercises. Clinical features
The startling picture of a patient with his arm locked
in almost full abduction should make diagnosis quite
Complications easy. The head of the humerus may be felt in or below
the axilla. Always examine for neurovascular damage.
Unreduced dislocation At least half the patients with
posterior dislocation have ‘unreduced’ lesions when X-ray
first seen. Sometimes weeks or months elapse before
the diagnosis is made and up to two thirds of poste- The humeral shaft is shown in the abducted position
rior dislocations are not recognised initially. Typically with the head sitting below the glenoid. It is impor-
the patient holds the arm internally rotated; he cannot tant to search for associated fractures of the glenoid or
abduct the arm more than 70–80 degrees, and if he proximal humerus.
lifts the extended arm forwards he cannot then turn NOTE: True inferior dislocation must not be con-
the palm upwards. If the patient is young, or is fused with postural downward displacement of the
uncomfortable and the dislocation fairly recent, open humerus, which results quite commonly from weak-
reduction is indicated. This is a difficult procedure. It ness and laxity of the muscles around the shoulder,
is generally done through a delto-pectoral approach; especially after trauma and shoulder splintage; here
the shoulder is reduced and the defect in the humeral
head can then be treated by transferring the sub-
scapularis tendon into the defect (McLaughlin proce-
dure). Alternatively, the defect on the humeral head
can be bone grafted. A useful technique for treating a 24.14 Inferior dislocation
defect smaller than 40 per cent of the humeral head is of the shoulder You can
to transfer of the lesser tuberosity together with the see why the condition is
subscapularis into the defect. For defects larger than called luxatio erecta. The
shaft of the humerus points
this a hemiarthroplasty may be considered.
upwards and the humeral
Late dislocations, especially in the elderly, are best head is displaced down-
left, but movement is encouraged. wards.
Recurrent dislocation or subluxation Chronic posterior
instability of the shoulder is discussed in Chapter
13. 743
24 the shaft of the humerus lies in the normal anatomical ment should be directed. A prolonged exercise pro-
position at the side of the chest. The condition is gramme may also help. Only if the child is genuinely
harmless and resolves as muscle tone is regained. distressed by the disorder, and provided psychological
factors have been excluded, should one consider
reconstructive surgery.
Treatment
Inferior dislocation can usually be reduced by pulling

upwards in the line of the abducted arm, with
counter-traction downwards over the top of the FRACTURES OF THE PROXIMAL
shoulder. If the humeral head is stuck in the soft tis- HUMERUS
sues, open reduction is needed. It is important to
examine again, after reduction, for evidence of neu-
Fractures of the proximal humerus usually occur after
rovascular injury.
middle age and most of the patients are osteoporotic,
The arm is rested in a sling until pain subsides and
postmenopausal women. Fracture displacement is
movement is then allowed, but avoiding abduction
usually not marked and treatment presents few prob-
for 3 weeks to allow the soft tissues to heal.
lems. However, in about 20 per cent of cases there is
considerable displacement of one or more fragments
and a significant risk of complications due to bone
fragility, damage to the rotator cuff and the prevailing
SHOULDER DISLOCATIONS IN co-morbidities. Deciding between operative and non-
CHILDREN operative treatment can be very difficult.
Traumatic dislocation of the shoulder is exceedingly

rare in children. Children who give a history of the
Mechanism of injury
shoulder ‘slipping out’ almost invariably have either Fracture usually follows a fall on the out-stretched
voluntary or involuntary (atraumatic) dislocation or arm – the type of injury which, in younger people,
subluxation. With voluntary dislocation, the child can might cause dislocation of the shoulder. Sometimes,
demonstrate the instability at will. With involuntary indeed, there is both a fracture and a dislocation.
dislocation, the shoulder slips out unexpectedly dur-
ing everyday activities. Most of these children have
generalized joint laxity and some have glenoid dyspla-
Classification and pathological anatomy
sia or muscle patterning disorders (Chapter 13). The most widely accepted classification is that of Neer
Examination may show that the shoulder subluxates (1970) who drew attention to the four major seg-
in almost any direction; x-rays may confirm the diag- ments involved in these injuries: the head of the
nosis. humerus, the lesser tuberosity, the greater tuberosity
and the shaft. Neer’s classification distinguishes
between the number of displaced fragments, with dis-
Treatment placement defined as greater than 45 degrees of angu-
Atraumatic dislocation should be viewed with great lation or 1 cm of separation. Thus, however many frac-
caution. Some of these children have behavioural or ture lines there are, if the fragments are undisplaced it
muscle patterning problems and this is where treat- is regarded as a one-part fracture; if one segment is sep-
24.15 Fractures of the

2 2 proximal humerus Diagram of
3 3 (a) the normal and (b) a fractured
4 proximal humerus, showing the
4 four main fragments, two or
more of which are seen in almost
all proximal humeral fractures.
1=shaft of humerus; 2=head of
5 humerus; 3=greater tuberosity;
4=lesser tuberosity. In this figure
1 there is a sizeable medial calcar
1
spike; 5=suggesting a low risk of
avascular necrosis.
744 (a) (b)

24

(a) (b) (c) (d)
24.16 X-rays of proximal humeral fractures Classification is all very well, but x-rays are more difficult to interpret than
line drawings. (a) Two-part fracture. (b) Three-part fracture involving the neck and the greater tuberosity. (c) Four-part
fracture. (1=shaft of humerus; 2=head of humerus; 3=greater tuberosity; 4=lesser tuberosity). (d) X-ray showing fracture-
dislocation of the shoulder.
arated from the others, it is a two-part fracture; if two X-ray

fragments are displaced, that is a three-part fracture; if
all the major parts are displaced, it is a four-part frac- In elderly patients there often appears to be a single,
ture. Furthermore, a fracture-dislocation exists when impacted fracture extending across the surgical neck.
the head is dislocated and there are two, three or four However, with good x-rays, several undisplaced frag-
parts. This grading is based on x-ray appearances, al- ments may be seen. In younger patients, the frag-
though observers do not always agree with each other ments are usually more clearly separated. Axillary and
on which class a particular fracture falls into. scapular-lateral views should always be obtained, to
exclude dislocation of the shoulder.
It has always been difficult to apply Neer’s classifi-
Clinical features cation when based on plain x-rays and not surprisingly
Because the fracture is often firmly impacted, pain there is a relatively high level of both inter- and intra-
may not be severe. However, the appearance of a large observer disagreement. Neer himself later noted that
bruise on the upper part of the arm is suspicious. when this classification was developed the criteria for
Signs of axillary nerve or brachial plexus injury should displacement (distance >1 cm, angulation >45
be sought. degrees) were set arbitrarily. The classification was not
intended to dictate treatment, but simply to help clar-
ify the pathoanatomy of the different fracture pat-
terns.
The advent of three-dimensional CT reconstruc-
tion has helped to reduce the degree of inter- and
intra-observer error, enabling better planning of treat-
ment than in the past.
As the fracture heals, the humeral head is some-
times seen to be subluxated downwards (inferiorly);
this is due to muscle atony and it usually recovers once
exercises are begun.
Treatment
MINIMALLY DISPLACED FRACTURES
These comprise the vast majority. They need no treat-
ment apart from a week or two period of rest with the
arm in a sling until the pain subsides, and then gentle
passive movements of the shoulder. Once the fracture
24.17 CT with three-dimensional reconstruction has united (usually after 6 weeks), active exercises are
Advanced imaging provides a much clearer picture of the encouraged; the hand is, of course, actively exercised
injury, allowing better pre-operative planning. from the start. 745
24 TWO-PART FRACTURES locked plating and nailing are biomechanically supe-
Surgical neck fractures The fragments are gently rior in osteoporotic bone.
manipulated into alignment and the arm is immobi-
lized in a sling for about four weeks or until the frac- FOUR-PART FRACTURES
ture feels stable and the x-ray shows some signs of The surgical neck and both tuberosities are displaced.
healing. Elbow and hand exercises are encouraged These are severe injuries with a high risk of complica-
throughout this period; shoulder exercises are com- tions such as vascular injury, brachial plexus damage, in-
menced at about four weeks. The results of conserva- juries of the chest wall and (later) avascular necrosis of
tive treatment are generally satisfactory, considering the humeral head. The x-ray diagnosis is difficult (how
that most of these patients are over 65 and do not many fragments are there, and are they displaced?). Of-
demand perfect function. However, if the fracture ten the most one can say is that there are ‘multiple dis-
cannot be reduced closed or if the fracture is very placed fragments’, sometimes together with gleno-
unstable after closed reduction, then fixation is humeral dislocation. In young patients an attempt
required. Options include percutaneous pins, bone should be made at reconstruction. In older patients,
sutures, intramedullary pins with tension band wiring closed treatment and attempts at open reduction and
or a locked intramedullary nail. Plate fixation requires fixation can result in continuing pain and stiffness and
a wider exposure and the newer locking plates offer a additional surgical treatment can compromise the
stable fixation without the need for extensive blood supply still further. If the fracture pattern is such
periosteal stripping. that the blood-supply is likely to be compromised, or
that reconstruction and internal fixation will be ex-
Greater tuberosity fractures Fracture of the greater
tremely difficult, then the treatment of choice is pros-
tuberosity is often associated with anterior dislocation
thetic replacement of the proximal humerus.
and it reduces to a good position when the shoulder is
The results of hemiarthroplasty are somewhat
relocated. If it does not reduce, the fragment can be
unpredictable. Anatomical reduction, fixation and
re-attached through a small incision with interosseous
healing of the tuberosities are prerequisites for a satis-
sutures or, in young hard bone, cancellous screws.
factory outcome; even then, secondary displacement
Anatomical neck fractures These are very rare. In young of the tuberosities may result in a poor functional out-
patients the fracture should be fixed with a screw. In come. In addition the prosthetic implant should be
older patients prosthetic replacement (hemi- perfectly positioned. Be warned – these are operations
arthroplasty) is preferable because of the high risk of for the expert; the subject is well covered by Boileau
avascular necrosis of the humeral head. et al. (2006).
THREE-PART FRACTURES
These usually involve displacement of the surgical
neck and the greater tuberosity; they are extremely FRACTURE-DISLOCATION
difficult to reduce closed. In active individuals this
injury is best managed by open reduction and internal Two-part fracture-dislocations (greater tuberosity
fixation. There is little evidence that one technique is with anterior dislocation and lesser tuberosity with
better than another although the newer implants with posterior) can usually be reduced by closed means.
(a) (b) (c) (d)
24.18 Proximal humerus fractures – treatment (a) Three-part fracture, treated by (b) locked nail fixation. (c) Four-part
746 fracture fixed with a locked plate; the intra-operative picture (d) shows how the plate was positioned.
Three-part fracture-dislocations, when the surgical Malunion Malunion usually causes little disability, but 24
neck is also broken, usually require open reduction loss of rotation may make it difficult for the patient to
and fixation; the brachial plexus is at particular risk reach behind the neck or up the back.
during this operation.
Four-part fracture-dislocations have a poor progno-
sis; prosthetic replacement is recommended in all but

young and very active patients.
FRACTURES OF THE PROXIMAL
HUMERUS IN CHILDREN
Complications
Vascular injuries and nerve injuries The patient should At birth, the shoulder is sometimes dislocated or the
always be carefully assessed for signs of vascular and proximal humerus fractured. Diagnosis is difficult and
nerve injuries, both at the initial examination and again a clavicular fracture or brachial plexus injury should
after any operation. The axillary nerve is at particular also be considered.
risk, both from the injury and from surgery. In infancy, the physis can separate (Salter–Harris I);
Avascular necrosis The reported incidence of avascular reduction does not have to be perfect and a good out-
necrosis (AVN) of the humeral head ranges from 10– come is usual.
30 per cent in three-part fractures and 10 to over In older children, metaphyseal fractures or Type II
50 per cent in four-part fractures. The ability to predict physeal fractures occur. Considerable displacement
the likelihood of this outcome is important in making and angulation can be accepted; because of the
the choice between internal fixation and hemi- marked growth and remodelling potential of the
arthroplasty for complex fractures. proximal humerus, malunion is readily compensated
The blood-supply of the humeral head is provided for during the remaining growth period.
mainly by the anterior circumflex artery and its Pathological fractures are not unusual, as the prox-
ascending branch (the arcuate artery) which pene- imal humerus is a common site of bone cysts and
trates into the humeral head and arches across sub- tumours in children. Fracture through a simple cyst
chondrally. Additional blood-supply is provided by usually unites and the cyst often heals spontaneously;
vessels entering the posteromedial aspect of the all that is needed is to rest the arm in a sling for 4–6
proximal humerus, metaphyseal vessels and vessels of weeks. Other lesions require treatment in their own
the greater and lesser tuberosities that anastomose right (See Chapter 9).
with the intraosseous arcuate artery. Thus, in three-
and four-part fractures with the only supply coming
from the posteromedial vessels, there may still be suf-
ficient perfusion of the humeral head if the head frag-
ment includes a sizeable part of the calcar on the
medial side of the anatomical neck. Hertel et al.
(2004) have made the point that fractures at the
anatomical neck with a medial metaphyseal (calcar)
spike shorter than 8 mm carry a high risk of develop-
ing humeral head avascular necrosis (see Fig. 24.15).
Disruption of the medial periosteal hinge is another
predictor of avascular necrosis and the presence of
these two factors combined has a positive predictive
value of 98 per cent for avascular necrosis of the
humeral head. Contrariwise, fractures with an intact
medial hinge and/or a large posteromedial metaphy-
seal spike carry a much better prognosis. The mere
number of fracture parts, their degree of displacement
and split-head fractures are rated as poor predictors of
avascular necrosis, as is the presence of dislocation.
Stiffness of the shoulder This is a common (a) (b)
complication, particularly in elderly patients. Unlike a
24.19 Fractures of the proximal humerus in children
frozen shoulder, the stiffness is maximal at the outset. (a) The typical metaphyseal fracture. Reduction need not
It can be prevented, or at least minimized, by starting be perfect as remodelling will compensate for malunion.
exercises early. (b) Fracture through a benign cyst. 747
24 FRACTURED SHAFT OF HUMERUS Treatment
Fractures of the humerus heal readily. They require
Mechanism of injury neither perfect reduction nor immobilization; the
weight of the arm with an external cast is usually
A fall on the hand may twist the humerus, causing a enough to pull the fragments into alignment. A
spiral fracture. A fall on the elbow with the arm
‘hanging cast’ is applied from shoulder to wrist with

abducted exerts a bending force, resulting in an the elbow flexed 90 degrees, and the forearm section
oblique or transverse fracture. A direct blow to the is suspended by a sling around the patient’s neck. This
arm causes a fracture which is either transverse or cast may be replaced after 2–3 weeks by a short
comminuted. Fracture of the shaft in an elderly (shoulder to elbow) cast or a functional polypropylene
patient may be due to a metastasis. brace which is worn for a further 6 weeks.
The wrist and fingers are exercised from the start.
Pendulum exercises of the shoulder are begun within
a week, but active abduction is postponed until the
With fractures above the deltoid insertion, the proxi- fracture has united (about 6 weeks for spiral fractures
mal fragment is adducted by pectoralis major. With but often twice as long for other types); once united,
fractures lower down, the proximal fragment is only a sling is needed until the fracture is consoli-
abducted by the deltoid. Injury to the radial nerve is dated.
common, though fortunately recovery is usual.
OPERATIVE TREATMENT
Clinical features Patients often find the hanging cast uncomfortable,
The arm is painful, bruised and swollen. It is impor- tedious and frustrating; they can feel the fragments
tant to test for radial nerve function before and after moving and that is sometimes quite distressing. The
treatment. This is best done by assessing active exten- temptation is to ‘do something’, and the ‘something’
sion of the metacarpophalangeal joints; active exten- usually means an operation. It is well to remember
sion of the wrist can be misleading because extensor (a) that the complication rate after internal fixation of
carpi radialis longus is sometimes supplied by a branch the humerus is high and (b) that the great majority of
arising proximal to the injury. humeral fractures unite with non-operative treatment.
(c) There is no good evidence that the union rate is
higher with fixation (and the rate may be lower if
X-ray there is distraction with nailing or periosteal stripping
with plating). There are, nevertheless, some well
The site of the fracture, its line (transverse, spiral or
defined indications for surgery:
comminuted) and any displacement are readily seen.
The possibility that the fracture may be pathological • severe multiple injuries
should be remembered. • an open fracture
(a) (b) (c) (d) (e)
24.20 Fractured shaft of humerus (a) Bruising is always extensive. (b,c) Closed transverse fracture with moderate
displacement. (d) Applying a U-slab of plaster (after a few days in a shoulder-to-wrist hanging cast) is usually adequate.
(e) Ready-made braces are simpler and more comfortable, though not suitable for all cases. These conservative methods
748 demand careful supervision if excessive angulation and malunion are to be prevented.
24.21 Fractured shaft of humerus 24
– treatment (a,b) Most shaft frac-
tures can be treated in a hanging
cast or functional brace, but beware
the upper third fracture which tends
to angulate at the proximal border of
a short cast. This fracture would

have been better managed by
(c) intramedullary nailing (and better
still with a locking nail).
(a) (b) (c)
• segmental fractures union; if this happens, exchange nailing and bone

• displaced intra-articular extension of the fracture grafting of the fracture may be needed.
• a pathological fracture Retrograde nailing with multiple flexible rods is not
• a ‘floating elbow’ (simultaneous unstable humeral entirely stable. Retrograde nailing with an interlock-
and forearm fractures) ing nail is suitable for some fractures of the middle
• radial nerve palsy after manipulation third.
• non-union External fixation may be the best option for high-
• problems with nursing care in a dependent person. energy segmental fractures and open fractures. How-
ever, great care must be taken in placing the pins as
Fixation can be achieved with either (1) a compression
the radial nerve is vulnerable.
plate and screws, (2) an interlocking intramedullary nail
or semi-flexible pins, or (3) an external fixator.
Plating permits excellent reduction and fixation,
and has the added advantage that it does not interfere Complications
with shoulder or elbow function. However, it requires
EARLY
wide dissection and the radial nerve must be pro-
Vascular injury If there are signs of vascular
tected. Too much periosteal stripping or inadequate
insufficiency in the limb, brachial artery damage must
fixation will probably increase the risk of non-union.
be excluded. Angiography will show the level of the
Antegrade nailing is performed with a rigid inter-
injury. This is an emergency, requiring exploration and
locking nail inserted through the rotator cuff under
either direct repair or grafting of the vessel. In these
fluoroscopic control. It requires minimal dissection
circumstances, internal fixation is advisable.
but has the disadvantage that it causes rotator cuff
problems in a significant proportion of cases (the Nerve injury Radial nerve palsy (wrist drop and
reported incidence ranges from 5–40 per cent). The paralysis of the metacarpophalangeal extensors) may
nail can also distract the fracture which will inhibit occur with shaft fractures, particularly oblique fractures
24.22 Fractured humerus –

other methods of fixation
(a,b) Compression plating,
and (c,d,e) external fixation.
(a) (b) (c) (d) (e) 749

24 at the junction of the middle and distal thirds of the Taking advantage of the robust periosteum and the
bone (Holstein–Lewis fracture). If nerve function was power of rapid healing in children, the humeral frac-
intact before manipulation but is defective afterwards, ture can usually be treated by applying a collar and
it must be assumed that the nerve has been snagged cuff bandage for 3 or 4 weeks. If there is gross short-
and surgical exploration is necessary. Otherwise, in ening, manipulation may be needed. Older children
closed injuries the nerve is very seldom divided, so may require a short plaster splint.
there is no hurry to operate as it will usually recover.

The wrist and hand must be regularly moved through
a full passive range of movement to preserve joint
motion until the nerve recovers. If there is no sign of FRACTURES OF THE DISTAL
recovery by 12 weeks, the nerve should be explored. It HUMERUS IN ADULTS
may just need a neurolysis, but if there is loss of
continuity of normal-looking nerve then a graft is Fractures around the elbow in adults – especially
needed. The results are often satisfactory but, if those of the distal humerus – are often high-energy
necessary, function can be largely restored by tendon injuries which are associated with vascular and nerve
transfers (see Chapter 11). damage. Some can be reduced and stabilized only by
complex surgical techniques; and the tendency to
LATE stiffness of the elbow means that with all severe
Delayed union and non-union Transverse fractures injuries the striving for anatomical perfection has to
sometimes take months to unite, especially if excessive be weighed up against the realities of imperfect post-
traction has been used (a hanging cast must not be too operative function.
heavy). Simple adjustments in technique may solve the The AO-ASIF Group have defined three types of
problem; as long as there are signs of callus formation distal humeral fracture:
it is worth persevering with non-operative treatment,
but remember to keep the shoulder moving. The rate Type A – an extra-articular supracondylar fracture;
of non-union in conservatively treated low-energy Type B – an intra-articular unicondylar fracture (one
fractures is less than 3 per cent. Segmental high energy condyle sheared off);
fractures and open fractures are more prone to both Type C – bicondylar fractures with varying degrees of
delayed union and non-union. comminution.
Intramedullary nailing may contribute to delayed
union, but if rigid fixation can be maintained (if nec-
essary by exchange nailing) the rate of non-union can TYPE A – SUPRACONDYLAR FRACTURES
probably be kept below 10 per cent.
A particularly vicious combination is incomplete These extra-articular fractures are rare in adults. When
union and a stiff joint. If elbow or shoulder move- they do occur, they are usually displaced and unstable
ments are forced before consolidation of the fracture, – probably because there is no tough periosteum to
or if an intramedullary nail is removed too soon (e.g., tether the fragments. In high-energy injuries there
because of shoulder problems), the humerus may re- may be comminution of the distal humerus.
fracture and non-union is then more likely.
The treatment of established non-union is Treatment
operative. The bone ends are freshened, cancellous
Closed reduction is unlikely to be stable and K-wire
bone graft is packed around them and the reduction
fixation is not strong enough to permit early mobi-
is held with an intramedullary nail or a compression
lization. Open reduction and internal fixation is there-
plate.
fore the treatment of choice. The distal humerus is
Joint stiffness is common. It can be
Joint stiffness approached through a posterior exposure. It is some-
minimized by early activity, but transverse fractures (in times possible to fix the fracture without recourse to
which shoulder abduction is ill-advised) may limit an olecranon osteotomy or triceps reflection. A simple
shoulder movement for several weeks. transverse or oblique fracture can usually be reduced
and fixed with a pair of contoured plates and screws.
SPECIAL FEATURES IN CHILDREN TYPES B AND C – INTRA-ARTICULAR

Fractures of the humerus are uncommon; in children FRACTURES
under 3 years of age the possibility of child abuse
should be considered and tactful examination for Except in osteoporotic individuals, intra-articular
750 other injuries performed. condylar fractures should be regarded as high-energy
injuries with soft-tissue damage. A severe blow on the fractures (some would say for all Type B and C 24
point of the elbow drives the olecranon process fractures – minor displacement is easily overlooked in
upwards, splitting the condyles apart. Swelling is con- the early post-injury x-rays). The danger with
siderable, but if the bony landmarks can be felt the conservative treatment is the strong tendency to
elbow is found to be distorted. The patient should be stiffening of the elbow and persistent pain.
carefully examined for evidence of vascular or nerve Good exposure of the joint is needed, if necessary

injury; if there are signs of vascular insufficiency, this by performing an intra-articular olecranon osteotomy.
must be addressed as a matter of urgency. The ulnar nerve should be identified and protected
throughout. The fragments are reduced and held
temporarily with K-wires. A unicondylar fracture
X-Ray without comminution can then be fixed with screws;
The fracture extends from the lower humerus into the if the fragment is large, a contoured plate is added to
elbow joint; it may be difficult to tell whether one or prevent re-displacement. First the articular block is
both condyles are involved, especially with an undis- reconstructed with a transverse screw; bone graft is
placed condylar fracture. There is often also com- sometimes needed. The distal block is then fixed to
minution of the bone between the condyles, the the humeral shaft with medial and lateral plates. Pre-
extent of which is usually underestimated. Sometimes contoured plates with locking screws are now avail-
the fracture extends into the metaphysis as a T- or Y- able. These hold the distal fragments more effectively.
shaped break, or else there may be multiple fragments Postoperatively the elbow is held at 90 degrees with
(comminution). The lesson is: ‘Prepare for the worst the arm supported in a sling. Movement is encour-
before operating’. CT scans can be helpful in planning aged but should never be forced. Fracture healing
the surgical approach. usually occurs by 12 weeks. Despite the best efforts,
the patient often does not regain full extension and in
the most severe cases movement may be severely
Treatment restricted.
A description of this sort fails to convey the real dif-
These are severe injuries associated with joint damage; ficulty of these operations. Unless the surgeon is more
prolonged immobilization will certainly result in a stiff than usually skilful, the elbow may end up stiffer than
elbow. Early movement is therefore a prime objective. if treated by activity (see below).
Undisplaced fractures These can be treated by applying
a posterior slab with the elbow flexed almost 90 ALTERNATIVE METHODS OF TREATMENT
degrees; movements are commenced after 2 weeks. If it is anticipated that the outcome of operative treat-
However, great care should be taken to avoid the dual ment will be poor (either because of the degree of
pitfalls of underdiagnosis (displacement and comminution and soft-tissue damage or because of
comminution are not always obvious on the initial x- lack of expertise and facilities) other options can be
ray) and late displacement (always obtain check x-rays considered.
a week after injury).
Elbow replacement The elderly patient with a
Displaced Type B and C fractures If the appropriate comminuted fracture, a low transverse fracture or
expertise and facilities are available, open reduction and osteopaenic bone, may be best served by replacement
internal fixation is the treatment of choice for displaced of the elbow.
24.23 Bicondylar
fractures X-rays taken
(a,b) before and
(c,d) after open
reduction and internal
fixation. An excellent
reduction was obtained
in this case; however,
the elbow sometimes
ends up with
considerable loss of
movement even though
the general anatomy
has been restored.
(a) (b) (c) (d) 751

24 The ‘bag of bones’ technique The arm is held in a collar
and cuff or, better, a hinged brace, with the elbow
flexed above a right angle; active movements are
encouraged as soon as the patient is willing. The
fracture usually unites within 6–8 weeks, but exercises
are continued far longer. A useful range of movement
(45–90 degrees) is often obtained.

Skeletal traction An alternative method of treating
either moderately displaced or severely comminuted
fractures is by skeletal traction through the olecranon
(beware the ulnar nerve!); the patient remains in bed
with the humerus held vertical, and elbow movements
are encouraged. Again, meticulous internal fixation or
elbow replacement are usually preferable. (a) (b)
24.24 Fractured capitulum Anteroposterior and lateral

Complications x-rays showing proximal displacement and tilting of the
capitular fragment.
EARLY
Vascular injury Always check the circulation (repeat-
edly!). Vigilance is required to make the diagnosis and
institute treatment as early as possible.
Nerve injury There may be damage to either the longer points directly towards it. Bryan and Morrey
median or the ulnar nerve. It is important to examine classify these as:
the hand and record the findings before treatment is
Type I Complete fracture
commenced. The ulnar nerve is particularly vulnerable
Type II Cartilaginous shell
during surgery.
Type III Comminuted fracture.
LATE CT scans can be helpful in clarifying the diagnosis.
Stiffness Comminuted fractures of the elbow always
result in some degree of stiffness. However, the
Treatment
disability may be reduced by encouraging an energetic
exercise programme. Late operations to improve elbow Undisplaced fractures can be treated by simple splin-
movement are difficult but can be rewarding. tage for 2 weeks.
Displaced fractures should be reduced and held.
Heterotopic ossification Severe soft-tissue damage may
Closed reduction is feasible, but prolonged immobi-
lead to heterotopic ossification. Forced movement
lization may result in a stiff elbow. Operative treat-
should be avoided.
ment is therefore preferred. The fragment is always
larger than expected. If it can be securely replaced, it
is fixed in position with a small screw. Headless bone
FRACTURED CAPITULUM screws are ideally passed from front to back; alterna-
tively, if the fragment is large enough, lag screws can
This is a rare articular fracture which occurs only in be passed from back to front. If this proves too diffi-
adults. The patient falls on the hand, usually with the cult, the fragment is best excised. Movements are
elbow straight. The anterior part of the capitulum is commenced as soon as discomfort permits. The
sheared off and displaced proximally. longer term outcome is not always good because of
stiffness and sometimes instability.
Clinical features
Fullness in front of the elbow is the most notable fea-
ture. The lateral side of the elbow is tender and flex-
ion is grossly restricted. FRACTURED HEAD OF RADIUS
Radial head fractures are common in adults but are

X-Ray
hardly ever seen in children (probably because the
In the lateral view the capitulum (or part of it) is seen proximal radius is mainly cartilaginous) whereas radial
752 in front of the lower humerus, and the radial head no neck fractures occur in children more frequently.
Mechanism of injury A single large fragment (Type II) If the fragment is 24
displaced, it should be reduced and held with one or
A fall on the outstretched hand with the elbow two small headless screws.
extended and the forearm pronated causes impaction
of the radial head against the capitulum. The radial A comminuted fracture (Type III) This is a challenging
head may be split or broken. In addition, the articular injury. Always assess for an associated soft tissue injury:

cartilage of the capitulum may be bruised or chipped; Rupture of the medial collateral ligament;
this cannot be seen on x-ray but is an important Rupture of the interosseous membrane (Essex
complication. The radial head is also sometimes frac- Lopresti lesion);
tured during elbow dislocation. Combined fractures of the radial head and coronoid
process plus dislocation of the elbow – the
Clinical features ‘terrible triad’.
This fracture is sometimes missed, but tenderness on If any of these is present, excision of the radial head is
pressure over the radial head and pain on pronation contra-indicated; this may lead to intractible insta-
and supination should suggest the diagnosis. bility of the elbow or forearm. The head must be
meticulously reconstructed with small headless screws
or replaced with a metal spacer. A medial collateral
X-ray rupture, if unstable after replacing or fixing the radial
Three types of fracture are identified and classified by head, should be repaired.
Mason as: Radial head excision usually gives a good long-term
result if there are no contra-indications; however,
Type I An undisplaced vertical split in the wrist pain from ulnar head impaction, valgus instabil-
radial head ity of the elbow and trochleo-olecranon arthritis can
Type II A displaced single fragment of the head develop.
Type III The head broken into several
fragments (comminuted).
Complications
An additional Type IV has been proposed, for those
fractures with an associated elbow dislocation. Joint stiffness is common and may involve both the
Special radial head views, rather than simple PA and elbow and the radioulnar joints. Even with minimally
lateral views are needed to fully assess the fracture. displaced fractures the elbow can take several months
The wrist also should be x-rayed to exclude a con- to recover, and stiffness may occur whether the radial
comitant injury of the distal radioulnar joint, which head has been excised or not.
would signify damage to the interosseous membrane Myositis ossificans is an occasional complication.
(acute longitudinal radioulnar dissociation). Recurrent instability of the elbow can occur if the
medial collateral ligament was also injured and the
radial head excised.
Treatment
Worthwhile pain relief can
An undisplaced split (Type I)
be achieved by aspirating the haematoma and injecting
local anaesthetic. The arm is held in a collar and cuff FRACTURE OF THE RADIAL NECK
for 3 weeks; active flexion, extension and rotation are
encouraged. The prognosis for this injury is very good, In adults, a displaced fracture of the radial neck may
although there is often some loss of elbow extension. need open reduction; if so, a mini-plate can be
24.25 Fractured head of

radius There are three main
types of adult radial head
fracture: (a) a chisel-like split
of head, (b) a marginal
fracture or (c) a comminuted
fracture. Displaced marginal
fractures can often be treated
by (d) internal fixation.
(a) (b) (c) (d) 753

24 applied, making sure not to damage the articular sur- therefore damages the articular cartilage. With trans-
face. An alternative is to use oblique headless screws. verse fractures, the triceps aponeurosis sometimes
remains intact, in which case the fracture fragments
stay together.
FRACTURES OF THE OLECRANON

Clinical features
Two broad types of injury are seen: (1) a comminuted A graze or bruise over the elbow suggests a commin-
fracture which is due to a direct blow or a fall on the uted fracture; the triceps is intact and the elbow can
elbow; and (2) a transverse break, due to traction be extended against gravity. With a transverse fracture
when the patient falls onto the hand while the triceps there may be a palpable gap and the patient is unable
muscle is contracted. These two types can be further to extend the elbow against resistance.
sub-classified into (a) displaced and (b) undisplaced
fractures. More severe injuries may be associated also
with subluxation or dislocation of the ulno-humeral
X-ray
joint. A properly orientated lateral view is essential to show
The fracture always enters the elbow joint and details of the fracture, as well as the associated joint
damage. Always check the position of the radial head
– it may be dislocated.
Treatment
A comminuted fracture with the triceps intact should
be treated as a severe ‘bruise’. Many of these patients
are old and osteoporotic, and immobilizing the elbow
will lead to stiffness. The arm is rested in a sling for a
week; a further x-ray is obtained to ensure that there
is no displacement and the patient is then encouraged
(a) (b)
to start active movements.
An undisplaced transverse fracture that does not
separate when the elbow is x-rayed in flexion can be
treated closed. The elbow is immobilized by a cast in
about 60 degrees of flexion for 2–3 weeks and then
exercises are begun. Repeat x-rays are needed to
exclude displacement.
Displaced transverse fractures can be held only by
splinting the arm absolutely straight – but stiffness in
that position would be disastrous. Operative treat-
(c) (d)
ment is therefore strongly recommended. The frac-
24.26 Fractured olecranon (a,b) Comminuted fractures, ture is reduced and held by tension band wiring.
undisplaced and displaced. (c,d) Transverse fractures, Oblique fractures may need a lag screw, neutralised by
undisplaced and displaced. a tension band system or plate.
(a) (b) (c)
24.27 Fractured olecranon (a) Slightly displaced transverse fracture. (b) Markedly displaced transverse fracture – the
754 extensor mechanism is no longer intact. Treatment in this case was by open reduction and tension-band wiring (c).
Displaced comminuted fractures need a plate and Mechanism of injury and pathology 24
often bone graft. In the osteoporotic bone of low-
demand elderly patients, good results can be achieved The cause of posterior dislocation is usually a fall on
with excision of fragments and re-attachment of tri- the outstretched hand with the elbow in extension.
ceps to the ulna. If the coronoid portion of the joint Disruption of the capsule and ligaments structures
is intact it will reduce the risk of instability. Following alone can result in posterior or posterolateral disloca-

operation, early mobilization should be encouraged. tion. However, provided there is no associated frac-
ture, reduction will usually be stable and recurrent
dislocation unlikely. The combination of ligamentous
Complications disruption and fracture of the radial head, coronoid
Stiffness used to be common, but with secure internal process or olecranon process (or, worse still, several
fixation and early mobilization the residual loss of fractures) will render the joint more unstable and,
movement should be minimal. unless the fractures are reduced and fixed, liable to re-
Non-union sometimes occurs after inadequate dislocation.
reduction and fixation. If elbow function is good, it Once posterior dislocation has taken place, lateral
can be ignored; if not, rigid internal fixation and bone shift may also occur. Soft tissue disruption is often
grafting will be needed. considerable and surrounding nerves and vessels may
Ulnar nerve symptoms can develop. These usually be damaged. Although certain common patterns of
settle spontaneously. fracture-dislocation are recognized (based on the par-
Osteoarthritis is a late complication, especially if ticular combination of structures involved), high-
reduction is less than perfect. This can usually be energy injuries do not necessarily follow any rules. A
treated symptomatically. classic example is the so-called side-swipe injury which
occurs, typically, when a car-driver’s elbow, protrud-
ing through the window, is struck by another vehicle.
The result is forward dislocation with fractures of any
DISLOCATION OF THE ELBOW or all of the bones around the elbow; soft-tissue dam-
age (including neurovascular injury) is usually severe.
Dislocation of the ulno-humeral joint is fairly com-
mon – more so in adults than in children. Injuries are
Clinical features
usually classified according to the direction of dis-
placement. However, in 90% of cases the radioulnar The patient supports his forearm with the elbow in
complex is displaced posteriorly or posterolaterally, slight flexion. Unless swelling is severe, the deformity
often together with fractures of the restraining bony is obvious. The bony landmarks (olecranon and epi-
processes. condyles) may be palpable and abnormally placed.
(b)
24.28 Dislocation of the elbow X-rays

showing (a) lateral and (b) posterior
displacement.
(a) 755
24 However, in severe injuries pain and swelling are so Head of radius The combination of ligament
marked that examination of the elbow is impossible. disruption and a type II or III radial head fracture is
Nevertheless, the hand should be examined for signs an unstable injury; stability is restored only by healing
of vascular or nerve damage. or repair of the ligaments and restoration of the radial
pillar – either by fracture fixation or (in the case of a
comminuted fracture) by prosthetic replacement of the
X-ray
radial head. The medial collateral ligament may also be

X-ray examination is essential (a) to confirm the pres- repaired to protect the radial head fixation or implant
ence of a dislocation and (b) to identify any associated from undue valgus stress.
fractures. It is often only when the elbow is screened
Olecranon process In the rare forward dislocation of
at the time of surgery that the full extent of the injury
the elbow, the olecranon process may fracture; a large
can be established.
piece of the olecranon is left behind as a separate
fragment. Open reduction with internal fixation is the
Treatment best treatment.
UNCOMPLICATED DISLOCATION Side-swipe injuries These severe fracture-dislocations
The patient should be fully relaxed under anaesthesia. are often associated with damage to the large vessels of
The surgeon pulls on the forearm while the elbow is the arm. The priorities are repair of any vascular injury,
slightly flexed. With one hand, sideways displacement skeletal stabilization and soft tissue coverage. This is
is corrected, then the elbow is further flexed while the demanding surgery, necessitating a high level of
olecranon process is pushed forward with the thumbs. expertise, and is best undertaken in a unit specialising
Unless almost full flexion can be obtained, the olecra- in upper limb injuries.
non is not in the trochlear groove.
In cases where the elbow remains
Persistent instability
After reduction, the elbow should be put through a
unstable after the bone and joint anatomy has been
full range of movement to see whether it is stable. The
restored, a hinged external fixator can be applied in
distal nerves and circulation are checked again. In
order to maintain mobility while the tissues heal.
addition, an x-ray is obtained to confirm that the joint
is reduced and to disclose any associated fractures.
The arm is held in a collar and cuff with the elbow Complications
flexed above 90 degrees. After 1 week the patient gen-
Complications are common; some are potentially so
tly exercises his elbow; at 3 weeks the collar and cuff
serious that the patient with a dislocation or a frac-
is discarded. Elbow movements are allowed to return
ture-dislocation of the elbow must be observed with
spontaneously and are never forced. The long-term
the closest attention.
results are usually good.
EARLY
DISLOCATION WITH ASSOCIATED FRACTURES
Vascular injury The brachial artery may be damaged.
Coronoid process Coronoid fractures have been
Absence of the radial pulse is a warning. If there are
classified by Regan and Morrey as:
other signs of ischaemia, this should be treated as an
Type I Avulsion of the tip. A benign enough emergency. Splints must be removed and the elbow
injury, but it can represent a should be straightened somewhat. If there is no
substantial soft-tissue injury of the improvement, an arteriogram is performed; the
elbow brachial artery may have to be explored.
Type II A single or comminuted fracture of the
Nerve injury The median or ulnar nerve is sometimes
coronoid with 50 per cent or less
injured. Spontaneous recovery usually occurs after 6–
involved. This is usually not repaired
8 weeks.
surgically, as the elbow remains stable
Type III A single or comminuted fracture
LATE
involving more than 50 per cent. If the
Stiffness Loss of 20 to 30 degrees of extension is not
elbow is unstable after reduction, then
uncommon after elbow dislocation; fortunately this is
fixation is usually needed.
usually of little functional significance. The most com-
Medial epicondyle An avulsed medial epicondyle is, for mon cause of undue stiffness is prolonged immobi-
practical purposes, a medial ligament disruption. If the lization. In the management of all elbow injuries the
epicondylar fragment is displaced, it must be reduced joint should be moved as soon as possible, with due
and fixed back in position. The arm and wrist are consideration to stability of the fractures and soft tis-
splinted with the elbow at 90 degrees; after 3 weeks sues and without undue passive stretching of the soft
756 movements are begun under supervision. tissues. For injuries requiring prolonged splintage, a
hinged elbow brace, or on some occasions a hinged Osteoarthritis Secondary osteoarthritis is quite 24
external fixator, can allow some movement in the common after severe fracture-dislocations. In older
flexion-extension plane whilst protecting against patients, total elbow replacement can be considered.
collateral stress.
Persistent stiffness of severe degree can often be
improved by anterior capsular release. However, oper-
ISOLATED DISLOCATION OF THE

ative treatment should not be rushed; remember that
sometimes the stiffness is due to myositis ossificans, RADIAL HEAD
which is usually undetectable on plain x-ray examina-
tion until a month or more after injury. A true isolated dislocation of the radial head is very
rare; if it is seen, search carefully for an associated frac-
Heterotopic ossification (myositis ossificans)Heterotopic ture of the ulna (the Monteggia injury). In a child,
bone formation may occur in the damaged soft tissues the ulnar fracture may be difficult to detect if it is
in front of the joint. It is due to muscle bruising or incomplete, either green-stick or plastic deformation
haematoma formation; however the precise of the shaft; it is very important to identify these
pathogenesis is not known. In former years ‘myositis incomplete fractures because even a minor deformity,
ossificans’ was a fairly common complication of elbow if it is allowed to persist, may prevent full reduction of
injury, usually associated with forceful reduction and the radial head dislocation.
overenthusiastic passive movement of the elbow.
Nowadays it is rarely seen, but it is as well to be alert
for signs such as slight swelling, excessive pain and
tenderness around the front of the elbow, along with FRACTURES AROUND THE ELBOW
tardy recovery of active movements.
X-ray examination is initially unhelpful; soft-tissue
IN CHILDREN
ossification is usually not visible until 4–6 weeks after
injury. If the condition is suspected, exercises are The elbow is second only to the distal forearm for fre-
stopped and the elbow is splinted in comfortable flex- quency of fractures in children. Most of these injuries
ion until pain subsides; gentle active movements and are supracondylar fractures, the remainder being
continuous passive motion are then resumed. Anti- divided between condylar, epicondylar and proximal
inflammatory drugs may help to reduce stiffness; they radial and ulnar fractures. Boys are injured more often
are also used prophylactically to reduce the risk of het- than girls and more than half the patients are under
erotopic bone formation. 10 years old.
A bone mass which markedly restricts movement The usual accident is a fall directly on the point of
and elbow function can be excised, though not before the elbow or – more often – onto the outstretched
the bone is fully ‘mature’, i.e. has well-defined corti- hand with the elbow forced into valgus or varus. Pain
cal margins and trabeculae (as seen on x-ray). and swelling are often marked and examination is dif-
ficult. X-ray interpretation also has its problems: The
Unreduced dislocation A dislocation may not have been bone ends are largely cartilaginous and therefore radi-
diagnosed; or only the backward displacement ographically incompletely visualized. A good knowl-
corrected, leaving the olecranon process still displaced edge of the normal anatomy is essential if fracture
sideways. Up to 3 weeks from injury, manipulative displacements are to be recognized.
reduction is worth attempting but care is needed to
avoid fracturing one of the bones. Other than this,
Points of anatomy
there is no satisfactory treatment. Open reduction can
be considered, but a wide soft tissue release is required, The elbow is a complex hinge, providing sufficient
which predisposes to yet further stiffness. Alternatively, mobility to permit the upper limb to reach through
the condition can be left, in the hope that the elbow wide ranges of flexion, extension and rotation, yet also
will regain a useful range of movement. If pain is a enough stability to support the necessary gripping,
problem, the patient can be offered an arthrodesis or pushing, pulling and carrying activities of daily life. Its
an arthroplasty. stability is due largely to the shape and fit of the bones
that make up the joint – especially the humero-ulnar
Recurrent dislocation This is rare unless there is a large component – and this is liable to be compromised by
coronoid fracture or radial head fracture. If recurrent any break in the articulating structures. The sur-
elbow instability occurs, the lateral ligament and rounding soft-tissue structures also are important,
capsule can be repaired or re-attached to the lateral especially the capsular and collateral ligaments and, to
condyle. A cast with the elbow at 90 degrees is worn a lesser extent, the muscles. Ligament disruption is
for 4 weeks. also, therefore, a destabilizing factor. 757
24 The forearm is normally in slight valgus in relation orly, sometimes injuring the brachial artery or median
to the upper arm, the average carrying angle in chil- nerve.
dren being about 15 degrees. (Published measure- Anterior displacement is rare; it is thought to be
ments range from 5 to 25 degrees). When the elbow due to direct violence (e.g. a fall on the point of the
is flexed, the forearm comes to lie directly upon the elbow) with the joint in flexion.
upper arm. Doubts about the normality of these fea-
tures can usually be resolved by comparing the injured

Classification
with the normal arm.
With the elbow flexed, the tips of the medial and Type I is an undisplaced fracture.
lateral epicondyles and the olecranon prominence Type II is an angulated fracture with the posterior
form an isosceles triangle; with the elbow extended, cortex still in continuity.
they lie transversely in line with each other. IIA – a less severe injury with the distal fragment
Though all the epiphyses are in some part cartilagi- merely angulated.
nous, the secondary ossific centres can be seen on x- IIB – a severe injury; the fragment is both
ray; they should not be mistaken for fracture angulated and malrotated.
fragments! The average ages at which the ossific cen- Type III is a completely displaced fracture (although
tres appear are easily remembered by the mnemonic the posterior periosteum is usually still preserved,
CRITOE: Capitulum – 2 years. Radial head – 4 years. which will assist surgical reduction).
Internal (medial) epicondyle – 6 years. Trochlea – 8
years. Olecranon – 10 years. External (lateral) epi-
condyle – 12 years. Obviously epiphyseal displace-
Clinical features
ments will not be detectable on x-ray before these Following a fall, the child is in pain and the elbow is
ages. Fracture displacement and accuracy of reduction swollen; with a posteriorly displaced fracture the
can be inferred from radiographic indices such as Bau- S-deformity of the elbow is usually obvious and the
mann’s angle (see Fig. 24.30). bony landmarks are abnormal. It is essential to feel the
pulse and check the capillary return; passive extension
of the flexor muscles should be pain-free. The wrist
SUPRACONDYLAR FRACTURES and the hand should be examined for evidence of
nerve injury.
These are among the commonest fractures in chil-
dren. The distal fragment may be displaced either pos-
X-ray
teriorly or anteriorly.
The fracture is seen most clearly in the lateral view. In
an undisplaced fracture the ‘fat pad sign’ should raise
Mechanism of injury suspicions: there is a triangular lucency in front of the
Posterior angulation or displacement (95 per cent of distal humerus, due to the fat pad being pushed for-
all cases) suggests a hyperextension injury, usually due wards by a haematoma.
to a fall on the outstretched hand. The humerus In the common posteriorly displaced fracture the
breaks just above the condyles. The distal fragment is fracture line runs obliquely downwards and forwards
pushed backwards and (because the forearm is usually and the distal fragment is tilted backwards and/or
in pronation) twisted inwards. The jagged end of the shifted backwards. In the anteriorly displaced fracture
proximal fragment pokes into the soft tissues anteri- the crack runs downwards and backwards and the
(a) (b) (c) (d)
24.29 Supracondylar fractures X-rays showing supracondylar fractures of increasing severity. (a) Undisplaced.
(b) Distal fragment posteriorly angulated but in contact. (c) Distal fragment completely separated and displaced posteri-
758 orly. (d) A rarer variety with anterior angulation.
24.30 Baumann’s angle 24
Anteroposterior x-rays are sometimes
difficult to make out, especially if the
elbow is held flexed after reduction of
the supracondylar fracture.
Measurement of Baumann’s angle is
helpful. This is the angle subtended

by the longitudinal axis of the
humeral shaft and a line through the
coronal axis of the capitellar physis, as
shown in (a) the x-ray of a normal
elbow and the accompanying
diagram (b). Normally this angle is
less than 80 degrees. If the distal
fragment is tilted in varus, the
(a) (b) (c) increased angle is readily detected (c).
distal fragment is tilted forwards. On a normal lateral general anaesthesia by the following step-wise
x-ray, a line drawn along the anterior cortex of the manoeuvre: (1) traction for 2–3 minutes in the length
humerus should cross the middle of the capitulum. If of the arm with counter-traction above the elbow;
the line is anterior to the capitulum then a Type II (2) correction of any sideways tilt or shift and rotation
fracture is suspected. (in comparison with the other arm); (3) gradual
An anteroposterior view is often difficult to obtain flexion of the elbow to 120 degrees, and pronation of
without causing pain and may need to be postponed the forearm, while maintaining traction and exerting
until the child has been anaesthetized. It may show finger pressure behind the distal fragment to correct
that the distal fragment is shifted or tilted sideways, posterior tilt. Then feel the pulse and check the capil-
and rotated (usually medially). Measurement of Bau- lary return – if the distal circulation is suspect, imme-
mann’s angle is useful in assessing the degree of diately relax the amount of elbow flexion until it
medial angulation before and after reduction (Fig. improves. X-rays are taken to confirm reduction,
24.30). checking carefully to see that there is no varus or val-
gus angulation and no rotational deformity. The
anteroposterior view is confusing and unreliable with
Treatment
the elbow flexed, but the important features can be
If there is even a suspicion of a fracture, the elbow is inferred by noting Baumann’s angle. Again, subtle
gently splinted in 30 degrees of flexion to prevent medial tilt and rotation of the distal fragment must be
movement and possible neurovascular injury during recognised. If the acutely flexed position cannot be
the x-ray examination. maintained without disturbing the circulation, or if
the reduction is unstable, (and most of these fractures
TYPE I: UNDISPLACED FRACTURE are unstable!) the fracture should be fixed with percu-
The elbow is immobilized at 90 degrees and neutral taneous crossed K-wires (take care not to skewer the
rotation in a light-weight splint or cast and the arm is ulnar nerve!).
supported by a sling. It is essential to obtain an x-ray Following reduction, the arm is held in a collar and
5–7 days later to check that there has been no dis- cuff; the circulation should be checked repeatedly
placement. The splint is retained for 3 weeks and during the first 24 hours. An x-ray is obtained after 3–
supervised movement is then allowed. 5 days to confirm that the fracture has not slipped.
The capitulum normally angles forward about 30 The splint is retained for 3 weeks, after which move-
degrees; if the capitulum is in a straight line with the ments are begun.
humerus on the lateral x-ray, it will still remodel.
Even with Type I fractures, care must be taken to TYPES II B AND III: ANGULATED AND MALROTATED
recognise any medial tilt of the distal fragment on the OR POSTERIORLY DISPLACED
anteroposterior x-ray, otherwise cubitus varus can These are usually associated with severe swelling, are
result. Measure Baumann’s angle. difficult to reduce and are often unstable; moreover,
there is a considerable risk of neurovascular injury or
TYPE II A: POSTERIORLY ANGULATED FRACTURE – circulatory compromise due to swelling. The fracture
MILD should be reduced under general anaesthesia as soon
In these cases swelling is usually not severe and the as possible, by the method described above, and then
risk of vascular injury is low. If the posterior cortices held with percutaneous crossed K-wires; this obviates
are in continuity, the fracture can be reduced under the necessity to hold the elbow acutely flexed. 759
24
(a) (b) (c) (d)
(e) (f) (g) (h) (i)
24.31 Supracondylar fractures – treatment (a) The uninjured arm is examined first; (b) traction of the fractured arm;
(c) correcting lateral shift and tilt; (d) correcting rotation; (e) correcting backwards shift and tilt; (f) feeling the pulse;
the elbow is kept well flexed while x-ray films are taken. (h) For the first 3 weeks the arm is kept under the clothes; after
this (i) it is outside the clothes.
Smooth wires should be used (this lessens the risk of with the elbow semi-flexed, applying thumb pressure
physeal injury) and great care should be taken not to over the front of the distal fragment and then extend-
injure the ulnar, radial and median nerves. Postopera- ing the elbow fully. Crossed percutaneous pins are
tive management is the same as for Type II A. used if unstable. A posterior slab is bandaged on and
retained for 3 weeks. Thereafter, the child is allowed
OPEN REDUCTION to regain flexion gradually.
This is sometimes necessary for (1) a fracture which
simply cannot be reduced closed; (2) an open frac-
ture; or (3) a fracture associated with vascular dam-
Complications
age. The fracture is exposed (preferably through two EARLY
incisions, one on each side of the elbow), the Vascular injury The great danger of supracondylar
haematoma is evacuated and the fracture is reduced fracture is injury to the brachial artery, which, before
and held by two crossed K-wires. the introduction of percutaneous pinning, was
reported as occurring in over 5 per cent of cases.
CONTINUOUS TRACTION Nowadays the incidence is probably less than 1 per
Traction through a screw in the olecranon, with the cent. Peripheral ischaemia may be immediate and
arm held overhead, can be used (1) if the fracture is severe, or the pulse may fail to return after reduction.
severely displaced and cannot be reduced by manipu- More commonly the injury is complicated by forearm
lation; (2) if, with the elbow flexed 100 degrees, the oedema and a mounting compartment syndrome
pulse is obliterated and image intensification is not which leads to necrosis of the muscle and nerves
available to allow pinning and then straightening of without causing peripheral gangrene. Undue pain plus
the elbow; or (3) for severe open injuries or multiple one positive sign (pain on passive extension of the
injuries of the limb. Once the swelling subsides, a fur- fingers, a tense and tender forearm, an absent pulse,
ther attempt can be made at closed reduction. blunted sensation or reduced capillary return on
pressing the finger pulp) demands urgent action. The
TREATMENT OF ANTERIORLY DISPLACED FRACTURES flexed elbow must be extended and all dressings
This is a rare injury (less than 5 per cent of supra- removed. If the circulation does not promptly improve,
condylar fractures). However, ‘posterior’ fractures are then angiography (on the operating table if it saves
sometimes inadvertently converted to ‘anterior’ ones time) is carried out, the vessel repaired or grafted and
by excessive traction and manipulation. a forearm fasciotomy performed. If angiography is not
760 The fracture is reduced by pulling on the forearm available, or would cause much delay, then Doppler
imaging should be used. In extreme cases, operative Elbow stiffness and myositis ossifficansStiffness is an 24
exploration would be justified on clinical criteria alone. ever-present risk with elbow injuries. Extension in
particular may take months to return. It must not be
Nerve injury The radial nerve, median nerve hurried. Passive movement (which includes carrying
(particularly the anterior interosseous branch) or the weights) or forced movement is prohibited – this will
ulnar nerve may be injured. Tests for nerve function only make matters worse and may contribute to the

are described in Chapter 11. Fortunately loss of development of myositis ossificans. As it is, myositis
function is usually temporary and recovery can be ossificans is extremely rare, and should remain so if
expected in 3 to 4 months. If there is no recovery the rehabilitation is properly supervised.
nerve should be explored. However, if a nerve,
documented as intact prior to manipulation, is then
found to have failed after manipulation, then
entrapment in the fracture is suspected and immediate FRACTURES OF THE LATERAL CONDYLE
exploration should be arranged.
The lateral condylar (or capitellar) epiphysis begins to
The ulnar nerve may be damaged by careless pin-
ossify during the first year of life and fuses with the
ning. If the injury is recognized, and the pin removed,
shaft at 12–16 years. Between these ages it may be
recovery will usually follow.
sheared off or avulsed by forceful traction.
LATE
Malunion Malunion is common. However, backward
or sideways shifts are gradually smoothed out by The child falls on the hand with the elbow extended
modelling during growth and they seldom give rise to and forced into varus. A large fragment, which
visible deformity of the elbow. Forward or backward includes the lateral condyle, breaks off and is pulled
tilt may limit flexion or extension, but consequent upon by the attached wrist extensors. Sometimes
disability is slight. there is a compression, rather than avulsion, mecha-
Uncorrected sideways tilt (angulation) and rotation nism of injury. The fracture line usually runs along the
are much more important and may lead to varus (or physis and into the trochlea; less often it continues
rarely valgus) deformity of the elbow; this is permanent through the medial epiphysis and exits through the
and will not improve with growth (Fig. 24.32). The capitulatrochlear groove. It crosses the growth plate
fracture is extra-physeal and so physeal damage should and so is a Salter Harris Type IV injury. In severe
not be blamed for the deformity; usually it is faulty re- injuries the elbow may dislocate posterolaterally; the
duction which is responsible. Cubitus varus is disfigur- condyle is ‘capsized’ by muscle pull and remains cap-
ing and cubitus valgus may cause late ulnar palsy. If de- sized while the elbow reduces spontaneously.
formity is marked, it will need correction by The extent of this injury is often not appreciated.
supracondylar osteotomy usually once the child ap- Because the condylar epiphysis is largely cartilaginous,
proaches skeletal maturity. the bone fragment may look deceptively small on
(a) (b) (c)
24.32 Supracondylar fracture – malunion (a) Varus deformity of the right elbow, due to incomplete correction of the
varus and rotational displacements in a supracondylar fracture. (b) It is most obvious when the boy raises his arms, dis-
playing the typical ‘gunstock deformity’. (c) X-ray showing the characteristic malunion. 761
24
(a) (b)
24.33 Physeal fractures of the lateral condoyle (a) (a) (b)

The commonest is a fracture starting in the metaphysi and
running along the physis of the lateral condyle into the 24.34 Fractured lateral condyle If displacement is more
trochlea (Salter–Harris Type II injury). (b) Less common is a than 2 mm, open reduction and internal fixation is the
fracture running right through the lateral condyle to reach treatment of choice.
the articular surface in the capitulotrochlear groove
(Salter–Harris Type IV): though uncommon, this latter injury
is important because of its potential for causing growth X-ray
defects.
X-ray examination must include oblique views or else
the full extent of the fracture may be missed. Two
types of fracture are recognized and classified by
Milch:
x-ray. Displacement can be quite marked due to Type I: A fracture lateral to the trochlea: the elbow
muscle pull. The fracture is important for two reasons: joint is not involved and is stable.
(a) it may damage the growth plate and (b) it always Type II: A fracture through the middle of the
involves the joint. Early recognition and accurate trochlea: this injury is more common; the
reduction are therefore essential if a poor outcome is elbow is unstable as the radius and ulna are
to be avoided. carried along with the fragment.. The
fragment is often grossly displaced and
capsized, and it may carry with it a
Clinical features triangular piece of the metaphysis.
The elbow is swollen and deformed. There is tender- Remember that the fragment (partly
ness over the lateral condyle. Passive flexion of the cartilaginous) is much larger than it seems
wrist (pulling on the extensors) may be painful. on x-ray.
24.35 Fractured lateral condyle –

complications (a,b) A large frag-
ment of bone and cartilage is
avulsed; even with reasonable reduc-
tion, union is not inevitable. (c) Open
reduction with fixation is often wise.
(d) Sometimes the condyle is
capsized; if left unreduced
non-union is inevitable (e) and a
(a) (b) (c)
valgus elbow with delayed ulnar
palsy (f) the likely sequel.
762 (d) (e) (f)

Treatment to 3 months) open reduction and fixation should be 24
attempted.
If there is no displacement the arm can be splinted in
a backslab with the elbow flexed 90 degrees, the fore- Recurrent dislocation Occasionally condylar displace-
arm neutral and the wrist extended (this position ment results in posterolateral dislocation of the elbow.
relaxes the extensor mechanism which attaches to the The only effective treatment is reconstruction of the
bony and soft tissues on the lateral side.

fragment). However, it is essential to repeat the x-ray
after 5 days to make sure that the fracture has not dis-
placed. The splint is removed after 2 weeks and exer-
cises are encouraged. SEPARATION OF THE MEDIAL
A displaced fracture requires accurate reduction and
internal fixation. If the fragment is only moderately EPICONDYLE
displaced (hinged), it may be possible to manipulate it
into position by extending the elbow and pressing
upon the condyle, and then fixing the fragment with The medial epicondyle begins to ossify at the age of
percutaneous pins. If this fails, and for all separated about 5 years and fuses to the shaft at about 16;
fractures, open reduction and internal fixation with between these ages it may be avulsed by a severe mus-
pins is required. The arm is immobilized in a cast; cast cle or ligament strain. The child falls on the out-
and pins are removed after 3 or 4 weeks. stretched hand with the wrist and elbow extended; the
elbow is wrenched into valgus. The unfused epi-
condylar apophysis is avulsed by tension on either the
Complications
wrist flexor muscles or the medial ligament of the
Non-union and malunion If the condyle is left capsized, elbow. If the elbow subluxates (even momentarily),
non-union is inevitable; with growth the elbow the small apophyseal fragment may be dragged into
becomes increasingly valgus, and ulnar nerve palsy is the joint. With more severe injuries the joint dislo-
then likely to develop. Stiffness and pain can result. cates laterally.
Even minor displacements sometimes lead to non-
union, and even slight malunion may lead to ulnar
palsy in later life; it is for these reasons that open
Clinical features
reduction (and internal fixation) is preferred for any The diagnosis should be suspected if injury is fol-
displaced fracture. The fracture is a Salter Harris Type lowed by pain, swelling and bruising on the medial
IV injury and so imperfect reduction can result in side of the elbow. If the joint is dislocated, deformity
growth arrest. Even if a fracture presents late (e.g. up is of course obvious. Sensation and power in the fin-
24.36 Fractured medial

epicondyle (a) Avulsion of
the medial epicondyle
following valgus train.
(b) Avulsion associated with
dislocation of the elbow;
(c) after reduction.
Sometimes the epicondylar
fragment is trapped in the
joint (d,e); the serious
nature is then liable to be
missed unless the surgeon
(a) (b) (c)
specifically looks for the
trapped fragment, which is
emphasized in the tracings
(f,g).
(d) (e) (f) (g) 763

24 gers should be tested to exclude concomitant ulnar elbow forced into valgus; in the latter case it would be
nerve damage. an avulsion injury. The fracture line runs through the
physis, exiting in the trochlear notch or even further
laterally, and the medial fragment may be displaced by
X-ray
the pull of the flexor muscle group.
In the anteroposterior view the medial epicondylar
epiphysis may be tilted or shifted downwards; if the

Clinical features and x-ray
joint is dislocated the fragment lies distal to the lower
humerus. A lateral view may show the epicondyle This is an intra-articular fracture, resulting in consid-
looking like a loose body in the joint. If in any doubt, erable pain and swelling. In older children the meta-
the normal side should be x-rayed for comparison (see physeal component is usually easily visualized on
Fig. 24.36 d–g). x-ray. However, in young children much of the medial
condylar epiphysis is cartilaginous and therefore not
visible on x-ray, so the full extent of the fracture may
Treatment not be recognized; seeing only the epicondylar ossific
Minor displacement may be disregarded. This is an centre in a displaced position on the x-ray may mis-
extra-articular fracture, so the elbow can be mobilized lead the surgeon into thinking that this is only an epi-
as soon as the child wishes. condylar fracture. In doubtful cases an arthrogram
If the epicondyle is trapped in the joint it must be may be helpful.
freed. Manipulation with the elbow in valgus and the
wrist hyperextended (to pull on the flexor muscles)
may be successful; if this fails, the joint must be
Treatment
opened (the ulnar nerve must be visualized and pro- Undisplaced fractures are treated by splintage; x-rays
tected) and the fragment retrieved and fixed back in are repeated until the fracture has healed, so as to
position. ensure that it does not become displaced.
Displaced fractures which are not trapped in the Displaced fractures are treated by either closed
joint usually do not need to be operated upon: how- reduction (sometimes with percutaneous pinning) or
ever, if there is valgus instability (because the medial by open reduction and fixation with pins.
collateral ligament complex is attached to the frag- Postoperative management is similar to that of
ment) then reduction and pinning is recommended. lateral condyle fractures.
Complications Complications
EARLY EARLY
Ulnar nerve damage is not uncommon. Mild symp- Lateral dislocation of the elbow occasionally occurs
toms recover spontaneously; even a complete palsy with a severe valgus strain and avulsion of the medial
will usually recover but, if there is the possibility that condyle. Early reduction of both the dislocation and
the nerve is kinked in the joint, exploration should be the fracture, if necessary by open operation and pin-
considered. ning, is important.
Ulnar nerve damage is not uncommon, but recov-
LATE ery is usual unless the nerve is left kinked in the joint.
Stiffness of the elbow is common and extension often
limited for months; but, provided movement is not LATE
forced, it will eventually return. Stiffness of the elbow is common and extension often
limited for months; but, provided movement is not
forced, it will eventually return.
FRACTURES OF THE MEDIAL CONDYLE
This is much rarer than either a fracture of the lateral
condyle or a separation of the medial epicondylar
FRACTURE-SEPARATION OF THE
apophysis. DISTAL HUMERAL PHYSIS
Up to the age of 7 years the distal humeral epiphysis

Mechanism of injury is a solid cartilaginous segment with maturing centres
The injury is usually caused by a fall from a height, of ossification. With severe injury it may separate en
involving either a direct blow to the point of the bloc. This is likely to occur with fairly severe violence;
764 elbow or a landing on the outstretched hand with the for example, in birth injuries or child abuse.
Clinical features 24.37 Fractured neck of 24
radius in a child Up to
The child is in pain and the elbow is markedly swollen. 30° of tilt is acceptable.
The history may be deceptively uninformative. Greater degrees of angula-
tion should be reduced;
never excise the radial head
X-ray in a child.

In a very young child, in whom the bony outlines are
still unformed, the x-ray may look normal. All that can
be seen of the epiphysis is the pea-like ossification cen-
tre of the capitulum; its position should be compared
with that of the normal side. Medial displacement of
either the capitellar ossification centre or the proximal
radius and ulna is very suspicious. In the older child
the deformity is usually obvious.
Treatment head tilt and up to 3 mm of transverse displacement

If the diagnosis is uncertain, arthrography or ultra- are acceptable. The arm is rested in a collar and cuff,
sound can help. If the fracture is undisplaced, the and exercises are commenced after a week.
elbow is merely splinted for 3 weeks; if displaced then Displacement of more than 30 degrees requires
the fracture should be accurately reduced and held reduction. With the patient’s elbow extended, trac-
with smooth percutaneous wires (otherwise there is a tion and varus force are applied; the surgeon then
high incidence of cubitus varus). The wires are pushes the displaced radial fragment into position
removed at 3 weeks. with his thumb. If this fails, a percutaneous imple-
ment can be used to push the fragment back into
place. Open reduction is occasionally performed if
significant displacement persists. The radial head tilt is
FRACTURED NECK OF RADIUS corrected but internal fixation is unnecessarily med-
dlesome. The head of the radius must never be
Mechanism of injury and pathology excised in children because this will interfere with the
A fall on the outstretched hand forces the elbow into synchronous growth of radius and ulna.
valgus and pushes the radial head against the capitu- Fractures that are seen a week or longer after injury
lum. In children the bone fractures through the neck should be left untreated (except for light splintage).
of the radius; in adults the injury is more likely to frac- Following operation, the elbow is splinted in 90
ture the radial head. degrees of flexion for a week or two and then move-
ments are encouraged.
Clinical features
Following a fall, the child complains of pain in the SUBLUXATION OF THE RADIAL HEAD
elbow. There may be localized tenderness over the
radial head and pain on rotating the forearm.
(‘PULLED ELBOW’)
In young children the elbow may be injured by
X-ray pulling on the arm, usually with the forearm
pronated. It is sometimes called subluxation of the
The fracture line is transverse. It is either situated radial head; more accurately, it is a subluxation of the
immediately distal to the physis or there is true sepa- orbicular ligament which slips up over the head of the
ration of the epiphysis with a triangular fragment of radius into the radiocapitellar joint.
shaft (a Salter-Harris II injury). The proximal frag- A child aged 2 or 3 years is brought with a painful,
ment is tilted distally, forwards and outwards. Some- dangling arm: there is usually a history of the child
times the upper end of the ulna is also fractured or being jerked by the arm and crying out in pain. The
there may be a posterior dislocation of the elbow. forearm is held in pronation and extension, and any
attempt to supinate it is resisted. There are no x-ray
Treatment changes.
A dramatic cure is achieved by forcefully supinating
In children there is considerable potential for remod- and then flexing the elbow; the ligament slips back
elling after these fractures. Up to 30 degrees of radial with a snap. 765
24 Modabber MR, Jupiter JB. Reconstruction for post-
FRACTURE OF THE OLECRANON IN traumatic conditions of the elbow joint. J Bone Joint Surg
CHILDREN 1995; 77A: 1431–46.
Morrey BF. Current concepts in the treatment of fractures
This is rare. When it does occur it is usually due to a
of the radial head, the olecranon and coronoid. J Bone
direct blow onto the tip of the flexed elbow or a fall
Joint Surg 1995; 77A: 316–27.
onto the outstretched hand. Most are undisplaced and
Neer CS II. Displaced proximal humeral fractures. Classifi-

are treated in a splint for 3 or 4 weeks. If displaced, then
cation and evaluation. J Bone Joint Surg 1970; 52A:
they should be reduced and held with wires.
1077–89.
O’Hara LJ, Barlow JW, Clarke NMP. Displaced supra-
REFERENCES AND FURTHER READING condylar fractures of the humerus in children. J Bone Joint
Surg 2000; 82B: 204–210.
Boileau P, Sinnerton RJ, Chuinard C, Walch G. Arthro- Ring D, Jupiter JB. Fracture-dislocation of the elbow.
plasty of the shoulder. J Bone Joint Surg 2006; 88B: 562– J Bone Joint Surg 1998; 80A: 566–80.
75. Robinson CM. Fractures of the clavicle in the adult. Epi-
Goss TP. Fractures of the glenoid cavity. J Bone Joint Surg demiology and classification. J Bone Joint Surg 1998;
1992; 74A: 299–305. 80B: 476–84.
Hertel R, Hempfing A, Stiehler M, Leunig M. Predictors Rockwood CA Jr, Green DP, Bucholz RW, Heckman JD
of humeral head ischemia after intracapsular fracture of (eds). Rockwood and Green’s Fractures in Adults, 4th
the proximal humerus. J Shoulder Elbow Surg, 2004; 13: Edition. 1996 Lippincott-Raven, Philadelphia.
427–33. Snow M, Funk L. Technique of arthroscopic Weaver–Dunn
Jupiter JB. Complex fractures of the distal part of the in chronic acromioclavicular joint dislocation. Techniques
humerus J Bone Joint Surg 1994; 76A: 1252–63. in Shoulder and Elbow Surgery 2006; 7: 155–9.
McKee MD, Pedersen EM, Jones C. Deficits following Williams GR, Naranja J, Klimkiewcz J et al. The floating
nonoperative treatment of displaced midshaft clavicular shoulder: a biomechanical basis for classification and
fractures. J Bone Joint Surg 2006; 88A: 35–40. management. J Bone Joint Surg 2001; 83A: 1182–7.
766
Injuries of the forearm
and wrist 25
David Warwick
ral deficit. Repeated examination is necessary in order

FRACTURES OF THE RADIUS AND to detect an impending compartment syndrome.
ULNA
X-RAY
Both bones are broken, either transversely and at the
same level or obliquely with the radial fracture usually
Fractures of the shafts of both forearm bones occur at a higher level. In children, the fracture is often
quite commonly. A twisting force (usually a fall on the incomplete (greenstick) and only angulated. In adults,
hand) produces a spiral fracture with the bones displacement may occur in any direction – shift, over-
broken at different levels. An angulating force causes lap, tilt or twist. In low-energy injuries, the fracture
a transverse fracture of both bones at the same level. tends to be transverse or oblique; in high-energy
A direct blow causes a transverse fracture of just one injuries it is comminuted or segmental.
bone, usually the ulna. Additional rotation deformity
may be produced by the pull of muscles attached to
the radius: they are the biceps and supinator muscles
Treatment
to the upper third, the pronator teres to the middle CHILDREN
third, and the pronator quadratus to the lower third. In children, closed treatment is usually successful
Bleeding and swelling of the muscle compartments of because the tough periosteum tends to guide and
the forearm may cause circulatory impairment. then control the reduction. The fragments are held in
a well-moulded full-length cast, from axilla to
metacarpal shafts (to control rotation). The cast is
Clinical features
applied with the elbow at 90 degrees. If the fracture is
The fracture is usually quite obvious, but the pulse must proximal to pronator teres, the forearm is supinated;
be felt and the hand examined for circulatory or neu- if it is distal to pronator teres, then the forearm is held
25.1 Fractured radius and ulna in children

Greenstick fractures (a) need only correction
of angulation (b), and plaster splintage. Com-
plete fractures (c) are harder to reduce; but
provided alignment is corrected and held in
plaster (d), slight lateral shift remodels with
growth (e).
(a) (b) (c) (d) (e)

25 in neutral. The position is checked by x-ray after a
week and, if it is satisfactory, splintage is retained until
both fractures are united (usually 6–8 weeks).
Throughout this period hand and shoulder exercises
are encouraged. The child should avoid contact sports
for a few weeks to prevent re-fracture.
Occasionally an operation is required, either if the (a)

fracture cannot be reduced or if the fragments are
unstable. Fixation with intramedullary rods is pre-
ferred, but they should be inserted with great care to
avoid injury to the growth plates. Alternatively, a plate
or K-wire fixation can be used.
Childhood fractures usually remodel well, but not if
there is any rotational deformity or an angular defor-
mity of more than 15 degrees in children under 6 (b)
years or 10 degrees in children between 6 and 12. In
those over 12 years old even slight angular deformities
are unlikely to remodel satisfactorily.
ADULTS
Unless the fragments are in close apposition, reduction
(c)
is difficult and re-displacement in the cast almost in-
variable. So predictable is this outcome that most sur- 25.3 Fractured radius and ulna – cross-union If the
geons opt for open reduction and internal fixation from interosseous membrane is severely damaged, even
successful plating (a,b) cannot guarantee that cross-union
the outset. The fragments are held by interfragmentary will not occur (c).
compression with plates and screws. Bone grafting is
advisable if there is comminution. The deep fascia is left
open to prevent a build-up of pressure in the muscle range of movement exercises are commenced but lift-
compartments, and only the skin is sutured. ing and sports are avoided. It takes 8–12 weeks for the
After the operation the arm is kept elevated until bones to unite. With comminuted fractures or unreli-
the swelling subsides, and during this period active able patients, immobilization in plaster is safer.
exercises of the hand are encouraged. If the fracture is
not comminuted and the patient is reliable, early OPEN FRACTURES
Open fractures of the forearm must be managed
meticulously. Antibiotics and tetanus prophylaxis are
given as soon as possible; the wounds are copiously
washed and nerve function and circulation are
checked. At operation the wounds are excised and
extended and the bone ends are exposed and thor-
oughly cleaned. The fractures are primarily fixed with
compression screws and plates; if the wounds are
absolutely clean, the soft tissues can be closed. If bone
grafting is necessary, this is best deferred until the
wounds are healed. If there is major soft-tissue loss,
the bones are better stabilized by external fixation.
The aim is to obtain skin cover as soon as possible; if
plastic surgery services are available, these should be
enlisted from the outset.
If there is any question of a compartment syn-
drome, the wounds should be left open and closed
24–48 hours later, with a skin graft if needed.
(a) (b) (c) (d)
25.2 Fractured radius and ulna in adults (a, b) These Complications

fractures are usually treated by internal fixation with sturdy
plates and screws. However, removal of the implants is not EARLY
without risk. (c,d) In this case, the radius fractured Nerve injury Nerve injuries are rarely caused by the
768 through one of the screw holes. fracture, but they may be caused by the surgeon!
osteotomy. However, it can be very difficult to 25
calculate the deformity and subsequent correction.
Complications of plate removalRemoval of plates and
screws is often regarded as a fairly innocuous
procedure. Beware! Complications are common and
they include damage to vessels and nerves, infection
Injuries of the forearm and wrist

(a) and fracture through a screw-hole.
FRACTURE OF A SINGLE FOREARM BONE

Fracture of the radius alone is very rare and fracture
of the ulna alone is uncommon. These injuries are
usually caused by a direct blow – the ‘nightstick
(b) fracture’. They are important for two reasons:
25.4 Compartment syndrome Incisions to relieve a • An associated dislocation may be undiagnosed; if
compartment syndrome in the forearm. only one forearm bone is broken along its shaft and
there is displacement, then either the proximal or
the distal radio-ulnar joint must be dislocated. The
Exposure of the radius in its proximal third risks entire forearm, elbow and wrist should always be
damage to the posterior interosseous nerve where it is x-rayed.
covered by the superficial part of the supinator muscle. • Non-union is liable to occur unless it is realized that
The proximal fragment of radius may have rotated so one bone takes just as long to consolidate as two.
the nerve may not be where it is expected. Surgical
technique is particularly important here; the anterior Clinical features
Henry approach is safest.
Ulnar fractures are easily missed – even on x-ray. If
Vascular injury Injury to the radial or ulnar artery there is local tenderness, a further x-ray a week or two
seldom presents any problem, as the collateral later is wise.
circulation is excellent.
X-ray The fracture may be anywhere in the radius or
Compartment syndrome Fractures (and operations) of ulna. The fracture line is transverse and displacement is
the forearm bones are always associated with swelling slight. In children, the intact bone sometimes bends
of the soft tissues, with the attendant risk of a without actually breaking (‘plastic deformation’).
compartment syndrome. The threat is even greater,
and the diagnosis more difficult, if the forearm is
wrapped up in plaster. A distal pulse does not exclude Treatment
compartment syndrome! The byword is ‘watchfulness’; Isolated fracture of the ulna The fracture is rarely
if there are any signs of circulatory embarrassment, displaced; a forearm brace leaving the elbow free can
treatment must be prompt and uncompromising. be sufficient. However, it takes about 8 weeks before
full activity can be resumed. Rigid internal fixation will
LATE allow earlier activity and avoids the risk of displacement
Delayed union and non-union Most fractures of the or non-union.
radius and ulna heal within 8–12 weeks; high energy
fractures and open fractures are less likely to unite. Isolated fracture of the radius Radius fractures are prone
Delayed union of one or other bone (usually the ulna) to rotary displacement; to achieve reduction in
is not uncommon; immobilization may have to be children the forearm needs to be supinated for upper
continued beyond the usual time. Non-union will third fractures, neutral for middle third fractures and
require bone grafting and internal fixation. pronated for lower third fractures. The position is
sometimes difficult to hold in children and just about
Malunion With closed reduction there is always a risk impossible in adults; if so, then internal fixation with a
of malunion, resulting in angulation or rotational compression plate and screws in adults, and preferably
deformity of the forearm, cross-union of the intramedullary rods in children, is better.
fragments, or shortening of one of the bones and
disruption of the distal radio-ulnar joint. If pronation Middle/distal third fractures of the radius in children These
or supination is severely restricted, and there is no are particularly unstable, being deformed by the pull
cross-union, mobility may be improved by corrective of the thumb abductors and pronator quadratus. They 769
25
(a) (b) (c) (d) (e) (f) (g)
25.5 Fracture of one forearm bone Fracture of the ulna: A fracture of the ulna alone (a) usually joins satisfactorily (b);
in children the intact radius may be bowed (c). Fracture of the radius: In a child, fracture of the radius alone (d) may join
in plaster (e), but in adults a fractured radius (f) is better treated by plating (g).
can be treated with an above-elbow cast in supination head usually dislocates forwards and the upper third
but, failing that, fixation with an intramedullary rod, of the ulna fractures and bows forwards. Sometimes
Kirschner (K-) wires or a plate is advisable. the causal force is hyperextension.
Clinical features
MONTEGGIA FRACTURE- The ulnar deformity is usually obvious but the dislo-
DISLOCATION OF THE ULNA cated head of radius is masked by swelling. A useful
clue is pain and tenderness on the lateral side of the
elbow. The wrist and hand should be examined for
The injury described by Monteggia in the early nine-
signs of injury to the radial nerve.
teenthth century (without benefit of x-rays!) was a
fracture of the shaft of the ulna associated with dislo- X-ray With isolated fractures of the ulna, it is essential
cation of the proximal radio-ulnar joint; the radio- to obtain a true anteroposterior and true lateral view of
capitellar joint is inevitably dislocated or subluxated as the elbow. In the usual case, the head of the radius
well. More recently the definition has been extended (which normally points directly to the capitulum) is
to embrace almost any fracture of the ulna associated dislocated forwards, and there is a fracture of the upper
with dislocation of the radio-capitellar joint, including third of the ulna with forward bowing. Backward or
trans-olecranon fractures in which the proximal radio- lateral bowing of the ulna (which is much less
ulnar joint remains intact. If the ulnar shaft fracture is common) is likely to be associated with, respectively,
angulated with the apex anterior (the commonest posterior or lateral displacement of the radial head.
type) then the radial head is displaced anteriorly; if the Trans-olecranon fractures, also, are often associated
fracture apex is posterior, the radial dislocation is pos- with radial head dislocation.
terior; and if the fracture apex is lateral then the radial
head will be laterally displaced. In children, the ulnar
injury may be an incomplete fracture (greenstick or
Treatment
plastic deformation of the shaft). The key to successful treatment is to restore the length of
the fractured ulna; only then can the dislocated joint
be fully reduced and remain stable. In adults, this
Mechanism of injury means an operation through a posterior approach.
Usually the cause is a fall on the hand; if at the The ulnar fracture must be accurately reduced, with
moment of impact the body is twisting, its momen- the bone restored to full length, and then fixed with a
770 tum may forcibly pronate the forearm. The radial plate and screws; bone grafts may be added for safety.
with chronic subluxation of the radial head. Because 25
of incomplete ossification of the radial head and
capitellar epiphysis in children, these landmarks may
not be easily defined on x-ray and a proximal disloca-
tion could be missed. The x-rays should be studied
very carefully and if there is any doubt, x-rays should

be taken of the other side for comparison.
Incomplete ulnar fractures can often be reduced
closed, although considerable force is needed to
straighten the ulna with plastic deformation. The
position of the radial head is then checked; if it is not
perfect, closed reduction can be completed by flexing
and supinating the elbow and pressing on the radial
head. The arm is then immobilized in a cast with the
elbow in flexion and supination, for 3 weeks.
Complete fractures are best treated by open reduc-
(a) (b) (c) (d) tion and fixation using an intramedullary rod or a
25.6 Monteggia fracture-dislocation (a) The ulna is
small plate.
fractured and the head of the radius no longer points to
the capitulum. In a child, closed reduction and plaster
(b) is usually satisfactory; in the adult (c) open reduction
and plating (d) is preferred.
GALEAZZI FRACTURE-DISLOCATION
OF THE RADIUS
The radial head usually reduces once the ulna has
been fixed. Stability must be tested through a full
range of flexion and extension. If the radial head does Mechanism of injury
not reduce, or is not stable, open reduction should be
This injury was first described in 1934 by Galeazzi.
performed.
The usual cause is a fall on the hand; probably with a
If the elbow is completely stable, then flexion–
superimposed rotation force. The radius fractures in
extension and rotation can be started after very soon
its lower third and the inferior radio-ulnar joint sub-
after surgery. If there is doubt, then the arm should be
luxates or dislocates.
immobilized in plaster with the elbow flexed for 6
weeks.
Clinical features
Complications The Galeazzi fracture is much more common than the
Monteggia. Prominence or tenderness over the lower
Nerve injury Nerve injuries can be caused by over-
end of the ulna is the striking feature. It may be pos-
enthusiastic manipulation of the radial dislocation or
sible to demonstrate the instability of the radio-ulnar
during the surgical exposure. Always check for nerve
joint by ‘ballotting’ the distal end of the ulna (the
function after treatment. The lesion is usually a
‘piano-key sign’) or by rotating the wrist. It is impor-
neurapraxia, which will recover by itself.
tant also to test for an ulnar nerve lesion, which may
Malunion Unless the ulna has been perfectly reduced, occur.
the radial head remains dislocated and limits elbow
X-ray A transverse or short oblique fracture is seen in
flexion. In children, no treatment is advised. In adults,
the lower third of the radius, with angulation or
osteotomy of the ulna or perhaps excision of the radial
overlap. The distal radio-ulnar joint is subluxated or
head may be needed.
dislocated.
Non-union Non-union of the ulna should be treated
by plating and bone grafting.
Treatment
As with the Monteggia fracture, the important step is
Special features in children to restore the length of the fractured bone. In chil-
The general features of Monteggia fracture-disloca- dren, closed reduction is often successful; in adults,
tions are similar to those in adults. However, it is reduction is best achieved by open operation and
important to remember that the ulnar fracture may be compression plating of the radius. An x-ray is taken to
incomplete (greenstick or plastic deformation); if this ensure that the distal radio-ulnar joint is reduced.
is not detected, and corrected, the child may end up There are three possibilities: 771
25 25.7 Galeazzi fracture-
dislocation The diagrams show
age, transfer of energy, mechanism of injury and bone
quality.
the contrast between With any of these fractures, the wrist also can suffer
(a) Monteggia and (b) Galeazzi
fracture-dislocations. substantial ligamentous injury causing instability to
(c,d) Galeazzi type before and the carpus or distal radio-ulnar joint. These injuries
after reduction and plating. are easily missed because the x-rays may look normal.
COLLES’ FRACTURE
The injury that Abraham Colles described in 1814 is
a transverse fracture of the radius just above the wrist,
with dorsal displacement of the distal fragment. It is
(a) (b) the most common of all fractures in older people, the
high incidence being related to the onset of post-
menopausal osteoporosis. Thus the patient is usually
an older woman who gives a history of falling on her
outstretched hand.
Mechanism of injury and pathological

anatomy
Force is applied in the length of the forearm with the
wrist in extension. The bone fractures at the cortico-
cancellous junction and the distal fragment collapses
into extension, dorsal displacement, radial tilt and
(c) (d) shortening.
The distal radio-ulnar joint is reduced and stable No

further action is needed. The arm is rested for a
few days, then gentle active movements are
encouraged. The radio-ulnar joint should be
checked, both clinically and radiologically, during
the next 6 weeks.
The distal radio-ulnar joint is reduced but unstable
The forearm should be immobilized in the
position of stability (usually supination),
supplemented if required by a transverse K-wire.
The forearm is splinted in an above-elbow cast for
6 weeks. If there is a large ulnar styloid fragment, (a) (b)
it should be reduced and fixed.
The distal radio-ulnar joint is irreducible This is
unusual. Open reduction is needed to remove the
interposed soft tissues. The triangular fibrocartilage
complex (TFCC) and dorsal capsule are then
carefully repaired and the forearm immobilized in
the position of stability (again, usually supination,
supported by a wire if needed) for 6 weeks.
(c) (d)
FRACTURES OF THE DISTAL RADIUS
IN ADULTS 25.8 Colles’ fracture (a,b) The typical Colles‘ fracture is
both displaced and angulated towards the dorsum and
towards the radial side of the wrist. (c,d) Note, how, after
The distal end of the radius is subject to many differ- successful reduction, the radial articular surface faces
772 ent types of fracture, depending on factors such as correctly both distally and slightly volarwards.
Clinical features circumference of the wrist. It is held in position by a 25
crepe bandage. Extreme positions of flexion and ulnar
We can recognize this fracture (as Colles did long deviation must be avoided; 20 degrees in each direc-
before radiography was invented) by the ‘dinner-fork’ tion is adequate.
deformity, with prominence on the back of the wrist The arm is kept elevated for the next day or two;
and a depression in front. In patients with less shoulder and finger exercises are started as soon as

deformity there may only be local tenderness and pain possible. If the fingers become swollen, cyanosed or
on wrist movements. painful, there should be no hesitation in splitting the
X-ray There is a transverse fracture of the radius at the bandage.
corticocancellous junction, and often the ulnar styloid At 7–10 days fresh x-rays are taken; re-displacement
process is broken off. The radial fragment is impacted is not uncommon and should be treated, if the
into radial and backward tilt. Sometimes there is an patient’s functional demands are high, by re-manipu-
intra-articular fracture; sometimes it is severely lation and internal fixation. However, in some elderly
comminuted. patients with low functional demands, modest degrees
of displacement should be accepted because (a) out-
come in these patients is not so dependent upon
Treatment anatomical perfection, and (b) fixation of the fragile
UNDISPLACED FRACTURES bone can be very difficult.
If the fracture is undisplaced (or only very slightly dis- The fracture unites in about 6 weeks and, even in
placed), a dorsal splint is applied for a day or two until the absence of radiological proof of union, the slab
the swelling has resolved, then the cast is completed. may safely be discarded and exercises begun.
An x-ray is taken at 10–14 days to ensure that the frac-
ture has not slipped; if it has, surgery may be required; IMPACTED OR COMMINUTED COLLES’ FRACTURES
if not, the cast can usually be removed after four With substantial impaction or comminution in osteo-
weeks to allow mobilization. porotic bone, manipulation and plaster immobiliza-
tion alone may be insufficient. The fracture can some-
DISPLACED FRACTURES times be reduced and held with percutaneous wires, but
Displaced fractures must be reduced under anaesthe- if impaction is severe even this may not be enough to
sia (haematoma block, Bier’s block or axillary block). maintain length; in that case, an external fixator is used
The hand is grasped and traction is applied in the to neutralize the compressive force of the 25 tendons
length of the bone (sometimes with extension of the crossing the wrist, and bone graft or bone substitute is
wrist to disimpact the fragments); the distal fragment placed into the gap. The fixator is attached to the dis-
is then pushed into place by pressing on the dorsum tal radius and the second metacarpal shaft. It should be
while manipulating the wrist into flexion, ulnar devia- used only as a neutralizing device; too much distraction
tion and pronation. The position is then checked by will lead to stiffness. The fixation is removed after 5–6
x-ray. If it is satisfactory, a dorsal plaster slab is applied, weeks and exercises begun.
extending from just below the elbow to the Plate fixation is increasingly being used for some
metacarpal necks and two-thirds of the way round the Colles’ fractures. The so-called ‘volar locking plate’ is
23°
12mm
1mm
11°
(a) (b) (c)
25.9 Colles‘ fracture – operative fixation (a) Comminuted Colles’ fracture reduced and held with percutaneous wires.
Make sure that the articular surface angles are correctly restored (b,c). 773
25 applied to the front of the radius through the bed of be swelling and tenderness of the finger joints, a
flexor carpi radialis. The screws are fixed to the plate warning not to neglect the daily exercises. In about 5
itself and are passed into the relatively stronger sub- per cent of cases, by the time the plaster is removed the
chondral bone distally. These devices, which are flour- hand is stiff and painful and there are signs of
ishing in the orthopaedic marketplace, allow stable vasomotor instability. X-rays show osteoporosis and
fixation and thus early mobilization of the forearm. there is increased activity on the bone scan.
Other devices, such as a locked intramedullary nail or

TFCC injury TFCC injury is more common than is
crossed K-wires, are also suitable for the distal radius.
generally appreciated. As the distal radius displaces
dorsally, the TFCC is damaged; the ulnar styloid
Outcome fracture which commonly accompanies a Colles’
fracture illustrates the forces which are transmitted to
As Colles himself recognized, the outcome of these
the TFCC, which attaches in part to it.
fractures in an older age group with lower functional
demands is usually good, regardless of the cosmetic or
LATE
the radiographic appearance. Poor outcomes can
Malunion Malunion is common, either because
often be improved by performing a corrective
reduction was not complete or because displacement
osteotomy. The amount of displacement that can be
within the plaster was overlooked. The appearance is
accepted depends on patient factors such as age, co-
ugly, and weakness and loss of rotation may persist. In
morbidity, functional demands, handedness, and qual-
most cases treatment is not necessary. Where the
ity of bone, and treatment factors such as surgical skill
disability is severe and the patient relatively young, the
and implants available. As a rule, shortening of more
lower 1.5 cm of the ulna may be excised to restore
than 2 mm at the distal radio-ulnar joint, dorsal tilt of
rotation, and the radial deformity corrected by
more than 10 degrees and dorsal translation of more
osteotomy.
than 30 per cent are likely to lead to a poor outcome
and early correction should be considered. This advice Delayed union and non-union Non-union of the radius
applies to older osteopaenic fractures; in younger is rare, but the ulnar styloid process often joins by
patients the tolerances are far less! fibrous tissue only and remains painful and tender for
several months.
Complications Stiffness Stiffness of the shoulder, elbow and fingers
from neglect is a common complication. Stiffness of
EARLY
the wrist may follow prolonged splintage.
Circulatory problems The circulation in the fingers must
be checked; the bandage holding the slab may need to Tendon rupture Rupture of extensor pollicis longus
be split or loosened. occasionally occurs a few weeks after an apparently
trivial undisplaced fracture of the lower radius. The
Nerve injury Direct injury is rare, but compression of
patient should be warned of the possibility and told
the median nerve in the carpal tunnel is fairly common.
that operative treatment is available.
If it occurs soon after injury and the symptoms are
mild, they may resolve with release of the dressings and
elevation. If symptoms are severe or persistent, the
transverse ligament should be divided. SMITH’S FRACTURE
Reflex sympathetic dystrophy This condition is probably Smith (a Dubliner, like Colles) described a similar
quite common, but fortunately it seldom progresses to fracture about 20 years later. However, in this injury
the full-blown picture of Sudeck’s atrophy. There may the distal fragment is displaced anteriorly (which is
(a) (b) (c) (d)
25.10 Colles’ fracture-complications (a) Rupture of extensor pollicis longus; (b) malunion – CT scan showing
incongruity of the distal radio-ulnar joint; (c) infected K-wire; (d) failed fixation as the wires have cut through the
774 osteoporotic bone.
fractures should be fixed with percutaneous wires or a 25
plate.
DISTAL FOREARM FRACTURES IN

CHILDREN
The distal radius and ulna are among the commonest

sites of childhood fractures. The break may occur
through the distal radial physis or in the metaphysis of
one or both bones. Metaphyseal fractures are often
(a) (b) incomplete or greenstick.
25.11 Smith’s fracture (a,b) Here, in contrast to Colles’
fracture, the displacement of the lower radial fragment is Mechanism of injury
forwards – not backwards.
The usual injury is a fall on the outstretched hand
with the wrist in extension; the distal fragment is
why it is sometimes called a ‘reversed Colles’). It is
forced posteriorly (this is often called a ‘juvenile
caused by a fall on the back of the hand.
Colles’ fracture’). However, sometimes the wrist is in
flexion and the fracture is angulated anteriorly. Lesser
Clinical features force may do no more than buckle the metaphyseal
cortex (a type of compression fracture, or torus frac-
The patient presents with a wrist injury, but there is
ture).
no dinner-fork deformity. Instead, there is a ‘garden
spade’ deformity.
Clinical features
X-ray There is a fracture through the distal radial
metaphysis; a lateral view shows that the distal There is usually a history of a fall, though this may be
fragment is displaced and tilted anteriorly – the passed off as one of many childhood spills. The wrist
opposite of a Colles’ fracture. The entire metaphysis is painful, and often quite swollen; sometimes there is
can be fractured, or there can be an oblique fracture an obvious ‘dinner-fork’ deformity.
exiting at the dorsal or volar rim of the radius.
X-ray The precise diagnosis is made on the x-ray
appearances.
Treatment Physeal fractures are almost invariably Salter–Harris
The fracture is reduced by traction, supination and type I or II, with the epiphysis shifted and tilted
extension of the wrist, and the forearm is immobilized backwards and radially. Type V injuries are unusual;
in a cast for 6 weeks. X-rays should be taken at 7–10 sometimes they are diagnosed in retrospect when
days to ensure the fracture has not slipped. Unstable premature epiphyseal fusion occurs.
(a) (b) (c) (d) (e) (f)
25.12 Distal forearm fractures in children (a,b) In older children the fracture is usually slightly more proximal than a
true Colles’, and often merely a greenstick or buckling injury. (c,d) In young children physeal fractures are usually Salter–
Harris type I or II. In this case, accurate reduction has been achieved (e,f). 775
25 Metaphyseal injuries may appear as mere buckling of Radio-ulnar discrepancy Premature fusion of the radial
the cortex (easily missed unless appropriate views epiphysis may result in bone length disparity and sub-
are obtained), as angulated greenstick fractures or luxation of the radio-ulnar joint. If this is troublesome,
as complete fractures with displacement and the radius can be lengthened and, if the child is near to
shortening. If only the radius is fractured, the ulna skeletal maturity, the ulnar physis fused surgically.
may be bent though not fractured.
Treatment RADIO-CARPAL FRACTURES

Physeal fractures are reduced, under anaesthesia, by
pressure on the distal fragment. The arm is immobi-
lized in a full-length cast with the wrist slightly flexed
FRACTURED RADIAL STYLOID
and ulnar deviated, and the elbow at 90 degrees. The This injury is caused by forced radial deviation of the
cast is retained for 4 weeks. These fractures very rarely wrist and may occur after a fall, or when a starting
interfere with growth. Even if reduction is not handle ‘kicks back’ – the so-called ‘chauffeur’s frac-
absolutely perfect, further growth and modelling will ture‘. The fracture line is transverse, extending later-
obliterate any deformity. Patients seen more than 2 ally from the articular surface of the radius; the
weeks after injury are best left untreated. fragment, much more than the radial styloid, is often
Buckle fractures require no more than 2 weeks in undisplaced. The radial styloid can also be fractured as
plaster, followed by another 2 weeks of restricted part of the far more serious trans-scaphoid perilunate
activity. fracture dislocation.
Greenstick fractures are usually easy to reduce – but
apt to re-displace in the cast! Some degree of angula-
tion can be accepted: in children under 10, up to 30 Treatment
degrees and in children over 10, up 15 degrees. If the If there is displacement it is reduced, and the wrist is
deformity is greater, the fracture is reduced by thumb held in ulnar deviation by a plaster slab round the outer
pressure and the arm is immobilized with three-point forearm extending from below the elbow to the
fixation in a full-length cast with the wrist and forearm metacarpal necks. Imperfect reduction may lead to os-
in neutral and the elbow flexed 90 degrees. The cast teoarthritis; therefore if closed reduction is imperfect the
is changed and the fracture re-x-rayed at 2 weeks; if it fragment should be screwed back, or held with K-wires.
has re-displaced a further manipulation can be carried
out. The cast is finally discarded after 6 weeks.
Complete fractures can be embarrassingly difficult to
reduce – especially if the ulna is intact. The fracture is
FRACTURE-SUBLUXATION (BARTON’S
manipulated in much the same way as a Colles’ frac- FRACTURE)
ture; the reduction is checked by x-ray and a full-
VOLAR SUBLUXATION
length cast is applied with the wrist neutral and the
The true Barton’s injury is a volar fracture of the distal
forearm supinated. After 2 weeks, a check x-ray is
radius associated with volar subluxation of the carpus.
obtained; the cast is kept on for 6 weeks. If the frac-
ture slips, especially if the ulna is intact, it should be
stabilized with a percutaneous K-wire.
Complications
EARLY
Forearm swelling and threatened compartment syndrome
This dire combination can be prevented by avoiding
over-forceful or repeated manipulations, splitting the
plaster, elevating the arm for the first 24–48 hours and
encouraging exercises.
LATE
Malunion This late sequel is uncommon in children
under 10 years of age. Deformity of as much as 30
degrees will straighten out with further growth and (a) (b)
remodelling over the next 5 years. This should be 25.13 Fractured radial styloid (a) X-ray; (b) fixation
776 carefully explained to the worried parents. with cannulated percutaneous screw.
25
(a)

(a)
(b) (c) (b) (c)
25.14 Fracture-subluxation (Barton’s fracture) 25.15 Comminuted fracture of the distal radius The
(a,b) The true Barton’s fracture is a split of the volar edge ‘die punch fragment’ of the lunate fossa of the distal
of the distal radius with anterior (volar) subluxation of the radius (a,b) must be perfectly reduced and fixed; here this
wrist. This has been reduced and held (c) with a small has been achieved by closed reduction and percutaneous
anterior plate. K-wire fixation (c). The wires can be used as ‘joy sticks’ to
manipulate the fragment back before fixation.
It is sometimes mistaken for a Smith’s fracture, but it closed K-wiring or open reduction and plating is
differs from the latter in that the fracture line runs advisable.
obliquely across the volar lip of the radius into the
wrist joint; the distal fragment is displaced anteriorly,
carrying the carpus with it. Because the fragment is
small and unsupported, the fracture is inherently COMMINUTED INTRA-ARTICULAR
unstable. FRACTURES IN YOUNG ADULTS
Treatment The fracture can be easily reduced, but it is
In the young adult, a comminuted intra-articular frac-
just as easily re-displaced. Internal fixation, using a
ture is a high energy injury. A poor outcome will
small anterior buttress plate, is recommended.
result unless intra-articular congruity, fracture align-
ment and length are restored and movements started
DORSAL SUBLUXATION
as soon as possible. For these patients a much higher
This is sometimes called a ‘dorsal Barton’s fracture’.
standard must be set than would be accepted for the
Here the line of fracture runs obliquely across the
typical osteoporotic fracture. In addition to the usual
dorsal lip of the radius and the carpus is carried pos-
posteroanterior and lateral x-rays, oblique views and
teriorly.
often CT scans are useful to show the fragment align-
Treatment The fracture is easier to control than the ment.
volar Barton’s. It is reduced closed and the forearm is The simplest option is a manipulation and cast. If
immobilized in a cast for 6 weeks. If it re-displaces, the anatomy is not restored, then an open reduction
25.16 High energy

injuries in younger
patients Perfect
reduction is required.
(a) (b) (c) (d) 777

25 COMPLICATIONS OF RADIO-CARPAL
FRACTURES
Associated injuries of the carpus Injuries of the carpus
are easily overlooked while attention is focussed on the
radius. Carpal injuries must be excluded by careful
clinical and x-ray examination, occasionally

supplemented by MRI or arthroscopy.
Re-displacement There is a strong tendency for
Barton’s fracture to re-displace if it is held in a cast;
hence our preference for internal fixation.
(a)
The patient may present years later
Carpal instability
with chronic carpal instability. The wrist injury may
have been overlooked at the time.
Secondary osteoarthritis Fractures into the joint and
carpal instability may eventually lead to secondary os-
teoarthritis. It is difficult to predict when (or even
whether) this is likely to occur; symptoms develop
slowly and disability is often not severe. Warning symp-
toms are restricted wrist movement and loss of grip
strength. If pain and weakness interfere significantly
with function, arthrodesis of the wrist may be need, es-
pecially if it is the dominant side which is affected.
(b) CARPAL INJURIES

25.17 Distal radius fracture Options include simple
plaster (a) or external fixation (b) depending on the Fractures and dislocations of the carpal bones are
amount of comminution, stability of the fracture and common. They vary greatly in type and severity. These
patient demands. should never be regarded as isolated injuries; the entire
carpus suffers, and sometimes, long after the fracture
may be necessary. The medial complex must be has healed, the patient still complains of pain and
anatomically reduced, which may require open reduc- weakness in the wrist.
tion through dorsal and palmar approaches and a The commonest wrist injuries are: sprains of the
combination of wires, plates, screws and bone grafts. capsule and ligaments; fracture of a carpal bone
(a) (b) (c)

25.18 Don’t forget the ulna (a) Fracture of radius and ulna, both unstable. (b) Both bones fixed. (c) Ulnar styloid
778 fracture fixed to prevent instability of distal radio-ulnar joint.
(usually the scaphoid); injury of the triangular fibro- fractures, the ‘jump spot’ is in the anatomical snuff- 25
cartilage complex (TFCC) and distal radio-ulnar box and scaphoid tubercle; for scapho-lunate injuries,
joint; dislocations of the lunate or the bones around just beyond Lister’s tubercle; for lunate dislocation, in
it; and subluxations and ‘carpal collapse’, which may the middle of the wrist; for triquetral injuries, beyond
be acute or chronic. the head of the ulna; for hamate fractures, at the base
of the hypothenar eminence; for triangular fibrocarti-

lage complex injuries, over the dorsum of the ulno-
Clinical assessment
carpal joint. Movements are often limited (more by
Following a fall, the patient complains of pain in the pain than by stiffness) and they may be accompanied
wrist. There may be swelling or well-marked defor- by a palpable catch or an audible clunk.
mity of the joint. Tenderness should be carefully local-
ized; undirected prodding will confuse both the
patient and the examiner. The blunt end of a pencil is
Imaging
helpful in testing for point tenderness. For scaphoid X-rays are the key to diagnosis. There are three
golden rules:
• Accept only high-quality films
• If the initial x-rays are ‘normal’, treat the clinical
diagnosis
• Repeat the x-ray examination 2 weeks later.
Initially three standard views are obtained: antero-
posterior and lateral with the wrist neutral, and an
oblique ‘scaphoid’ view. If these are normal and clini-
cal features suggest a carpal injury, further views are
(a) obtained: anteroposterior x-rays with the wrist first in
maximum ulnar and then in maximum radial deviation,
and an anteroposterior view with the fist clenched.
The examiner should be familiar with the normal
x-ray anatomy of the carpus in all the standard views,
so that he or she can visualize a three-dimensional pic-
ture from the two-dimensional, overlapping images of
the carpal bones.
In the anteroposterior x-rays note the shape of the
carpus, whether the individual bones are clearly out-
lined and whether there are any abnormally large gaps
(b) suggesting disruption of the ligaments. The scaphoid
may be fractured; or it may have lost its normal bean
shape and look squat and foreshortened, sometimes
with an inner circular density (the cortical ring sign) –
features of an end-on view when the bone is hyper-
flexed because of damage to the restraining scapho-
lunate ligament. The lunate is normally quadrilateral
in shape, but if it is dislocated it looks triangular.
In the lateral x-ray the axes of the radius, lunate,
capitate and third metacarpal are co-linear, and the
scaphoid projects at an angle of about 45 degrees to
(c) this line. With traumatic instability the linked carpal
segments collapse (like the buckled carriages of a
25.19 Carpal instability – x-ray patterns (a) Normal
derailed train). Two patterns are recognized: dorsal
lateral view. The radius, capitate and middle metacarpal lie
in a straight line and the scaphoid axis is angled at 45º to intercalated segment instability (DISI), in which the
the line of the radius. (b) Dorsal intercalated segmental lunate is torn from the scaphoid and tilted backwards;
instability (DISI). The lunate is tilted dorsally and the and volar intercalated segment instability (VISI), in
scaphoid is tilted somewhat volarwards; the axes of the which the lunate is torn from the triquetrum and
capitate and metacarpals now lie behind (dorsal to) that of
turns towards the palm; the capitate shows a comple-
the radius. (c) Volar intercalated segmental instability
(VISI). The lunate and scaphoid are tilted somewhat mentary dorsal tilt. There may be a flake fracture off
volarwards and the capitate and metacarpals lie anterior the back of a carpal bone (usually the triquetrum).
(volar) to the radius. Special x-ray studies are sometimes helpful: a carpal 779
25 Principles of management
‘Wrist sprain’ should not be diagnosed unless a more
serious injury has been excluded with certainty. Even
with apparently trivial injuries, ligaments are some-
times torn and the patient may later develop carpal
instability.
If the x-rays are normal but the clinical signs
strongly suggest a carpal injury, a splint or plaster
(b)
should be applied for 2 weeks, after which time the x-
rays are repeated. A fracture or dislocation may
(a) become more obvious after a few weeks, but a second
negative x-ray still does not exclude a serious injury. A
bone scan or MRI at this stage will confirm the diag-
nosis and avoid an unnecessary period of immobiliza-
tion and time from work. If these tests are not readily
available, then the patient should be re-examined
repeatedly until the symptoms settle or a firm diagno-
sis is made.
The more common lesions are dealt with below.
(d)
(c)
FRACTURED SCAPHOID
Scaphoid fractures account for almost 75 per cent of
all carpal fractures although they are rare in the elderly
and in children. With unstable fractures there may
also be disruption of the scapho-lunate ligaments and
dorsal rotation of the lunate.
(f)
(e)
25.20 Carpal injuries (a,b) Normal appearances in

antero-posterior and lateral x-rays. (c,d) Following a
‘sprained wrist’ this patient developed persistent pain and
weakness. X-rays showed (c) scapho-lunate dissociation
and (d) dorsal rotation of the lunate (the typical DISI
pattern). (e,f) This patient, too, had a sprained wrist. The
anteroposterior and lateral x-rays show foreshortening of e g
the scaphoid and volar rotation of the lunate (VISI). d
f
c
tunnel view may show a fractured hook of hamate, a
and motion studies in different positions may reveal a b
subluxation. A radioisotope scan will confirm a wrist
injury although it may not precisely localize it.
MRI is sensitive and specific (especially for detect-
ing undisclosed fractures or Kienböck’s disease), but
unless very fine cuts are taken it may miss TFCC and
interosseous ligament tears.
25.21 X-ray appearance of the normal carpus X-ray of

Arthroscopy a normal wrist showing the shape and disposition of the
eight carpal bones: (a) scaphoid; (b) lunate;
Wrist arthroscopy is the best way of demonstrating (c) triquetrum overlain by pisiform; (d) trapezium;
780 TFCC or interosseous ligament tears. (e) trapezoid; (f) capitate; and (g) hamate.
Mechanism of injury and pathological the same place is an important diagnostic sign; the 25
anatomy scaphoid can of course also be palpated from the front
and back of the wrist and it may be tender there as
The scaphoid lies obliquely across the two rows of well. Proximal pressure along the axis of the thumb
carpal bones, and is also in the line of loading between may be painful.
the thumb and forearm. The combination of forced

carpal movement and compression, as in a fall on the
dorsiflexed hand, exerts severe stress on the bone and
it is liable to fracture. Most scaphoid fractures are sta-
X-ray
ble; with unstable fractures the fragments may Anteroposterior, lateral and oblique views are all
become displaced. The distal fragment, unrestrained essential; often a recent fracture shows only in the
by the scapho-lunate ligament, flexes and the proxi- oblique view. Usually the fracture line is transverse,
mal fragment tilts dorsally with the lunate (a DISI and through the narrowest part of the bone (waist),
deformity); the hump-backed deformity of the but it may be more proximally situated (proximal pole
scaphoid is permanent. fracture). Sometimes only the tubercle of the scaphoid
The blood supply of the scaphoid diminishes prox- is fractured.
imally. This accounts for the fact that 1 per cent of dis- It is very important to look for subtle signs of dis-
tal third fractures, 20 per cent of middle third placement or instability: e.g. obliquity of the fracture
fractures and 40 per cent of proximal fractures result line, opening of the fracture line, angulation of the
in non-union or avascular necrosis of the proximal distal fragment and foreshortening of the scaphoid
fragment. image.
A few weeks after the injury the fracture may be
more obvious; if union is delayed, cavitation appears
Clinical features
on either side of the break. Old, un-united fractures
The appearance may be deceptively normal, but the have ‘hard’ borders, making it seem as if there is an
astute observer can usually detect fullness in the extra carpal bone. Relative sclerosis of the proximal
anatomical snuffbox; precisely localized tenderness in fragment is pathognomonic of avascular necrosis.
(a) (b) (c)
(d) (e) (f) (g)
25.22 Fractures of the scaphoid – diagnosis (a) The initial anteroposterior view often fails to show the fracture;
(b) always ask for a ‘scaphoid series’, including two oblique views. If the clinical features are suggestive of a fracture, then
immobilize the wrist and repeat the x-ray 2 weeks later when the fracture is more likely to be apparent. (c) A CT scan is
useful for showing the fracture configuration. The fracture may be (d) through the proximal pole, (e) the waist, or (f) the
scaphoid tubercle. Occasionally these fractures are seen in children (g). 781
25
(a) (b) (c) (d) (e)
(f) (g) (h)
25.23 Fractures of the scaphoid –treatment (a) Scaphoid plaster – position and extent. (b,c) Before and after treat-
ment: in this case radiological union was visible at 10 weeks. (d) Delayed union, treated successfully by (e) bone grafting
and screw fixation. (f) Long-standing stable non-union. (g) Non-union with avascular necrosis and secondary osteoarthritis
treated by (h) scaphoid excision and four-corner fusion.
Treatment that stage, one of two pictures may emerge: (a) the
wrist is painless and the fracture has healed – the cast
Fracture of the scaphoid tubercle needs no splintage can be discarded; (b)the x-ray shows signs of delayed
and should be treated as a wrist sprain; a crepe band- healing (bone resorption and cavitation around the
age is applied and movement is encouraged. Other fracture) – union can be hastened by bone grafting
scaphoid fractures are treated as follows. and internal fixation.
Undisplaced fractures need no reduction and are Displaced fractures can also be treated in plaster,
treated in plaster; 90 per cent of waist fractures should but the outcome is less predictable. It is better to
heal. The cast is applied from the upper forearm to reduce the fracture openly and to fix it with a com-
just short of the metacarpo-phalangeal joints of the pression screw. This should increase the likelihood of
fingers, but incorporating the proximal phalanx of the union and reduce the time of immobilization.
thumb. The wrist is held dorsiflexed and the thumb Some patients may not want to endure a prolonged
forwards in the ‘glass-holding’ position. The plaster period in plaster. Early percutaneous fixation with a
must be carefully moulded into the hollow of the compression screw, though technically demanding,
hand, and is not split. It is retained (and if necessary can dramatically reduce the time away from work and
repaired or renewed) for 8 weeks. the difficulties associated with personal care.
After 8 weeks the plaster is removed and the wrist
examined clinically and radiologically. If there is no
tenderness and the x-ray shows signs of healing, the Complications
wrist is left free; a CT scan is the most reliable means Avascular necrosis The proximal fragment may die,
of confirming union if in doubt. especially with proximal pole fractures, and then at 2–
If the scaphoid is tender, or the fracture still visible 3 months it appears dense on x-ray. Although
782 on x-ray, the cast is reapplied for a further 4 weeks. At revascularization and union are theoretically possible,
they take years and meanwhile the wrist collapses and a trough carved into the front of the scaphoid and 25
arthritis develops. Bone grafting, as for delayed union, again stabilized with a screw or wires. If these
may be successful, in which case the bone, though techniques fail to achieve union then the options are a
abnormal, is structurally intact. If the wrist becomes vascularized bone graft, scaphoidectomy with
painful, the dead fragment can be excised. However, proximal-to-distal-row (four-corner) fusion, proximal
the wrist tends to collapse after this procedure; a better row carpectomy or radio-carpal arthrodesis.

option would be to remove the entire proximal row of In older patients, and those who are completely
carpal bones or else to remove the scaphoid and fuse asymptomatic, non-union may be left untreated.
the proximal to the distal row (four-corner fusion: Sometimes a patient is seen for the first time with a
capitate–hamate–triquetrum–lunate). ‘sprain’, but x-rays show an old, un-united fracture
with sclerosed edges; 3–4 weeks in plaster may suffice
Non-union By 3 months it may be obvious that the
to make him or her comfortable once again, and no
fracture will not unite. Bone grafting should be
further treatment is required.
attempted, especially in the younger, more vigorous
type of patient, because this probably reduces the Osteoarthritis Non-union or avascular necrosis may
chance of later, symptomatic osteoarthritis. Two types lead to secondary osteoarthritis of the wrist. If the
of graft are used. If the scaphoid has folded into a arthritis is localized to the distal pole, excising the
flexed ‘humpback’ shape, then it is approached from radial styloid may help. As the arthritis progresses,
the front and a wedge of cortico-cancellous iliac crest changes appear in the scapho-capitate joint then the
graft is inserted to restore the shape of the bone. The capitate-lunate joint. The lunate-radius joint is never
graft is fixed with a buried screw and/or K-wires. If affected, thus allowing salvage procedures – either
the scaphoid has not collapsed, the graft is inserted into proximal row carpectomy or four-corner fusion.
(a) (b) (c)
(d) (e) (f)
(g) (h)
25.24 Fractures of other carpal bones (a) Fracture of body of trapezium; (b) lunate fracture; (c) lunate fracture; (d)
hook of hamate; (e) hook of hamate CT; (f) capitate fracture fixed (g) with a screw; (h) fracture of body of hamate. 783
25 FRACTURES OF OTHER CARPAL BONES styloid avulsed or the articular surfaces of the ulno-
carpal joint or distal radio-ulnar joint damaged.
Triquetrum
Avulsion of the dorsal ligaments is not uncommon; Clinical features
analgesics and splintage for a few days are all that is There is tenderness over the distal radio-ulnar joint
required. Occasionally the body is fractured; it usually and pain on rotation of the forearm. The distal ulna
heals after 4–6 weeks in plaster. may be unstable; the piano-key sign is elicited by hold-
ing the patient’s forearm pronated and pushing
Hamate sharply forwards on the head of the ulna.
A fracture of the hook of hamate follows a direct blow

to the palm of the hand. These fractures cannot be Imaging and arthroscopy
seen on routine x-rays; a carpal tunnel view, CT or A lateral x-ray in pronation and supination shows
MRI is needed. The fracture does not heal readily; if incongruity of the distal radio-ulnar joint. The antero-
symptoms are prolonged then the fragment is excised, posterior view may show an avulsed ulnar styloid.
taking care not to damage the ulnar nerve. Fractures Arthrography, MRI and arthroscopy may be needed
of the body are rare. They are also difficult to define to confirm the diagnosis.
on plain x-rays. If the CT scan shows a fracture, fixa-
tion may occasionally be needed.
Treatment
Trapezium Instability usually resolves if the arm is held in supina-
tion for 6 weeks; occasionally a K-wire is needed to
The body of the trapezium can be fractured if the maintain the reduction. If the dislocation is irre-
shaft of the first metacarpal impacts onto it; the ridge ducible, this may be due to trapped soft tissue, which
(to which the transverse carpal ligament attaches) can will have to be removed. Chronic instability may
be fractured by a direct blow. The latter fracture can require reconstructive surgery.
usually be seen on a carpal tunnel view rather than A TFCC tear should be repaired and the ulno-
standard x-rays. The body fracture may need open carpal capsule reefed. A displaced fracture at the base
reduction and internal fixation if displaced; the ridge of the ulnar styloid, if painful or associated with insta-
fracture usually settles with splintage for a week or bility of the radio-ulnar joint, should be fixed with a
two. small screw.
Capitate
The capitate is relatively protected within the carpus.
However, in severe trauma the wrist can be fractured; CARPAL DISLOCATIONS,
the distal fragment can rotate, in which case open SUBLUXATIONS AND INSTABILITY
reduction and internal fixation is required.
The wrist functions as a system of intercalated seg-
Lunate ments or links, stabilized by the intercarpal ligaments
and the scaphoid which acts as a bridge between the
Fractures of the lunate are rare and follow a hyperex- proximal and distal rows of the carpus. Fractures and
tension injury to the wrist. There is a real risk of non- dislocations of the carpal bones, or even simple liga-
union; undisplaced fractures should be immobilized ment tears and sprains, may seriously disturb this sys-
in a cast for 6 weeks; displaced fractures should be tem so that the links collapse into one of several
reduced and fixed with a screw. well-recognized patterns (see Chapter 16).
ULNAR-SIDE WRIST INJURIES LUNATE AND PERILUNATE DISLOCATIONS

(see also Chapter 16) A fall with the hand forced into dorsiflexion may tear
the tough ligaments that normally bind the carpal
The distal radio-ulnar joint is often injured with a bones. The lunate usually remains attached to the
radial fracture; it can also be damaged in isolation, radius and the rest of the carpus is displaced back-
particularly after hyperpronation. The triangular wards (perilunate dislocation). Usually the hand
784 fibrocartilage complex (TFCC) can be torn, the ulnar immediately snaps forwards again but, as it does so,
the lunate may be levered out of position to be dis- wards, and the capitate and metacarpals lie behind the 25
placed anteriorly (lunate dislocation). Sometimes the line of the radius (DISI pattern); if there is an associ-
scaphoid remains attached to the radius and the force ated scaphoid fracture, the distal fragment may be
of the perilunar dislocation causes it to fracture flexed.
through the waist (trans-scaphoid perilunate disloca-
tion).
Treatment

Closed reduction The surgeon pulls strongly on the
Clinical features dorsiflexed hand; then, while maintaining traction, he
The wrist is painful and swollen and is held immobile. or she slowly palmarflexes the wrist, at the same time
If the carpal tunnel is compressed there may be squeezing the lunate backwards with his or her other
paraesthesia or blunting of sensation in the territory thumb. These manoeuvres usually effect reduction;
of the median nerve, and weakness of palmar abduc- they also prevent conversion of a perilunate to a lunate
tion of the thumb. dislocation. A plaster slab is applied holding the wrist
neutral. Percutaneous K-wires may be needed to hold
the reduction.
X-ray
Open reduction Reduction is imperative, and if closed
Most dislocations are perilunate. In the antero-poste-
reduction fails, or if a later x-ray shows that the wrist
rior view the carpus is diminished in height and the
has collapsed into the familiar DISI pattern, open
bone shadows overlap abnormally. One or more of
reduction is performed. The carpus is exposed by an
the carpal bones may be fractured (usually the
anterior approach which has the advantage of
scaphoid and radial styloid). If the lunate is dislocated,
decompressing the carpal tunnel. While an assistant
it has a characteristic triangular shape instead of the
pulls on the hand, the lunate is levered into place and
normal quadrilateral appearance.
kept there by a K-wire which is inserted through the
In the lateral view it is easy to distinguish a lunate
lunate into the capitate. If the scaphoid is fractured,
from a perilunate dislocation. The dislocated lunate is
this too can be reduced and fixed with a Herbert screw
tilted forwards and is displaced in front of the radius,
or K-wires. Where possible, the torn soft tissues should
while the capitate and metacarpal bones are in line
be repaired through palmar and dorsal approaches. At
with the radius. With a perilunate dislocation the
the end of the procedure, the wrist is splinted in a
lunate is tilted only slightly and is not displaced for-
plaster slab, which is retained for 3 weeks. Finger,
elbow and shoulder exercises are practised throughout
this period. The K-wires are removed at 6 weeks.
This injury is frequently accompanied by severe
compression of the median nerve, which should be
released.
SCAPHO-LUNATE DISSOCIATION
A wrist sprain may be followed by persistent pain and
tenderness over the dorsum just distal to Lister’s
tubercle.
(a) (c) (e) X-rays show an excessively large gap between the
scaphoid and the lunate. The scaphoid may appear
foreshortened, with a typical cortical ring sign. In the
lateral view, the lunate is tilted dorsally and the
scaphoid anteriorly (DISI pattern).
Treatment
Scapho-lunate instability causes weakness of the wrist
and recurrent discomfort. If seen early (i.e. less than 4
(b) (d) (f) weeks after injury) the scapho-lunate ligament should
25.25 Lunate and perilunate dislocations. be repaired directly with interosseous sutures, pro-
(a,b) Lateral x-ray of normal wrist; (c,d) lunate dislocation; tected by K-wires for 6 weeks and a cast for 8–12
(e,f) perilunate dislocation. weeks. If seen between 4 and 24 weeks, then the 785
25
(a) (b) (c) (d)
25.26 Perilunate dislocation (a,b) Lunate still in its original position while the rest of the carpus is dislocated around it.
(c) The dislocation has been reduced and held with K-wires. (d) The luno-triquetral ligament is re-attached with ligament
anchors.
ligament is unlikely to heal. Blatt’s capsulodesis is RADIO-CARPAL DISLOCATION

helpful: a proximally based flap of dorsal capsule is
attached to the back of the scaphoid to haul it back The most common injuries of this type involve a
from flexion into a normal position. In chronic lesions fracture of the anterior or posterior rim of the distal
without secondary osteoarthritis, a capsulodesis or radius (Barton’s fracture – see page 776). However,
ligament reconstruction is attempted. If there is occasionally the ligaments which bind the carpus to
severe symptomatic osteoarthritis then a limited inter- the distal radius can rupture; the carpus tends to
carpal arthrodesis or radio-carpal arthrodesis is per- translate medially. Repair of the ligaments and tempo-
formed. rary K-wire stabilization is needed.
TRIQUETRO-LUNATE DISSOCIATION MIDCARPAL DISLOCATION

A medial sprain followed by weakness of grip and ten- The extrinsic ligaments which bind the proximal to
derness distal to the head of the ulna should suggest the distal row can rupture (there are, by definition, no
disruption of the triquetro-lunate ligaments. intrinsic ligaments between these two rows). The
X-rays show a noticeable gap between the tri- diagnosis is difficult but is more readily suggested in
quetrum and the lunate, with a VISI carpal collapse those with generalized ligament laxity and a chronic
pattern in the lateral view. wrist problem. The patient complains of a painful,
recurrent snap in the wrist; the two rows can be pas-
sively ‘clunked’ apart when shifted backwards and for-
Treatment
wards. If an acute ligament rupture is diagnosed, then
Acute tears should be repaired with interosseous sutures, repair and temporary K-wire stabilization should be
supported by temporary K-wires for 6 weeks and a cast carried out. In a chronic lesion, fusion of the proximal
for 8–12 weeks. In chronic injuries, a ligament substi- row to the distal row is the most effective treatment
tution (e.g. a slip of extensor carpi ulnaris) or a limited but this operation will restrict wrist movement and
intercarpal fusion may be considered. may predispose to later arthritis.
786
Hand injuries
26
David Warwick
Hand injuries – the commonest of all injuries – are is needed, only the injured finger should be splinted. If
important out of all proportion to their apparent the entire hand needs splinting, this must always be in
severity, because of the need for perfect function. the ‘position of safety’ – with the metacarpo-phalangeal
Nowhere else do painstaking evaluation, meticulous joints flexed at least 70 degrees and the interphalangeal
care and dedicated rehabilitation yield greater joints almost straight. Sometimes an external splint, to
rewards. The outcome is often dependent upon the be effective, would need to immobilize undamaged
judgement of the doctor who first sees the patient. fingers or would need to hold the joints of the injured
If there is skin damage the patient should be exam- finger in an unfavourable position (e.g. flexion of the
ined in a clean environment with the hand displayed interphalangeal joints). If so, internal fixation may be
on sterile drapes. required (K-wires, screws or plates).
A brief but searching history is obtained; often the
Skin cover Skin damage demands wound toilet
mechanism of injury will suggest the type and severity
followed by suture, skin grafting, local flaps, pedicled
of the trauma. The patient’s age, occupation and
flaps or (occasionally) free flaps. Treatment of the skin
‘handedness’ should be recorded.
takes precedence over treatment of the fracture.
Superficial injuries and severe fractures are obvious,
but deeper injuries are often poorly disclosed. It is Nerve and tendon injury Generally, the best results will
important in the initial examination to assess the cir- follow primary repair of tendons and nerves.
culation, soft-tissue cover, bones, joints, nerves and Occasionally grafts are required.
tendons.
X-rays should include at least three views (postero-
anterior, lateral and oblique), and with finger injuries
the individual digit must be x-rayed. METACARPAL FRACTURES
The metacarpal bones are vulnerable to blows and

falls upon the hand, or the longitudinal force of the
GENERAL PRINCIPLES OF
boxer’s punch. Injuries are common and the bones
TREATMENT may fracture at their base, in the shaft or through the
neck.
Most hand injuries can be dealt with under local or Angular deformity is usually not very marked, and
regional anaesthesia; a general anaesthetic is only even if it persists, it does not interfere much with
rarely required.
CirculationIf the circulation is threatened, it must be
promptly restored, if necessary by direct repair or vein
grafting.
Swelling Swelling must be controlled by elevating the
hand and by early and repeated active exercises.
Splintage Incorrect splintage is a potent cause of (a) (b) (c)
stiffness; it must be appropriate and it must be kept to
26.1 Splintage of the hand Three positions of the hand:
a minimum length of time. If a finger has to be (a) The position of relaxation, (b) the position of function
splinted, it may be possible simply to tape it to its (ready for action) and (c) the position of safe
neighbour so that both move as one; if greater security immobilization, with the ligaments taut.
26
(a) (b) (c) (d)
26.2 Metacarpal fractures (a) A spiral fracture

(especially an ‘inboard’ one) can be adequately
held by the surrounding muscles and ligaments
but internal fixation (b) allows early mobilization.
A displaced fracture (c), especially an ‘outboard’
one, can be held by a plate or transverse wires to
allow early mobilization (d); multiple metacarpal
fractures should be fixed with rigid plates for
wires (e). A boxer’s fracture (f) should be treated
(e) (f) by early mobilization.
function. Rotational deformity, however, is serious. practised assiduously. As the patient moves the fingers,
Close your hand with the distal phalanges extended, the fracture may shorten until the intertacarpal liga-
and look: the fingers converge across the palm to a ments between the metacarpal necks tighten, thus
point above the thenar eminence; malrotation of the limiting further shortening and rotational deformity.
metacarpal (or proximal phalanx) will cause that fin- Transverse fractures with considerable displacement
ger to diverge and overlap one of its neighbours. are reduced by traction and pressure. Reduction can
Thus, with a fractured metacarpal it is important to sometimes be held by a plaster slab extending from
regain normal rotational alignment. the forearm over the fingers (only the damaged ones).
The fourth and fifth metacarpals are more mobile at The slab is maintained for 3 weeks and the undam-
their base than the second and third, and therefore are aged fingers are exercised. However, these fractures
better able to compensate for residual angular defor- are usually unstable and should be fixed surgically
mity. with compression plates or percutaneous K-wires
Fractures of the thumb metacarpal usually occur placed either across the fracture or transversely
near the base and pose special problems. They are through the neighbouring undamaged metacarpals.
dealt with separately below. Spiral fractures are liable to rotate; if so, they
should be perfectly reduced and fixed with lag screws
and a plate, or percutaneous wires.
FRACTURES OF THE METACARPAL SHAFT
A direct blow may fracture one or several metacarpal FRACTURES OF THE METACARPAL NECK
shafts transversely, often with associated skin damage.
A twisting or punching force may cause a spiral frac- A blow may fracture the metacarpal neck, usually of
ture of one or more shafts. There is local pain and the fifth finger (the ‘boxer’s fracture’) and occasion-
swelling, and sometimes a dorsal ‘hump’. ally one of the others. There may be local swelling,
with flattening of the knuckle. X-rays show an
impacted transverse fracture with volar angulation of
Treatment the distal fragment.
Oblique or transverse fractures with slight displacement
require no reduction. Splintage also is unnecessary,
Treatment
but a firm crepe bandage may be comforting; this
should not be allowed to discourage the patient from The main function of the fifth and fourth fingers is
788 active movements of the fingers, which should be firm flexion (‘power grip’) and, as can be readily
joint is so badly damaged that primary replacement is 26
considered (Silastic, pyrocarbon or polythene–
metal).
FRACTURES OF THE METACARPAL BASE
Hand injuries
Excepting fractures of the thumb metacarpal, these
are usually stable injuries which can be treated by
ensuring that rotation is correct and then splinting the
(a) (b) digit in a volar slab extending from the forearm to the
26.3 Fracture of the metacarpal head (a) Depressed proximal finger joint. The splint is retained for 3
head fracture which was reduced and held with buried weeks and exercises are then encouraged.
mini-screws. (b) A ‘fight-bite’, with metacarpal head Displaced intra-articular fractures of the base of the
damage from an opponent’s tooth. fourth or fifth metacarpal may cause marked incon-
gruity of the joint. This is a mobile joint and it may,
demonstrated on a normal hand, there is ‘spare’ therefore, be painful. The fracture should be reduced
extension available at the metacarpo-phalangeal by traction on the little finger and then held with a
(MCP) joint. Therefore in these digits, a flexion percutaneous K-wire or compression screw. In the
deformity of up to 40 degrees can be accepted; as long term, if painful arthritis supervenes, treatment
long as there is no rotational deformity, a good out- would be with either arthrodesis or joint excision.
come can be expected. The hand is immobilized in a
gutter splint with the MCP joint flexed and the inter- FRACTURE OF THE THUMB METACARPAL
phalangeal (IP) joints straight until discomfort settles
– a week or two – and then the hand is mobilized. The Three types of fracture are encountered: impacted
patient is warned that the knuckle profile may be per- fracture of the metacarpal base; Bennett’s fracture-dis-
manently lost. In the index and middle fingers, which location of the carpo-metacarpal (CMC) joint; and
function mainly in extension, no more than 20 Rolando’s comminuted fracture of the base.
degrees of flexion at the fracture is acceptable.
If the fracture needs reduction, this can be done Impacted fracture
under a local block. The reduced finger is held with a A boxer may, while punching, sustain a fracture of the
gutter splint moulded at three points to support the base of the first metacarpal. Localized swelling and
fracture; the MCP joints are flexed and the IP joints tenderness are found, and x-ray shows a transverse
are straight. Unfortunately, these fractures are usually fracture about 6 mm distal to the CMC joint, with
fairly unstable because of the tone of the flexor ten- outward bowing and impaction.
dons and the palmar comminution of the fracture. If
there is a tendency to redisplacement, fixation should Treatment If the angulation is less than 20–30
be used. Plates are not really suitable because the frac- degrees and the fragments are impacted, the thumb is
ture is so distal. A bouquet of two or three bent wires rested in a plaster of Paris cast extending from the
passed distally through a hole in the styloid process of forearm to just short of the interphalangeal thumb
the fifth metacarpal base is particularly effective. joint with the thumb fully abducted and extended.
The cast is removed after 2–3 weeks and the thumb is
mobilized.
Complications If the angulation is greater than 30 degrees, then
Malunion, with volar angulation of the distal frag- the reduced thumb web span will be noticeable and so
ment, is poorly tolerated if this occurs in the second the fracture should be reduced. The surgeon pulls on
or third rays. The patient may be aware of a bump in the abducted thumb and, by levering the metacarpal
the palm from the prominent metacarpal head and the outwards against his own thumb, corrects the bow-
digit may take on a ‘Z’ appearance as the knuckle joint ing. A plaster cast is applied. If the fracture is still
hyperextends to compensate for the deformity. unstable, then a percutaneous K-wire is inserted. An
alternative would be a low profile plate.
FRACTURES OF THE METACARPAL HEAD Bennett’s fracture-dislocation

These fractures occur after a direct blow. They are This fracture, too, occurs at the base of the first
often quite comminuted and sometimes ‘open’. Oper- metacarpal bone and is commonly due to punching;
ative reduction is usually required and fixation with however the fracture is oblique, extends into the
small headless buried screws is ideal. Occasionally the CMC joint and is unstable. 789
26
(a) (b) (c) (d) (e)
26.4 Fractures of the first metacarpal base A transverse fracture (a) can be reduced and held in plaster (b). Bennett’s
fracture-dislocation (c) is best held with a small screw (d) or a percutaneous K-wire (e).
The thumb looks short and the carpo-metacarpal usually requires open reduction. This is, by definition,
region swollen. X-rays show that a small triangular a Salter-Harris type III fracture-separation of the
fragment has remained in contact with the medial physis; it must be accurately reduced and fixed with a
edge of the trapezium, while the remainder of the K-wire.
thumb has subluxated proximally, pulled upon by the
abductor pollicis longus tendon.
Treatment It is widely supposed (with little evidence) FRACTURES OF THE PHALANGES
that perfect reduction is essential. It should, however,
be attempted and can usually be achieved by pulling The fingers are usually injured by direct violence, and
on the thumb, abducting it and extending it. Reduc- there may be considerable swelling or open wounds.
tion can then be held in one of two ways: plaster or Injudicious treatment may result in a stiff finger
internal fixation. which, in some cases, can be worse than no finger.
Plaster may be applied with a felt pad over the frac-
ture, and the first metacarpal held abducted and
extended (usually best achieved by flexing the MCP FRACTURES OF THE PROXIMAL AND
joint). However, plaster only works if it is applied with MIDDLE PHALANGEAL SHAFTS
great skill, and the pressure required to maintain a
reduction can cause skin damage; it has, therefore, The phalanx may fracture in various ways:
generally been abandoned in favour of surgery. • Transverse fracture of the shaft, often with forward
Surgical fixation is achieved by passing a K-wire angulation.
across the metacarpal base into the carpus. If the frag- • Spiral fracture of the shaft, from a twisting injury.
ment is large and cannot be reduced and held with a • Comminuted fracture, usually due to a crush injury
wire, then open reduction and fixation with a lag and often associated with significant tendon dam-
screw is effective. age and skin loss.
• Avulsion of a small fragment of bone.
ROLANDO’S FRACTURE • Metaphyseal fracture at the base of the proximal
This is an intra-articular comminuted fracture of the phalanx, commonly seen in osteopaenic bone. The
base of the first metacarpal with a T or Y configura- shaft is pulled into extension and at the distal end
tion. Closed reduction and K-wiring or open reduc- the entire head may displace. This is most com-
tion and plate fixation can be used. With more severe monly seen in children.
comminution, external fixation is needed. • Intra-articular fractures: At the distal end of the
phalanx, the entire head may rotate or, more com-
METACARPAL FRACTURES IN CHILDREN monly, one condyle rotates through a longitudinal
midline fracture into the joint. At the proximal end,
Metacarpal fractures are less common in children than displacement tends to lead to an angular deformity.
in adults. In general they also present fewer problems:
the vast majority can be treated by manipulation and
plaster splintage; angular deformities will almost
Treatment
always be remodelled with further growth. However, UNDISPLACED FRACTURES
rotational alignment is as important as it is in adults. These can be treated by ‘functional splintage’. The
790 Bennett’s fracture is rare; but when it does occur it finger is strapped to its neighbour (‘buddy strapping’)
26
Hand injuries
(a) (b)
26.5 Phalangeal fractures These
can be treated, depending on the
‘personality’ of the fracture,
experience of the surgeon and
equipment available, with neighbour
strapping (a), plate fixation
(b), percutaneous screw fixation (c) or
percutaneous wires (d).
(c) (d)
and movements are encouraged from the outset. CHILDHOOD FRACTURES

Splintage is retained for 2–3 weeks, but during this In children the phalangeal neck can be broken, often
time it is wise to check the position by x-ray in case after a crush injury. The distal fragment displaces dor-
displacement has occurred. sally and extends. These are serious injuries and
should be reduced as soon as possible and then held
DISPLACED FRACTURES with a percutaneous wire.
Displaced fractures must be reduced and immobi-
lized. It is essential to check for rotational correction by
(1) noting the convergent position of the finger when FRACTURES OF THE TERMINAL PHALANX
the MCP joint is flexed, and (2) seeing that the fin-
gernails are all in the same plane. The technique The terminal phalanx, small though it is, is subject to
depends on the fracture pattern. Most need simple five different types of fracture.
manipulation and can then be held in a splint. Basal
fractures with extension are manipulated and held
with a dorsal blocking splint with the MCP joint at 90
Fracture of the tuft
degrees. Angulated basal fractures are manipulated The tip of the finger may be struck by a hammer or
with a pencil between the digits as a lever and then caught in a door, and the bone shattered. The fracture
held with neighbour strapping which pulls the injured is disregarded and treatment is focused on controlling
finger to the next one. Spiral fractures are held with swelling and regaining movement. The painful
‘de-rotation taping’ to the next digit, using tension in haematoma beneath the finger nail should be drained
the tape to unwind the fracture. Transverse fractures by piercing the nail with a hot paper clip. If the nail
may be held in a gutter splint or neighbour splint. bed is shattered and cosmesis is important, it should
If a reduction cannot be achieved, or if it is unsta- be meticulously repaired under magnification.
ble and the position slips, then surgery is needed. The
technique depends upon the configuration of the frac-
ture. K-wires are less invasive and are perfect for some
Mallet finger injury
fractures; other techniques include percutaneous lag After a sudden flexion injury (e.g. stubbing the tip of
screw fixation (for spiral fractures and distal condylar the finger) the terminal phalanx droops and cannot be
fractures) and plate fixation (which risks stiffness in straightened actively. Three types of injury are recog-
the proximal phalanx due to the soft-tissue exposure nized: avulsion of the most distal part of the extensor
and subsequent tendon adhesion). External fixation tendon; avulsion of a small flake of bone from the base
may be needed for comminuted fractures. of the terminal phalanx; and avulsion of a large dorsal 791
26
(a)
(b)
(c)
(d)
26.6 Distal phalangeal injury A fracture of the tuft (a),

caused by a hammer blow, is treated by a protective
dressing. The subungual haematoma should be evacuated
using a red-hot paper clip tip (b) or a small drill. A mallet
finger (c) is best treated with a splint for 6 weeks (d).
Mallet fractures (e) are also better splinted – surgery can
make the outcome worse.
(e)
bone fragment, sometimes with subluxation of the Persistent droop About 85 per cent of mallet fingers
terminal interphalangeal (TIP) joint. recover full extension. If there is a persistent droop
this can be treated by tendon repair supported by
TREATMENT K-wire fixation of the joint, but the results are often
The TIP joint should be immobilized in slight hyper- disappointing. The alternative would be joint arthro-
extension, using a special mallet-finger splint which desis, best achieved with a buried intramedullary
fixes the distal joint but leaves the proximal joints free. double-pitch screw.
For tendinous avulsions (which usually occur pain-
Swan neck deformity Imbalance of the extensor mech-
lessly) the splint should be kept in place constantly
anism can cause this in lax-jointed individuals. A cen-
for 8 weeks and then only at night for another 4
tral slip tenotomy is straightforward and can give a
weeks. Even if there has been a delay of 3 or 4 weeks
very good result.
after injury, this prolonged splintage is usually suc-
cessful.
Bone avulsions are also treated in a splint, but 6 Fracture of the terminal shaft
weeks should suffice as bone heals quicker than ten-
don. Operative treatment is generally avoided, even Undisplaced fractures of the shaft need no treatment
for large bone fragments, unless there is subluxation. apart from analgesia. If angulated, they should be
Surgery carries a high complication rate (wound fail- reduced and held with a longitudinal K-wire through
ure, metalwork problems) without evidence that the the pulp for 4 weeks. The nail is often dislocated from
outcome is improved. However, if there is subluxation its fold; if so it must be carefully tucked back in and
then K-wires or small screws are used to fix the frag- held with a suture in each corner.
ment in place.
COMPLICATIONS OF MALLET FINGER

Avulsion of the flexor tendon
Non-union This is usually painless and treatment is This injury is caused by sudden hyperextension of the
792 not needed. distal joint, typically when a game player catches his
JOINT INJURIES 26
Any finger joint may be injured by a direct blow

(often the overlying skin is damaged), or by an angu-
lation force, or by the straight finger being forcibly
Hand injuries
stubbed. The affected joint is swollen, tender and too
painful to move. X-rays may show that a fragment of
bone has been sheared off or avulsed.
CARPO-METACARPAL DISLOCATION
(a) (b)
The thumb is most frequently affected and clinically
26.7 Flexor tendon avulsion (a) Large fragment and the injury then resembles a Bennett’s fracture-
(b) smaller fragment lodged in front of the PIP joint. dislocation; however, x-rays reveal proximal subluxa-
tion or dislocation of the first metacarpal bone
without a fracture. The displacement is easily reduced
by traction and hyperpronation, but reduction is
unstable and can be held only by a K-wire driven
through the metacarpal into the carpus. The wire is
finger on an opponent’s shirt. The ring finger is most
removed after 5 weeks but a protective splint should
commonly affected. The flexor digitorum profundus
be worn for 8 weeks because of the risk of instability.
tendon is avulsed, either rupturing the tendon itself or
Chronic instability can occur. This is treated prior
taking a fragment of bone with it. If the bone frag-
to arthritis developing, by using part of the flexor
ment is small, or if only the tendon is ruptured, it can
carpi radialis tendon to reconstruct the ruptured and
recoil into the palm. If the lesion is detected within a
incompetent palmar ligament of the CMC joint.
few days (and the diagnosis is easily missed if not
The other carpo-metacarpal joints are also some-
thought about), then the tendon can be re-attached.
times dislocated, typically when a motorcyclist, hold-
If the diagnosis is much delayed, repair is likely to be
ing the handlebars, strikes an object and the hand is
unsuccessful. Two-stage tendon reconstruction is pos-
driven backwards. The hand swells up rapidly and the
sible but difficult, and the finger may end up stiff.
diagnosis is easily missed unless a true lateral x-ray is
Thus, for late cases, tenodesis or fusion of the distal
carefully examined. Closed manipulation is usually
joint is usually preferable.
successful, although a K-wire is recommended to
prevent the joint from dislocating again.
Physeal fracture Late presentation Late presentation or secondary
The basal physis can break, usually producing a arthritis is treated by joint fusion. However, if just the
Salter–Harris I fracture (Seymour fracture). The nail fifth CMC joint is involved, a neat operation is to fuse
may be dislocated from its fold and the germinal the base of the fourth to the fifth metacarpal and then
matrix can be trapped in the fracture. The injury is excise the articular surface of the fifth. This will main-
easily overlooked if the finger is very swollen. The nail tain movement at the fourth CMC, so allowing the
must be cleaned and carefully replaced into its bed. ulnar side of the hand to ‘cup’ around during grip.
(d)
26.8 Carpo-metacarpal dislocation
(a) Thumb dislocation. (b) Dislocation of
the fourth and fifth CMC joints treated
by closed reduction and K-wires (c).
Complete carpo-metacarpal dislocation
(d).
(a) (b) (c) 793
26 METACARPO-PHALANGEAL DISLOCATION the spindle-like swelling of the joint to settle and for
full extension to recover. If there is a large palmar
Usually the thumb is affected, sometimes the fifth fin- fragment with displacement, then this should be
ger, and rarely the other fingers. The entire finger is reduced and fixed. If closed reduction is successful,
suddenly forced into hyperextension and the capsule then an extension splint or temporary transarticular
and muscle insertions in front of the joint may be wire is used. If it cannot be reduced or remains unsta-
torn. There are two types of dislocation: ble then screw fixation or a small wire loop can be
used. If there is marked comminution and instability,
Simple dislocation The finger is extended about 75
the joint is exposed from the palmar surface, the dam-
degrees. It is easily reduced by traction, firstly in
aged fragments are excised and the palmar plate is re-
hyperextension then pulling the finger around. The
attached to the base of the proximal phalanx
finger is strapped to its neighbour and early mobiliza-
(‘palmar-plate arthroplasty’).
tion is encouraged.
Complex dislocation The avulsed palmar plate sits in
the joint, blocking reduction. Furthermore, the
metacarpal head can be clasped between the flexor ‘PILON’ FRACTURES OF THE MIDDLE
tendon and lumbrical tendon. The finger is extended PHALANX
only about 30 degrees and there is usually a tell-tale
dimple in the palm. Very occasionally the fracture can These are quite common injuries and can be very
be reduced closed by hyperextending the MCP joint troublesome. The head of the proximal phalanx
and flexing the IP joints to release the clasp. If this impacts into the base of the middle phalanx, causing
fails, open reduction is required. A dorsal approach is the latter to splay open in several pieces. These injuries
safest. After reduction the joint is stable and should be are best treated with dynamic distraction using a
mobilized in a neighbour-splint. spring-loaded external fixator which rotates around
the head of the proximal phalanx and disimpacts the
This is treated
Chronic instability in the thumb MCP joint
distal fragment. The results can be surprisingly good.
by a sesamoid arthrodesis. The abductor sesamoid is
fused to the underside of the metacarpal neck. This
preserves some flexion yet prevents hyperextension.
An alternative is formal arthrodesis. The use of a low- CONDYLAR FRACTURE
profile compression plate allows early mobilization.
The functional result is usually very good. The basal joint surface or distal joint surface of the pha-
langes can be fractured, usually by an angulation force.
If the fragment is not displaced, it is best to disregard
INTERPHALANGEAL JOINT DISLOCATION the fracture, strap the finger to its neighbour and con-
centrate on regaining movement. An x-ray should be
Distal joint dislocation is rare; proximal joint disloca- taken after a week to ensure there is no displacement.
tion is more common. The dislocation is easily If the fracture is displaced, there is a risk of perma-
reduced by pulling. The joint is strapped to its neigh- nent angular deformity and loss of movement at the
bour for a few days and movements are begun imme- joint. The fracture should be anatomically reduced,
diately. The lateral x-ray may show a small flake of either closed or by open operation and fixed with
bone, representing a palmar plate avulsion; this small K-wires or mini-screws. The finger is splinted for
should be ignored. The patient must be warned that a few days and then supervised movements are com-
it can take many months (and sometimes forever) for menced.
26.9 Finger
dislocation (a)
Metacarpo-phalangeal
dislocation in the
thumb occasionally
buttonholes and needs
open reduction; (b,c)
interphalangeal
dislocations are easily
reduced (and easily
missed if not x-rayed!).
794 (a) (b) (c)
VOLAR FRACTURE-DISLOCATIONS is tested with the MP joint flexed (if extended, even a 26
normal ligament is very lax!).
When the proximal interphalangeal joint dislocates, a In children, the injury may be accompanied by a
fragment of bone may be avulsed from the base of the Salter–Harris III fracture at the base of the proximal
middle phalanx. If this fragment is large, the joint can phalanx.
subluxate forwards. Surgical fixation is very difficult A large bone fragment, if displaced, can be re-
Hand injuries
and can lead to permanent stiffness of the joint. The attached from a palmar approach, using a tension
fracture can be reduced by flexing the joint to 40 band suture or small screw. Smaller fragments are
degrees. The joint is then held in a splint which allows treated by splintage with the MP joints flexed.
flexion but not extension. The amount of extension
block is reduced over the next 4 weeks and the splint
is then discarded. If the fragment is large enough, ULNAR COLLATERAL LIGAMENT OF THE
then miniscrew fixation may be attempted, but failure THUMB METACARPO-PHALANGEAL JOINT
of fixation, tendon adhesion or joint stiffness are risks.
(‘GAMEKEEPER’S THUMB’; ‘SKIER’S
THUMB’)
LIGAMENT INJURIES In former years, gamekeepers who twisted the necks
of little animals ran the risk of tearing the ulnar col-
PROXIMAL INTERPHALANGEAL lateral ligament of the thumb metacarpo-phalangeal
LIGAMENTS joint, either acutely or as a chronic injury. Nowadays
this injury is seen in skiers who fall onto the extended
Partial or complete tears of the proximal interpha- thumb, forcing it into hyperabduction. A small flake
langeal ligaments are quite common, due to forced of bone may be pulled off at the same time. The
angulation of the joint. Mild sprains require no treat- resulting loss of stability may interfere markedly with
ment but with more severe injuries the finger should be prehensile (pinching) activities.
splinted in extension for 2 or 3 weeks, If the joint is The ulnar collateral ligament inserts partly into the
frankly unstable, especially the index and middle which palmar plate. In a partial rupture, only the ligament
oppose load from the thumb, repair is considered. proper is torn and the thumb is unstable in flexion but
Occasionally, the bone to which the ligament is still more or less stable in full extension because the
attached is avulsed; if the fragment is markedly dis- palmar plate is intact. In a complete rupture, both the
placed (and large enough), it should be re-attached. ligament proper and the palmar plate are torn and the
The patient must be warned that the joint is likely to thumb is unstable in all positions. If the ligament rup-
remain swollen and slightly painful for at least 6–12 tures completely (usually at its distal attachment to
months. If the instability persists – which is rare – it the base of the proximal phalanx), it will not heal
can be treated by using spare tendon (e.g. palmaris unless it is repaired; this is because the proximal end
longus) for reconstruction. gets trapped in front of the adductor pollicis aponeu-
rosis (the Stener lesion).
METACARPO-PHALANGEAL JOINTS
Clinical assessment
The radial collateral ligament of the index finger is
most vulnerable, although with a suitable force any On examination there is tenderness and swelling pre-
ligament can be injured. The tension of the ligament cisely over the ulnar side of the thumb metacarpo-
(a) (b) (c)
26.10 Skier’s thumb (a,b) The ulnar collateral ligament has ruptured. Urgent repair is indicated (c).
795
26 phalangeal joint. An x-ray is essential, to exclude a frac- Clinical assessment
ture before carrying out any stress tests. Laxity is often
obvious but if in doubt, then the joint can be examined Open injuries comprise tidy or ‘clean’ cuts, lacera-
under local anaesthetic. If there is no undue laxity (com- tions, crushing and injection injuries, burns and pulp
pare with the normal side) in both extension and 30 defects.
degrees flexion, then a serious injury can be excluded. If The precise mechanism of injury must be under-
there is more than a few degrees of laxity there is prob- stood. Was the instrument sharp or blunt? Clean or
ably a complete rupture which will require operative dirty? The position of the fingers (flexed or extended)
repair. at the time of injury will influence the relative damage
to the deep and superficial flexor tendons. A history of
high pressure injection predicts major soft-tissue
Treatment damage, however innocuous the wound may seem.
Partial tears can be treated by a short period (2–4 What are the patient’s occupation, hobbies and
weeks) of immobilization in a splint followed by increas- aspirations? Is he or she right-handed or left-
ing movement. Pinch should be avoided for 6–8 weeks. handed?
Complete tears need operative repair. Care should Examination should be gentle and painstaking. Skin
be taken during the exposure not to injure the super- damage is important, but it should be remembered
ficial radial nerve branches. The Stener lesion is found that even a tiny, clean cut may conceal nerve or ten-
at the proximal edge of the adductor aponeurosis. don damage.
The aponeurosis is incised and retracted to expose the The circulation to the hand and each digit must be
ligaments and capsule and the torn structures are then assessed. The Allen test can be applied to the hand as
carefully repaired. Postoperatively, the joint is immo- a whole or to an individual finger. The radial and ulnar
bilized in a thumb splint for 6 weeks, but can be arteries at the wrist are simultaneously compressed by
moved early in the flexion–extension plane as the lig- the examiner while the patient clenches his fist for sev-
ament is isometric (i.e. the same length in flexion and eral seconds before relaxing; the hand should now be
extension). The thumb interphalangeal joint should pale. The radial artery is then released; if the hand
be left free from the outset to avoid the adductor flushes it means that the radial blood supply is intact.
aponeurosis becoming adherent (which would limit The test is repeated for the ulnar artery. An injured
flexion). A neglected tear leads to weakness of pinch. finger can be assessed in the same way. The digital
In early cases without articular damage, stability may arteries are occluded by pinching the base of the fin-
be restored by using a free tendon graft. If this fails, ger. When blood is squeezed out of the finger the
or if the joint is painful, MP joint arthrodesis is reli- pulp will become noticeably pale; one digital artery is
able and leaves minimal functional deficit. then released and the pulp should pink up; the test is
In children, the injury may be accompanied by a repeated for the other digital artery.
Salter–Harris Type III fracture through the physis. Sensation is tested in the territory of each nerve.
This should be reduced and fixed with smooth K- Two-point discrimination may be reduced in partial
wires which should not cross the growth plate. injuries. In children, who are more difficult to exam-
ine, the plastic pen test is helpful: if a plastic pen is
brushed along the skin it will tend to ‘stick’ due to the
normal thin layer of sweat on the surface; absence of
OPEN INJURIES OF THE HAND sweating (due to a nerve injury) is revealed by noting
that the pen does not adhere as it should (compared
Over 75 per cent of work injuries affect the hands; to the normal side). Another observation is that the
inadequate treatment costs the patient (and society) skin in the territory of a divided nerve will not wrin-
dear in terms of functional disability. kle if immersed in water.
26.11 Open injuries (a) A

mangled hand; (b) open
finger fracture treated with
external fixation.
796 (a) (b)

26
Hand injuries
(a) (b)
(c) (d)
26.12 Testing the flexor tendons Testing for (a) flexor

digitorum profundus (FDP) lesser fingers, (b) flexor
digitorum superficialis (FDS) lesser fingers, (c) FDP index,
(d) FDS index. 26.13 Hand incisions ‘Permissable’ incisions in hand
surgery. Incisions must not cross a skin crease or an
interdigital web or else scarring may cause contracture and
Tendons must be examined with similar care. Start deformity.
by testing for ‘passive tenodesis’. When the wrist is
extended passively, the fingers automatically flex in a
gentle and regular cascade; when the wrist is flexed, as possible. Prophylaxis against tetanus and gas gan-
the fingers fall into extension. These actions rely upon grene may also be needed. The hand is lightly splinted
the balanced tension of the opposing flexor and and the wound is covered with an iodine-soaked
extensor tendons to the fingers; if a tendon is cut, the dressing.
cascade will be disturbed.
Active movements are then tested for each individ- WOUND EXPLORATION
ual tendon. Flexor digitorum profundus is tested by Under general or regional anaesthesia, the wound is
holding the proximal finger joint straight and cleaned and explored. A pneumatic tourniquet is
instructing the patient to bend the distal joint. Flexor essential unless there is a crush injury where muscle
digitorum superficialis is tested by the examiner hold- viability is in doubt. Skin is too precious to waste and
ing all the fingers together out straight, then releasing only obviously dead skin should be excised. For ade-
one and asking the patient to bend the proximal joint. quate exposure the wound may need enlarging, but
Holding the fingers out straight ‘immobilizes’ all the incisions must not cross a skin crease or an interdigi-
deep flexors (including that of the finger being tested) tal web. Through the enlarged wound, loose debris is
which have a common muscle belly. However, in the picked out, dead muscle is excised and the tissues are
index finger this test is not 100 per cent reliable thoroughly irrigated with isotonic crystalloid solu-
because the deep flexor is sometimes separate. It is tion. A further assessment of the extent of the injury
better to ask the patient to make a ‘circle’ between is then undertaken.
thumb and index (FDP intact) and a ‘buttonhole‘
(FDS intact). TISSUE REPAIR
If a tendon is only partly divided, it will still work Fractures are reduced and held appropriately (splin-
although it may be painful. In full thickness skin lac- tage, K-wires, external fixator or plate and screws)
erations, if there is any doubt about the integrity of unless there is some specific contraindication.
the tendons, the wound should be explored. Joint capsule and ligaments are repaired.
X-rays may show fractures, foreign bodies, air or Artery and vein repair may be needed if the hand or
paint. finger is ischaemic. This done with the aid of an oper-
ating microscope. Any gap should be bridged with a
vein graft.
Primary treatment Severed nerves are sutured under an operating
PREOPERATIVE CARE microscope (or at least loupe magnification) with the
The patient may need treatment for pain and shock. If finest, non-reactive material. If the repair cannot be
the wound is contaminated, it should be rinsed with achieved without tension then a nerve graft (e.g. from
sterile crystalloid; antibiotics should be given as soon the posterior interosseous nerve at the wrist) should 797
26
26.15 The flexor tendon sheath and pulleys Fibrous

pulleys – designated A1 to A5 – hold the flexor tendons to
the phalanges and prevent bowstringing during
movement. A1, 3 and 5 are attached to the palmar plate
near each joint; A2 and 4 have a crucial tethering effect
and must always be preserved or reconstructed.
26.14 The zones of injury I – Distal to the insertion of
flexor digitorum superficialis. II – Between the opening of
the flexor sheath (the distal palmar crease) and the joint where both the superficial and deep tendons run
insertion of flexor superficialis. IIII – Between the end of together in a tight sheath (Zone II or, more dramati-
the carpal tunnel and the beginning of the flexor sheath.
IV – Within the carpal tunnel. V – Proximal to the carpal cally, ‘no man’s land’ because injuries in this zone are
tunnel. the most dangerous). Primary repair with fastidious
postoperative supervision gives the best outcome but
calls for a high level of expertise and specialized phys-
be performed. More recently, dissolvable nerve guides iotherapy. If the necessary facilities are not available,
have been used to bridge the gap, allowing a biologi- then the wound should be washed out and loosely
cal regeneration across the gap). closed, and the patient transferred to a special centre.
Extensor tendon repair is not as easy and the results A delay of several days, with a clean wound, is unlikely
not as reliable as some have suggested. Repair and to affect the outcome. The tendon repair must be
postoperative management should be meticulous. strong and accurate enough to allow early mobiliza-
Flexor tendon repair is even more challenging, par- tion (usually passive) so that the tendons can glide
ticularly in the region between the distal palmar crease freely and independently from each other and the
and the flexor crease of the proximal interphalangeal sheath. Four strands of locked core suture are placed
26.16 Flexor tendon repair A core suture

(a) is supplemented by circumferential
sutures (b). (c) The relationship of the
important structures in ‘no man’s land’:
1 – the tendon sheath; 2 – flexor digitorum
profundus; 3 – flexor digitorum superficialis;
4 – digital nerve; 5 – artery; 6 – extensor
tendon.
(a)
798 (b) (c)

26
Hand injuries
(a) (b) (c)
26.17 Pulp and finger-tip injuries (a) Cross-finger graft for a palmar oblique finger-tip injury
with exposed bone. (b) V-to-Y advancement for a transverse finger-tip injury with exposed bone.
Thumb tip loss (c) must always be reconstructed – never amputate.
without handling the tendon any more than is usually a sign of infection and antibiotics should be
absolutely necessary; this is supplemented by a con- avoided. The wound is inspected only infrequently,
tinuous circumferential suture which strengthens the then re-covered with the non-adherent dressing, until
repair and smoothes it, thus making the gliding action it heals.
through the sheath easier. The A2 and A4 pulleys If the open area is greater than 1 cm in diameter,
must be repaired or reconstructed, otherwise the ten- healing will be quicker with a split-skin or full thick-
dons will bowstring. Cuts above the wrist (Zone V), ness graft but the residual pulp cover may not be as
in the palm (Zone III) or distal to the superficialis satisfactory as a wound that has been left to heal nat-
insertion (Zone I) generally have a better outcome urally by granulation and re-epithelialization.
than injuries in the carpal tunnel (Zone IV) or flexor If bone is exposed and length of the digit is impor-
sheath (Zone II). Division of the superficialis tendon tant for the individual patient, then an advancement
noticeably weakens the hand and a swan neck defor- flap or neurovascular island flap should be considered.
mity can develop in those with lax ligaments. At least The precise type of flap depends on the orientation of
one slip should therefore always be repaired. the cut. Otherwise, primary cover can be achieved by
Amputation of a finger as a primary procedure shortening the bone and tailoring the skin flaps (‘ter-
should be avoided unless the damage involves many minalization’).
tissues and is clearly irreparable. Even when a finger In young children, the finger-tips recover extraordi-
has been amputated by the injury, the possibility of re- narily well from injury and they should be treated
attachment should be considered (see below). with dressings rather than grafts or terminalization.
Ring avulsion is a special case. When a finger is Thumb length should never be sacrificed lightly and
caught by a ring, the soft tissues are sheared away every effort should be made to provide a long, sensate
from the underlying skeleton. Depending on the digit.
amount of damage, skin reattachment, microvascular
Nail bed injuries Nail bed injuries are often seen in
reconstruction or even amputation may be required.
association with fractures of the terminal phalanx. If
appearance is important, meticulous repair of the nail
CLOSURE
bed under magnification, replacing any loss with a split
The tourniquet is deflated and bipolar diathermy is
thickness nail bed graft from one of the toes, will give
used to stop bleeding. Haematoma formation leads to
the best cosmetic result. In children, these injuries are
poor healing and tendon adhesions. Unless the
associated with a physeal fracture.
wound is contaminated, the skin is closed – either by
direct suture without tension or, if there is skin loss,
DRESSING AND SPLINTAGE
by skin grafting. Skin grafts are conveniently taken
The wound is covered with a single layer of paraffin
from the inner aspect of the upper arm. If tendon or
gauze and ample wool roll. A light plaster slab holds
bare bone is exposed, this must be covered by a rota-
the wrist and hand in the position of safety (wrist
tion or pedicled flap. Sometimes a severely mutilated
extended, metacarpo-phalangeal joints flexed to 90
finger is sacrificed and its skin used as a rotation flap
degrees, interphalangeal joints straight, thumb
to cover an adjacent area of loss.
abducted). This is the position in which the
Pulp and finger-tip injuries In full thickness wounds metacarpo-phalangeal and interphalangeal ligaments
without bone exposure, the wound should be are fully stretched and fibrosis therefore least likely to
thoroughly cleaned and then covered with a non- cause contractures. Failure to appreciate this point is
adherent dressing. This is left well alone for 7 days; the the commonest cause of irrecoverable stiffness after
accumulation of fluid beneath the dressing is not injury (see Fig 16.26). 799
26 This position is modified in two circumstances. (1) used, particularly for injuries at the level of the exten-
After primary flexor tendon suture, the wrist is held sor retinaculum and the metacarpo-phalangeal joint.
with a dorsal splint in about 20 degrees of flexion to Various protocols are followed for flexor tendon
take tension off the repair (too much wrist flexion injuries, including passive, active or elastic-band
invites wrist stiffness and carpal tunnel symptoms) but assisted flexion. Early movement promotes tendon
the interphalangeal joints must remain straight. There healing and excursion. In all cases the risk of rupture
should be minimal restriction at the front of the fin- is balanced against the need for early mobilization.
gers, otherwise the resistance can precipitate rupture Close supervision and attention to detail are essential.
of the tendon. (2) After extensor tendon repair, the Once the tissues have healed, the hand is increasingly
metacarpo-phalangeal joints are flexed to only about used for more and more arduous and complex tasks, es-
30 degrees so that there is less tension on the repair; pecially those that resemble the patient’s normal job,
the wrist is extended to 30 degrees and the interpha- until he or she is fit to start work; if necessary, his or her
langeal joints remain straight. work is modified temporarily. If secondary surgery is re-
quired, tendon or nerve repair is postponed until the
skin is healthy, there is no oedema and the joints have
regained a normal range of passive movement.
Replantation
With modern microsurgical techniques and appropri-
ate skill, amputated digits or hands can be replanted.
An amputated part should be wrapped in sterile saline
(a) (b)
gauze and placed in a plastic bag, which is itself placed
26.18 Splintage Always splint in the safe position (wrist in watery ice. The ‘cold ischaemic time’ for a finger,
slightly extended, MP joint flexed, PIP extended). Only which contains so little muscle, is about 30 hours, but
immobilize the affected ray if there is a metacarpal or
phalangeal injury. the ‘warm time’ less than six. For a hand or forearm,
the cold ischaemic time is only about 12 hours and
the warm time much less. After resuscitation and
attention to other potentially life-threatening injuries,
Postoperative management the patient and the amputated part should be trans-
IMMEDIATE AFTERCARE ferred to a centre where the appropriate surgical skills
Following an operation, the hand is kept elevated in a and facilities are available.
roller towel or high sling. If the latter is used, the sling
must be removed several times a day to exercise the INDICATIONS
elbow and shoulder. Too much elbow flexion can stop The decision to replant depends on the patient’s age,
venous return and make swelling worse. Antibiotics his or her social and professional requirements, the
are continued as necessary. condition of the part (whether clean-cut, mangled,
crushed or avulsed), and the warm and cold ischaemic
REHABILITATION time. Furthermore, and perhaps most importantly, it
Movements of the hand must be commenced within a depends on whether the replanted part is likely to give
few days at most. Splintage should allow as many better function than an amputation.
joints as possible to be exercised, consistent with pro- The thumb should be replanted whenever possible.
tecting the repair. Most extensor tendon injuries are Even if it functions only as a perfused ‘post’ with pro-
splinted for about 4 weeks. Dynamic splintage can be tective sensation, it will give useful service. Multiple dig-
26.19 Avulsion This is not

replantable.
(a) (b)
800
its also should be replanted, and in a child even a single 26
digit. Proximal amputations (through the palm, wrist or
forearm) likewise merit an attempt at replantation.
RELATIVE CONTRAINDICATIONS
Single digits do badly if replanted. There is a high
Hand injuries
complication rate, including stiffness, non-union,
poor sensation, and cold intolerance; a replanted sin-
gle finger is likely to be excluded from use. The excep-
tion is an amputation beyond the insertion of flexor
digitorum superficialis, when a cosmetic, functioning
finger-tip can be retrieved. Severely crushed, mangled
or avulsed parts may not be replantable; and parts with
a long ischaemic time may not survive. General med- 26.20 Frostbite
ical disorders or other injuries may engender unac-
ceptable risks from the prolonged anaesthesia needed
icity or both. The thumb or index finger is usually
for replantation.
involved. Substances can gain entry even through
intact skin. Air or lead paint may show on x-ray.
Immediate decompression and removal of the for-
MANAGEMENT OF BURNS eign substance offers the best hope. This means an
extensive dissection. The outcome is often poor, with
Generally, hand burns should be dealt with in a spe- amputation sometimes being necessary.
cialized unit. Superficial burns are covered with moist
non-adherent dressings; the hand is elevated and fin-
ger movements are encouraged. Partial thickness
FROSTBITE
burns can usually be allowed to heal spontaneously;
the hand is dressed with an antimicrobial cream and
splinted in the position of safety. Frostbite requires special treatment. The limb is re-
Full thickness burns will not heal. Devitalized tissue warmed in a water bath at 40–42 degrees for 30 min-
should be excised; the wound is cleaned and dressed utes. Oedema is minimized by elevation, and blisters
and 2–5 days later skin-grafted. Full thickness circum- are drained. Digits sometimes need amputation.
ferential burns may need early escharotomy to pre-
serve the distal circulation. Skin flaps are sometimes
needed in sites such as the thumb web which are
SECONDARY OPERATIONS
prone to contracture. The hand should be splinted in
the position of safety; K-wires may be needed to
maintain this position. The primary treatment of hand injuries should always
Electric burns may cause extensive damage and be carried out with an eye to any future reconstructive
thrombosis which become apparent only after several procedures that might be necessary. These are of three
days. The patient may of course need resuscitation kinds:
(treating cardiac anomalies and myoglobinuria). The • secondary repair or replacement of damaged struc-
arm needs to be monitored and fasciotomy with tures
debridement of dead tissue is often needed. • amputation of fingers
Chemical burns should be irrigated copiously for 20 • reconstruction of a mutilated hand.
or 30 minutes, usually with water or saline but some-
times with a specific reagent (calcium gluconate for hy-
drogen fluoride burns, soda lime or magnesium solu- Delayed repair
tion for hydrochloric acid, mineral oil for sodium). SKIN
If the skin cover is unsuitable for primary closure or
has broken down it is replaced by a graft or flap. As
always, the skin creases must be respected. Contrac-
MANAGEMENT OF INJECTION tures are dealt with by Z-plasty, skin grafting, or local
INJURIES flaps, regional flaps or free flaps. When important
volar surfaces such as the thumb or index tip are
Oil, grease, solvents, hydraulic fluid or paint injected insensate, a flap of skin complete with its neurovascu-
under pressure are damaging because of tension, tox- lar supply may be transposed. 801
26 Split thickness skin contracts and so full thickness NERVES
grafts are preferred. The upper inner arm can provide Late-presenting nerve injuries must be carefully
a fair amount of skin leaving a reasonable cosmetic assessed. The results of repair deteriorate with time,
defect. Larger amounts of skin can be harvested from particularly for motor nerves where the end plate
the groin or abdomen. Bear in mind that grafts will begins to fail and the muscle begins to fibrose. If sev-
not adhere to raw tendon or bone. eral months have passed, tendon transfer may be a
more reliable alternative. If nerve repair is attempted,

the scar is excised and the stumps pared back until
TENDONS healthy nerve is found proximally and distally; a nerve
Primary suture may have been contraindicated by graft or tubular nerve guide is usually needed to avoid
wound contamination, undue delay between injury tension at the suture line.
and repair, massive skin loss or inadequate operating
facilities. In these circumstances secondary repair or JOINTS
tendon grafting may be necessary. The proximal interphalangeal joint is most prone to a
In a late-presenting injury of the profundus tendon flexion contracture. Active and passive exercises can
with an intact superficialis, advancement of a retracted be supplemented by serial static splints or dynamic
tendon can cause a flexion deformity of the entire fin- splints. Surgery (capsulotomy, palmar plate and collat-
ger. Tendon grafting also is risky: the finger could end eral ligament release) may be required but these oper-
up even stiffer. Unless the patient’s work or hobby ations themselves can invite further stiffness. Unstable
demands flexion of the distal joint and maximum or painful joints are best fused.
power in the finger, fusion or tenodesis of the distal
interphalangeal joint is a more reliable option. BONES
If both the superficialis and profundus tendons have Malunion, especially if rotational, may require treat-
been divided and have retracted, a tendon graft is ment. Non-union is very uncommon, but if present
needed. Full passive joint movement is a prerequisite. grafting may be required. Extensor tendons may stick
If the pulley system is in good condition and there to bone, most commonly after plate fixation of the
are no adhesions, the tendons are excised from the proximal phalanx.
flexor sheath and replaced with a tendon graft (pal- Plate removal and tenolysis is followed by aggres-
maris longus, plantaris or a toe extensor). Rehabilita- sive active and passive movements: a fair result is usu-
tion is the same as for a primary repair. ally achieved.
If the pulleys are damaged, the skin cover poor, the
passive range of movement limited or the sheath
scarred, a two-stage procedure is preferred. The ten-
dons are excised and the pulleys reconstructed with AMPUTATION
extensor retinaculum or excised tendon. A Silastic rod
is sutured to the distal stump of the profundus tendon
Indications A finger is amputated only if it remains
and left free proximally either in the palm or distal
painful or unhealed, or if it is a nuisance (i.e. the
forearm. Rehabilitation is planned to maintain a good
patient cannot bend it, straighten it or feel with it),
passive range of movement. A smooth gliding surface
and then only if repair is impossible or uneconomic.
forms around the rod. At least 3 months later, the rod
is removed through two smaller incisions and a ten- Technique In the finger-tip, the aim is a mobile digit
don graft (palmaris longus, plantaris or a lesser toe covered by healthy skin with normal sensation. This
extensor) is sutured to the proximal and distal stumps can be achieved by local advancement flaps or
of flexor digitorum profundus. Rehabilitation is the neurovascular island flaps, or by bone shortening
same as that for a primary repair. (‘terminalization’). A cross-finger flap is fairly
Tenolysis is sometimes indicated. After flexor tendon straightforward and provides good skin cover, but
repair in Zone II, a poor excursion is not infrequent sensation is limited and a flexion contracture can
because of adhesions between the tendons and the develop in the donor finger. The final choice depends
sheath. There is some active movement – indicating on the patient’s requirements and the surgeon’s skill.
that the tendon is intact – but not enough for good In the thumb every millimetre is worth preserving;
function. The passive range of movement should be even a stiff or deformed thumb is worth keeping.
good if the tenolysis is to succeed. The tendons are The middle and ring fingers should not be ampu-
painstakingly freed through small windows in the tated through the knuckle joint because cosmetically
flexor sheath. Postoperatively an intensive programme this is unsatisfactory and small objects will fall through
of movement is essential, otherwise there will be even the gap (‘incontinence of grip’). If the proximal pha-
more scar tissue than before and the tenolysis will lanx can be left, the appearance is still abnormal but
802 have made matters worse. function is better. The extensor tendon must never be
sutured to the flexor tendon; this will act as a tether on 26
the common belly of flexor digitorum profundus and
prevent the other digits from flexing fully (the
‘Quadriga effect’). If the middle phalanx is amputated
distal to the flexor digitorum superficialis insertion, the
profundus tendon continues to pull, but now through
Hand injuries
the lumbrical, making the proximal interphalangeal
joint paradoxically extend rather than flex. This irritat-
ing anomaly is avoided by suturing the superficialis
stump to the flexor sheath or by dividing the lumbrical. 26.21 Late reconstruction The second toe has been
For more proximal injuries, the entire finger with transferred to replace the thumb, which was severed in an
most of its metacarpal may be amputated; the hand is accident.
weakened but the appearance is usually satisfactory. If
the middle ray is amputated through the metacarpal,
the index finger may ‘scissor’ across it in flexion; this
can be overcome by dividing the adjacent index
metacarpal and transposing it to the stump of the exceptional cases. If all the fingers have been lost but
middle metacarpal. the thumb is present, a new finger can sometimes be
constructed with cortical bone, covered by a tubular
flap of skin; an alternative is a neurovascular micro-
surgical transfer from the second toe. If the thumb has
LATE RECONSTRUCTION been lost, the options include pollicization (rotating a
finger to oppose the other fingers), second toe trans-
A severely mutilated hand should be dealt with by a fer and osteoplastic reconstruction (a cortical bone
hand expert. Certain options may be considered in graft surrounded by a skin flap).
803
Injuries of the spine
27
Stephen Eisenstein, Wagih El Masry
in a stable injury, if the neural elements are undam-

PATHOPHYSIOLOGY OF SPINE aged there is little risk of them becoming damaged.
INJURIES An unstable injury is one in which there is a signifi-
cant risk of displacement and consequent damage – or
further damage – to the neural tissues.
Stable and unstable injuries
In assessing spinal stability, three structural ele-
Spinal injuries carry a double threat: damage to the ments must be considered: the posterior osseoligamen-
vertebral column and damage to the neural tissues. tous complex (or posterior column) consisting of the
While the full extent of the damage may be apparent pedicles, facet joints, posterior bony arch, inter-
from the moment of injury, there is always the fear spinous and supraspinous ligaments; the middle col-
that movement may cause or aggravate the neural umn comprising the posterior half of the vertebral
lesion; hence the importance of establishing whether body, the posterior part of the intervertebral disc and
the injury is stable or unstable and treating it as unsta- the posterior longitudinal ligament; and the anterior
ble until proven otherwise. column composed of the anterior half of the vertebral
A stable injury is one in which the vertebral com- body, the anterior part of the intervertebral disc and
ponents will not be displaced by normal movements; the anterior longitudinal ligament (Denis, 1983). All
fractures involving the middle column and at least one
other column should be regarded as unstable. Fortu-
nately, only 10 per cent of spinal fractures are unsta-
ble and less than 5 per cent are associated with cord
damage.
Pathophysiology
Primary changes Physical injury may be limited to the
vertebral column, including its soft-tissue components,
and varies from ligamentous strains to vertebral
fractures and fracture-dislocations. The spinal cord
and/or nerve roots may be injured, either by the initial
trauma or by ongoing structural instability of a
vertebral segment, causing direct compression, severe
energy transfer, physical disruption or damage to its
blood supply.
Secondary changes During the hours and days
following a spinal injury biochemical changes may lead
to more gradual cellular disruption and extension of
the initial neurological damage.
27.1 Structural elements of the spine The vertical lines
show Denis’ classification of the structural elements of the
spine. The three elements are: the posterior complex, the Mechanism of injury
middle component and the anterior column. This concept
is particularly useful in assessing the stability of lumbar There are three basic mechanisms of injury: traction
injuries. (avulsion), direct injury and indirect injury.
27 PRINCIPLES OF DIAGNOSIS AND
INITIAL MANAGEMENT
Diagnosis and management go hand in hand; inap-

propriate movement and examination can irretriev-
ably change the outcome for the worse.
Early management
The adherence to the resuscitation protocol (airway
with cervical spine control, breathing, circulation and
haemorrhage control) supersedes the assessment of
(a) (b) the spinal injury. Adequate oxygenation, ventilation
27.2 Mechanism of injury The spine is usually injured in and circulation will minimize secondary spinal cord
one of two ways: (a) a fall onto the head or the back of injury. The essential principle is that if there is the
the neck; and (b) a blow on the forehead, which forces
the neck into hyperextension.
slightest possibility of a spinal injury in a trauma
patient, the spine must be immobilized until the
patient has been resuscitated and other life-threaten-
ing injuries have been identified and treated. Immobi-
Traction injury In the lumbar spine resisted muscle lization is abandoned only when spinal injury has
effort may avulse transverse processes; in the cervical been excluded by clinical and radiological assessment.
spine the seventh spinous process can be avulsed (‘clay-
shoveller’s fracture’).
Methods of temporary immobilization
Direct injury Penetrating injuries to the spine,
particularly from firearms and knives, are becoming CERVICAL SPINE
increasingly common. In-line immobilization The head and neck are supported
in the neutral position.
Indirect injury This is the most common cause of
significant spinal damage; it occurs most typically in a QUADRUPLE IMMOBILIZATION
fall from a height when the spinal column collapses in A backboard, sandbags, a forehead tape and a semi-
its vertical axis, or else during violent free movements rigid collar are applied. Because children have a rela-
of the neck or trunk. A variety of forces may be applied tively prominent occiput, care must be taken to
to the spine (often simultaneously): axial compression, ensure that the neck is not flexed: padding may be
flexion, lateral compression, flexion-rotation, shear, required behind the shoulders.
flexion-distraction and extension.
NOTE: Insufficiency fractures may occur with Thoracolumbar spine The patient should be moved
minimal force in bone which is weakened by osteo- without flexion or rotation of the thoracolumbar spine.
porosis or a pathological lesion. A scoop stretcher and spinal board are very useful;
however in the paralysed patient, there is a high risk of
pressure sores – adequate padding is essential and
Healing transfer to a special bed must be undertaken as soon as
Spinal injuries may damage both bone and soft tissue possible.
(ligaments, facet joint capsule and intervertebral disc). If the back is to be examined, or if the patient is to
Non-union of fractures is very rare while malunion is be placed onto a scoop stretcher or spinal board, the
common. The bone injury will usually heal; however, logrolling technique should be used.
if the bone structures heal in an abnormal position the
healed soft tissues may not always protect against pro-
gressive deformity. This may occur with flexion
injuries in which there is anterior wedging of the ver- DIAGNOSIS
tebral body of more than 40 per cent. An increasing
flexion-deformity (kyphosis) may occur. Injuries with
a predominant soft-tissue element – for example History
flexion-distraction with bilateral facet dislocation and A high index of suspicion is essential; symptoms and
disruption of the posterior ligaments and disc – heal signs may be minimal; the history is crucial. Every
with fibrous tissue and can become completely stable; patient with a blunt injury above the clavicle, a head
806 sometimes, however, they do not regain stability. injury or loss of consciousness should be considered
to have a cervical spine injury until proven otherwise. BACK 27
Every patient who is involved in a fall from a height The patient is ‘log-rolled’ (i.e. turned over ‘in one
or a high-speed deceleration accident should similarly piece’) to avoid movement of the vertebral column.
be considered to have a thoracolumbar injury. The The back is inspected for deformity, penetrating
safe approach is to consider the presence of a vertebral injury, haematoma or bruising. The bone and soft-tis-
column injury in all patients with multiple injuries. sue structures are palpated, again with particular ref-

Lesser injuries also should arouse suspicion if they are erence to the interspinous spaces. A haematoma, a gap
followed by pain in the neck or back or neurological or a step are signs of instability.
symptoms in the limbs.
GENERAL EXAMINATION – ‘SHOCK’
Early examination of the severely injured patent is con-
Examination sidered in Chapter 22. The ABC sequence of advanced
NECK trauma life support (ATLS) always takes precedence.
The patient may be supporting his or her head with Three types of shock may be encountered in
their hands – a warning to the examiner to be equally patients with spinal injury:
careful! The head and face are thoroughly inspected Hypovolaemic shock is suggested by tachycardia,
for bruises or grazes which could indicate indirect peripheral shutdown and, in later stages, hypotension.
trauma to the cervical spine. The neck is inspected for Neurogenic shock reflects loss of the sympathetic
deformity, bruising or penetrating injury. The bones pathways in the spinal cord; the peripheral vessels
and soft tissues of the neck are gently palpated for ten- dilate causing hypotension but the heart, deprived of
derness and areas of ‘bogginess’, or increased space its sympathetic innervation, does not respond by
between the spinous processes, suggesting instability increasing its rate. The combination of paralysis, warm
due to posterior column failure. The back of the neck and well-perfused peripheral areas, bradycardia and
must also be examined but throughout the entire hypotension with a low diastolic blood pressure sug-
examination the cervical spine must not be moved gests neurogenic shock. Over-enthusiastic use of
because of the risk of injuring the cord in an unstable fluids can cause pulmonary oedema; atropine and
injury (see below). vasopressors may be required.
(a) (b) (c)
27.3 Spinal injuries – early management (a) Quadruple immobilization: the patient is on a backboard, the head is
supported by sandbags and held with tape across the forehead, and a semi-rigid collar has been applied. (b,c) The
log-rolling technique for exposure and examination of the back.
27.4 Spinal injuries – suspi-

cious signs First appearances
do matter. (a) With severe
facial bruising always suspect a
hyperextension injury of the
neck. (b) Bruising over the
lower back should raise the
suspicion of a lumbar vertebral
fracture.
(a) (b) 807

27 ‘Spinal shock’ occurs when the spinal cord fails tem- Sacral sparing should be tested for. Preservation of
porarily following injury. Even parts of the cord with- active great toe flexion, active anal squeeze (on digital
out structural damage may not function. Below the examination) and intact peri-anal sensation suggest a
level of the injury, the muscles are flaccid, the reflexes partial rather than complete lesion. Further recovery
absent and sensation is lost. This rarely lasts for more may occur.
than 48 hours and during this period it is difficult to The unconscious patient is difficult to examine; a
tell whether the neurological lesion is complete or spinal injury must be assumed until proven otherwise.
incomplete. If the primitive reflexes (anal ‘wink’ and Clues to the existence of a spinal cord lesion are a his-
the bulbocavernosus reflex) are absent, their return tory of a fall or rapid deceleration, a head injury,
usually does not mark the end of ‘spinal shock’; some diaphragmatic breathing, a flaccid anal sphincter,
neurological improvement can occur as time passes. hypotension with bradycardia and a pain response
above, but not below, the clavicle.
NEUROLOGICAL EXAMINATION
A full neurological examination is carried out in every IMAGING
case; this may have to be repeated several times dur- • X-ray examination of the spine is mandatory for all
ing the first few days. Each dermatome, myotome and accident victims complaining of pain or stiffness in
reflex is tested. the neck or back or peripheral paraesthesiae, all
Cord longitudinal column functions are assessed: patients with head injuries or severe facial injuries
corticospinal tract (posterolateral cord, ipsilateral (cervical spine), patients with rib fractures or severe
motor power), spinothalamic tract (anterolateral cord, seat-belt bruising (thoracic spine), and those with
contralateral pain and temperature) and posterior severe pelvic or abdominal injuries (thoracolumbar
columns (ipsilateral proprioception). spine). This is performed during the secondary
survey.
• Accident victims who are unconscious should have
spine x-rays as part of the routine work-up.
• Elderly people and patients with known vertebral
pathology (e.g. ankylosing spondylitis) may suffer
fractures after comparatively minor back injury. The
spine should be x-rayed even if pain is not marked.
Table 27.1 Tests for nerve root motor function

Nerve root Test
C5 Elbow flexion
C6 Wrist extension
C7 Wrist flexion, finger extension
C8 Finger flexion
T1 Finger abduction
L1,2 Hip abduction
L3,4 Knee extension
L5,S1 Knee flexion
L5 Great toe extension
S1 Great toe flexion
Table 27.2 Root values for tendon reflexes

Root value Tendon reflex
C5 Biceps
C6 Brachioradialis
C7 Triceps
L3,4 Quadriceps
27.5 Spine injuries – neurological examination L5,S1 Achilles tendon
808 Dermatomes supplied by the spinal nerve roots.
• Pain is often poorly localized; views should include 27
several segments above and below the painful area. PRINCIPLES OF DEFINITIVE
• X-ray examination should be carried out with a TREATMENT
minimum of movement and manipulation. No
attempt should be made to obtain ‘flexion-and- The objectives of treatment are:
extension’ views during the initial work-up.

• ‘Difficult’ areas, such as the upper cervical spine, • to preserve neurological function;
the cervico-thoracic junction and the upper thoracic • to minimize a perceived threat of neurological com-
segments which are often obscured by shoulder and pression;
rib images, may require plain film tomography, CT • to stabilize the spine;
or MRI. Odontoid fractures also are sometimes bet- • to rehabilitate the patient.
ter shown on axial tomograms than on routine CT. The indications for urgent surgical stabilization are:
• In addition to anteroposterior and lateral views, (a) an unstable fracture with progressive neurological
open-mouth views are needed for the upper two deficit and MRI signs of likely further neurological
cervical vertebrae and oblique views may be needed deterioration; and (b) controversially an unstable frac-
for the cervical as well as the thoracolumbar region. ture in a patient with multiple injuries.
• CT is ideal for showing structural damage to indi-
vidual vertebrae and displacement of bone frag-
Patients with no neurological injury
ments into the vertebral canal. In fact, screening
CT is employed routinely in many centres; the Stable injuries If the spinal injury is stable, the patient
drawback is its high level of radiation exposure. is treated by supporting the spine in a position that will
• MRI is the method of choice for displaying the cause no further strain; a firm collar or lumbar brace
intervertebral discs, ligamentum flavum and neural will usually suffice, but the patient may need to rest in
structures, and is indicated for all patients with neu- bed until pain and muscle spasm subside. The
rological signs and those who are considered for exception is a burst fracture of the vertebral body: a
surgery. CT should be arranged which may show displaced
• CT myelography, with the intrathecal introduction fragments within the spinal canal; however, even if a
of contrast agent, provides information on the retropulsed fragment is identified, operative treatment
dimensions of the spinal canal, impingement by is not imperative, though rehabilitation may be easier
fracture fragments or intervertebral disc, and root if surgery is performed. Furthermore, these patients
avulsion. This investigation has been largely are potentially ‘neurologically unstable’. A progressive
replaced by MRI. neurological deficit may occasionally develop, which
• Three-dimensional reconstruction of CT images could be an indication for decompression and fusion.
defines certain complex fracture patterns. Spiral CT The correction of deformity by surgery is also con-
allows high resolution sagittal reconstruction and, troversial. It is not clear that symptoms are related to
when available, is useful for displaying fractures of minor deformity, although a kyphosis of greater than
the odontoid process. 30 degrees may on occasions be associated with back
• Remember that the spine may be damaged in more pain in the long term. The patient should be given the
than one place. choice between surgery for early mobilization and dis-
• Do not accept poor quality images. charge, and conservative management which is likely
• Consult with the radiologist. to take longer.
27.6 X-ray diagnosis Plain x-

ray alone may be insufficient to
show the true state of affairs.
(a) This x-ray showed the
fracture, but it needed a CT scan
(b) to reveal the large fragment
encroaching on the spinal canal.
(a) (b) 809

27 Unstable injuries If the spinal injury is unstable it ‘Medical treatment’ to counteract the secondary
should be held secure until the tissues heal and the pathophysiological changes associated with cord
spine becomes stable. In the cervical spine this should injury has been (and still is being) pursued. Of the
be done as soon as possible by traction, using tongs or various methods the one that gained most attention
a halo device attached to the skull. If the halo is was the use of corticosteroids. However, after several
attached to a body cast the combination can be used trials in the USA and elsewhere, the use of intra-
as an external fixator for prolonged immobilization venous methylprednisolone is considered to be of

(see below). Alternatively (particularly in the thora- dubious benefit and is currently viewed as an ‘option’
columbar spine) internal fixation can be carried out. for patients seen within the first few hours of injury,
Attempts to reduce dislocations and subluxations rather than a ‘recommendation’ (Short et al., 2000;
should be made whether by adjusting the posture, by Molano et al., 2002; Hugenholtz et al., 2002).
traction or by open operation if the patient so
chooses.
Patients with a neurological injury TREATMENT METHODS

Once spinal shock has recovered, the full extent of the
neurological injury is assessed. Caring for patients Cervical spine
with neurological injury requires the infrastructure of
Collars Soft collars offer very little biomechanical
an experienced multidisciplinary team that can opti-
support to the cervical spine and their use is restricted
mally manage their multisystem physiological impair-
to minor sprains for the first few days after injury. Semi-
ment and malfunction, including the spinal injury.
rigid collars limit motion quite effectively and are
Whenever feasible, they should be transferred to a
widely used in the acute setting. They are not adequate
Spinal Injury Centre as soon as possible after injury.
for very unstable injury patterns. Four-poster braces are
If the spinal injury is stable (which is rare), the
more stable, applying pressure to the mandible,
patient can be treated conservatively and rehabilitated
occiput, sternum and upper thoracic spine. They can
as soon as possible.
be uncomfortable.
With the usual unstable injury, conservative treat-
ment can be still be used; this is highly demanding Tongs A pin is inserted into the outer table on each
and is best carried out in a special unit equipped for side of the skull; these are mounted on a pair of tongs
round-the-clock nursing, 2-hourly turning routines, and traction is applied to reduce the fracture or
skin toilet, bladder care and specialized physiotherapy dislocation and to maintain the reduced position.
and occupational therapy. After a few weeks the injury
Halo ring At least four pins are inserted into the outer
stabilizes spontaneously and the patient can be got
table of the skull and a ring is applied. The use of tita-
out of bed for intensive rehabilitation. This approach
nium pins and graphite ring allows an MRI scan to be
is applicable to almost all injuries. Early operative
performed. The halo ring can be used for initial trac-
stabilization is preferred by many; it facilitates nursing
tion and reduction of the fracture or dislocation, and
by inexperienced carers and reduces the risk of spinal
then can be attached to a plaster vest. Proper position-
deformity.
ing and torque-pressure of the pins is essential. Bear in
The benefit of surgery on ease and speed of
mind that the use of a halo-vest carries a significant risk
rehabilitation, total period of hospitalization and neu-
of complications such as pin loosening, pin-site infec-
rological recovery is uncertain. A positive indication
tion and (in elderly patients) respiratory distress.
for early operative reduction or decompression and
stabilization is progressive neurological deterioration Fixation Various operative procedures are available,
with evidence (or a serious risk) of further neural com- depending on the level and pattern of injury. Odontoid
pression on MRI. fractures can be fixed with lag screws, burst fractures
Patients with incomplete lesions are also sometimes can be decompressed through an anterior approach,
considered for operation, but there is little enthusiasm and facet dislocations can be reduced through a
for this approach in specialized centres. Significant posterior approach. The spine can be stabilized
neurological recovery occurs without surgery in the anteriorly with plates between the vertebral bodies or
majority of those who present with sensory and/or posteriorly with wires between the spinous processes,
motor sparing in the first 48–72 hours. Furthermore, or with small plates between the lateral masses.
such recovery can theoretically be endangered by
operative manoeuvres, arterial injury, hypoxia,
Thoracolumbar spine
hypotension, hypothermia, further damage to the
blood–brain barrier or sepsis associated with spinal Beds Special beds are used in the management of
810 surgery. spinal injuries. They are designed to avoid pressure
systems provide fixation between intact vertebrae sev- 27
eral segments above and below the injury. The advent
of segmental spinal instrumentation, with the fixation
device attached to the spinal column through pedicle
screws, allows secure fixation of a much shorter
implant, reaching only one or two segments away

from the injury. These devices also allow correction of
the deformity by distraction and extension. Bone graft
is required so that a biological fusion can supplement
the implants.
(a) (b) CERVICAL SPINE INJURIES

27.7 Spine injuries –
treatment (a) Standard The patient will usually give a history of a fall from a
cervical collar. (b) More rigid height, a diving accident or a vehicle accident in
variety. (c) Halo-body cast. which the neck is forcibly moved. In a patient uncon-
scious from a head injury, a fractured cervical spine
should be assumed (and acted upon) until proved
otherwise.
An abnormal position of the neck is suggestive, and
careful palpation may elicit tenderness. Movement is
best postponed until the neck has been x-rayed. Pain
or paraesthesia in the limbs is significant, and the
(c)
patient should be examined for evidence of spinal
cord or nerve root damage.
sores (with special mattresses or the facility to turn the Imaging

patient frequently). Some beds allow postural Plain x-rays must be of high quality and should be
reduction of fractures. inspected methodically.
Brace A thoracolumbar brace avoids flexion by three- • In the anteroposterior view the lateral outlines
point fixation. It is suitable for some burst fractures, should be intact, and the spinous processes and tra-
seat-belt injuries and compression fractures. cheal shadow in the midline. An open-mouth view
Decompression and stabilization The aim of surgery is
is necessary to show C1 and C2 (for odontoid and
to reduce the fracture, hold the reduction and lateral mass fractures).
decompress the neural elements. The surgical approach
can be either anterior or posterior.
The anterior approach is suitable for burst fractures
with significant canal impingement or as a supplement 27.8 Cervical spine
to posterior fixation in those compression fractures injury Look at the
with considerable loss of anterior bone stock. With an position of this patient’s
anterior approach, the spine is exposed through a neck. He complained of
pain and stiffness after
transthoracic, transdiaphragmatic or transperitoneal a fall. It could have
approach depending on the level of the fracture. The been no more than a
vertebral body is removed so that the spinal canal is soft-tissue strain, but
decompressed; a bone graft (rib, fibula or iliac crest) x-ray examination
is then inserted and special plates are applied between revealed an odontoid
fracture.
the intact vertebral bodies above and below the
injured level.
The posterior approach is more suitable for flexion-
compression injuries, seat-belt injuries and fracture-
dislocations. Some burst fractures can also be reduced
indirectly from a posterior approach using implants
that apply distraction to the fracture. Hook and rod 811
27
(a) (b)
27.10 Cervical spine injuries – x-ray diagnosis

(a) Following a traffic accident this patient had a painful
neck and consulted her doctor three times; on each occa-
sion she was told ‘the x-rays are normal‘. But count the
vertebrae! There are only six in this film. (b) When a
shoulder ‘pull-down view’ was obtained to show the
entire cervical spine, a dislocation of C6 on C7 could be
seen at the very bottom of the film.
27.9 Cervical spine – normal x-ray In the lateral projec-

tion, four parallel lines can be traced unbroken from C1 to
C7. They are formed by: (1) the anterior surfaces of the then a ‘swimmer’s view’ is obtained. If this, too,
vertebral bodies; (2) the posterior surfaces of the bodies; fails, then tomography or a CT scan is required.
(3) the posterior borders of the lateral masses; and (4) the
• The distance between the odontoid peg and the
bases of the spinous processes.
back of the anterior arch of the atlas should be no
more than 3 mm in adults and 4.5 mm in children.
• Compare the shape of each vertebral body with that
of the others; note particularly any loss of height,
fragmentation or backward displacement of the
• In the lateral view the smooth lordotic curve should posterior border of the vertebral body.
be followed, tracing four parallel lines formed by • Examine the soft-tissue shadows. The retropharyn-
the front of the vertebral bodies, the back of the geal space may contain a haematoma; the preverte-
bodies, the posterior borders of the lateral masses bral soft-tissue shadow should be less than 5 mm in
and the bases of the spinous processes; any irregu- thickness above the level of the trachea and less
larity suggests a fracture or displacement. Forward than one vertebral body’s width in thickness below.
shift of the vertebral body by 25 per cent suggests The interspinous space may be widened after liga-
a unilateral facet dislocation and by 50 per cent a ment rupture.
bilateral facet dislocation.
• The lateral view must include all seven cervical ver-
Diagnostic pitfalls in children
tebrae and the upper half of T1, otherwise a serious
injury at the cervico-thoracic junction will be Children are often distressed and difficult to examine;
missed. If the cervico-thoracic junction cannot be more than usual reliance may be placed on the x-rays.
seen, then the lateral view should be repeated while It is well to recall some common pitfalls.
812 the patient’s shoulders are pulled down. If this fails, An increased atlanto-dental interval (up to 4.5mm)
may be quite normal; this is because the skeleton is 27
incompletely ossified and the ligaments relatively lax
during childhood. There may also be apparent sub-
luxation of C2 on C3 (pseudosubluxation).
An increased retropharyngeal space can be brought
about by forced expiration during crying.

Growth plates and synchondroses can be mistaken
for fractures. The normal synchondrosis at the base of
the dens has usually fused by the age of 6 years, but it
can be mistaken for an undisplaced fracture; the spin-
ous process growth plates also resemble fractures; and
the growth plate at the tip of the odontoid can be
taken for a fracture in older children.
SCIWORA is an acronym for spinal cord injury
without obvious radiographic abnormality. Normal
radiographs in children do not exclude the possibility
of spinal cord injury.
UPPER CERVICAL SPINE

Occipital condyle fracture
This is usually a high-energy fracture and associated
skull or cervical spine injuries must be sought. The
diagnosis is likely to be missed on plain x-ray exami-
nation and CT is essential. 27.11 Occipito–cervical fusion X-ray showing one of
the devices used for internal fixation in occipito-cervical
Impacted and undisplaced fractures can be treated fusion operations.
by brace immobilization for 8–12 weeks. Displaced
fractures are best managed by using a halo-vest or by
operative fixation. C1 ring fracture
Sudden severe load on the top of the head may cause
a ‘bursting’ force which fractures the ring of the atlas
Occipito-cervical dislocation (Jefferson’s fracture). There is no encroachment on
This high-energy injury is almost always associated the neural canal and, usually, no neurological damage.
with other serious bone and/or soft-tissue injuries, The fracture is seen on the open-mouth view (if the
including arterial and pharyngeal disruption, and the lateral masses are spread away from the odontoid peg)
outcome is often fatal. Patients are best dealt with by and the lateral view. A CT scan is particularly helpful
a multidisciplinary team of surgeons and physicians. in defining the fracture. If it is undisplaced, the injury
The diagnosis can sometimes be made on the lateral
cervical radiograph: the tip of the odontoid should be
no more than 5mm in vertical alignment and 1mm in
horizontal alignment from the basion (anterior rim of
the foramen magnum). Greater distances are allow-
able in children. CT scans are more reliable.
The injury is likely to be unstable and requires
immediate reduction (without traction!) and stabiliza-
tion with a halo-vest, pending surgical treatment.
After appropriate attention to the more serious soft-
tissue injuries and general resuscitation, the disloca-
tion should be internally fixed; specially designed
occipito-cervical plates and screws are available for the
purpose. In severely unstable injuries, halo-vest
stabilization should be retained for another 6–8 27.12 Fracture of C1 ring Jefferson’s fracture – bursting
weeks. apart of the lateral masses of C1. 813
27 is stable and the patient wears a semi-rigid collar or
halo-vest until the fracture unites. If there is sideways
spreading of the lateral masses (more than 7 mm on
the open-mouth view), the transverse ligament has
ruptured; this injury is unstable and should be treated
by a halo-vest for several weeks. If there is persisting
instability on x-ray, a posterior C1/2 fixation and

fusion is needed.
A hyperextension injury can fracture either the 27.13 Fracture of C2 ‘Hangman’s fracture’ – fracture of
anterior or posterior arch of the atlas. These injuries the pars interarticularis of C2.
are usually relatively stable and are managed with a
halo-vest or semi-rigid collar until union occurs.
velocity accidents or severe falls. However, they also
Fractures of the atlas are associated with injury else-
occur in elderly, osteoporotic people as a result of
where in the cervical spine in up to 50 per cent of
low-energy trauma in which the neck is forced into
cases.
hyperextension, e.g. a fall onto the face or forehead.
A displaced fracture is really a fracture-dislocation
C2 pars interarticularis fractures of the atlanto-axial joint in which the atlas is shifted
forwards or backwards, taking the odontoid process
In the true judicial ‘hangman’s fracture’ there are
with it. At this level about a third of the internal diam-
bilateral fractures of the pars interarticularis of C2 and
eter of the atlas is free space, a third filled with the
the C2/3 disc is torn; the mechanism is extension
odontoid and a third with the cord; thus there is room
with distraction. In civilian injuries, the mechanism is
for displacement without neurological injury. How-
more complex, with varying degrees of extension,
ever, cord damage is not uncommon and in old peo-
compression and flexion. This is one cause of death in
ple there is a considerable mortality rate.
motor vehicle accidents when the forehead strikes the
dashboard. Neurological damage, however, is unusual
because the fracture of the posterior arch tends to Classification
decompress the spinal cord. Nevertheless the fracture
Odontoid fractures have been classified by Anderson
is potentially unstable.
and D’Alonzo (1974) as follows:
Undisplaced fractures which are shown to be stable
on supervised flexion–extension views (less than 3mm • Type I – An avulsion fracture of the tip of the odon-
of C2/3 subluxation) can be treated in a semi-rigid toid process due to traction by the alar ligaments.
orthosis until united (usually 6–12 weeks). The fracture is stable (above the transverse liga-
Fractures with more than 3mm displacement but ment) and unites without difficulty.
no kyphotic angulation may need reduction; however, • Type II – A fracture at the junction of the odontoid
because the mechanism of injury usually involves dis- process and the body of the axis. This is the most
traction, traction must be avoided. After reduction, the common (and potentially the most dangerous) type.
neck is held in a halo-vest until union occurs. C2/3 The fracture is unstable and prone to non-union.
fusion is sometimes required for persistent pain and • Type III – A fracture through the body of the axis.
instability (‘traumatic spondylolisthesis’). The fracture is stable and almost always unites with
Occasionally, the ‘hangman’s fracture’ is associated immobilization.
with a C2/3 facet dislocation. This is a severely unsta-
ble injury; open reduction and stabilization is required.
Clinical features
The history is usually that of a severe neck strain
C2 Odontoid process fracture
followed by pain and stiffness due to muscle spasm.
Odontoid fractures are uncommon. They usually The diagnosis is confirmed by high quality x-ray
occur as flexion injuries in young adults after high- examination; it is important to rule out an associated
27.14 Odontoid fractures –

classification (a) Type I –
fracture through the tip of the
odontoid process. (b) Type II –
fracture at the junction of the
odontoid process and the body.
(c) Type III – fracture through
the body of the axis. (Anderson
and D’Alonzo, 1974.)
814 (a) (b) (c)
or – in elderly patients – a rigid collar. Displaced 27
fractures should be reduced by traction and can then
be held by operative posterior C1/2 fusion; a
drawback is that neck rotation will be restricted.
Anterior screw fixation is suitable for Type II fractures
that run from anterior-superior to posterior-inferior,

provided the fracture is not comminuted, that the
(a) (b) transverse ligament is not ruptured, that the fracture is
fully reduced and the bone solid enough to hold a
27.15 Fractured odontoid process (a) Anteroposterior
‘open-mouth’ x-ray showing a Type II odontoid fracture. screw; in that case neck rotation is retained. If full
(b) Lateral x-ray of the same patient. operative facilities are not available, immobilization can
be applied by using a halo-vest with repeated x-ray
monitoring to check for stability.
occipito-cervical injury which commands immediate
Type III fractures If undisplaced, these are treated in a
attention. In some cases the clinical features are mild
halo-vest for 8–12 weeks. If displaced, attempts should
and continue to be overlooked for weeks on end.
be made at reducing the fracture by halo traction,
Neurological symptoms occur in a significant number
which will allow positioning in either flexion or
of cases.
extension, depending on whether the displacement is
forward or backward; the neck is then immobilized in
Imaging a halo-vest for 8–12 weeks. For elderly patients with
poor bone a collar may suffice, though this carries a
Plain x-rays usually show the fracture, although the
higher risk of non-union.
extent of the injury is not always obvious – e.g. there
may be an associated fracture of the atlas or displace-
ment at the occipito-atlanto level. Tomography is
helpful but MRI has the advantage that it may reveal LOWER CERVICAL SPINE
rupture of the transverse ligament; this can cause
Fractures of the cervical spine from C3 to C7 tend to
instability in the absence of a fracture.
produce characteristic fracture patterns, depending on
the mechanism of injury: flexion, axial compression,
Treatment flexion–rotation or hyperextension.
Type I fractures Isolated fractures of the odontoid tip
are uncommon. They need no more than Posterior ligament injury
immobilization in a rigid collar until discomfort
Sudden flexion of the mid-cervical spine can result in
subsides.
damage to the posterior ligament complex (the inter-
Type II fractures These are often unstable and prone to spinous ligament, facet capsule and supraspinous liga-
non-union, especially if displaced more than 5 mm. ment). The upper vertebra tilts forward on the one
Undisplaced fractures can be held by fitting a halo-vest below, opening up the interspinous space posteriorly.
(a) (b) (c) (d)
27.16 Fractured odontoid – treatment (a) A severely displaced Type II odontoid fracture. (b) The fracture was reduced
by skull traction and held by fixing the spinous process of C1 to that of C2 with wires. (c) An undisplaced Type II fracture,
which was suitable for (d) anterior screw fixation. 815
27
(a) (b)
27.17 Cervical spine – posterior ligament injury

(a) The film taken in extension shows no displacement of
the vertebral bodies, but there is an unduly large gap
between the spinous processes of C4 and 5. (b) With the 27.18 Cervical compression fracture A wedge com-
neck slightly flexed the subluxation is obvious. pression fracture of a single cervical vertebral body. This is
NB: flexion–extension views are potentially dangerous and a stable injury because the middle and posterior elements
should be used only in specific situations under direct are intact. Compare and contrast with Figure 27.19.
supervision of an experienced surgeon.
fragment, i.e. features of a burst fracture (see below)

The patient complains of pain and there may be which is potentially dangerous. If there is the least
localized tenderness posteriorly. X-ray may reveal a doubt, an axial CT or MRI should be obtained.
slightly increased gap between the adjacent spines;
however, if the neck is held in extension this sign can Burst and compression-flexion (‘tear-
be missed, so it is always advisable to obtain a lateral
view with the neck in the neutral position. A flexion
drop’) fractures
view would, of course, show the widened interspinous These severe injuries are due to axial compression of
space more clearly, but flexion should not be permitted the cervical spine, usually in diving or athletic acci-
in the early post-injury period. This is why the diagno- dents (Fig. 27.19). If the vertebral body is crushed in
sis is often made only some weeks after the injury, neutral position of the neck the result is a ‘burst
when the patient goes on complaining of pain. fracture’. With combined axial compression and flex-
The assessment of stability is essential in these cases. ion, an antero-inferior fragment of the vertebral body
If the angulation of the vertebral body with its neigh- is sheared off, producing the eponymous ‘tear-drop’
bour exceeds 11 degrees, if there is anterior transla- on the lateral x-ray. In both types of fracture there is a
tion of one vertebral body upon the other of more risk of posterior displacement of the vertebral body frag-
than 3.5 mm or if the facets are fractured or displaced, ment and spinal cord injury.
then the injury is unstable and it should be treated as Plain x-rays show either a crushed vertebral body
a subluxation or dislocation. If it is certain that the (burst fracture) or a flexion deformity with a triangu-
injury is stable, a semi-rigid collar for 6 weeks is ade- lar fragment separated from the antero-inferior edge
quate; if the injury is unstable then posterior fixation of the fractured vertebra (the innocent-looking ‘tear-
and fusion is advisable. drop’). The x-ray images should be carefully exam-
ined for evidence of middle column damage and
posterior displacement (even very slight displace-
Wedge compression fracture
ment) of the main body fragment. Traction must be
A pure flexion injury results in a wedge compression applied immediately and CT or MRI should be per-
fracture of the vertebral body (Fig. 27.18). The mid- formed to look for retropulsion of bone fragments
dle and posterior elements remain intact and the into the spinal canal.
injury is stable. All that is needed is a comfortable col-
lar for 6–12 weeks. TREATMENT
A note of warning: The x-ray should be carefully If there is no neurological deficit, the patient can be
examined to exclude damage to the middle column treated surgically or by confinement to bed and trac-
816 and posterior displacement of the vertebral body tion for 2–4 weeks, followed by a further period of
27

(a) (b) (c)
27.19 Tear-drop fracture (a) This comminuted vertebral body fracture has produced a large anterior fragment and obvi-
ous posterior displacement of the posterior fragment. (b) In this case the anterior ‘tear-drop’ was noted but the severity of
the injury was underestimated; careful examination shows that the main body fragment is displaced slightly posteriorly.
The patient was treated in a collar; 3 weeks later (c) the fracture had collapsed and the large body fragment was now
very obviously tilted and displaced posteriorly. By then he was complaining of tingling and weakness in his right arm.
Beware the innocent tear-drop!
immobilization in a halo-vest for 6–8 weeks. (The The lateral x-ray shows forward displacement of a
halo-vest is unsuitable for initial treatment because it vertebra on the one below of greater than half the ver-
does not provide axial traction). tebra’s antero-posterior width.
If there is any deterioration of neurological status The displacement must be reduced as a matter of
while the fracture is believed to be unstable, and the urgency. Skull traction is used, starting with 5 kg and
MRI shows that there is a threat of cord compression, increasing it step-wise by similar amounts up to about
then urgent anterior decompression is considered – 30kg; intravenous muscle relaxants and a bolster
anterior corpectomy, bone grafting and plate fixation, beneath the shoulders may help. The entire procedure
and sometimes also posterior stabilization. should be done without anaesthesia (or under mild
sedation only) and neurological examination should
be repeated after each incremental step. If neuro-
Fracture-dislocations logical symptoms or signs develop, or increase,
Bilateral facet joint dislocations are caused by severe further attempts at closed reduction should be
flexion or flexion–rotation injuries. The inferior artic- stopped.
ular facets of one vertebra ride forward over the supe- When x-rays show that the dislocation has been
rior facets of the vertebra below. One or both of the reduced, traction is diminished to about 5 kg and then
articular masses may be fractured or there may be a maintained for 6 weeks. During this time MRI can be
pure dislocation – ‘jumped facets’. The posterior performed to rule out the presence of an associated
ligaments are ruptured and the spine is unstable; often disc disruption. At the end of that period the patient
there is cord damage. should still wear a collar for another 6 weeks;
(a) (b) (c) (d)
27.20 Cervical fracture-dislocation (a) Fracture-dislocation in the lower cervical spine. (b,c) Stages in the reduction of
this fracture-dislocation by skull traction; (d) subsequent posterior wiring to ensure stability. 817
27 however, it may be more convenient to immobilize weeks. However, in about 50 per cent of the patients
the neck in a halo-vest for 12 weeks. surgery may still have to be considered at the end of
Another alternative is to carry out a posterior fusion this period. If there is an associated facet fracture or
as soon as reduction has been achieved; the patient is recurrent dislocation in the external fixator, then pos-
then allowed up in a cervical brace which is worn for terior fusion again becomes necessary. Patients left
6–8 weeks. Posterior open reduction and fusion is also with an unreduced unilateral facet dislocation may
indicated if closed reduction fails. develop neck pain and nerve root symptoms long-
The need for pre-reduction MRI is controversial. In term if poorly managed.
its favour is the ability to diagnose an extruded disc Remember that halo vests can cause pressure sores
fragment which may further compromise any neuro- over the scapula in sensory impaired patients.
logical lesion but can be dealt with by anterior decom-
pression. This is particularly applicable to elderly
patients in whom immediate closed reduction may be Hyperextension injury
hazardous and long periods on their backs can lead to
Hyperextension strains of soft-tissue structures are
pressure sores. An argument against pre-reduction
common and may be caused by comparatively mild
MRI is that there is insufficient correlation between
acceleration forces. Bone and joint disruptions, how-
various degrees of disc extrusion and neurological
ever, are rare.
deterioration to justify another surgical assault on the
The more severe injuries are suggested by the his-
traumatized patient.
tory and the presence of facial bruising or lacerations.
Unilateral facet dislocation This is a flexion–rotation The posterior bone elements are compressed and may
injury in which only one apophyseal joint is dislocated. fracture; the anterior structures fail in tension, with
There may be an associated fracture of the facet. On tearing of the anterior longitudinal ligament or an
the lateral x-ray the vertebral body appears to be avulsion fracture of the anterosuperior or anteroinfe-
partially displaced (less than one-half of its width); on rior edge of the vertebral body, opening up of the
the anteroposterior x-ray the alignment of the spinous anterior part of the disc space, fracture of the back of
processes is distorted. Cord damage is unusual and the the vertebral body and/or damage to the interverte-
injury is stable. bral disc. In patients with pre-existing cervical
Management is the same as for bilateral dislocation. spondylosis, the cord can be pinched between the
Sometimes complete reduction is prevented by the bony spurs or disc and the posterior ligamentum
upper facet becoming perched upon the lower. When flavum; oedema and haematomyelia may cause an
no further progress occurs, it is tempting to assist in acute central cord syndrome (quadriplegia, sacral
the final reduction by gently manipulating the sparing and more upper limb than lower limb deficit,
patient’s head in extension and rotation; this should a flaccid upper limb paralysis and spastic lower limb
be attempted only by an experienced operator. As a paralysis).
general rule, if closed reduction fails, open reduction These injuries are stable in the neutral position, in
and posterior fixation are advisable. which they should be held by a collar for 6–8 weeks.
After reduction, if the patient is neurologically Healing may lead to spontaneous fusion between
intact the neck is immobilized in a halo-vest for 6–8 adjacent vertebral bodies.
5
5 5
6 6 6
7 7
(a) (b) (c) (d)
27.21 Hyperextension injuries (a) The anterior longitudinal ligament has been torn; in the neutral position the gap will
close and reduction will be stable, but a collar or brace will be needed until the soft tissues are healed. (b) X-ray in this
case showed a barely visible flake of bone anteriorly at the C6/7 disc space. (c) 1 month later the traction fracture at C6/7
was more obvious, as was the disc lesion at C5/6. (d) A year later C6/7 has fused anteriorly; the patient still has neck pain
818 due to the C5/6 disc degeneration.
Double injuries Sudden paresis will need immediate surgical 27
decompression. With lesser symptoms and signs, one
With high-energy trauma the cervical spine may be can afford to wait a few days for improvement; if this
injured at more than one level. Discovery of the most does not occur, then anterior discectomy and inter-
obvious lesion is no reason to drop one’s guard. Two body fusion will be needed.
salutary examples are shown in Figures 27.22 and 27.23.

Avulsion injury of the spinous process Neurapraxia of the cervical cord
Fracture of the C7 spinous process may occur with Accidents causing sudden, severe axial loading with
severe voluntary contraction of the muscles at the the neck in hyperflexion or hyperextension are occa-
back of the neck; it is known as the clay-shoveller’s sionally followed by transient pain, paraesthesia and
fracture. The injury is painful but harmless. No treat- weakness in the arms or legs, all in the absence of any
ment is required; as soon as symptoms permit, neck x-ray or MRI abnormality. Symptoms may last for as
exercises are encouraged. little as a few minutes or as long as two or three days.
The condition has been called neurapraxia of the
cervical cord and is ascribed to pinching of the cord
Cervical disc herniation by the bony edges of the mobile spinal canal and/or
Acute post-traumatic disc herniation may cause severe local compression by infolding of the posterior longi-
pain radiating to one or both upper limbs, and neu- tudinal ligament or the ligamentum flavum (Thomas
rological symptoms and signs ranging from mild et al., 1999). Congenital narrowing of the spinal canal
paraesthesia to weakness, loss of a reflex and blunted may be a predisposing factor.
sensation. Rarely a patient presents with full-blown Treatment consists of reassurance (after full neuro-
paresis. The diagnosis is confirmed by MRI or CT- logical investigation) and graded exercises to improve
myelography. strength in the neck muscles.
27.22 Double cervical injuries (a) This

patient with a neck injury was suspected of
having an odontoid fracture. This was con-
firmed and a posterior stabilization was per-
formed. Only when the brace was removed
and he started flexing his neck did the x-ray
show an obvious subluxation lower down
(b). This was treated by anterior fusion (c).
(a) (b) (c)
27.23 Avulsions (a) The clay-shoveller’s frac-

ture. Jerking the neck backwards has resulted
in avulsion of one of the spinous processes – a
benign injury. (b) This patient might be
thought to have a similar fracture, but a subse-
quent flexion film (c) shows the serious nature
of the injury – a severe fracture-dislocation.
(a) (b) (c) 819

27 SPRAINED NECK (WHIPLASH INJURY) MRI may show early degenerative changes, but no
more commonly than in the age-matched population
Soft-tissue sprains of the neck are so common after at large; the examination is not indicated except in
motor vehicle accidents that they now constitute a patients with convincing neurological signs.
veritable epidemic. There is usually a history of a low- For purposes of comparison, the severity grading
velocity rear-end collision in which the occupant’s system proposed by the Quebec Task Force on
body is forced against the car seat while his or her Whiplash-Associated Disorders is useful.
head flips backwards and then recoils in flexion. This
mechanism has generated the imaginative term
whiplash injury, which has served effectively to
enhance public apprehension at its occurrence. How- The diagnosis of sprained neck is reached largely by a
ever, similar symptoms are often reported with flexion process of exclusion, i.e. the inability to demonstrate
and rotation injuries. Women are affected more often any other credible explanation for the patient’s
than men, perhaps because their neck muscles are symptoms. X-rays should be carefully scrutinized to
more gracile. There is disagreement about the exact avoid missing a vertebral fracture or a mid-cervical
pathology but it has been suggested that the anterior subluxation. The presence of neurological signs such
longitudinal ligament of the spine and the capsular as muscle weakness and wasting, a depressed reflex or
fibres of the facet joints are strained and in some cases definite loss of sensibility should suggest an acute disc
the intervertebral discs may be damaged in some lesion and is an indication for MRI.
unspecified manner. There is no correlation between Seat-belt injuries often accompany neck sprains.
the amount of damage to the vehicle and the severity They do not always cause bruising of the chest, but
of complaints. they can produce pressure or traction injuries of the
suprascapular nerve or the brachial plexus, either of
which may cause symptoms resembling those of a
Clinical features whiplash injury. The examining doctor should be
Often the victim is unaware of any abnormality imme- familiar with the clinical features of these conditions.
diately after the collision. Pain and stiffness of the
neck usually appear within the next 12–48 hours, or
Treatment
occasionally only several days later. Pain sometimes
radiates to the shoulders or interscapular area and may Collars are more likely to hinder than help recovery.
be accompanied by other, more ill-defined, symptoms Simple pain-relieving measures, including analgesic
such as headache, dizziness, blurring of vision, paraes- medication, may be needed during the first few weeks.
thesia in the arms, temporomandibular discomfort However, the emphasis should be on graded exer-
and tinnitus. Neck muscles are tender and movements cises, beginning with isometric muscle contractions
often restricted; the occasional patient may present and postural adjustments, then going on gradually to
with a ‘skew neck’. Other physical signs – including active movements and lastly movements against resist-
neurological defects – are uncommon. ance. The range of movement in each direction is
X-ray examination may show straightening out of slowly increased without subjecting the patient to
the normal cervical lordosis, a sign of muscle spasm; unnecessary pain. Many patients find osteopathy and
in other respects the appearances are usually normal. chiropractic treatment to be helpful.
In some cases, however, there are features of long-
standing intervertebral disc degeneration or degener-
Progress and outcome
ative changes in the uncovertebral joints; it may be
that these patients suffer more, and for longer spells, The natural history of whiplash injury is reflected in
than others. the statistics appearing in the medical literature on this
subject. Details and references are presented in a
Table 27.3 Proposed grading of whiplash-associated recent review by Bannister et al. (2009).
injuries Many people who are involved in road collisions do
Grade Clinical pattern
not seek medical attention at all; this is particularly the
case in countries where medical and legal costs are not
0 No neck symptoms or signs
compensated. Some patients start improving within a
1 Neck pain, stiffness and tenderness few weeks and reports in the medical literature sug-
No physical signs gest that 50–60 per cent eventually make a full recov-
2 Neck symptoms and musculoskeletal signs ery; in most cases symptoms diminish after about 3
3 Neck symptoms and neurological signs months and go on improving over the next year or
two; however, 2–5 per cent continue to complain of
4 Neck symptoms and fracture or dislocation
820 symptoms and loss of functional capacity more or less
indefinitely (Bannister et al., 2009). Negative prog- compressive, tensile or tortional strains; and high- 27
nostic indicators are increasing age, severity of symp- energy fractures or fracture-dislocations due to major
toms at the outset, prolonged duration of symptoms injuries sustained in motor vehicle collisions, falls or
and the presence of pre-existing intervertebral disc diving from heights, sporting events, horse-riding and
degeneration. Other factors that presage a poor out- collapsed buildings. It is mainly in the third group
come are a history of pre-accident psychological dys- that one encounters neurological complications, but

function, unduly frequent attendance with unrelated lesser fractures also sometimes cause nerve damage.
physical complaints, a record of unemployment and a The common mechanisms of injury are:
general tendency to underachievement.
• Flexion–compression – failure of the anterior
It should be borne in mind that outcome studies
column and wedge-compression of the vertebral
are almost invariably based on a selected group of
body. Usually stable, but greater than 50 per cent
patients, namely those who attend for medical treat-
loss of anterior height suggests some disruption of
ment after the accident, and little is known of the nat-
the posterior ligamentous structures.
ural progress in the thousands of people who
• Lateral compression – lateral wedging of the
experience similar injuries and either do not develop
vertebral body resulting in a localized ‘scoliotic’
symptoms or do not report them.
deformity.
• Axial compression – failure of anterior and middle
Chronic whiplash-associated disorder columns causing a ‘burst’ fracture and the danger
of retropulsion of a posterior fragment into the
Those patients who, in the absence of any objective
spinal canal. Often unstable.
clinical or imaging signs, continue almost indefinitely
• Flexion–rotation – failure of all three columns and
to complain of pain, restriction of movement, loss of
a risk of displacement or dislocation. Usually
function, depression and inability to work constitute a
unstable.
sizeable problem in terms of medical resources, com-
• Flexion–distraction – the so-called ‘jack-knife’
pensation claims, legal costs and – not least – personal
injury causing failure of the posterior and middle
suffering. As yet, no convincing evidence of a new
columns and sometimes also anterior
pathological lesion has been adduced to account for
compression.
this long-lasting disorder and it cannot be said with
• Extension – tensile failure of the anterior column
certainty how much of it is due to a physical abnor-
and compression failure of the posterior column.
mality and how much is an expression of a behavioural
Unstable.
disorder. The subject is well reviewed in the Current
Concepts monograph edited by Gunzburg and
Szpalski (1997). Examination
Patients complaining of back pain following an injury
or showing signs of bruising and tenderness over the
THORACOLUMBAR INJURIES spine, as well as those suffering head or neck injuries,
chest injuries, pelvic fractures or multiple injuries else-
Most injuries of the thoracolumbar spine occur in the where, should undergo a careful examination of the
transitional area – T11 to L2 – between the somewhat spine and a full neurological examination, including
rigid upper and middle thoracic column and the flex- rectal examination to assess sphincter tone.
ible lumbar spine. The upper three-quarters of the
thoracic segments are also protected to some extent
by the rib-cage and fractures in this region tend to be
Imaging
mechanically stable. However, the spinal canal in that X-rays The anteroposterior x-ray may show loss of
area is relatively narrow so cord damage is not uncom- height or splaying of the vertebral body with a crush
mon and when it does occur it is usually complete fracture. Widening of the distance between the pedicles
(Bohlman, 1985). The spinal cord actually ends at L1 at one level, or an increased distance between two
and below that level it is the lower nerve roots that are adjacent spinous processes, is associated with posterior
at risk. column damage. The lateral view is examined for
alignment, bone outline, structural integrity, disc space
defects and soft-tissue shadow abnormalities. Always
Pathogenesis look carefully for evidence of fragment retropulsion
Pathogenetic mechanisms fall into three main groups: towards the spinal canal. Plain x-rays, while showing
low-energy insufficiency fractures arising from com- the lower thoracic and lumbar spine quite clearly, are
paratively mild compressive stress in osteoporotic less revealing of the upper thoracic vertebrae because
bone; minor fractures of the vertebral processes due to the scapulae and shoulders get in the way 821
27 CT and MRI Rapid screening CT scans are now routine Fracture of the pars interarticularis
in many accident units. Not only are they more reliable
than x-rays in showing bone injuries throughout the A stress fracture of the pars interarticularis should be
spine, and indispensable if axial views are necessary, but suspected if a gymnast or athlete or weight-lifter com-
they also eliminate the delay, discomfort and anxiety plains of the sudden onset of back pain during the
so often associated with multiple attempts at ‘getting course of strenuous activity. The injury is often
the right views’ with plain x-rays. In some cases MRI ascribed to a disc prolapse, whereas in fact it may be a
also may be needed to evaluate neurological or other stress fracture of the pars interarticularis (traumatic
soft-tissue injuries. spondylolysis). This is best seen in the oblique x-rays,
but a thin fracture line is easily missed; a week or two
later, an isotope bone scan may show a ‘hot’ spot.
Treatment Bilateral fractures occasionally lead to spondylolisthe-
Treatment depends on: (a) the type of anatomical dis- sis. The fracture usually heals spontaneously, provided
ruption; (b) whether the injury is stable or unstable; the patient is prepared to forego his (more often her)
(c) whether there is neurological involvement or not; athletic passion for several months.
and (d) the presence or absence of concomitant
injuries. Details are discussed under each fracture
type.
MAJOR INJURIES
Flexion–compression injury
MINOR INJURIES
This is by far the most common vertebral fracture and
Fractures of the transverse processes is due to severe spinal flexion, though in osteoporotic
individuals fracture may occur with minimal trauma.
The transverse processes can be avulsed with sudden The posterior ligaments usually remain intact,
muscular activity. Isolated injuries need no more than although if anterior collapse is marked they may be
symptomatic treatment. More ominous than usual is a damaged by distraction. CT shows that the posterior
fracture of the transverse process of L5; this should part of the vertebral body (middle column) is unbro-
alert one to the possibility of a vertical shear injury of ken. Pain may be quite severe but the fracture is usu-
the pelvis. ally stable. Neurological injury is extremely rare.
Patients with minimal wedging and a stable fracture
pattern are kept in bed for a week or two until pain sub-
sides and are then mobilized; no support is needed.
Those with moderate wedging (loss of 20–40 per cent
of anterior vertebral height) and a stable injury can be
allowed up after a week, wearing a thoracolumbar
brace or a body cast applied with the back in exten-
sion. At 3 months, flexion–extension x-rays are
obtained with the patient out of the orthosis; if there
is no instability, the brace is gradually discarded. If the
deformity increases and neurological signs appear, or
if the patient cannot tolerate the orthosis, surgical
stabilization is indicated.
If loss of anterior vertebral height is greater than 40
per cent, it is likely that the posterior ligaments have
been damaged by distraction and will be unable to
resist further collapse and deformity. If the patient is
neurologically intact, surgical correction and internal
fixation is the preferred treatment, though if necessary
even these patients can be treated conservatively with
vigilant monitoring of their neurological status.
In the rare cases of patients with a wedge compression
fracture and neurological impairment treatment will
depend on the degree of dysfunction and the risk of
27.24 Thoracolumbar injuries – minor fractures progression. If nerve loss is incomplete there is the
Fracture of the transverse processes on the right at L3 and potential for further recovery; any increase in kyphotic
822 L4. deformity or MRI signs of impending cord
27.25 Wedge-compression fractures 27
(a) Central compression fracture of the vertebral
body and (b) anterior wedge-compression fracture
with less than 20 per cent loss of vertebral body
height. In both cases the middle and posterior
columns are intact; further collapse can be
prevented by immobilization for 8–12 weeks in

(c) a plaster ‘jacket’ or (d) a lightweight removable
orthosis. (e,f) More severe and potentially
unstable compression fractures may need posterior
internal fixation.
(a) (b) (c)
d) (e) (f)
neurological compression would be an indication for is kept in bed until the acute symptoms settle (usually
operative decompression and stabilization through a under a week) and is then mobilized in a thoracolum-
trans-thoracic approach. bar brace or body cast which is worn for about 12
If there is complete paraplegia with no improvement weeks. Wood et al. (2003) carried out a prospective
after 48 hours, conservative management is adequate; randomized trial comparing operative and non-opera-
the patient can be rested in bed for 5–6 weeks, then tive treatment of stable thoracolumbar burst fractures
gradually mobilized in a brace. With severe bony with no neurological impairment; they found no dif-
injury, however, increasing kyphosis may occur and ference in the long-term results in the two groups,
internal fixation should be considered. but complications were more frequent in the surgical
group.
Axial compression or burst injury
Severe axial compression may ‘explode’ the vertebral
body, causing failure of both the anterior and the mid-
dle columns. The posterior column is usually, but not
always, undamaged. The posterior part of the verte-
bral body is shattered and fragments of bone and disc
may be displaced into the spinal canal. The injury is
usually unstable.
Anteroposterior x-rays may show spreading of the
vertebral body with an increase of the interpedicular
distance. Posterior displacement of bone into the (a) (b)
spinal canal (retropulsion) is difficult to see on the
27.26 Lumbar burst fracture Severe compression may
plain lateral radiograph; a CT is essential. shatter the middle column and cause retropulsion of the
If there is minimal anterior wedging and the frac- vertebral body (a). The extent of spinal canal
ture is stable with no neurological damage, the patient encroachment is best shown by CT (b). 823
27
(a) (b) (c) (d) (e)
27.27 Burst fracture – treatment (a) Burst fracture in a 44-year-old man who fell from his horse; 3 months later he
developed paraesthesia in both legs. (b–e) Internal fixation and grafting through a transthoracic transdiaphragmatic
approach provided total stability (the Kaneda method).
Even if CT shows that there is considerable com- The Chance fracture (being an ‘all bone’ injury)
promise of the spinal canal, provided there are no heals rapidly and requires 3 months in a body cast or
neurological symptoms or signs non-operative treat- well-fitting brace. Flexion–extension lateral views
ment is still appropriate; the fragments usually should then be taken to ensure that there is no unsta-
remodel. However, any new symptoms such as tin- ble deformity.
gling, weakness or alteration of bladder or bowel Severe ligamentous injuries are less predictable and
function must be reported immediately and should posterior spinal fusion is advisable.
call for further imaging by MRI; anterior decompres-
sion and stabilization may then be needed if there are
signs of present or impending neurological compro-
Fracture-dislocation
mise. Segmental displacement may occur with various com-
binations of flexion, compression, rotation and shear.
All three columns are disrupted and the spine is
Jack-knife injury
Combined flexion and posterior distraction may cause
the mid-lumbar spine to jack-knife around an axis that
is placed anterior to the vertebral column. This is seen
most typically in lap seat-belt injuries, where the body
is thrown forward against the restraining strap. There
is little or no crushing of the vertebral body, but the
posterior and middle columns fail in distraction; thus
these fractures are unstable in flexion.
The tear passes transversely through the bones or
the ligament structures, or both. The most perfect
example of tensile failure is the injury described by
Chance in 1948, in which the split runs through the
spinous process, the transverse processes, pedicles and
the vertebral body. Neurological damage is uncom-
mon, though the injury is (by definition) unstable. X- (a) (b)
rays may show horizontal fractures in the pedicles or
transverse processes, and in the anteroposterior view 27.28 Jack-knife injuries (a) Whereas flexion usually
crushes the vertebral body and leaves the posterior
the apparent height of the vertebral body may be ligaments intact, the jack-knife injury disrupts the posterior
increased. In the lateral view there may be opening up ligaments causing only slight anterior compression. (b) The
824 of the disc space posteriorly. rare Chance fracture.
27

(a) (b) (c) (d)
27.29 Thoracolumbar fracture-dislocation (a) Fracture-dislocation at T11/12 in a 32-year-old woman who was a pas-
senger in a truck that overturned. She was completely paraplegic and operation was not thought worthwhile. (b) Four
weeks later the deformity has increased, leaving her with a marked gibbus. (c,d) A similar injury in a 17-year-old man,
treated by open reduction and internal fixation.
grossly unstable. These are the most dangerous with neurological impairment who have the benefit of
injuries and are often associated with neurological being treated in a specialized spinal injuries unit, a
damage to the lowermost part of the cord or the strong case can be made for managing them also by
cauda equina. non-operative methods.
The injury most commonly occurs at the thora-
columbar junction. X-rays may show fractures
through the vertebral body, pedicles, articular
processes and laminae; there may be varying degrees NEURAL INJURIES
of subluxation or even bilateral facet dislocation.
Often there are associated fractures of transverse In spinal injuries the displaced structures may damage
processes or ribs. CT is helpful in demonstrating the the cord or the nerve roots, or both; cervical lesions
degree of spinal canal occlusion. may cause quadriplegia, thoracolumbar lesions para-
In neurologically intact patients, most fracture- plegia. The damage may be partial or complete. Three
dislocations will benefit from early surgery. varieties of lesion occur: neurapraxia, cord transection
In fracture-dislocation with paraplegia, there is no and root transection.
convincing evidence that surgery will facilitate
nursing, shorten the hospital stay, help the patient’s
rehabilitation or reduce the chance of painful defor-
Neurapraxia
mity. In fracture-dislocation with a partial neuro- Motor paralysis (flaccid), burning paraesthesia, sen-
logical deficit, there is also no evidence that surgical sory loss and visceral paralysis below the level of the
stabilization and decompression provides a better cord lesion may be complete, but within minutes or a
neurological outcome than conservative treatment. If few hours recovery begins and soon becomes full. The
surgical decompression and stabilization are per- condition is most likely to occur in patients who, for
formed, this may require a combined posterior and some reason other than injury, have a small-diameter
anterior approach. anteroposterior canal; there is, however, no radiolog-
In fracture-dislocation without neurological deficit, ical evidence of recent bony damage.
surgical stabilization will prevent future neurological
complications and allow earlier rehabilitation.
When specialized surgery cannot be performed,
Cord transection
these injuries can be managed non-operatively with Motor paralysis, sensory loss and visceral paralysis
postural reduction, bed rest and bracing. For patients occur below the level of the cord lesion; as with cord 825
27 concussion, the motor paralysis is at first flaccid. This • motor power to the muscles controlling the ankle
is a temporary condition known as cord shock, but and foot;
the injury is anatomical and irreparable. • the anal and penile reflexes, plantar responses and
After a time the cord below the level of transection ankle jerks;
recovers from the shock and acts as an independent • bladder and bowel continence.
structure; that is, it manifests reflex activity. Within 48
The lumbar roots innervate:
hours the primitive anal wink and bulbocavernosus

reflexes return. Within 4 weeks of injury tendon • sensation to the groins and entire lower limb other
reflexes return and the flaccid paralysis becomes spas- than that portion supplied by the sacral segment;
tic, with increased tone, increased tendon reflexes and • motor power to the muscles controlling the hip and
clonus; flexor spasms and contractures may develop knee;
with inadequate management. • the cremasteric reflexes and knee jerks.
It is essential, when the bony injury is at the thora-
columbar junction, to distinguish between cord tran-
Root transection
section with root escape and cord transection with
Motor paralysis, sensory loss and visceral paralysis root transection. A patient with root escape is much
occur in the distribution of the damaged roots. Root better off than one with cord and root transection.
transection, however, differs from cord transection in
two ways: recovery may occur and residual motor
DIAGNOSIS
paralysis remains permanently flaccid.
Clinical examination of the back nearly always shows
the signs of an unstable fracture; however, a ‘burst’
ANATOMICAL LEVELS
fracture with paraplegia is stable as long as the patient
Cervical spine With cervical spine injuries the segmen-
is in recumbency or very well braced until the fracture
tal level of cord transection nearly corresponds to the
heals. The nature and level of the bone lesion are
level of bony damage. Not more than one or two
demonstrated by x-ray, and that of the neural lesion
additional roots are likely to be transected. High cer-
by CT or MRI.
vical cord transection is fatal because all the respiratory
Neurological examination should be painstaking.
muscles are paralysed. At the level of the C5 vertebra,
Without detailed information, accurate diagnosis and
cord transection isolates the lower cervical cord (with
prognosis are impossible; rectal examination is
paralysis of the upper limbs), the thoracic cord (with
mandatory.
paralysis of the trunk) and the lumbar and sacral cord
(with paralysis of the lower limbs and viscera). With Complete cord lesions Complete paralysis and
injury below the C5 vertebra, the upper limbs are anaesthesia below the level of injury suggest cord
partially spared and characteristic deformities result. transection. During the stage of spinal shock when the
anal reflex is absent (seldom longer than the first 24
Between T1 and T10 vertebrae The first lumbar cord
hours) the diagnosis cannot be absolutely certain; if the
segment in the adult is at the level of the T10 vertebra.
anal reflex returns and the neural deficit (sensory and
Consequently, cord transection at that level spares the
motor) persists, the cord lesion is complete. Complete
thoracic cord but isolates the entire lumbar and sacral
lesions lasting more than 72 hours have only a small
cord, with paralysis of the lower limbs and viscera. The
chance of neurological recovery.
lower thoracic roots may also be transected but are of
relatively little importance. Incomplete cord lesions Persistence of any sensation
distal to the injury (peri-anal pinprick is most
Below T10 vertebra The cord forms a slight bulge (the
important) suggests an incomplete lesion.
conus medullaris) between the T10 and L1 vertebrae,
The commonest is the central cord syndrome where
and tapers to an end at the interspace between the L1
the initial flaccid weakness is followed by lower motor
and L2 vertebrae. The L2 to S4 nerve roots arise from
neuron paralysis of the upper limbs with upper motor
the conus medullaris and stream downwards in a bunch
neuron (spastic) paralysis of the lower limbs, and
(the cauda equina) to emerge at successive levels of the
intact peri-anal sensation (sacral sparing). Bladder
lumbosacral spine. Therefore, spinal injuries above the
control may or may not be preserved from an early
T10 vertebra cause cord transection, those between
stage.
the T10 and L1 vertebrae cause cord and nerve root le-
With the less common anterior cord syndrome there
sions, and those below the L1 vertebra only root lesions.
is complete paralysis and anaesthesia but deep pres-
The sacral roots innervate:
sure and position sense are retained in the lower limbs
• sensation in the ‘saddle’ area (S3, S4), a strip down (dorsal column sparing).
the back of the thigh and leg (S2) and the outer The posterior cord syndrome is rare; only deep pres-
826 two-thirds of the sole (S1); sure and proprioception are lost.
The Brown-Séquard syndrome (due to cord hemi- Bladder and bowel For the first 24 hours the bladder 27
section) is usually associated with penetrating thoracic distends only slowly, but, if the distension is allowed to
injuries. There is loss of motor power on the side of progress, overflow incontinence occurs and infection is
the injury and loss of pain and temperature sensation probable. In special centres it is usual to manage the pa-
on the opposite side. Most of these patients improve tient from the outset by intermittent catheterization
and regain bowel and bladder function and some under sterile conditions. If early transfer to a paraple-

walking ability. gia centre is not possible, continuous drainage through
High root lesions sometimes cause confusion. Below a fine Silastic catheter is advised. The catheter drains in
the T10 vertebra, discrepancies between neurological a closed manner into a disposable bag, and is changed
and skeletal levels are due to transection of roots twice weekly to prevent urethral and bladder compli-
descending from cord segments higher than the ver- cations, catheter blockage and infection. When infec-
tebral lesion. tion supervenes, antibiotics are given.
Bladder training is begun as early as possible.
FRANKEL GRADING Although retention is complete to begin with, partial
A well-established method of recording the functional recovery may lead to either an automatic bladder
deficit after an incomplete spinal cord injury was that which works reflexly or an expressible bladder which
described by Frankel: is emptied by manual suprapubic pressure.
A few patients are left with a high residual urine
Grade A = Absent motor and sensory function.
after emptying the bladder. They need special investi-
Grade B = Sensation present, motor power absent.
gations, including cystography and cystometry;
Grade C = Sensation present, motor power present
transurethral resection of the bladder neck or sphinc-
but not useful.
terotomy may be indicated but should not be per-
Grade D = Sensation present, motor power present
formed until at least 3 months of bladder training
and useful (grade 4 or 5).
have been completed.
Grade E =Normal motor and sensory function.
The bowel is more easily trained, with the help of
Frankel observed that 60 per cent of patients with enemas, aperients and abdominal exercises.
partial cord lesions (Grades B, C or D) improved
Muscles and joints The paralysed muscles, if not
(spontaneously) by one grade regardless of the treat-
treated, may develop severe flexion contractures. These
ment type and a significant number are able to walk
are usually preventable by moving the joints passively
again. Although many of the patients who present in
through their full range twice daily. Later, splints may
Frankel Grade A improve to B or C, only 5 per cent
be necessary.
of these patients improve to Frankel D or E.
With lesions below the cervical cord, the patient
should be up within 6 weeks; standing and walking
are valuable in preventing contractures.
Callipers are usually necessary to keep the knees
MANAGEMENT OF TRAUMATIC straight and the feet plantigrade. The callipers are
PARAPLEGIA AND QUADRIPLEGIA removed at intervals during the day while the patient
lies prone, and while he or she is having physiother-
With both complete and incomplete paralysis it is the apy. The upper limbs must be trained until they
overall management of the patient that is most impor- develop sufficient power to enable the patient to use
tant – from the early stages onwards. crutches and a wheelchair.
The patient must be transported with great care to If flexion contractures have been allowed to
prevent further damage, and preferably taken to a develop, tenotomies may be necessary. Painful flexor
spinal centre. The strategy is outlined below. spasms are rare unless skin or bladder infection occurs.
They can sometimes be relieved by tenotomies,
Skin Within a few hours anaesthetic skin may develop
neurectomies, rhizotomies or the intrathecal injection
large pressure sores; this can be prevented by
of alcohol.
meticulous nursing. Creases in the sheets and crumbs
Heterotopic ossification is a common and disturb-
in bed are not permitted. Every 2 hours the patient is
ing complication. It is more likely to occur with high
gently rolled onto his or her side and the back is
lesions and complete lesions. It may restrict or abolish
carefully washed (without rubbing), dried and
movement, especially at the hip. Once the new bone
powdered. After a few weeks the skin becomes a little
is mature it should be considered for excision if it
more tolerant and the patient can turn him- or herself.
interferes with function.
Later he or she should be taught how to relieve skin
pressure intermittently during periods of sitting. If Tendon transfers Some function can be regained in the
sores have been allowed to develop, they may never upper limb by the use of tendon transfers. The aim
heal without surgical closure. with patients who have a low cervical cord injury is to 827
27 use the limited number of functioning muscles in the Bohlman HH. Treatment of fractures and dislocations of
arm to provide a primitive pinch mechanism (normally the thoracic and lumbar spine – current concepts review.
powered by C8 or T1 which, being below the level of J Bone Joint Surg 1985; 67A: 165–9.
injury, are lost). One must establish which muscles are Chance CQ. Note on a type of flexion fracture of the spine.
working, which are not and which are available for Br J Radiol 1948; 21: 452–3.
transfer. Denis F. The three column spine and its significance in the
classification of acute thoracolumbar spinal injuries. Spine

• If only deltoid and biceps are working (C5, C6) then
1983; 8: 817–31.
a posterior-deltoid to triceps transfer using interpo-
El Masry WS. Management of Traumatic Spinal Cord
sition tendon grafts will replace the lost C7 func-
Injuries: current standard of care revisited. Ad Clin Neu-
tion of elbow extension; this will enable the patient
roscience Rehab. 2010; 10: 37–40.
to orient his or her hand in space.
El Masry WS. Traumatic spinal cord injury: the relationship
• If brachioradialis (C6) is working, this can be trans-
between pathology and clinical implications. Trauma
ferred to become a wrist extensor (since its prime
2006; 8: 29–46.
function as an elbow flexor is duplicated by biceps).
El Masri WS. Physiological instability of the spinal cord fol-
A primitive thumb pinch can be achieved by the
lowing injury. Paraplegia 1993; 31: 273–5.
Moberg procedure in which the thumb interpha-
Gunzburg R, Szpalski M. Whiplash injuries. Current con-
langeal joint is fused and the basal joint of the
cepts in prevention, diagnosis and treatment of the cervical
thumb is tenodesed with a loop of the redundant
whiplash syndrome. Philadelphia, Lippincott-Raven, 1997.
flexor pollicis longus. On active extension of the
Hugenholtz H, Cass DE, Dvorak MF. High-dose methyl-
wrist, the basal joint of the thumb is passively
prednisolone for acute closed spinal cord injury: Only a
flexed.
treatment option. Can J Neurol Sci 2002; 29: 227–35.
• If extensor carpi radialis longus and brevis (C7) are
Katoh S, El Masry WS, Jaffray D et al. The neurologic
both available, one of them can be transferred into
outcome in conservatively treated patients with incom-
the flexor pollicis longus to provide active thumb
plete closed traumatic cervical spinal cord injuries. Spine;
flexion (normally supplied by C8).
21: 2345–2351.
Morale The morale of a paraplegic patient is liable to Molano M, Broton JG, Bean JA et al. Complications asso-
reach a low ebb, and the restoration of his or her self- ciated with prophylactic use of methylprednisolone dur-
confidence is an important part of treatment. Constant ing surgical stabilization after spinal cord injury.
enthusiasm and encouragement by doctors, J Neurosurg 2002; 96: 267–72.
physiotherapists and nurses is essential. Their Short DJ, El Masry WS, Jones PW. High dose methyl-
scrupulous attention to the patient’s comfort and toilet prednisolone in the management of acute spinal cord
are of primary importance; the unpleasant smells of injury: A systematic review from a clinical perspective.
bowel accidents, or those associated with skin or Spinal Cord 2000; 38: 273–86.
urinary infection must be prevented. The patient Slucky AV, Eismont FJ. Treatment of acute injury of the
should find a hobby or be trained for a new job as cervical spine. J Bone Joint Surg 1994; 76A: 1882–95.
quickly as possible. Solomon L, Pearse MF. Osteonecrosis following low-dose
short-course corticosteroids. J Orthop Rheumatol 1994;
7: 203–5.
Spitzer WO, Skovron ML, Salmi LR et al. Scientific mono-
graph of the Quebec Task Force on whiplash-associated
REFERENCES AND FURTHER READING disorders: redefining whiplash and its management. Spine
1995; 20(8): 1S–73S.
Advanced Trauma Life Support. American College of Thomas BE, McCullen GM, Yuan HA. Cervical spine
Surgeons 1997. injuries in football players. J Am Acad Orthop Surg 1999;
Anderson LD, D’Alonzo RT. Fractures of the odontoid 7: 338–47.
process of the axis. J Bone Joint Surg 1974; 56A: 1663– Wood K, Butterman G, Mehbod A et al. Operative com-
74. pared with non-operative treatment of a thoracolumbar
Bannister G, Amirfeyz R, Kelley S, Gargan M. Whiplash burst fracture without neurological deficit. J Bone Joint
injury. J Bone Joint Injury 2009; 91B: 845–50. Surg 2003; 85A: 773–81.
828
Injuries of the pelvis
28
Louis Solomon
Fractures of the pelvis account for less than 5 per cent iac joints. The strongest and most important of the
of all skeletal injuries, but they are particularly impor- tethering ligaments are the sacroiliac and iliolumbar
tant because of the high incidence of associated soft- ligaments; these are supplemented by the sacro-
tissue injuries and the risks of severe blood loss, shock, tuberous and sacrospinous ligaments and the liga-
sepsis and adult respiratory distress syndrome ments of the symphysis pubis. As long as the bony
(ARDS). Like other serious injuries, they demand a ring and the ligaments are intact, load-bearing is
combined approach by experts in various fields. unimpaired.
About two-thirds of all pelvic fractures occur in The major branches of the common iliac arteries
road accidents involving pedestrians; over 10 per cent arise within the pelvis between the level of the sacroil-
of these patients will have associated visceral injuries, iac joint and the greater sciatic notch. With their
and in this group the mortality rate is probably in accompanying veins they are particularly vulnerable in
excess of 10 per cent. fractures through the posterior part of the pelvic ring.
The nerves of the lumbar and sacral plexuses, likewise,
are at risk with posterior pelvic injuries.
Surgical anatomy
The bladder lies behind the symphysis pubis. The
The pelvic ring is made up of the two innominate trigone is held in position by the lateral ligaments of
bones and the sacrum, articulating in front at the sym- the bladder and, in the male, by the prostate. The
physis pubis (the anterior or pubic bridge) and poste- prostate lies between the bladder and the pelvic floor.
riorly at the sacroiliac joints (the posterior or sacroiliac It is held laterally by the medial fibres of the levator
bridge). This basin-like structure transmits weight ani, whilst anteriorly it is firmly attached to the pubic
from the trunk to the lower limbs and provides pro- bones by the puboprostatic ligament. In the female
tection for the pelvic viscera, vessels and nerves. the trigone is attached also to the cervix and the ante-
The stability of the pelvic ring depends upon the rior vaginal fornix. The urethra is held by both the
rigidity of the bony parts and the integrity of the pelvic floor muscles and the pubourethral ligament.
strong ligaments that bind the three segments Consequently in females the urethra is much more
together across the symphysis pubis and the sacroil- mobile and less prone to injury.
(a) (b)
28.1 Ligaments supporting the pelvis (a) Anterior view. (b) Posterior view. Some ligaments run transversely and will
resist rotational forces which separate the two halves (the posterior sacroiliac and iliolumbar ligaments can be thought of as
a posterior band), whilst those that are oriented longitudinally tend to resist vertical shear.
28 In severe pelvic injuries the membranous urethra is bleeding. The pelvic ring can be gently compressed
damaged when the prostate is forced backwards whilst from side to side and back to front. Tenderness over
the urethra remains static. When the puboprostatic the sacroiliac region is particularly important and may
ligament is torn, the prostate and base of the bladder signify disruption of the posterior bridge.
can become grossly dislocated from the membranous A rectal examination is then carried out in every
urethra. case. The coccyx and sacrum can be felt and tested for
The pelvic colon, with its mesentery, is a mobile tenderness. If the prostate can be felt, which is often
structure and therefore not readily injured. However, difficult due to pain and swelling, its position should
the rectum and anal canal are more firmly tethered to be gauged; an abnormally high prostate suggests a
the urogenital structures and the muscular floor of the urethral injury.
pelvis and are therefore vulnerable in pelvic fractures. Enquire when the patient passed urine last and look
for bleeding at the external meatus. An inability to
void and blood at the external meatus are the classic
Pelvic instability
features of a ruptured urethra. However, the absence
If the pelvis can withstand weightbearing loads with- of blood at the meatus does not exclude a urethral
out displacement, it is stable; this situation exists only injury, because the external sphincter may be in
if the bony and key ligamentous structures are intact. spasm, halting the passage of blood from the site of
An anterior force applied to both halves of the injury. Thus every patient who has a pelvic fracture
pelvis forces apart the symphysis pubis. If a diastasis must be considered to be at risk.
occurs because of capsular rupture, the extent of sep- The patient can be encouraged to void; if he is able
aration is checked by the anterior sacroiliac and to do so, either the urethra is intact or there is only
sacrospinous ligaments. Should these restraints fail minimal damage which will not be made worse by the
through the application of a still greater force, the passage of urine. No attempt should be made to pass a
pelvis opens like a book until the posterior iliac spines catheter, as this could convert a partial to a complete
abut; because the more vertically oriented long poste- tear of the urethra. If the urethral injury is suspected,
rior sacroiliac and sacrotuberous ligaments remain this can be diagnosed more accurately and more safely
intact, the pelvis will still resist vertical shear but it is by retrograde urethrography.
rotationally unstable. If, however, the posterior A ruptured bladder should be suspected in patients
sacroiliac and sacrotuberous ligaments are damaged, who do not void or in whom a bladder is not palpable
then the pelvis is not only rotationally and vertically after adequate fluid replacement. This palpation is
unstable, but there will also be posterior translation of often difficult because of abdominal wall haematoma.
the injured half of the pelvis. Vertical instability is The physical findings initially can be minimal, with
therefore ominous as it suggests complete loss of the normal bowel sounds, as extravasation of sterile urine
major ligamentous support posteriorly. produces little peritoneal irritation. Only a very small
It should be remembered that some fracture pat-
terns can cause instability which mimics that of liga-
mentous disruption; e.g. fractures of both pubic rami
may behave like symphyseal disruptions, and fractures
of the iliac wing combined with ipsilateral pubic rami
fractures are unstable to vertical shear.
Clinical assessment
Fracture of the pelvis should be suspected in every
patient with serious abdominal or lower limb injuries.
There may be a history of a road accident or a fall from
a height or crush injury. Often the patient complains of
severe pain and feels as if he has fallen apart, and there
may be swelling or bruising of the lower abdomen, the
thighs, the perineum, the scrotum or the vulva. All
these areas should be rapidly inspected, looking for evi-
dence of extravasation of urine. However, the first prior- 28.2 Fractures of the pelvis This young man crashed on
ity, always, is to assess the patient’s general condition and his motorcycle and was brought into the Accident and
look for signs of blood loss. It may be necessary to start Emergency Department with a fractured femur. His
perineum and scrotum were swollen and bruised, he was
resuscitation before the examination is completed. unable to pass urine and a streak of blood appeared at the
The abdomen should be carefully palpated. Signs of external meatus. X-rays confirmed that he had a fractured
830 irritation suggest the possibility of intraperitoneal pelvis.
28

(a) (c) (e)
(b) (d) (f)

28.3 Pelvic fractures – x-ray diagnosis (1) (a,b) The anteroposterior view is usually taken during the initial assessment
of the multiply-injured patient as part of a ‘trauma series’. It is useful in quickly diagnosing gross disruptions or fractures.
The x-ray should be read systematically: Is the picture well centred? Look for asymmetry in the pubic symphysis, the pubic
rami, the iliac blades, the sacroiliac joints and the sacral foramina. If the patient’s condition permits, at least two additional
views should be obtained: (c,d) an inlet view with the tube titled 30° downwards and (e,f) an outlet view with the tube
titled 40° upwards.
(a) (b) (c) (d)

28.4 Pelvic fractures – x-ray diagnosis (2) Oblique views are helpful for defining the ilium and acetabulum on each
side. (a,b) the right oblique view; and (c,d) the left oblique view. These can be omitted if facilities for CT are available.
proportion of patients with a ruptured bladder are Imaging of the pelvis

hypotensive, so if a patient is hypotensive another
cause must be sought. During the initial survey of every severely injured
Neurological examination is important; there may patient, a plain anteroposterior x-ray of the pelvis
be damage to the lumbar or sacral plexus. should be obtained at the same time as the chest x-ray.
If the patient is unconscious, the same routine is In most cases this film will give sufficient information
followed. However, early x-ray examination is essen- to make a preliminary diagnosis of pelvic fracture. The
tial in these cases. exact nature of the injury can be clarified by more 831
28 the contrast agent to ensure that the urethra is fully
distended. This technique will confirm a urethral tear
and will show whether it is complete or incomplete.
In a patient with possible rupture of the bladder (so
long as there is no evidence of a urethral injury) a cys-
togram should be performed.
Types of injury
Injuries of the pelvis fall into four groups: (1) isolated
fractures with an intact pelvic ring; (2) fractures with
a broken ring – these may be stable or unstable; (3)
fractures of the acetabulum – although these are ring
fractures, involvement of the joint raises special prob-
(a) (b) lems and therefore they are considered separately; and
28.5 Pelvic fractures and bladder injury (4) sacrococcygeal fractures.
(a) Intravenous urogram outlining the bladder and
showing the typical globular appearance due to
compression by blood and extravasated urine. There is also
marked gastric dilation suggesting retroperitoneal ISOLATED FRACTURES
bleeding. (b) Cystogram showing extravasation of radio-
opaque material. This patient had a ruptured bladder.
Avulsion fractures
A piece of bone is pulled off by violent muscle con-
detailed radiography once it is certain that the patient traction; this is usually seen in sportsmen and athletes.
can tolerate an extended period of positioning and The sartorius may pull off the anterior superior iliac
repositioning on the x-ray table. Five views are neces- spine, the rectus femoris the anterior inferior iliac
sary: anteroposterior, an inlet view (tube cephalad to spine, the adductor longus a piece of the pubis, and
the pelvis and tilted 30° downwards), an outlet view the hamstrings part of the ischium. All are essentially
(tube caudad to the pelvis and tilted 40° upwards), muscle injuries, needing only rest for a few days and
and right and left oblique views. reassurance.
If any serious injury is suspected, a CT scan at the Pain may take months to disappear and, because
appropriate level is extremely helpful (some would say there is often no history of impact injury, biopsy of
essential). This is particularly true for posterior pelvic the callus may lead to an erroneous diagnosis of a
ring disruptions and for complex acetabular fractures, tumour. Rarely, avulsion of the ischial apophysis by
which cannot be properly evaluated on plain x-rays. the hamstrings may lead to persistent symptoms, in
Three-dimensional CT re-formation of the pelvic which case open reduction and internal fixation is
image gives the most accurate picture of the injury; indicated (Wootton, Cross and Holt, 1990).
however, with practice almost as much information
can be gleaned from a good set of plain radiographs
and standard CT images. Direct fractures
A direct blow to the pelvis, usually after a fall from a
Imaging of the urinary tract height, may fracture the ischium or the iliac blade.
Bed rest until pain subsides is usually all that is
If there is evidence of upper abdominal injury, and the needed.
patient has haematuria, an intravenous urogram is
performed to exclude renal injury. This will also show
whether there is any ureteric or major bladder dam- Stress fractures
age. In a case of urethral rupture, the base of the blad-
der may be riding high (dislocated prostate) or there Fractures of the pubic rami are fairly common (and
may be a teardrop deformity of the bladder owing to often quite painless) in severely osteoporotic or osteo-
compression by blood and extravasated urine malacic patients. More difficult to diagnose are stress
(prostate-in-situ). fractures around the sacroiliac joints; this is an
When a urethral injury is considered likely, an ure- uncommon cause of ‘sacroiliac’ pain in elderly osteo-
throgram should be undertaken using 25–30ml of porotic individuals and long distance runners.
water-soluble contrast agent with suitable aseptic Obscure stress fractures are best demonstrated by
832 technique. A film must be taken during injection of radioisotope scans.
springy. Often, however, the second break is not visi- 28
ble – either because it reduces immediately or because
the sacroiliac joints are only partially disrupted.
Mechanisms of injury

The basic mechanisms of pelvic ring injury are antero-
posterior compression, lateral compression, vertical
(a) (b) shear and combinations of these.
Anteroposterior compression This injury is usually
caused by a frontal collision between a pedestrian and
a car. The pubic rami are fractured or the innominate
bones are sprung apart and externally rotated, with
disruption of the symphysis – the so-called ‘open
book’ injury. The anterior sacroiliac ligaments are
strained and may be torn, or there may be a fracture
of the posterior part of the ilium.
(c) (d)
Lateral compression Side-to-side compression of the
28.6 Isolated injuries (a,b) Avulsion fractures. Unusually pelvis causes the ring to buckle and break. This is usu-
powerful muscle contraction may tear off a piece of bone ally due to a side-on impact in a road accident or a fall
at its attachment. Two examples are shown here:
(a) avulsion of sartorius attachment; (b) avulsion of rectus from a height. Anteriorly the pubic rami on one or
femoris origin. (c,d) Fractured iliac blade. The bruise both sides are fractured, and posteriorly there is a
suggests the site of the injury. The fracture looks alarming severe sacroiliac strain or a fracture of the sacrum or
and is certainly painful but, if the remainder of the bony ilium, either on the same side as the fractured pubic
pelvis is intact, it poses no threat to the patient. rami or on the opposite side of the pelvis. If the sacroil-
iac injury is much displaced, the pelvis is unstable.
Vertical shear The innominate bone on one side is dis-
FRACTURES OF THE PELVIC RING placed vertically, fracturing the pubic rami and dis-
rupting the sacroiliac region on the same side. This
occurs typically when someone falls from a height
It has been cogently argued that, because of the rigid-
onto one leg. These are usually severe, unstable
ity of the pelvis, a break at one point in the ring must
injuries with gross tearing of the soft tissues and
be accompanied by disruption at a second point;
retroperitoneal haemorrhage.
exceptions are fractures due to direct blows (including
fractures of the acetabular floor), or ring fractures in Combination injuries In severe pelvic injuries there
children, whose symphysis and sacroiliac joints are may be a combination of the above.
(a) (b) (c)
28.7 Types of pelvic ring fracture The three important types of injury are shown. (a) Anteroposterior compression with
lateral rotation may cause the ‘open book’ injury, the hallmark of which is diastasis of the pubic symphysis. Widening of the
anterior portion of the sacroiliac joint is best seen on an inlet view. (b) Lateral compression causing the ring to buckle and
break; the pubic rami are fractured, sometimes on both sides. Posteriorly the iliac blade may break or the sacrum is crushed.
(c) Vertical shear, with disruption of both the sacroiliac and symphyseal regions on one side. 833
28 Stable and unstable fractures metrical appearance of the pelvis. As with APC-III
injuries, the hemi-pelvis is totally disconnected and
A stable pelvic ring injury is usually defined as one the pelvic ring is unstable.
that will (theoretically) allow full weightbearing with-
out the risk of pelvic deformity. Of course one cannot COMBINATION INJURIES
actually perform the test in an acutely injured patient. Combination patterns do occur but, in the main, the
However, because the mechanisms which cause these above classification defines the most common types of
injuries are fairly consistent, typical patterns and dis- injury. The LC-II pattern is linked to abdominal, head
placements are defined which make it possible to and chest injuries; all the unstable patterns carry a
deduce the mechanism of injury, the type of ligament high risk of severe haemorrhage and are life-threaten-
damage and the degree of pelvic instability. Occa- ing (Dalal et al., 1989).
sionally the decision on stability cannot be made until
the patient is examined under anaesthesia.
Several classifications are in use. The one presented here Clinical features
is based on that of Young and Burgess (1986; 1987).
Stable ring injuries The patient is not severely shocked
ANTEROPOSTERIOR COMPRESSION (APC) INJURIES but has pain on attempting to walk. There is localized
The ‘open book’ pattern appears as either diastasis of tenderness but seldom any damage to pelvic viscera.
the pubic symphysis or fracture(s) of the pubic rami; Plain x-rays reveal the fractures.
as the pelvis is sprung open, the posterior (sacroiliac) Unstable ring injuries The patient is severely shocked,
elements also are strained. This general pattern is sub- in great pain and unable to stand. He or she may also
classified according to the severity of the injury: be unable to pass urine and there may be blood at the
In APC-I injuries there may be only slight (less external meatus. Tenderness is widespread, and
than 2 cm) diastasis of the symphysis; however, attempting to move one or both blades of the ilium is
although invisible on x-ray, there will almost certainly very painful. Clinical assessment for stability is diffi-
be some strain of the anterior sacroiliac ligaments. cult; few patients will allow pulling or pushing to
The pelvic ring is stable. reveal abnormal vertical movement (Olson and Pol-
In APC-II injuries diastasis is more marked and the lack, 1996). One leg may be partly anaesthetic
anterior sacroiliac ligaments (often also the sacro- because of sciatic nerve injury.
tuberous and sacrospinous ligaments) are torn. CT
Haemodynamic instability High-energy fractures of the
may show slight separation of the sacroiliac joint on
one side. Nevertheless, the pelvic ring is still stable. pelvis are extremely serious injuries, carrying a great
In APC-III injuries the anterior and posterior risk of associated visceral damage, intra-abdominal
sacroiliac ligaments are torn. CT shows a shift or sep- and retroperitoneal haemorrhage, shock, sepsis and
aration of the sacroiliac joint; the one hemi-pelvis is ARDS; the mortality rate is considerable. The patient
effectively disconnected from the other anteriorly and should be repeatedly assessed and re-assessed for signs
from the sacrum posteriorly. The ring is unstable. of blood loss and hypovolaemia. Bear in mind that,
although the pelvis may be the main focus of atten-
tion, haemorrhage may occur also in areas outside the
LATERAL COMPRESSION (LC) INJURIES
pelvis.
The hallmark of this injury is a transverse fracture of
the pubic ramus (or rami), often best seen on an inlet
view x-ray. There may also be a compression fracture Imaging
of the sacrum. In its simplest form this would be clas-
sified as a LC-I injury. The ring is stable. This may show fractures of the pubic rami, ipsilateral
The LC-II injury is more severe; in addition to the or contralateral fractures of the posterior elements,
anterior fracture, there may be a fracture of the iliac separation of the symphysis, disruption of the sacroil-
wing on the side of impact. However, the ring iac joint or combinations of these injuries. The films
remains stable. are often difficult to interpret and CT scans are much
The LC-III injury is worse still. As the victim is run the best way of visualizing the nature of the injury.
over, the lateral compression force on one iliac wing
results in an opening anteroposterior force on the
opposite ilium, causing injury patterns typical for that Management
mechanism. EARLY MANAGEMENT
Treatment should not await full and detailed diagno-
VERTICAL SHEAR (VS) INJURIES sis. It is vital to keep a sense of priorities and to act on
The hemi-pelvis is displaced in a cranial direction, and any information that is already available while moving
834 often posteriorly as well, producing a typically asym- along to the next diagnostic hurdle. ‘Management’ in
this context is a combination of assessment and treat- release the tamponade effect and lead to uncontrol- 28
ment, following the ATLS protocols. lable haemorrhage.
Six questions must be asked and the answers acted If there is no evidence of intra-abdominal bleeding
upon as they emerge: and laparotomy is not contemplated, but the patient
shows signs of continuing blood loss, then angiogra-
• Is there a clear airway?
phy should be performed with a view to carrying out
• Are the lungs adequately ventilated?

embolization. If blood loss continues after emboliza-
• Is the patient losing blood?
tion, angiography can again be performed to seek
• Is there an intra-abdominal injury?
other sites of bleeding. However, angiography will
• Is there a bladder or urethral injury?
not reveal any source of venous bleeding and repeated
• Is the pelvic fracture stable or unstable?
procedures are time-wasting.
With any severely injured patient, the first step is to An alternative approach is the application of pelvic
make sure that the airway is clear and ventilation is packing to provide a tamponade effect (Ertel et al.,
unimpaired. Resuscitation must be started immedi- 2001).
ately and active bleeding controlled. The patient is The management of severe haemorrhage in pelvic
rapidly examined for multiple injuries and, if neces- injuries is well-described in a recent review paper by
sary, painful fractures are splinted. A single anteropos- Hak et al. (2009).
terior x-ray of the pelvis is obtained.
A more careful examination is then carried out, pay- MANAGEMENT OF THE URETHRA AND BLADDER
ing attention to the pelvis, the abdomen, the per- Urological injury occurs in about 10 per cent of
ineum and the rectum. The urethral meatus is patients with pelvic ring fractures. As these patients
inspected for signs of bleeding. The lower limbs are are often seriously ill from other injuries, a urinary
examined for signs of nerve injury. catheter may be required to monitor urinary output,
If the patient’s general condition is stable, further and therefore the urologist is placed under pressure to
x-rays can then be obtained. If a urethral tear is sus- make a rapid diagnosis of urethral damage.
pected, an urethrogram is gently performed. The There is no place for passing a diagnostic catheter
findings up to that stage may dictate the need for an as this will most probably convert any partial tear to a
intravenous urogram. complete tear. For an incomplete tear, the insertion of
By now the examining doctor will have a good idea a suprapubic catheter as a formal procedure is all that
of the patient’s general condition, the extent of the is required. Around half of all incomplete tears will
pelvic injury, the presence or absence of visceral injury heal and require little long-term management.
and the likelihood of continued intra-abdominal or The treatment of a complete urethral tear is contro-
retroperitoneal bleeding. Ideally, a team of experts versial. Primary realignment of the urethra may be
will be on hand to deal with the individual problems achieved by performing suprapubic cystostomy, evacu-
or undertake further investigations. ating the pelvic haematoma and then threading a
catheter across the injury to drain the bladder. If the
MANAGEMENT OF SEVERE BLEEDING bladder is floating high it is repositioned and held
Severe bleeding is the main cause of death following down by a sling suture passed through the lower ante-
high-energy pelvic fractures. The general treatment of rior part of the prostatic capsule, through the per-
shock is described in Chapter 22. If there is an unsta- ineum on either side of the bulbar urethra and an-
ble fracture of the pelvis, haemorrhage will be reduced chored to the thighs by elastic bands. An alternative –
by rapidly applying an external fixator. and much simpler – approach is to perform the cys-
If either the expertise or the necessary equipment is tostomy as soon as possible, making no attempt to
lacking, unstable APC injuries can initially be man- drain the pelvis or dissect the urethra, and to deal with
aged by applying a pelvic binder to achieve side-to- the resulting stricture 4–6 months later. The latter
side compression; the rationale is to try and close the method is contraindicated if there is severe prostatic dis-
‘open book’ and reduce the internal pelvic volume. location or severe tears of the rectum or bladder neck.
The diagnosis of persistent bleeding is often diffi- With both methods there is a significant incidence of
cult, and even when it seems clear that continuing late stricture formation, incontinence and impotence.
shock is due to haemorrhage, it is not easy to deter-
mine the source of the bleeding. Patients with suspi- TREATMENT OF THE FRACTURE
cious abdominal signs should be further investigated For patients with very severe injuries, early external
by peritoneal aspiration or lavage. If there is a positive fixation is one of the most effective ways of reducing
diagnostic tap, the abdomen should be explored in an haemorrhage and counteracting shock (Poka and
attempt to find and deal with the source of bleeding. Libby, 1996; Hak et al., 2009). If there are no life-
However, if there is a large retroperitoneal threatening complications, definitive treatment is as
haematoma, it should not be evacuated as this may follows. 835
28
(a) (b) (c)
28.8 Internal fixation (a) Severe open-book injury with complete disruption of the symphysis pubis. (b) Reduction and
stabilization by external fixator. (c) The symphysis was then firmly held by internal fixation with a plate and screws.
Isolated fractures and minimally displaced fractures These Fractures of the iliac blade can often be treated with
injuries need only bed rest, possibly combined with bed rest. However, if displacement is marked, or if there
lower limb traction. Within 4–6 weeks the patient is is an associated anterior ring fracture or symphysis sep-
usually comfortable and may then be allowed up aration, then open reduction and internal fixation with
using crutches. plates and screws will need to be considered (e.g. in dis-
placed LC-II injuries causing a leg length discrepancy
Open-book injuries Provided the anterior gap is less
greater than 1.5 cm). It is also possible to reduce and
than 2 cm and it is certain that there are no displaced
hold some of these fractures by external fixation.
posterior disruptions, these injuries can usually be
treated satisfactorily by bed rest; a posterior sling or a APC-III and VS injuries These are the most dangerous
pelvic binder helps to ‘close the book’. injuries and the most difficult to treat. It may be pos-
The most efficient way of maintaining reduction is sible to reduce some or all of the vertical displacement
by external fixation with pins in both iliac blades con- by skeletal traction combined with an external fixator;
nected by an anterior bar; ‘closing the book’ may also even so, the patient needs to remain in bed for at least
reduce the amount of bleeding. Placing the pins is 10 weeks. This prolonged recumbency is not without
made easier if two temporary pins are first inserted risk. As these injuries represent loss of both anterior
hugging the medial and lateral surfaces of each iliac and posterior support, both areas will need to be
blade and then directing the fixing pins between stabilized. Two techniques are used: (a) anterior
them. Internal fixation by attaching a plate across the external fixation and posterior stabilization using
symphysis should be performed: (1) during the first screws across the sacroiliac joint, or (b) plating anteri-
few days after injury only if the patient needs a laparo- orly and iliosacral screw fixation posteriorly. Posterior
tomy; and (2) later on if the gap cannot be closed by operations are hazardous (the dangers include massive
less radical methods. haemorrhage, neurological damage and infection)
(a) (b) (c)
28.9 Treatment of vertical sheer fracture (a) X-ray showing a fractured superior pubic ramus and disruption of the
right sacroiliac joint. (b) This was initially treated by traction and external fixation. (c) X-ray showing the pelvic ring restored.
836 Thereafter, the sacroiliac joint was stabilized with plates and screws.
and should be attempted only by surgeons with con- a blow on the side (as in a fall from a height) or by a 28
siderable experience in this field. blow on the front of the knee, usually in a dashboard
Persisting with skeletal traction and external fixa- injury when the femur also may be fractured.
tion is probably safer, though the malposition is likely Acetabular fractures combine the complexities of
to leave a legacy of posterior pain. It should be pelvic fractures (notably the frequency of associated
emphasized that more than 60 per cent of pelvic frac- soft-tissue injury) with those of joint disruption

tures need no fixation. (namely, articular cartilage damage, noncongruent
loading and secondary osteoarthritis).
Open pelvic fractures Open fractures are best managed
by external fixation. A diversion colostomy may be
necessary.
Patterns of fracture
Several classifications of acetabular fractures are cur-
Complications rently popular (Letournel, 1981; Müller et al., 1991;
Thromboembolism A careful watch should be kept for Tile, 1995). All use similar anatomical descriptions,
signs of deep vein thrombosis or pulmonary embolism but Tile’s universal classification has much to com-
(Montgomery et al., 1996). Prophylactic anticoagu- mend it for simplicity.
lants are advocated in some hospitals. The fractures are divided into four major types;
though they are distinguished on anatomical grounds,
Sciatic nerve injury It is essential to test for sciatic nerve
it is important to recognize that they also differ in
function both before and after treating the pelvic frac-
their ease of reduction, their stability after reduction
ture. If the nerve is injured it is usually a neuropraxia and
and their long-term prognosis.
one can afford to wait several weeks for signs of recov-
ery. Occasionally, though, nerve exploration is necessary. Acetabular wall fractures Fractures of the anterior or
posterior part of the acetabular rim affect the depth of
Urogenital problems Urethral injuries sometimes
the socket and may lead to hip instability unless they
result in stricture, incontinence or impotence and may
are properly reduced and fixed.
require further treatment.
Persistent sacroiliac pain Unstable pelvic fractures are COLUMN FRACTURES
often associated with partial or complete sacroiliac The anterior column extends from the pubic symph-
joint disruption, and this can lead to persistent pain at ysis, along the superior pubic ramus, across the
the back of the pelvis. Occasionally arthrodesis of the acetabulum to the anterior part of the ilium. On the
sacroiliac joint is needed. x-ray it is shown in profile by the iliopectineal line in
the oblique view. Anterior column fractures are
uncommon, do not involve the weightbearing area
and have a good prognosis.
FRACTURES OF THE ACETABULUM The posterior column extends from the ischium,
across the posterior aspect of the acetabular socket to
Fractures of the acetabulum occur when the head of the the sciatic notch and the posterior part of the innom-
femur is driven into the pelvis. This is caused either by inate bone. In an iliac oblique x-ray it is seen in
28.10 Acetabular fractures

(a) Fractures occur through the
wall (rim) or supporting
columns. (b) Of particular
importance is the roof
(superior dome – which
carries a high proportion of the
load in walking).
(a) (b) 837

28
(a) (b) (c) (d) (e)
28.11 The classification of acetabular fractures There are four types of injury: (a,b) a simple fracture involving either
the anterior or the posterior wall or column; (c) a transverse or (d) a T-type fracture involving two columns; (e) the both-
column fracture, resulting in a ‘floating’ acetabulum with no part of the socket attached to the ilium (compare this with the
transverse or T-type fractures).
profile as the ilioischial line. A posterior column frac- fractures, a portion of the acetabulum remains
ture usually runs upwards from the obturator foramen attached to the ilium. These fractures are usually diffi-
into the sciatic notch, separating the posterior ischiop- cult to reduce and to hold reduced.
ubic column of bone and breaking the weightbearing
part of the acetabulum. It is usually associated with a COMPLEX FRACTURES
posterior dislocation of the hip and may injure the sci- Many acetabular fractures are complex injuries which
atic nerve. Treatment is more urgent and usually damage either the anterior or the posterior columns
involves internal fixation to obtain a stable joint. (or both) as well as the roof or the walls of the acetab-
ulum. Of particular note, and sometimes a cause of
TRANSVERSE FRACTURE confusion, is the ‘both-column fracture’ – this is really
This fracture runs transversely through the acetabu- a variant of the T-fracture in that the two columns are
lum, involving both the anterior and posterior involved but the transverse part of the ‘T’ lies just
columns, and separating the iliac portion above from above the acetabulum; effectively, no portion of the
the pubic and ischial portions below. A vertical split acetabulum remains connected to the rest of the
into the obturator foramen may coexist, resulting in a pelvis. Understandably, the confusion arises when the
T-fracture. Note that in both transverse and T-type term ‘both-column’ is used to refer to a transverse
(a) (b) (c)
28.12 Imaging the pelvis for acetabular fractures Although CT scans have become the standard in assessing
acetabular fractures, plain x-rays have much to offer. The obturator oblique (a), standard anteroposterior (b) and iliac
oblique (c) views will allow the trained eye to picture the structures involved in the injury. The iliopectineal line represents a
profile of the anterior column whereas the ilioschial line defines the posterior column. The margins of the anterior and
838 posterior walls are usually seen in all three views.
fracture – perhaps the term ‘high T’ would have been scans and three-dimensional re-formations are added 28
better! refinements, and are particularly helpful if surgical
Complex fracture patterns share the following fea- reconstruction is planned.
tures: (1) the injury is severe; (2) the joint surface is
disrupted; (3) they usually need operative reduction
and internal fixation; and (4) the end result is likely to Treatment

be less than perfect, unless surgical restoration has EMERGENCY TREATMENT
been exact. The first priority is to counteract shock and reduce a
dislocation. Skeletal traction is then applied to the
Clinical features distal femur (10 kg will suffice) and during the next
3–4 days the patient’s general condition is brought
There has usually been a severe injury; either a traffic under control. Occasionally, additional lateral traction
accident or a fall from a height. Associated fractures through the greater trochanter is needed for central
are not uncommon and, because they may be more hip dislocations. Definitive treatment of the fracture is
obvious, are liable to divert attention from the more delayed until the patient is fit and operation facilities
urgent pelvic injuries. Whenever a fractured femur, a are optimal.
severe knee injury or a fractured calcaneum is diag-
nosed, the hips also should be x-rayed. NON-OPERATIVE TREATMENT
The patient may be severely shocked, and the com- In recent years opinion has moved in favour of opera-
plications associated with all pelvic fractures should be tive treatment for displaced acetabular fractures. How-
excluded. Rectal examination is essential. There may ever, conservative treatment is still preferable in certain
be bruising around the hip and the limb may lie in well-defined situations: (1) acetabular fractures with
internal rotation (if the hip is dislocated). No attempt minimal displacement (in the weightbearing zone, less
should be made to move the hip. than 3 mm); (2) displaced fractures that do not involve
Careful neurological examination is important, test- the superomedial weightbearing segment (roof) of the
ing the function of the sciatic, femoral, obturator and acetabulum – usually distal anterior column and distal
pudendal nerves. transverse fractures; (3) a both-column fracture that
retains the ball and socket congruence of the hip by
virtue of the fracture line lying in the coronal plane
Imaging
and displacement being limited by an intact labrum;
At least four x-ray views should be obtained in every (4) fractures in elderly patients, where closed reduc-
case: a standard anteroposterior view, the pelvic inlet tion seems feasible; (5) patients with ‘medical’ con-
view and two 45 degrees oblique views. Each view traindications to operative treatment (including local
shows a different profile of the acetabulum; with prac- sepsis). Comminution in itself is not a contraindication
tice the various landmarks (iliopectineal line, ilioischial to operative treatment, provided adequate facilities
line and the boundaries of the anterior and posterior and expertise are available.
walls) can be identified, thus providing a fairly good Matta and Merritt (1988) have listed certain crite-
mental picture of the fracture type, the degree of ria which should be met if conservative treatment is
comminution and the amount of displacement. CT expected to succeed: (1) when traction is released, the
(a) (b) (c) (d)
28.13 Fractured acetabulum – conservative treatment This severely displaced acetabular fracture (a) was almost
completely reduced by (b) longitudinal and lateral traction. (c) The fracture healed and the patient regained a congruent
joint with a fairly good range of movement. (d) X-ray two years later. 839
28 hip should remain congruent; (2) the weightbearing is useful to monitor somatosensory evoked potentials
portion of the acetabular roof should be intact; and during the operation, in order to avoid damaging the
(3) associated fractures of the posterior wall should be sciatic nerve (separate electrodes are required for
excluded by CT. Non-operative treatment is more medial and lateral popliteal branches).
suitable for patients aged over 50 years than for ado- Prophylactic antibiotics are used, and postopera-
lescents and young adults. tively hip movements are started as soon as possible.
If there are medical contraindications to operative Some prophylaxis against heterotopic ossification is
treatment, closed reduction under general anaesthesia often used, usually indomethacin. The patient is
is attempted. In all patients treated conservatively, allowed up, partial weightbearing with crutches, after
longitudinal traction, if necessary supplemented by 7 days. Exercises are continued for 3–6 months; it
lateral traction, is maintained for 6–8 weeks; this will may take a year or longer for full function to return.
unload the articular cartilage and will help to prevent
further displacement of the fracture. During this
Complications
period, hip movement and exercises are encouraged.
The patient is then allowed up, using crutches with Operative treatment should aim for a perfect anatom-
minimal weightbearing for a further 6 weeks. ical reduction and is best undertaken in centres that
specialize in this form of treatment.
OPERATIVE TREATMENT
Iliofemoral venous thrombosis This is potentially seri-
Operative treatment is indicated for all unstable hips
ous and in some clinics prophylactic anticoagulation is
and fractures resulting in significant distortion of the
used.
ball and socket congruence. The hip may be dislo-
cated centrally, anteriorly or posteriorly. Patients with Sciatic nerve injury Nerve injury may occur either at
isolated posterior wall fractures and dislocation may the time of fracture or during the subsequent opera-
require immediate open reduction and stabilization. tion. Unless the nerve is seen to be unharmed during
In other cases operation is usually deferred for 4 or 5 the operation, there can be no certainty about the
days. prognosis. Intra-operative somatosensory monitoring
Matta and Merritt (1988) have made the important is advocated as a means of preventing serious nerve
point that open reduction is an operation on the pelvis damage. For an established lesion, it is worth waiting
and not merely the acetabular socket. Adequate expo- for 6 weeks to see if there is any sign of recovery. If
sure is essential, if possible through a single approach there is none, the nerve should be explored in order
which is selected according to the type of fracture. to establish the diagnosis and ensure that the nerve is
The posterior Kocher–Langenbach exposure allows not being compressed.
good access to the posterior wall and column but may
Hereterotopic bone formation Periarticular ossification
have to be combined with a trochanteric osteotomy to
is common after severe soft-tissue injury and extended
gain adequate sight in transverse fractures. The ante-
surgical dissections. In cases where this is anticipated,
rior ilioinguinal approach is suited for anterior wall
prophylactic indomethacin is useful.
and column fractures. Both exposures are usually
needed in T-type and both-column fractures – this is Avascular necrosis Osteonecrosis of the femoral head
a considerable undertaking, encouraging some sur- may occur even if the hip is not fully dislocated. The
geons to adopt the singular triradiate or extended condition is probably overdiagnosed because of
iliofemoral approaches instead. The fracture (or frac- erroneous interpretation of the x-ray appearances fol-
tures) is fixed with lag screws or special buttressing lowing impacted marginal fractures of the acetabulum
plates which can be shaped in the operating theatre. It (Gruen, Mears and Tauxe, 1988).
28.14 Fractured acetabulum

– internal fixation (a) X-ray
and (b) three-dimensional CT
before reduction, showing a
large posterior fragment which
needed accurate repositioning
and internal fixation (c).
(Courtsey of Mr RN Brueton
and Dr RL Guy).
840 (a) (b) (c)

reduction is stable, which is fortunate. The patient is 28
allowed to resume normal activity, but is advised to
use a rubber ring cushion when sitting. Occasionally,
sacral fractures are associated with urinary problems,
necessitating sacral laminectomy.
Persistent pain, especially on sitting, is common

after coccygeal injuries. If the pain is not relieved by
the use of a cushion or by the injection of local anaes-
thetic into the tender area, excision of the coccyx may
be considered.
Dalal SA, Burgess AR, Siegel JH, et al. Pelvic fracture in

multiple trauma. J Trauma 1989; 29: 981–1000.
(a) (b) Ertel W, Keel M, Eid K, et al. Control of severe haemor-
rhage using C-clamp and pelvic packing in multiply
28.15 Sacrococcygeal fractures (a) Fractured sacrum; injured patients with pelvic ring disruption. J Orthop
(b) fractured coccyx.
Trauma 2001; 15: 468–74.
Gruen GS, Mears DC, Tauxe WN. Distinguishing avascular
necrosis from segmental impaction of the femoral head
following an acetabular fracture. J Orthop Trauma 1988;
Loss of joint movement and secondary osteoarthritis Dis- 2: 5–9.
placed fractures involving the weightbearing portion Hak DJ, Smith WR, Suzuki T. Management of haemor-
of the joint may result in loss of movement and early rhage in life-threatening pelvic fracture. J Am Acad
onset osteoarthritis. If a joint replacement operation is Orthop Surg 2009; 17: 447–57.
contemplated it should be deferred until the fractures Letournel E. Fractures of the Acetabulum 1981. Springer,
have consolidated; the acetabular implant is bound to Berlin, 1981.
work loose if there is any movement of the innomi- Matta JM, Merritt PO. Displaced acetabular fractures. Clin
nate segments. Orthop Relat Res 1988; 230: 83–97.
Montgomery KD, Geerts WH, Potter HG, Helfet DL.
Thromboembolic complications in patients with pelvic
INJURIES TO THE SACRUM AND trauma. Clin Orthop Relat Res 1996; 329: 68–87.
COCCYX Müller ME, Allgower M, Schneider R, Willeneger H.
Manual of Internal Fixation, 3rd edition. Springer Ver-
lag. Berlin, Heidelberg, New York, 1991.
A blow from behind, or a fall onto the ‘tail’ may frac-
Olson SA, Pollak AN. Assessment of pelvic ring stability
ture the sacrum or coccyx, or sprain the joint between
after injury. Indications for surgical stabilisation. Clin
them. Women seem to be affected more commonly
than men.
Poka A, Libby EP. Indications and techniques for external
Bruising is considerable and tenderness is elicited
fixation of the pelvis. Clin Orthop Relat Res 1996; 329:
when the sacrum or coccyx is palpated from behind or
54–9.
per rectum. Sensation may be lost over the distribu-
Tile M. Fractures of the pelvis and acetabulum. 2nd edition.
tion of sacral nerves.
Williams and Wilkins, Baltimore, 1995.
X-rays may show: (1) a transverse fracture of the
Wootton JR, Cross MJ, Holt KWG. Avulsion of the ischial
sacrum, in rare cases with the lower fragment pushed
apophysis. J Bone Joint Surg 1990; 72B: 625–7.
forwards; (2) a fractured coccyx, sometimes with the
Young JWR, Burgess AR, Brumback RJ, Poka A. Lateral
lower fragment angulated forwards; or (3) a normal
compression fractures of the pelvis: the importance of
appearance if the injury was merely a sprained sacro-
plain radiographs in the diagnosis and surgical manage-
coccygeal joint.
ment. Skeletal Radiol 1986; 15: 103–9.
Treatment If the fracture is displaced, reduction is Young JWR, Burgess AR. Radiologic management of
worth attempting. The lower fragment may be pelvic ring fractures: Systematic radiographic diagnosis.
pushed backwards by a finger in the rectum. The Urban and Schwarzenberg. Baltimore, 1987.
841
Injuries of the hip and
femur 29
Selvadurai Nayagam
dashboard. The femur is thrust upwards and the

DISLOCATION OF THE HIP femoral head is forced out of its socket; often a piece
of bone at the back of the acetabulum (usually the
The magnitude of force needed to dislocate the hip, a posterior wall) is sheared off, making it a fracture-dis-
joint particularly well-contained by virtue of its bony location. Seat-belt restraints can reduce the number of
and soft-tissue anatomy, is so great that the disloca- posterior hip dislocations.
tion is often associated with fractures – either around
the joint or elsewhere in the same limb. Small frag-
ments of bone are often chipped off, usually from the Clinical features
femoral head or from the wall of the acetabulum. If In a straightforward case the diagnosis is easy; the leg
there is a major fragment, the injury is regarded as a is short and lies adducted, internally rotated and
fracture-dislocation. slightly flexed. However, if one of the long bones is
Hip dislocations are classified according to the di- fractured – usually the femur – the injury can easily be
rection of the femoral head displacement: posterior (by missed as the limb can adopt almost any position. The
far the commonest variety), anterior and central (a golden rule is to x-ray the pelvis in every case of severe
comminuted or displaced fracture of the acetabulum). injury and, with femoral fractures, to insist on an x-ray
that includes both the hip and knee. The lower limb
should be examined for signs of sciatic nerve injury
POSTERIOR DISLOCATION (Figure 29.1).
Mechanism of injury
X-ray
This is a posterior dislocation, usually occurring in a
road accident when someone seated in a truck or car In the anteroposterior film the femoral head is seen
is thrown forward, striking the knee against the out of its socket and above the acetabulum. A
(a) (b) (c) (d)
29.1 Posterior dislocation of the hip (a) This is the typical posture in a patient with posterior dislocation: the left hip is
slightly flexed and internally rotated. (b) The x-ray in this case showed a simple dislocation, with the femoral head lying
above and behind the acetabulum. (c) Another patient with dislocation and an associated acetabular rim fracture. However,
in some cases it may need a CT scan and three-dimensional image reconstruction to appreciate the full extent of the
associated acetabular injury (d).
29 Table 29.1 Classification of hip dislocation (Thompson
and Epstein).
Treatment
The dislocation must be reduced as soon as possible
Types Thompson and Epstein classification of hip
under general anaesthesia. In the vast majority of cases
dislocations
this is performed closed, but if this is not achieved
I Dislocation with no more than minor chip fractures after two or three attempts an open reduction is
II Dislocation with single large fragment of posterior required. An assistant steadies the pelvis; the surgeon
acetabular wall
starts by applying traction in the line of the femur as
III Dislocation with comminuted fragments of posterior it lies (usually in adduction and internal rotation), and
acetabular wall
then gradually flexes the patient’s hip and knee to
IV Dislocation with fracture through acetabular floor
90 degrees, maintaining traction throughout. At 90
V Dislocation with fracture through acetabular floor and degrees of hip flexion, traction is steadily increased
femoral head
and sometimes a little rotation (either internal or
external) is required to accomplish reduction.
Another assistant can help by applying direct medial
and anterior pressure to the femoral head through the
buttock. A satisfying ‘clunk’ terminates the manoeu-
segment of acetabular rim or femoral head may have vre. An important test follows, to assess the stability of
been broken off and displaced; oblique films are use- the reduced hip. By flexing the hip to 90 degrees and
ful in demonstrating the size of the fragment. If any applying a longitudinal and posteriorly-directed force,
fracture is seen, other bony fragments (which may the hip is screened on an image-intensifier looking for
need removal) must be suspected. A CT scan is the signs of subluxation. Evidence of this should prompt
best way of demonstrating an acetabular fracture (or a repair to the posterior wall of the acetabulum.
any bony fragment) but detailed imaging at this stage Reduction is usually stable in type I injuries, but the
should be undertaken only if it does not delay reduc- hip has been severely injured and needs to be rested.
tion of the dislocation unduly. The simplest way is to apply traction and maintain it
Thompson and Epstein (1951) suggested a classifi- for a few days. Movement and exercises are begun as
cation which is helpful in planning treatment. Types I soon as pain allows; continuous passive movement
and II are relatively simple dislocations; these are asso- machines are helpful. The terminal ranges of hip
ciated with either minor chip fractures (small frag- movements are avoided to allow healing of the cap-
ments of the acetabular wall or fovea centralis) or a sule and ligaments. As soon as active limb control is
single large fragment from the posterior acetabular achieved, and this may take about 2 weeks, the patient
wall. In Type III the posterior wall is comminuted. is allowed to walk with crutches but without taking
type IV has an associated fracture of the acetabular weight on the affected side. The rationale for not
floor, and Type V an associated fracture of the femoral bearing weight is to prevent collapse of femoral head
head, which can be further subdivided according to due to an unsuspected avascular change.
Pipkin’s (1957) classification. (Figure 29.2) The period of hip ‘protection’ varies according to
Pipkin classification of femoral head fractures
Type I Type II Type III Type IV

The fracture The fracture As with types I and II but Any pattern of femoral
line is fragment with an associated femoral head fracture and an acetabular
inferior to includes the neck fracture fracture (coincides with
the fovea fovea Thompson and Epstein’s type V)
844 29.2 Pipkin classification of femoral head fractures
the risk of avascular necrosis: if the reduction was per- sciatic nerve lesion is diagnosed, the nerve should be 29
formed promptly (within 6 hours), then no more than explored to ensure it is not trapped by the reduction
6 weeks should suffice, but if there was a longer delay manoeuvre. Recovery often takes months and in the
then an extended period of 12 weeks may be wiser. meantime the limb must be protected from injury and
Progression of weightbearing should be graduated the ankle splinted to overcome the foot drop.
and the hip joint monitored by x-ray (Tornetta and
Vascular injury Occasionally the superior gluteal
Injuries of the hip and femur

Mostafavi 1997).
artery is torn and bleeding may be profuse. If this is
If the post-reduction x-rays or CT scans show the
suspected, an arteriogram should be performed. The
presence of intra-articular bone fragments or larger
torn vessel may need to be ligated.
femoral head pieces that are incompletely reduced, an
open procedure should be planned. The approach is Associated fractured femoral shaft When this occurs at
dictated by the location of the fragment on CT scan; the same time as the hip dislocation, the dislocation is
however, the operation is not an emergency and can often missed. It should be a rule that with every femoral
be done once the patient’s condition has stabilized. shaft fracture, the buttock and trochanter are palpated,
The joint needs to be thoroughly washed out at the and the hip clearly seen on x-ray. Even if this precaution
conclusion of the procedure to remove bone ‘grit’. has been omitted, a dislocation should be suspected if
Type II fracture-dislocations are often treated by the proximal fragment of a transverse shaft fracture is
immediate open reduction and anatomical fixation of seen to be adducted. Closed reduction of the disloca-
the detached fragment, the rationale being that many tion will be much more difficult. A prompt open
large posterior wall fragments either do not reduce reduction of the hip followed by internal fixation of
well or remain as a cause of instability even after the shaft fracture should be undertaken.
reduction. However, if the patient’s general condition
is suspect, or the necessary surgical skills are not avail-
LATE
able, the hip is reduced closed, as described above.
Avascular necrosis Avascular necrosis of the femoral
Traction can be applied until conditions are appropri-
head has been reported in about 10 per cent of trau-
ate for surgery – open reduction and internal fixation
matic hip dislocations; if reduction is delayed by more
will remedy the source of instability, return congruity
than 12 hours, the figure rises to over 40 per cent.
to the joint and remove any trapped bone fragments.
Changes are seen first on MRI or isotope bone scans.
Type III injuries are treated closed, but there may
X-ray features such as increased density of the femoral
be retained fragments and these should be removed
head may not be seen for at least 6 weeks, and some-
by open operation. Fixation of a comminuted poste-
times very much later (up to 2 years), depending on
rior wall is sometimes impossible – if persistent insta-
the rate of bone repair.
bility is present, referral to a specialist centre, where
Ischaemia is due to interruption of femoral head
reconstruction using a segment of iliac crest could be
blood supply when the hip is dislocated. There is evi-
undertaken, is advisable.
dence to suggest that this results from compression,
Types IV and V are treated initially by closed reduc-
traction and arterial spasm rather than actual disrup-
tion. The indications for surgery follow the principles
tion of blood vessels (Shim 1979), which means that
already outlined: instability, retained fragments or
the consequences of ischaemia are proportional to the
joint incongruity. In type V injuries, a femoral head
delay in starting treatment; blood flow is restored on
fragment may automatically fall into place, and this
reduction of the hip, especially if this is performed
can be confirmed by post-reduction CT. If the frag-
early – which highlights the need for emergency treat-
ment remains unreduced, operative treatment is indi-
ment with a target of less than 12 hours (preferably
cated: a small fragment can simply be removed, but a
less than 6) from the time of injury.
large fragment should be replaced; the joint is
If the femoral head develops signs of fragmenta-
opened, the femoral head dislocated and the fragment
tion, an operation may be needed. If the necrotic seg-
fixed in position with a countersunk screw. Postoper-
ment is small, realignment osteotomy is the method
atively, traction is maintained for 2–4 weeks and full
of choice; for extensive femoral head collapse, usually
weightbearing is deferred for 12 weeks.
with accompanying degenerative arthritis, the choice
is between joint replacement and hip arthrodesis
(never an easy procedure).
Complications Myositis ossificans This is an uncommon complication,
EARLY probably related to the severity of the injury. During
Sciatic nerve injury The sciatic nerve is damaged in recovery, movements should never be forced and in sev-
10–20 per cent of cases but it usually recovers. Nerve ere injuries the period of rest and non-weightbearing
function must be tested and documented before reduc- may need to be prolonged. Small areas of ossification
tion is attempted. If, after reducing the dislocation, a seen on x-ray usually bear no clinical significance. 845
29 29.3 Anterior hip dislocation (a,b)
The usual appearance of an anterior
dislocation: the hip is only slightly
abducted and the head shows clinically
as a prominent lump.
(b)
(a)
Unreduced dislocation After a few weeks an untreated upwards. Occasionally the leg is abducted almost to a
dislocation can seldom be reduced by closed manipu- right angle. Seen from the side, the anterior bulge of
lation and open reduction is needed. The incidence of the dislocated head is unmistakable, especially when
stiffness or avascular necrosis is considerably increased the head has moved anteriorly and superiorly. The
and the patient may later need reconstructive surgery. prominent head is easy to feel, either anteriorly (supe-
Osteoarthritis Secondary osteoarthritis is not uncom-
rior type) or in the groin (inferior type). Hip move-
mon and is due to (1) cartilage damage at the time of ments are impossible (Figure 29.3).
the dislocation, (2) the presence of retained fragments
in the joint or (3) ischaemic necrosis of the femoral X-ray
head. In young patients treatment presents a difficult In the anteroposterior view the dislocation is usually
problem. obvious, but occasionally the head is almost directly in
front of its normal position; any doubt is resolved by
a lateral film.
ANTERIOR DISLOCATION
Anterior dislocation is rare compared with posterior. Treatment and complications
Dislocation of one or even both hips may occur when The manoeuvres employed are similar to those used
a weight falls onto the back of a miner or building to reduce a posterior dislocation, except that while the
labourer who is working with his legs wide apart, knees flexed knee is being pulled and the hip gently flexed
straight and back bent forwards. However, nowadays upwards, it should be kept adducted; an assistant then
the usual cause is a road accident or air crash – even a helps by applying lateral pressure to the inside of the
posteriorly directed force on an abducted and externally thigh. The point of reduction is usually heard and felt.
rotated hip may cause the neck to impinge on the ac- The subsequent treatment is similar to that employed
etabular rim and lever the femoral head out in front of for posterior dislocation.
its socket. The femoral head will then lie superiorly Avascular necrosis occurs in less than 10 per cent of
(type I - pubic) or inferiorly (type II - obturator). cases.
Clinical features
CENTRAL DISLOCATION
The leg lies externally rotated, abducted and slightly
flexed. It is not short, because the attachment of rec- A fall on the side, or a blow over the greater
tus femoris prevents the head from displacing trochanter, may force the femoral head medially
29.4 Central dislocation

(a) The plain x-ray gives a good
picture of the displacement, but
(b) a CT scan shows the pelvic
injury more clearly.
(c) Skeletal traction, which often
needs both longitudinal and
lateral vectors, is an effective
method of reduction.
846 (a) (b) (c)

through the floor of the acetabulum. Although this is a height or a blow sustained in a road accident. These 29
called ‘central dislocation’, it is really a fracture of the patients often have multiple injuries and in 20 per
acetabulum (Figure 29.4). The condition is dealt with cent there is an associated fracture of the femoral
in the chapter on ‘Injuries of the pelvis’. shaft. Occasionally, stress fractures of the femoral neck
occur in runners or military personnel.

FRACTURES OF THE FEMORAL NECK Pathological anatomy and classification
The most useful classification is that of Garden, which
The femoral neck is the commonest site of fractures in is based on the amount of displacement apparent in
the elderly. The vast majority of patients are Caucasian the pre-reduction x-rays (Garden 1961). Once frac-
women in the seventh and eighth decades, and the tured, the head and neck become displaced in pro-
association with osteoporosis is so manifest that the gressively severe stages. Stage I is an incomplete
incidence of femoral neck fractures has been used as a impacted fracture, including the so-called abduction
measure of age-related osteoporosis in population fracture in which the femoral head is tilted into valgus
studies. Other risk factors include bone-losing or in relation to the neck. Stage II is a complete but
bone-weakening disorders such as osteomalacia, dia- undisplaced fracture. Stage III is a complete fracture
betes, stroke (disuse), alcoholism and chronic debili- with moderate displacement. And Stage IV is a
tating disease. In addition, old people often have weak severely displaced fracture. This is essentially a radi-
muscles and poor balance resulting in an increased ographic classification; the distinctive x-ray features
tendency to fall. are described below.
The association of femoral neck fracture with post- Garden I and II fractures, which are only slightly
menopausal bone loss has stimulated renewed interest displaced, have a much better prognosis for union and
in screening for osteoporosis and prophylactic meas- for viability of the femoral head than the more
ures in the ‘at risk’ population (see Chapter 7). By severely displaced Garden III and IV fractures
contrast, this injury is much less common among peo- (Barnes, Brown et al. 1976). This has an important
ple whose bone mass is above that of the population influence on the choice of treatment for the various
average, e.g. those with osteoarthritis of the hip. stages. However, there is little room for complacency
Femoral neck fractures are also much less common with any of these fractures; left untreated, a compara-
in black (Negroid) peoples than in whites and Asians. tively benign-looking Stage I fracture may rapidly dis-
The reasons for this phenomenon are poorly under- integrate to Stage IV.
stood. Slightly higher bone mass and a slower rate of Healing of femoral neck fractures is bedevilled by
bone loss after the menopause may be significant, but two problems: the threat of bone ischaemia and tardy
a qualitative difference in bone structure has also union. The femoral head gets its blood supply from
been suggested: even among people with the same three sources: (1) intramedullary vessels in the
bone mass, those with greater loss of trabecular inter- femoral neck; (2) ascending cervical branches of the
connectivity (typical in elderly whites) will suffer frac- medial and lateral circumflex anastomoses, which run
tures more easily than those with firmer structure. within the capsular retinaculum before entering the
The incidence of femoral neck fractures is set to bone at the articular margin of the femoral head; and
double over the next 30 years; this is a reflection of a (3) the vessels of the ligamentum teres. The
higher number of individuals living beyond 65 years intramedullary supply is always interrupted by the
and a parallel rise in those affected with osteoporosis. fracture; the retinacular vessels, also, may be kinked or
The economic impact of treating, rehabilitating and torn if the fracture is displaced. In elderly people, the
caring for this group of patients is increasingly being remaining supply in the ligamentum teres is at best
recognized, with many governments and healthcare fairly meagre and, in 20 per cent of cases, non-
administration bodies focusing on preventive strate- existent. Hence the high incidence of avascular necro-
gies. sis in displaced femoral neck fractures.
Transcervical fractures are, by definition, intracapsu-
lar. They have a poor capacity for healing because: (1)
Mechanism of injury by tearing the retinacular vessels the injury deprives
The fracture usually results from a simple fall; how- the head of its main blood supply; (2) intra-articular
ever, in very osteoporotic people, less force is required bone has only a flimsy periosteum and no contact with
–– perhaps no more than catching a toe in the carpet soft tissues which could promote callus formation; and
and twisting the hip into external rotation. Some (3) synovial fluid prevents clotting of the fracture
patients may have experienced minor symptoms of a haematoma. Accurate apposition and impaction of
preceding stress fracture of the femoral neck. bone fragments are therefore of more importance than
In younger individuals, the usual cause is a fall from usual. There is evidence that aspirating a haemarthro- 847
29 sis increases the blood flow in the femoral head by nal fixation, whereas displaced fractures have a high
relieving tension in the capsule, and the practice is rate of non-union and avascular necrosis.
encouraged at the time of surgery (Harper, Barnes et In Garden I fractures the femoral head is in its nor-
al. 1991; Bonnaire and Weber 2002). mal position or tilted into valgus and impacted on the
femoral neck stump. The medial cortex may be intact.
The femoral head stress trabeculae are normally
Clinical features aligned with the innominate trabeculae.

There is usually a history of a fall, followed by pain in In Garden II fractures the femoral head is normally
the hip. If the fracture is displaced, the patient lies placed and the fracture line may be difficult to discern.
with the limb in lateral rotation and the leg looks In Garden III fractures the anteroposterior x-ray
short. shows that the femoral head is tilted out of position
Beware, not all hip fractures are so obvious. With and the trabecular markings are not in line with those
an impacted fracture the patient may still be able to of the innominate bone; this is because the proximal
walk, and debilitated or mentally handicapped fragment retains some contact with the neck stump
patients may not complain at all – even with bilateral and is pushed out of alignment.
fractures. In Garden IV fractures the femoral head trabeculae
In contrast, femoral neck fractures in young adults are normally aligned with those of the innominate
result from road traffic accidents or falls from heights bone; the reason is that the proximal fragment has lost
and are often associated with multiple injuries. A good contact with the femoral neck and lies in its normal
rule is that young adults with severe injuries – whether position in the acetabular socket.
they complain of hip pain or not – should always be
examined for an associated femoral neck fracture. Diagnosis
There are four situations in which a femoral neck frac-
X-ray ture may be missed, sometimes with dire conse-
quences.
Two questions must be answered: is there a fracture,
and is it displaced? Usually the break is obvious, but • Stress fractures The elderly patient with unexplained
an impacted fracture can be missed by the unwary. pain in the hip should be considered to have a stress
Displacement is judged by the abnormal shape of the fracture until proved otherwise. A similar caution-
bone outlines and the degree of mismatch of the tra- ary note is raised for young athletes who do regular
becular lines in the femoral head and neck and the impact-loading sports and military personnel on
supra-acetabular (innominate) part of the pelvis (Fig- marching routines. The x-ray is usually normal but
ure 29.5). This assessment is important because a bone scan, or better still an MRI, will show the
impacted or undisplaced fractures do well after inter- lesion (Figure 29.6).
29.5 Garden’s classification

of femoral neck fractures
(a) Stage I: incomplete (so-
called abducted or impacted) –
the femoral head in this case is
in slight valgus.
(b) Stage II: complete without
displacement.
(c) Stage III: complete with
partial displacement – the
fragments are still connected
by the posterior retinacular
attachment; the femoral head
trabeculae are no longer in
line with those of the
innominate bone.
(d) Stage IV: complete with
full displacement – the
proximal fragment is free and
lies correctly in the acetabulum
so that the trabeculae appear
normally aligned with those of
the innominate.
848 (a) (b) (c) (d)

29

(a) (b) (c)
29.6 Fractures of the femoral neck – diagnosis (a) An elderly woman tripped on the pavement and complained of
pain in the left hip. The plain x-ray showed no abnormality. Two weeks later she was still in pain; (b) a bone scan showed a
‘hot’ area medially at the base of the femoral neck. MRI, if available, is an alternative investigation to confirm suspicions of
a femoral neck fracture (c).
• Undisplaced fractures Impacted fractures may be accurate reduction and stable internal fixation is
extremely difficult to discern on plain x-ray. If there needed as soon as possible. In older patients, also, the
is a fracture it will show up on MRI or a bone scan longer the delay, the greater is the likelihood of com-
after a few days. plications. However, here speed is tempered by the
• Painless fractures A bed-ridden patient may develop need for adequate preparation, especially in the very
a ‘silent’ fracture. Even a fit patient occasionally elderly, who are often ill and debilitated.
walks about without pain if the fracture is impacted. What if operation is considered too dangerous?
If the context suggests an injury, investigate – Lying in bed on traction may be even more danger-
whether the patient complains or not. ous, and leaving the fracture untreated too painful;
• Multiple fractures The patient with a femoral shaft the patient least fit for operation may need it most.
fracture may also have a hip fracture, which is easily
missed unless the pelvis is x-rayed. Internal fixation Notwithstanding the advances in
joint replacement, for most patients the principles of
treatment are as of old: accurate reduction, secure
Treatment internal fixation and early activity. Displaced fractures
Initial treatment consists of pain-relieving measures must first be reduced: with the patient under anaes-
and simple splintage of the limb. If operation is thesia, the fracture is disimpacted by applying traction
delayed, a femoral nerve block may be helpful. with the hip held in 45 degrees of flexion and slight
A case for non-operative treatment of undisplaced abduction; the limb is then slowly brought into exten-
(Garden Stages I and II) fractures can be made in sion and finally internally rotated; as traction is
treating patients with advanced dementia and little released, the fracture re-impacts in the reduced posi-
discomfort. For all others, operative treatment is tion.
almost mandatory. Displaced fractures will not unite The reduction is assessed by x-ray. The femoral
without internal fixation, and in any case elderly head should be positioned correctly with the stress
people should be got up and kept active without delay trabeculae in the femoral head and those in the
if pulmonary complications and bed sores are to be femoral neck aligned close to their normal position in
prevented. Impacted fractures can be left to unite, but both anteroposterior and lateral views, as shown in
there is always a risk that they may become displaced, Figure 29.7. In the AP x-ray the trabeculae in the
even while lying in bed, so fixation is safer. femoral head and a line along the medial border of the
Another indication for non-operative management femoral shaft should subtend an angle of 155–180
is an impacted Garden I fracture that is an ‘old’ injury, degrees.
where the diagnosis is made only after the patient has To fix an imperfectly reduced fracture is to risk fail-
been walking about for several weeks without delete- ure. If a stage III or IV fracture cannot be reduced
rious effect on the fracture position. closed, and the patient is under 60 years of age, open
When should the operation be performed? In reduction through an anterolateral approach is advis-
young patients operation is urgent; interruption of able. However, in older patients (and certainly in
the blood supply will produce irreversible cellular those over 70) this may not be justified; if two careful
changes after 12 hours and, to prevent this, an attempts at closed reduction fail, prosthetic 849
29 replacement is preferable. Some may even argue that
prosthetic replacement is always a preferable option
for this older group as it carries a much lower risk of
needing revision surgery.
Once the fracture is reduced, it is held with cannu-
lated screws or a sliding screw and side-plate which
attaches to the femoral shaft. A lateral incision is used

to expose the upper femur. When using cannulated
screws, guide wires –– inserted under fluoroscopic
control – are used to ensure correct placement of the
fixing device. Usually three cannulated screws will suf-
(a) (b)
fice; they should lie parallel and extend to within
5 mm of the subchondral bone plate. It is usual to
start with an inferior screw that skirts the inferior cor-
tex of the neck but remains centred in the lateral x-ray
view. This screw should be inserted through the lat-
eral cortex of the femur at a level proximal to the
lesser trochanter lest a stress riser is created and pro-
duces a subtrochanteric fracture. Two further screws
are inserted more proximally, this time centred in the
femoral neck on the anteroposterior x-ray but strad-
dling the anterior and posterior margins of the
femoral neck on the lateral x-ray (Figure 29.8). If a
(c) (d) sliding screw is used, the femoral neck will first have
29.7 Garden’s index for assessing reduction in to be reamed; a temporary guidewire should always
subcapital fractures On the anteroposterior x-ray (a,b), be introduced before reaming so as to prevent the
the medial femoral shaft and the axis of trabecular femoral head from rotating with the reamer and tear-
markings over the medial aspect of the femoral neck lie at ing the remaining soft-tissue attachments. Once the
an angle of 160°; an acceptable reduction is deemed to lie sliding screw is fixed, the guidewire is replaced by a
between 155° and 180°. On the lateral view (c,d), the
trabecular markings would be in line (i.e. 180°) if the single screw to reduce the risk of femoral head rota-
fracture was perfectly reduced; an acceptable reduction is tion during fracture healing – this screw must be par-
within 20° of this ideal. Garden (1974) noted that there allel to the sliding screw or else impaction of the
was a higher association with complications such as fracture will not occur!
avascular necrosis, non-union and osteoarthritis if the From the first day patients should sit up in bed or
quality of reduction was outside these acceptable limits.
in a chair. They are taught breathing exercises, and
(a) (b) (c) (d)
29.8 Femoral neck injuries – treatment (a,b) This Garden stage II fracture has been stabilized with 3 cannulated
screws. (c,d) An optimum position for the screws is: one to support the inferior portion of the neck (centrally); and another
two, central in level, skirting the anterior and posterior cortices of the femoral neck on the lateral x-ray. It is important the
most inferior screw enters the lateral cortex of the femur proximal to the level of the inferior margin of the lesser
850 trochanter.
29

(a) (b) (c)
29.9 Fracture of the femoral neck – treatment (a) A fracture as severely displaced as this (Stage IV), if treated by
reduction and internal fixation, will probably end up needing revision surgery; instead it could be treated by performing a
hemiarthroplasty using a cemented femoral prostheses (b). A total hip replacement (c) provides a better outcome for
younger patients (50–60 year olds) with this type of fracture.
encouraged to help themselves and to begin walking However, some studies suggest a longer survivorship
(with crutches or a walker) as soon as possible. To of bipolar implants and an argument can be made for
delay weightbearing may be theoretically appropriate their use in younger patients.
but is rarely practicable. Total hip replacement for femoral neck fractures
may be indicated: (1) if treatment has been delayed
Prosthetic replacement This procedure carries a longer for some weeks and acetabular damage is suspected,
operating time, greater blood loss and a higher infec- or (2) in patients with metastatic disease or Paget’s
tion rate than internal fixation. However, in its favour disease. Hip function and quality of life are reported
is a much lower need for revision surgery (nearly four to be better with total hip replacement, even when
times less) when compared to internal fixation for compared with hemiarthroplasty, and there is some
stage III and IV fractures. The mortality rates are justification for using this as a preferred option in the
equivalent for the two groups but there is insufficient healthy, active person who needs treatment for a stage
data to be certain there is a difference in morbidity III or IV fracture (Keating, Grant et al. 2006).
(Masson, Parker et al. 2003). Some argue that pros- Postoperatively, breathing exercises and early mobi-
thetic replacement is always preferable for stage III lization are important. Speed of recovery depends
and IV fractures so that patients, particularly the eld- largely on how active the patient was before the frac-
erly, are subject to one single surgical intervention ture; after 2–4 months, further improvement is
(Figure 29.9). This is also true for patients with unlikely.
pathological fractures and those in whom closed
reduction cannot be achieved.
Hip prostheses used for femoral neck fractures are
Complications
usually of the femoral part only (hemiarthroplasty) General complications These patients, most of whom
and may be inserted with or without cement. are elderly, are prone to general complications such as
Cemented prostheses have better mobility and less deep vein thrombosis, pulmonary embolism, pneu-
thigh pain; uncemented prostheses should be reserved monia and bed sores; not to mention disorders that
for the very frail where the pre-injury status suggests might have been present before the fracture and
that mobility is unlikely to be attained after operation which lead to death in a substantial proportion of
and those who will benefit significantly from the cases. Notwithstanding the advances in perioperative
reduced operating time. There is little evidence to care, the mortality rate in elderly patients may be as
support use of bipolar prostheses over unipolar types high as 20 per cent at 4 months after injury. Among
for the elderly group; the mortality, morbidity and the survivors over 80 years, about half fail to resume
functional recovery following use of either are similar. independent walking. 851
29 Avascular necrosis Ischaemic necrosis of the femoral In younger patients, the choice of treatment is con-
head occurs in about 30 per cent of patients with dis- troversial. Core decompression has no place in the
placed fractures and 10 per cent of those with undis- management of traumatic osteonecrosis. Realignment
placed fractures. There is no way of diagnosing this at or rotational osteotomy is suitable for those with a rel-
the time of fracture. A few weeks later, an isotope atively small necrotic segment. Arthrodesis is often
bone scan may show diminished vascularity. X-ray mentioned in armchair discussions, but in practice it is
changes may not become apparent for months or even seldom carried out. Provided the risks are carefully
years. Whether the fracture unites or not, collapse of explained, including the likelihood of at least one revi-
the femoral head will cause pain and progressive loss sion procedure, joint replacement may be justifiable
of function (Figure 29.10). In patients over 45 years, even in this group.
treatment is by total joint replacement.
Non-union More than 30 per cent of all femoral neck
fractures fail to unite, and the risk is particularly high
in those that are severely displaced. There are many
causes: poor blood supply, imperfect reduction, inad-
equate fixation, and the tardy healing that is charac-
teristic of intra-articular fractures. The bone at the
fracture site is ground away, the fragments fall apart
and the screw cuts out of the bone or is extruded lat-
erally. The patient complains of pain, shortening of
the limb and difficulty with walking. The x-ray shows
the sorry outcome.
The method of treatment depends on the cause of
the non-union and the age of the patient. In the rela-
tively young, three procedures are available: (1) if the
(a) (b) fracture is nearly vertical but the head is alive, sub-
trochanteric osteotomy with internal fixation changes
the fracture line to a more horizontal angle; (2) if the
reduction or fixation was faulty and there are no signs
of necrosis, it is reasonable to remove the screws,
reduce the fracture, insert fresh screws correctly and
also to apply a bone graft across the fracture, either a
segment of fibula or a muscle pedicle graft; and (3), if
the head is avascular but the joint unaffected, pros-
thetic replacement may be suitable; if the joint is dam-
aged or arthritic, total replacement is indicated.
In elderly patients, only two procedures should be
(c) (d) considered: (1) if pain is considerable then the
femoral head, no matter whether it is avascular or not,
is best removed and (provided the patient is reason-
ably fit) total joint replacement is performed; (2) if
the patient is old and infirm and pain not unbearable,
a raised heel and a stout stick or elbow crutch are
often sufficient.
Osteoarthritis Avascular necrosis or femoral head
collapse may lead, after several years, to secondary
osteoarthritis of the hip. If there is marked loss of
joint movement and widespread damage to the artic-
(e) (f) ular surface, total joint replacement will be needed.
29.10 Fracture of the femoral neck – avascular
necrosis (a) The post-reduction x-ray may look splendid
but the blood supply is compromised and 6 months later Combined fractures of the neck and
(b) there is obvious necrosis of the femoral head.
(c) Section across the excised femoral head, showing the shaft
large necrotic segment and splitting of the articular
cartilage. (d) Fine detail x-ray of the same. (e,f) Even an Young patients with high-energy fractures of both the
impacted fracture, if it is displaced in valgus, can lead to femoral neck and the ipsilateral femoral shaft present a
852 avascular necrosis. special problem. Both fractures must be fixed, and
there are several ways of doing this. The femoral neck 1. there is poor contact between the fracture 29
fracture takes priority as complications following this fragments, as in four-part intertrochanteric types
fracture are generally more difficult to address than (greater and lesser trochanter, proximal and distal
those of the shaft fracture. Anatomic reduction and femoral fragments), or if the posteromedial cortex
stable fixation of the femoral neck fracture must not be is comminuted.
compromised in order to accommodate fixation of the 2. the fracture pattern is such that forces of

shaft fracture. The femoral neck fracture is reduced weightbearing continually displace the fragments
using closed or, if necessary, open methods. The frac- further, as in those with a reverse oblique pattern
ture is fixed using multiple screws. The femoral shaft or with a subtrochanteric extension.
fracture can then be managed with a retrograde locked 3. osteoporosis leading to poor quality grip by the
intramedullary nail (inserted through the knee) or by fixation implants.
a lateral plate inserted in a submuscular fashion.
The importance of fracture pattern is detailed in the
classification by Kyle (1994) which distinguishes four
basic patterns that reflect increasing instability and
INTERTROCHANTERIC FRACTURES increasing difficulty at reduction and fixation (Figure
29.11).
Intertrochanteric fractures are, by definition, extra-
capsular. As with femoral neck fractures, they are com- Clinical features
mon in elderly, osteoporotic people; most of the
patients are women in the 8th decade. However, in The patient is usually old and is unable to stand. The
contrast to intracapsular fractures, extracapsular leg is shorter and more externally rotated than with a
trochanteric fractures unite quite easily and seldom transcervical fracture (because the fracture is extracap-
cause avascular necrosis. sular) and the patient cannot lift his or her leg.
Mechanism of injury X-ray

The fracture is caused either by a fall directly onto the Undisplaced, stable fractures may show no more than
greater trochanter or by an indirect twisting injury. a thin crack along the intertrochanteric line; indeed,
The crack runs up between the lesser and greater there is often doubt as to whether the bone is frac-
trochanter and the proximal fragment tends to dis- tured and the diagnosis may have to be confirmed by
place in varus. scintigraphy or MRI.
More often the fracture is displaced and there may
be considerable comminution. The lesser and greater
Pathological anatomy trochanters may be identifiable as separate fragments
Intertrochanteric fractures are divided into stable and and this calls for caution; surgery is technically more
unstable varieties. In essence, unstable fractures are difficult and, even with modern implants, stable fixa-
those where: tion may be hindered because of poor bone quality.
TYPE 1 TYPE 2 TYPE 3 TYPE 4

Undisplaced Displaced Displaced Severely comminuted
Uncomminuted Minimal comminuted Greater trochanter fracture Subtrochanter extension
Lesser trochanter fracture Comminuted (Also reverse oblique)
Varus Varus
29.11 Intertrochanteric fractures – classification Types 1 to 4 are arranged in increasing degrees of instability and
complexity. Types 1 and 2 account for the majority (nearly 60 per cent). The reverse oblique type of intertrochanteric
fracture represents a subgroup of Type 4; it causes similar difficulties with fixation. 853
29 Treatment
Intertrochanteric fractures are almost always treated
by early internal fixation – not because they fail to
unite with conservative treatment (they unite quite
readily), but (a) to obtain the best possible position
and (b) to get the patient up and walking as soon as

possible and thereby reduce the complications associ-
ated with prolonged recumbency. Non-operative
treatment may be appropriate for a small group who
are too ill to undergo anaesthesia; traction in bed until
there is sufficient reduction of pain to allow mobiliza-
tion can yield reasonable results but much depends on
the quality of nursing care and physical therapy (a) (b)
(Kaplan, Miyamoto et al. 2008). 29.13 Risk of screw cut-out The tip-apex distance is a
measure that estimates the risk of screw cut-out from the
femoral head. (a,b) It is the sum of the measured distances
(after adjustment for magnification on the x-ray) from the
tip of the screw to the apex of the femoral head – on both
the AP (x) and lateral views (y). The risk of cut-out is low if
the sum is less than 25 mm.
With the less common ‘reversed oblique’ fracture

(where the fracture line runs downwards obliquely
from medial to lateral cortex) there is a tendency for
the distal fragment to shift medially under the proxi-
mal fragment as the hip screw slides in the barrel;
often the screws from the slide plate lose their pur-
(a) (b) chase from the femoral shaft. In these cases a 95
degree screw-plate device or an intramedullary device
29.12 Intertrochanteric features Two contrasting types
of intertrochanteric fracture. (a) Type 2 fracture: the with a hip screw gives more stable fixation.
fracture runs obliquely downwards from the lateral to If closed reduction fails to achieve a satisfactory
medial cortex, in this case associated with a lesser position, open reduction and manipulation of the
trochanter fracture and resulting in a typical varus fragments will be necessary. A large posteromedial
deformity. This is an unstable fracture. (b) Type 4 ‘reverse fragment (often including the lesser trochanter) may
oblique’ fracture: here the fracture line runs downwards
from medial to lateral cortex, to give an even more need additional fixation. The addition of bone grafts
unstable geometry. may hasten union of the medial cortex. On the occa-
sion that anatomical reduction proves impossible, a
valgus osteotomy may be needed to allow the proxi-
Fracture reduction at surgery is performed on a frac- mal fragment to abut securely against the femoral
ture table that provides slight traction and internal ro- shaft (Dimon and Hughston 1967) (Figure 29.14 c,d).
tation; the position is checked by x-ray and the fracture Postoperatively, exercises are started on the day
is fixed with an angled device – preferably a sliding after operation and the patient allowed up and partial
screw in conjunction with a plate or intramedullary nail. weightbearing as soon as possible.
Positioning the screw is important if it is to be pre-
vented from cutting out of the osteoporotic bone. It
Complications
should pass up the femoral neck to end within the
centre of the femoral head, with the tip resting about EARLY
5 mm from the subchondral bone plate. A ‘tip-apex’ Early complications are the same as with femoral neck
distance is described to identify a ‘sweet-spot’ for po- fractures, reflecting the fact that most of these patients
sitioning this sliding screw: if within 25 mm, there is a are in poor health.
lower risk of the screw cutting out of the femoral head
(Figure 29.13). The side plate should be long enough LATE
to accommodate at least 4 screws below the fracture Failed fixation Screws may cut out of the osteoporotic
line. A small lesser trochanteric fragment may be bone if reduction is poor or if the fixation device is
854 ‘caught’ with additional screws. incorrectly positioned. If union is delayed, the implant
29

(a) (b) (c) (d) (e) (f)
29.14 Intertrochanteric fractures – treatment Anatomic reduction is the ideal; but stable fixation is equally important.
Types 1 and 2 fractures (a,b) can usually be held in good position with a compression screw and plate. If this is not possible,
an osteotomy of the lateral cortex (c,d) will allow a screw to be inserted up to the femoral neck and into the head of the
femur; this can be used as a lever to reduce the fracture so that the medial spike of the proximal fragment engages securely
into the femoral canal; fixation is completed with a side plate. Reverse oblique fractures (e,f) are inherently unstable even
after perfect reduction; here one can use an intramedullary device with an oblique screw that engages the femoral head.
(Courtesy of Mr M Manning and Mr JS Albert).
itself may break. In either event, reduction and fixa- Pathological fractures
tion may have to be re-done.
Intertrochanteric fractures may be due to metastatic
Malunion Varus and external rotation deformities are disease or myeloma. Unless patients are terminally ill,
common. Fortunately they are seldom severe and fracture fixation is essential in order to ensure an
rarely interfere with function. acceptable quality of life for their remaining years. In
Non-union Intertrochanteric fractures seldom fail to addition to internal fixation, methylmethacrylate
unite. If healing is delayed (say beyond 6 months) the cement may be packed in the defect to improve
fracture probably will not join and further operation is stability.
advisable; the fragments are repositioned as anatomi- If there is involvement of the femoral neck,
cally as is feasible, the fixation device is applied more replacement with a cemented prosthesis may be
securely and bone grafts are packed around the frac- preferable.
ture (Figure 29.15).
(a) (b) (c) (d)
29.15 Complications of treatment of intertrochanteric fractures (a,b) Failure to maintain reduction, which can be
early – usually in osteoporotic bone or from poor implant seating (c,d). The implant may fracture if union is not timely.
Revision surgery is complex and may involve bone grafts and a new implant. 855
29 PROXIMAL FEMORAL FRACTURES IN
fractures are easily mistaken for hip dislocation. Ultra-
sonography, MRI and arthrography may help. In
CHILDREN older children the diagnosis is usually obvious on
plain x-ray examination.
Hip fractures rarely occur in children but when they It is important to establish whether the fracture is
do they are potentially very serious. displaced or undisplaced; the former carries a much
The fracture is usually due to high velocity trauma; higher risk of complications. Type IV fractures are the
for example, falling from a height or a car accident. least likely to give rise to complications.
Pathological fractures sometimes occur through a bone
cyst or benign tumour. In children under two years, the Treatment
possibility of child abuse should be considered.
There is a high risk of complications, such as avas- These fractures should be treated as a matter of
cular necrosis, premature physeal closure and coxa vara. urgency, and certainly within 24 hours of injury. Ini-
At birth the proximal end of the femur is entirely tially the hip is supported or splinted while investiga-
cartilaginous and for several years, as ossification pro- tions are carried out. Early aspiration of the
ceeds, the area between the capital epiphysis and intracapsular haematoma is advocated by some
greater trochanter is unusually vulnerable to trauma. authors as a means of reducing the risk of epiphyseal
Moreover, between the ages of 4 and 8 the ligamen- ischaemia; however, the benefits are uncertain and the
tum teres contributes very little to the blood supply of matter is controversial.
the epiphysis; hence its susceptibility to post-trau- Undisplaced fractures may be treated by immobi-
matic ischaemia. lization in a plaster spica for 6–8 weeks. However,
fracture position is not always maintained and there is
a considerable risk of late displacement and malunion
Classification or non-union.
The most useful classification is that of Delbet, which Displaced type IV fractures also can be treated non-
is based on the level of the fracture (Hughes and operatively: closed reduction, traction and spica
Beaty 1994). Type I is a fracture-separation of the immobilization. Careful follow-up is essential; if posi-
epiphysis; sometimes the epiphyseal fragment is tion is lost, operative fixation will be needed.
dislocated from the acetabulum. Type II is a transcer- Type I, II and III fractures are treated by closed
vical fracture of the femoral neck; this is the com- reduction and then internal fixation with smooth pins
monest variety, accounting for almost half of the or cannulated screws. ‘Closed reduction’ means one
injuries. Type III is a basal (cervico-trochanteric) frac- gentle manipulation; if this fails, open reduction is
ture, the second most common injury. Type IV is an performed. In small children, operative fixation is sup-
intertrochanteric fracture (Figure 29.16). plemented by a spica cast for 6–12 weeks.
Clinical features Complications

Diagnosis can be difficult, especially in infants where Avascular necrosis of the femoral head This is the most
the epiphysis is not easily defined on x-ray. Type I common (and most feared) complication; it occurs in
(a) (b) (c) (d)
29.16 Proximal femoral fractures in children These are the result of strong forces or weak bone, e.g. through cysts.
There are 4 types (the Delbet classification), depending on the level of the fracture: (a) Type 1 at the physeal level; (b) Type
856 2 through the middle of the neck; (c) Type 3 at the base of the neck and (d) Type 4 at the intertrochanteric level.
29

(a) (b) (c) (d)
29.17 Femoral neck fractures in children: (a) Fracture of the femoral neck in a child is particularly worrying because,
even with perfect fixation (b), there is often ischaemia of the femoral head. This fracture united and the screws were
removed (c), but the radioisotope scan shows no activity in the left femoral head (d) i.e. ischaemic necrosis.
about 30 per cent of all cases. Important risk factors Occasionally, the greater trochanter is fractured and
are (1) an age of more than 10; (2) a high velocity the fragment widely separated in a young individual.
injury; (3) a type I or II fracture; and (4) displacement. It can be fixed back in position with cancellous screws
The child complains of pain and loss of movement; or tension band wiring. Full weightbearing is prohib-
x-ray changes usually appear within 3 months of injury. ited for 6–8 weeks.
Treatment is problematic. Non-weightbearing, or
‘containment splintage’ in abduction and internal
rotation, is sometimes advocated but there is little evi- SUBTROCHANTERIC FRACTURES
dence that this makes any difference. The outcome
depends largely on the size of the necrotic area; unfor- The part of the femoral shaft around the lesser
tunately most end up with intrusive pain and marked trochanter is substantially strengthened by a widening
restriction of movement. Arthrodesis may be advis- cortex and that stout pillar of bone posteromedially,
able, as a late salvage procedure. the calcar femorale. Therefore, large forces are needed
to cause fractures in this area – and that is usually the
Coxa vara Femoral neck deformity may result from case when this injury is diagnosed in young adults. By
malunion, avascular necrosis or premature physeal contrast, in the elderly, who are the second group
closure. If the deformity is mild, remodelling may who sustain this fracture quite frequently, the injury is
take care of it. If the neck-shaft angle is less than 110 relatively trivial; here the reason is a weakening of
degrees, subtrochanteric valgus osteotomy will prob- bone in this area by osteoporosis, osteomalacia,
ably be needed. Paget’s disease or a secondary deposit.
Diminished growth Physeal damage may result in
retarded femoral growth. Limb length equalization
may be needed.
ISOLATED FRACTURES OF THE

TROCHANTERS
In adolescents, the lesser trochanter apophysis may be

avulsed by the pull of the psoas muscle; the injury
nearly always occurs during hurdling. Treatment is rest,
followed by return to activity when comfortable. In the
elderly, separation of the lesser trochanter should
arouse suspicions of metastatic malignant disease. (a) (b) (c)
In the elderly, part of the greater trochanter can be 29.18 Subtrochanteric fractures of the femur –
fractured by a direct blow after a fall. The x-ray should warning signs on the x-ray X-ray findings that should
caution the surgeon: (a) comminution, with extension into
be scrutinised for a subtle associated intertrochanteric the piriform fossa; (b) displacement of a medial fragment
fracture. In the event this is absent, treatment is non- including the lesser trochanter and, (c) lytic lesions in the
operative and functional recovery is usually good. femur. 857
29 29.19 Subtrochanteric
fractures – internal
fixation Several
methods of fixation are
in use: (a) a 95° screw
and plate device; (b) an
intramedullary nail with
proximal interlocking
screw into the femoral
head; and (c) a proximal
femoral plate with
locking screws.
(a) (b) (c)
Subtrochanteric fractures have several features Treatment

which make them interesting (and challenging to
treat): Traction may help to reduce blood loss and pain. It is
an interim measure until the patient, especially if eld-
1. Blood loss is greater than with femoral neck or erly and with multiple medical problems, is stabilized
trochanteric fractures – the region is covered with and prepared for surgery.
anastomosing branches of the medial and lateral Open reduction and internal fixation is the treat-
circumflex femoral arteries which come off the ment of choice. Two main types of implant are used
profunda femoris trunk. for fracture fixation: (a) an intramedullary nail with a
2. There may be subtle extensions of the fracture proximal interlocking screw that can be directed into
into the intertrochanteric region, which may the femoral head or placed in the standard manner,
influence the manner in which internal fixation and (b) a 95 degree hip screw-and-plate device. Both
can be performed. implants are suitable but there are circumstances
3. The proximal part is abducted and externally where one may be preferable:
rotated by the gluteal muscles, and flexed by the
psoas. The shaft of the femur has to be brought 1. Intramedullary nails are generally stronger and can
into a position to match the proximal part or else tolerate stresses for longer if healing is slow; this
a malunion is created by internal fixation. may be the case if the fracture is very comminuted
or unstable, or if one suspects that operative
dissection may have compromised bone viability.
Clinical features 2. An intramedullary nail is also preferable for a
pathological fracture; a full-length nail should be
The leg lies in neutral or external rotation and looks used as there may be tumour deposits in the distal
short; the thigh is markedly swollen. Movement is part of the femur.
excruciatingly painful.
Key points to bear in mind when operating on these
fractures are: (a) an anatomic reduction will provide
X-ray
the greatest surface area of contact between the frag-
The fracture is through or below the lesser trochanter. ments and reduce stresses on the implant; with
It may be transverse, oblique or spiral, and is fre- intramedullary nails this has to be achieved before
quently comminuted. The upper fragment is flexed reaming is commenced; (b) as little soft-tissue dissec-
and appears deceptively short; the shaft is adducted tion as possible to accomplish reduction should be
and is displaced proximally. performed; and (c) it is important that the integrity of
Three important features should be looked for, as the medial cortex (around the lesser trochanter) be
the presence of any one will influence treatment: (1) established, particularly if a hip screw-and-plate device
an unusually long fracture line extending proximally is used.
towards the greater trochanter and piriform fossa; (2) Proximal interlocking screws with intramedullary
a large, displaced fragment which includes the lesser nails should be directed into the femoral head if the
858 trochanter; and (3) lytic lesions in the femur. fracture pattern extends above the lesser trochanter. If
the fracture enters the piriformis fossa, then an elderly patients should be considered ‘pathological’ 29
intramedullary nail designed to be inserted at the tip until proved otherwise. In children under 4 years the
of the greater trochanter is better; alternatively a 95 possibility of physical abuse must be kept in mind.
degree hip screw-and-plate device can be used. Fracture patterns are clues to the type of force that
Postoperatively the patient is allowed partial produced the break. A spiral fracture is usually caused
weightbearing (with crutches) until union is secure. It by a fall in which the foot is anchored while a twisting

is rarely feasible to impose significant weightbearing force is transmitted to the femur. Transverse and
restrictions on the elderly and it would be better to oblique fractures are more often due to angulation or
choose a stronger implant (and ensure a near- direct violence and are therefore particularly common
anatomic reduction of the fracture) so that early load- in road accidents. With severe violence (often a com-
ing can be tolerated. bination of direct and indirect forces) the fracture may
be comminuted, or the bone may be broken in more
than one place (a segmental fracture).
Complications
Malunion Varus and rotational malunions are fairly
common. This can be prevented by careful attention Pathological anatomy
to accurate reduction before internal fixation is
applied. If the degree of malunion produces symp- Most fractures of the femoral shaft have some degree
toms, it may need operative correction. of comminution, although it is not always apparent on
x-ray. Small bone fragments, or a single large ‘butter-
Non-union This occurs in about 5 per cent of cases; it
fly’ fragment, may separate at the fracture line but
will require operative correction of any deformity,
usually remain attached to the adjacent soft tissue and
renewed fixation and bone grafting.
retain their blood supply. With more extensive com-
minution there is no point of firm contact between
proximal and distal fragments and the fracture is com-
FEMORAL SHAFT FRACTURES pletely unstable (Figure 29.20). This is the basis of a
helpful classification (Winquist, Hansen et al. 1984).
Fracture displacement often follows a predictable
The femoral shaft is circumferentially padded with
pattern dictated by the pull of muscles attached to
large muscles. This provides advantages and disadvan-
each fragment.
tages: reduction can be difficult as muscle contraction
displaces the fracture; however, healing potential is • In proximal shaft fractures the proximal fragment is
improved by having this well-vascularized sleeve con- flexed, abducted and externally rotated because of
taining a source of mesenchymal stem cells, and open gluteus medius and iliopsoas pull; the distal frag-
fractures often need no more than split thickness skin ment is frequently adducted.
grafts to obtain satisfactory cover. • In mid-shaft fractures the proximal fragment is
again flexed and externally rotated but abduction is
less marked.
Mechanism of injury • In lower third fractures the proximal fragment is
This is usually a fracture of young adults and results adducted and the distal fragment is tilted by gas-
from a high energy injury. Diaphyseal fractures in trocnemius pull.
29.20 Femoral shaft fractures –

classification Winquist’s classification reflects
the observation that the degrees of soft-tissue
damage and fracture instability increase with
increasing grades of comminution. In Type 1
there is only a tiny cortical fragment. In Type 2
the ‘butterfly fragment’ is larger but there is
still at least 50 per cent cortical contact
between the main fragments. In Type 3 the
butterfly fragment involves more than 50 per
cent of the bone width. Type 4 is essentially a
segmental fracture.
859
29 The soft tissues are always injured and bleeding
from the perforators of the profunda femoris may be
severe. Over one litre may be lost into the tissues and,
in the case of bilateral femoral shaft fractures, the
patient can become hypotensive quickly if not ade-
quately resuscitated. Beware of the fracture at the
junction of the middle and distal thirds of the femoral

shaft – it can be responsible for damaging the femoral
artery in the adductor canal.
Clinical features
There is swelling and deformity of the limb, and any
attempt to move the limb is painful. With the excep-
tion of a fracture through pathological bone, the large
forces needed to break the femur usually produce
accompanying injuries nearby and sometimes further
afield. Careful clinical scrutiny is necessary to exclude
neurovascular problems and other lower limb or
(a) (b)
pelvic fractures. An ipsilateral femoral neck fracture
occurs in about 10 per cent of cases and, if present, 29.21 Femoral shaft fractures – diagnosis (a) The
upper fragment of this femur is adducted, which should
there is a one in three chance of a significant knee
alert the surgeon to the possibility of (b) an associated hip
injury as well. The combination of femoral shaft and dislocation. With this combination of injuries the
tibial shaft fractures on the same side, producing a dislocation is frequently missed; the safest plan is to x-ray
‘floating knee’, signals a high risk of multi-system the pelvis with every fracture of the femoral shaft.
injury in the patient. The effects of blood loss and
other injuries, some of which can be life-threatening,
may dominate the clinical picture.
a definitive plan of action instituted as soon as the
X-ray patient’s condition has been fully assessed.
It may be difficult to obtain adequate views in the

Accident and Emergency Room setting, especially
Definitive treatment
views that provide reliable information on proximal or The patient with multiple injuries The association of
distal fracture extensions or joint involvement; these femoral shaft fractures with other injuries, including
can be postponed until better facilities and easier head, chest, abdominal and pelvic trauma, increases
patient positioning are possible. But never forget to x- the potential for developing fat embolism, ARDS and
ray the hip and knee as well (Figure 29.21). A baseline multi-organ failure. The risk of systemic complica-
chest x-ray is useful as there is a risk of adult respira- tions can be significantly reduced by early stabilization
tory distress syndrome (ARDS) in those with multiple of the fracture, usually by a locked intramedullary nail.
injuries. However, surgery to introduce a reamed intra-
The fracture pattern should be noted; it will form a medullary nail may produce untoward effects in those
guide to treatment. with severe chest injuries, especially when carried out
within 24 hours of the fracture. It is thought the
trauma of surgery and blood loss induces inflamma-
Emergency treatment
tory changes that may increase both morbidity and
Traction with a splint is first aid for a patient with a mortality – this phenomenon is called ‘the second hit’,
femoral shaft fracture. It is applied at the site of the referring to a second episode of trauma, albeit surgi-
accident, and before the patient is moved. A Thomas’ cal, on the patient. Consequently, in the multiply-
splint, or one of the modern derivations of this prac- injured patient, particularly one with severe chest
tical device, is ideal: the leg is pulled straight and trauma, prompt stabilization with an external fixator
threaded through the ring of the splint; the shod foot may be wise; the fixator can be exchanged for an
is tied to the cross-piece so as to maintain traction and intramedullary nail when the patient’s condition sta-
the limb and splint are firmly bandaged together. This bilizes. The timing of this second procedure is prob-
temporary stabilization helps to control pain, reduces lematic. Some guidance can be sought from
bleeding and makes transfer easier. Shock should be measurement of circulating levels of interleukin-6, a
860 treated; blood volume is restored and maintained, and pro-inflammatory cytokine (Pape, van Griensven et al.
2001); when the levels start to decrease, it should be The main indications for traction are (1) fractures 29
safe to perform ‘second hit’ interventions. Clinically in children; (2) contraindications to anaesthesia; and
this occurs around 5–7 days after admission, but this (3) lack of suitable skill or facilities for internal fixa-
window is by no means applicable to all patients nor tion. It is a poor choice for elderly patients, for patho-
is it conclusive at this time. logical fractures and for those with multiple injuries.
Performing the exchange to an intramedullary nail The various methods of traction are described in

also carries the risk of transferring contaminants from Chapter 23. For young children, skin traction without
pin sites to the intramedullary nail; the earlier the a splint is usually all that is needed. Infants less than
operation is performed, the lower the risk. In the 12 kg in weight are most easily managed by suspending
patient who spends a protracted period in the inten- the lower limbs from overhead pulleys (‘gallows trac-
sive care unit, it may be safer to use external fixation tion’), but no more than 2 kg weight should be used
as definitive treatment, perhaps with a return to the- and the feet must be checked frequently for circulatory
atre later to allow insertion of new pins to increase the problems. Older children are better suited to Russell’s
stability of the construct. traction (Chapter 23) or use of a Thomas’ splint. Frac-
ture union will have progressed sufficiently by 2–4
weeks (depending on the age of the child) to permit a
THE ISOLATED FEMORAL SHAFT FRACTURE hip spica to be applied and the child is then allowed up.
Traction, bracing and spica casts Traction can reduce Consolidation is usually complete by 6–12 weeks.
and hold most fractures in reasonable alignment, Adults (and older adolescents) require skeletal trac-
except those in the upper third of the femur. Joint tion through a pin or a tightly strung Kirschner wire
mobility can be ensured by active exercises. The chief behind the tibial tubercle. Traction (8–10 kg for an
drawback is the length of time spent in bed (10–14 adult) is applied over pulleys at the foot of the bed.
weeks for adults) with the attendant problems of The limb is usually supported on a Thomas’ splint and
keeping the femur aligned until sufficient callus has a flexion piece allows movement at the knee (Figure
formed plus reducing patient morbidity and frustra- 29.22). However, a splint is not essential; indeed,
tion. Some of these difficulties are overcome by skeletal traction without a splint (Perkins’ traction) has
changing to a plaster spica or – in the case of lower the advantages of producing less distortion of the
third fractures – functional bracing when the fracture fracture and allowing freer movement in bed (Figure
is ‘sticky’, usually around 6–8 weeks. 29.23). Exercises are begun as soon as possible.
(a) (b)
(c) (d)
29.22 Femoral fractures – treatment by traction (a) Fixed traction on a Thomas’ splint: the
splint is tied to the foot of the bed which is elevated. This method should be used only rarely
because the knee may stiffen; (b) this was the range in such a case when the fracture had united.
(c,d) Balanced traction: one way to minimize stiffness is to use skeletal balanced traction; the
lower slings can be removed to permit knee flexion while traction is still maintained. 861
29
(b)
(f)
(c)
(a) (d) (e) (g)
29.23 Femoral fractures – treatment by traction Even in the adult, traction without a splint can be
satisfactory, but skeletal traction is essential. The patient with this rather unstable fracture (a) can lift his leg
and exercise his knee (b,c,d). At no time was the leg splinted, but clearly the fracture has consolidated
(e), and the knee range (f) is only slightly less than that of the uninjured left leg (g).
Once the fracture is sticky (at about 8 weeks in either end of the femoral shaft, especially those with
adults) traction can be discontinued and the patient extensions into the supracondylar or pertrochanteric
allowed up and partial weightbearing in a cast or areas, (2) a shaft fracture in a growing child, and (3)
brace. For fractures in the upper half of the femur, a a fracture with a vascular injury which requires repair
plaster spica is the safest but it will almost certainly (Figure 29.24).
prolong the period of knee stiffness. For fractures in
Intramedullary nailing Intramedullary nailing is the
the lower half of the femur, cast-bracing is suitable.
method of choice for most femoral shaft fractures.
This type of protection is needed until the fracture has
However, it should not be attempted unless the
consolidated (16–24 weeks).
appropriate facilities and expertise are available. The
basic implant system consists of an intramedullary nail
Plate and screw fixation Plating is a comparatively easy
(in a range of sizes) which is perforated near each end
way of obtaining accurate reduction and firm fixation.
so that locking screws can be inserted transversely at
The method was popular at one time but went out of
the proximal and distal ends; this controls rotation
favour because of a high complication rate. This
and length, and ensures stability even for sub-
occurred when plates were applied through a wide
trochanteric and distal third fractures (Figure 29.25).
open exposure of the fracture site and perfect anatom-
These important details should be remembered
ical reduction of all bone pieces. Such extensive sur-
when using locked intramedullary nails:
gery damaged the healing potential and led to tardy
union and implant failure. However, plates have 1. Reamed nails have a lower need for revision
encountered resurgence: today, they are inserted surgery when compared to unreamed nails.
through short incisions and placed in a submuscular 2. Select a nail that is approximately the size of the
plane, rather than deep to periosteum; an indirect medullary isthmus so that it fills the canal
(closed) reduction of the fracture is done; fewer reasonably well (after reaming) and adds to
screws are used, and usually placed at the ends of the stability – small diameter nails are quicker to insert
plate, leading to a less rigid hold on the fracture. This but more frequently lead to the need for revision
technique of minimally invasive plate osteosynthesis surgery.
(MIPO) has led to better union rates. However, post- 3. Consider alternative means of fracture fixation if
operative weightbearing will need to be modified as the isthmus is so narrow that a large amount of
the implant is not as strong as an intramedullary nail. canal reaming will have to be done in order to fit
862 The main indications for plates are (1) fractures at the smallest diameter nail available.
29

(a) (b) (c) (d) (e)
29.24 Plate fixation – past and present (a,b) Plate fixation was popular in the past, but it fell out of favour because of
the high complication rate (c). Modern techniques of minimally invasive plate osteosynthesis (d,e) have shown that it still
has place in the treatment of certain types of femoral shaft fracture.
4. Use a nail of sufficient length to fully span the canal. for each side); when there is a tibial shaft fracture
5. Antegrade insertion (through either the piriformis on the same side; and if there is a femoral neck
fossa or the tip of the greater trochanter, fracture more proximally, as screws can be inserted
depending on the design of nail) or retrograde to hold this fracture without being impeded by
insertion (through the intercondylar notch the nail.
distally) are equally suitable techniques to use;
there is a small incidence of hip and thigh pain Stability is improved by using interlocking screws;
with antegrade nails, whereas there is a small all locking holes in the nail should be used. Often
problem with knee pain with retrograde nails. there is enough shared stability between the nail and
Retrograde insertion of intramedullary nails is fracture ends to allow some weightbearing early on.
particularly useful for: obese patients; when there The fracture usually heals within 20 weeks and the
are bilateral femoral shaft fractures (as the complication rate is low; sometimes malunion (more
procedure can be performed without the need for likely malrotation) or delayed union (from leaving the
a fracture table and the added time for setting up fracture site over-distracted) occurs.
(a) (b) (c) (d) (e)
29.25 Intramedullary nailing Nowadays this is the commonest way of treating femoral shaft fractures. Ideally a range
of designs to suit different types of fracture should be available. (a,b) Antegrade nailing with insertion of the nail through
the pyriform fossa and transverse locking screws proximally and distally. (c) Retrograde nailing with insertion of the nail
through the intercondylar notch at the knee – useful for obese patients and those with bilateral femoral fractures.
(d,e) Proximal locking can be achieved in other ways e.g. by using parallel screws or a sliding hip screw. 863
29 Open medullary nailing is a feasible alternative Treatment of open fractures
where facilities for closed nailing are lacking. A limited
lateral exposure of the femur is made; the fracture is Open femoral fractures should be carefully assessed
reduced and a guidewire is passed between the main for (1) skin loss; (2) wound contamination; (3) mus-
proximal and distal fragments. A small exposure to cle ischaemia; and (4) injury to vessels and nerves.
achieve reduction does not significantly affect the risk The immediate treatment is similar to that of closed
of complications or fracture healing as compared to fractures; in addition, the patient is started on intra-
‘closed’ nailing. venous antibiotics. The wound will need cleansing: it
should be extended to give unhindered access, con-
External fixation The main indications for external fix- taminated areas and dead tissue must be excised and
ation are (1) treatment of severe open injuries; (2) the entire area should be washed thoroughly.
management of patients with multiple injuries where Stabilization of open femoral shaft fractures is best
there is a need to reduce operating time and prevent achieved with locked intramedullary nails unless there
the ‘second hit’; and (3) the need to deal with severe is heavy contamination or bone loss – in which case
bone loss by the technique of bone transport. Exter- external fixation (if necessary with the capacity to deal
nal fixation is also useful for (4) treating femoral frac- with bone loss through distraction osteogenesis) is
tures in adolescents (Figure 29.26). preferable.
Like closed intramedullary nailing, it has the advan-
tage of not exposing the fracture site and small
amounts of axial movement can be applied to the Complex injuries
bone by allowing a telescoping action in the fixator FRACTURES ASSOCIATED WITH VASCULAR INJURY
body (with some designs of external fixator). As the Warning signs of an associated vascular injury are
callus increases in volume and quality, the fixator can (1) excessive bleeding or haematoma formation; and
be adjusted to increase stress transfer to the fracture (2) paraesthesia, pallor or pulselessness in the leg and
site, thus promoting quicker consolidation. However, foot. Do not accept ‘arterial spasm’ as a cause of absent
there are still problems with pin-site infection, pin pulses; the fracture level on x-ray will indicate the region
loosening and (if the half-pins are applied close to of arterial damage and arteriography may only delay
joints) limitation of movement due to interference surgery to re-establish perfusion. Most femoral fractures
with sliding structures. with vascular injuries will have had warm ischaemia
The patient is allowed up as soon as he or she is times greater than 2–3 hours by the time the patient
comfortable and knee movement exercises are arrives in the operating theatre; when this exceeds 4–
encouraged to prevent tethering by the half pins. Par- 6 hours, salvage may not be possible and the risk of
tial weightbearing is usually possible immediately but amputation rises. This means that diagnosis must be
this will depend on the x-ray appearance of callus – prompt and re-establishing perfusion a priority; frac-
this may take some time (more than 6 weeks) if the ture stabilization is secondary.
fixator is a rigid device. Most femoral shaft fractures A recommended sequence for treatment, particu-
will unite in under 5 months but some take longer if larly if the warm ischaemia time is approaching the sal-
the fracture is badly comminuted or contact between vage threshold, is (a) to create a shunt from the
fracture ends is poor. femoral vessels in the groin to beyond the point of
29.26 External
fixation for femoral
shaft fractures in older
children (a–c) External
fixation is an option for
treating femoral shaft
fractures in adolescents.
Elastic stable
intramedullary nails
shown in Fig 29.31 may
not be strong enough
for this heavier group of
teenagers.
864 (a) (b) (c)

injury using plastic catheters; (b) to stabilize the frac- an x-ray of the pelvis. Both sites must be stabilized, 29
ture (usually by plating or external fixation) and then first the femoral neck and then the femur. Parallel
(c) to carry out definitive vascular repair. This screw fixation of the femoral neck followed by retro-
sequence establishes blood flow quickly and permits grade femoral nailing is a useful way to treat this prob-
fracture fixation and vascular repair to be carried out lem.
without pressure of time.

PATHOLOGICAL FRACTURES
FRACTURE ASSOCIATED WITH KNEE INJURY Fractures through metastatic lesions should be fixed
Femoral fractures are frequently accompanied by by intramedullary nailing. Provided the patient is fit
injury to the ligaments of the knee. Direct blows to enough to tolerate the operation, a short life
the knee from the dashboard of a car in an accident expectancy is not a contraindication. ‘Prophylactic fix-
will damage knee ligaments as well as break the ation’ is also indicated if a lytic lesion is (a) greater
femoral shaft and femoral neck – this triad of prob- than half the diameter of the bone; (b) longer than
lems should be recognized. With attention focused 3 cm on any view, or (c) painful, irrespective of its
on the femur, the knee injury is easily overlooked, size.
only to re-emerge as a persistent complaint weeks or Paget’s disease, fibrous dysplasia or rickets may
months later. As soon as the fracture has been stabi- present a problem. The femur is likely to be bowed
lized, the knee should be carefully examined and any and, in the case of Paget’s disease, abnormally hard.
associated abnormality treated. An osteotomy to straighten the femur may be neces-
sary to allow a nail to be inserted fully (Figure 29.27).
‘FLOATING KNEE’
Ipsilateral fractures of the femur and tibia may leave the PERIPROSTHETIC FRACTURES
knee joint ‘floating’. This is a very serious situation, and Femoral shaft fractures around a hip implant are
other injuries are often present. Both fractures will uncommon; they may happen during primary hip sur-
need immediate stabilization – an anterior approach to gery when reaming or preparing the medullary canal,
the knee joint will allow both fractures to be stabilized or when forcing in an over-sized uncemented pros-
by intramedullary nails – retrograde for the femur and thesis, or during revision surgery while extracting
antegrade for the tibia. It is usual to fix the femur first. cement or attempting to dislocate the hip if the soft
tissue release has been insufficient. Sometimes the
COMBINED NECK AND SHAFT FRACTURES fracture occurs much later, and there are usually x-ray
This is dealt with on page 850. The most important signs of osteolysis or implant loosening suggesting a
thing is diagnosis: always examine the hip and obtain reason for bone weakness.
(a) (b) (c) (d) (e)

29.27 Pathological fractures – internal fixation (a) Metastatic tumour, nailed before it actually causes a fracture.
(b) Fibrous dysplasia with a stress fracture; (c) nailing provided the opportunity to correct the deformity. (d,e) Paget’s
disease, with a fracture; in this case (because of its site) treated by fixation with a plate and screws. 865
29 If the prosthesis is worn or loose, it should be piratory distress syndrome). Blood gases should be
removed and replaced by one with a long stem, measured if this is suspected and signs such as short-
thereby treating both problems. If the primary ness of breath, restlessness or a rise in temperature or
implant is neither loose nor worn it can be left in place pulse rate should prompt a search for petechial
and the fracture treated by plate fixation with struc- haemorrhages over the upper body, axillae and con-
tural allografts bridging the fracture (Figure 29.28). junctivae. Treatment is supportive, with the emphasis
on preventing hypoxia and maintaining blood

volume.
Complications of femoral shaft fractures Thromboembolism Prolonged traction in bed predis-
All the complications described in Chapter 23, with poses to thrombosis. Movement and exercise are
the exception of visceral injury and avascular necrosis, important in preventing this, but high-risk patients
are encountered in femoral shaft fractures. The more should be given prophylactic anticoagulants as well.
common ones are as follows. Vigilance is needed and full anticoagulant treatment is
started immediately if thigh or pelvic vein thrombosis
EARLY is diagnosed.
Shock One or two litres of blood can be lost even
Infection In open injuries, and following internal fixa-
with a closed fracture, and if the injury is bilateral
tion, there is always a risk of infection. Prophylactic an-
shock may be severe. Prevention is better than cure;
tibiotics and careful attention to the principles of frac-
most patients will require a transfusion.
ture surgery should keep the incidence below 2 per
Fat embolism and ARDS Fracture through a large mar- cent. If the bone does become infected, the patient
row-filled cavity almost inevitably results in small should be treated as for an acute osteomyelitis. Antibi-
showers of fat emboli being swept to the lungs. This otic treatment may suppress the infection until the
can usually be accommodated without serious conse- fracture unites, at which time the femoral nail can be re-
quences, but in some cases (and especially in those moved and the canal reamed and washed out. How-
with multiple injuries and severe shock, or in patients ever, if there is pus or a sequestrum, a more radical ap-
with associated chest injuries) it results in progressive proach is called for: the wound is explored, all dead and
respiratory distress and multi-organ failure (adult res- infected tissue is removed and the nail as well; the
(a) (b) (c) (d)
29.28 Periprosthetic fracture This patient had two successive fractures around his hip prosthesis. The first was held with
866 cerclage wires (a,b). As the prosthesis was secure in the femur the second fracture was fixed with a plate and screws (c,d).
canal is reamed and washed out and the fracture is then Malunion Fractures treated by traction and bracing 29
stabilized by an external fixator. Replacement of the ex- often develop some deformity; no more than 15
ternal fixator by another intramedullary nail can be degrees of angulation should be accepted (Figure
risky, and much depends of the nature of the infecting 29.29). Even if the initial reduction was satisfactory,
organism (its sensitivity or resistance to antibiotics), the until the x-ray shows solid union the fracture is too
length of time during which the infection has been insecure to permit weightbearing; the bone will bend

present and the quality of the surgical debridement. and what previously seemed a satisfactory reduction
The long-term management of chronic osteo- may end up with lateral or anterior bowing.
myelitis is discussed in Chapter 2. Malunion is much less likely in those treated with
static interlocked nails; yet it does still occur – espe-
LATE cially malrotation – and this can be prevented only by
Delayed union and non-union The time-scale for meticulous intra-operative and post-operative assess-
declaring a delayed or non-union can vary with the ment followed, where necessary, by immediate cor-
type of injury and the method of treatment. If there is rection. Shortening is seldom a major problem unless
failure to progress by 6 months, as judged by serial there was bone loss; if it does occur, treatment will
x-rays, then intervention may be needed. A common depend on the amount and its clinical impact – some-
practice is to remove locking screws from the times all that is needed is a built-up shoe.
intramedullary nail to enable the fracture to ‘collapse’
Joint stiffness The knee is often affected after a
(‘dynamise’ in modern orthopaedic parlance). This
femoral shaft fracture. The joint may be injured at the
can be successful in a small proportion of cases; more
same time, or it stiffens due to soft-tissue adhesions
often it fails and results in pain as rotational control of
during treatment; hence the importance of repeated
the fracture is lost (the femur is often subject to tor-
evaluation and early physiotherapy.
sional forces in walking). A better course is to remove
the nail, ream the medullary canal and introduce a Refracture and implant failure Fractures which heal
larger diameter nail – exchange nailing. Bone grafts with abundant callus are unlikely to recur. By contrast,
should be added to the fracture site if there are gaps in those treated by internal fixation, callus formation
not closed at the revision procedure. is often slow and meagre. With delayed union or non-
(a) (b) (c) (d)
29.29 Malalignment after treatment Treatment of femoral shaft fractures by traction can produce good results but, in
some, a malunion can lead to symptoms. In this patient (a,b) the varus deformity produced knee symptoms from
overloading of the medial compartment; this was relieved by corrective osteotomy and intramedullary nailing (c,d).
867
29 union, the integrity of the femur may be almost children under 2 years of age the commonest cause is
wholly dependent on the implant and sooner or later child abuse; if there are several fractures in different
it will fail. If a comminuted fracture is plated, bone stages of healing, this is very suspicious.
grafts should be added and weightbearing delayed so Pathological fractures are common in generalized
as to protect the plate from reaching its fatigue limit disorders such as spina bifida and osteogenesis imper-
too soon. Intramedullary nails are less prone to break. fecta, and with local bone lesions (e.g. a benign cyst
However, sometimes they do, especially with a slow- or tumour).

healing fracture of the lower third and a static locked
nail; the break usually occurs through the screw-hole
Treatment
closest to the fracture. Treatment consists of replacing
the nail and adding bone grafts. In resistant cases, the The principles of treatment in children are the same as
fracture site may need excising (as viability of the bone in adults but it should be emphasized that in young
ends is poor) followed by distraction osteogenesis children open treatment is rarely necessary. The
which simultaneously stabilizes the limb and deals choice of closed method depends largely on the age
with the length discrepancy (Figure 29.30). and weight of the child. As children get older (and
larger), fractures take longer to heal and conservative
treatment is more likely to result in problems associ-
ated with long hospitalization and a greater risk of
FEMORAL SHAFT FRACTURES IN malunion (Poolman, Kocher et al. 2006). Coupled to
CHILDREN this is the cost of protracted bed occupancy. Conse-
quently there has been a trend towards treating
femoral shaft fractures in older children by operation,
Mechanism
but the argument is flawed if this is based on cost
Fractures of the femur are quite common in older alone – many of these children will have to return for
children and are usually due to direct violence (e.g. a implant removal. Perhaps it is the risk of malunion,
road accident) or a fall from a height. However, in particularly in unstable fracture patterns, that renders
(a) (b) (c) (d)
29.30 Implant failure and non-union (a) This was an open injury with poor vascularity of the fracture ends. It was fixed
with an intramedullary nail in the hope that it might unite. It didn’t, and one of the proximal screws broke. The fracture
ends were excised; an external fixator was applied (b); and an osteotomy was performed lower down (c); then the fracture
868 ends were brought together with distraction osteogenesis at the osteotomy site. The fracture united (d).
surgery a better option for older children and If a satisfactory reduction cannot be achieved by 29
adolescents. traction, internal (plates or flexible intramedullary
nails) or external fixation is justified. This applies to
Traction and casts Infants need no more than a few
older children and those with multiple injuries.
days in balanced traction, followed by a spica cast for
another 3–4 weeks. Angulation of up to 30 degrees Operative treatment This is growing in popularity as
can be accepted, as the bone remodels quite remark- there is: (1) a shorter in-patient stay (and for the child,

ably with growth. Immediate spica casting has also a quick return home); (2) a lower incidence of malu-
found favour and this approach does not appear to nion. Against this is the added risk of surgery, taking
increase the risk of complications. into account that many such fractures have good results
Children between 2 and 10 years of age can be when treated non-operatively. The tendency to adopt
treated either with balanced traction for 2–3 weeks this approach in older children and adolescents may be
followed by a spica cast for another 4 weeks, or by justified. Surgical options include fixation with flexible
early reduction and a spica cast from the outset. intramedullary nails or trochanteric entry-point rigid
Shortening of 1–2 cm and angulation of up to 20 nails with interlocking screws (neither of which
degrees are acceptable. damages the physes), plates inserted by the MIPO
Teenagers require somewhat longer (4–6 weeks) in technique and external fixation (Figure 29.31).
balanced traction, and those aged over 15 (or even
younger adolescents if they are large and muscular)
may need skeletal traction. Once the fracture feels
Complications
firm, traction is exchanged for either a spica cast (in Shortening Overlapping and comminution of the
the case of upper third and mid-shaft fractures) or a bone fragments may shorten the femur. However,
cast-brace (for lower third fractures), which is retained anything up to 2 cm is quite acceptable in young chil-
for a further 6 weeks. The position should be checked dren; indeed, some surgeons regard this as an advan-
every few weeks; the limit of acceptable angulation in tage because there is a tendency for the fractured
this age group is 15 degrees in the anteroposterior bone to grow faster for up to 2 years after the injury.
x-ray and 25 degrees in the lateral. This may be related to stimulation of the physes
(a) (b) (c) (d)
29.31 Fixation techniques for femoral shaft fractures in children Non-operative treatment is safest for children. If
surgery is indicated, options include: (a) flexible nailing; (b) trochanteric entry-point rigid nails; (c) plates and screws
inserted by the minimally invasive percutaneous osteosynthesis (MIPO) technique and, (d) external fixation. 869
29 derived from the increased blood flow that accompa- should always be checked to ensure the popliteal
nies fracture healing. Unfortunately, the effect on artery was not injured in the fracture.
growth is unpredictable.
X-RAY The entire femur should be x-rayed so as not
Malunion Angulation can usually be tolerated within to miss a proximal fracture or dislocated hip. The
the limits mentioned above. However, the fact that supracondylar fracture pattern will vary. Of impor-
bone modelling is excellent in children is no excuse tance are: (a) whether there is a fracture into the joint
for casual management; bone may be forgiving but and if it is comminuted; (b) the size of the distal seg-
parents are not! Certainly rotational malunion is not ment; and (c) whether the bone is osteoporotic.
corrected by growth or remodelling. It is probably These factors influence the type of internal fixation
wise to observe a malunited fracture for 2 years before required, if that is the chosen mode of treatment.
offering corrective osteotomy.
Treatment
SUPRACONDYLAR FRACTURES OF Non-operative If the fracture is only slightly displaced
THE FEMUR and extra-articular, or if it reduces easily with the knee
in flexion, it can be treated quite satisfactorily by trac-
tion through the proximal tibia; the limb is cradled on
Supracondylar fractures of the femur are encountered a Thomas’ splint with a knee flexion piece and move-
(a) in young adults, usually as a result of high energy ments are encouraged. If the distal fragment is dis-
trauma, and (b) in elderly, osteoporotic individuals. placed by gastrocnemius pull, a second pin above the
knee, and vertical traction, will correct this. At 4–6
Mechanism and pathological anatomy weeks, when the fracture is beginning to unite, traction
can be replaced by a cast-brace and the patient allowed
Direct violence is the usual cause. The fracture line is up and partially weightbearing with crutches. Non-
just above the condyles, but may extend between them. operative treatment should be considered as an option
In the worst cases the fracture is severely comminuted. if the patient is young or the facilities and skill to treat
A useful classification is from the AO group: type A by internal fixation are absent. Elderly patients tend not
fractures have no articular splits and are truly ‘supra- do as well with the 6 weeks of enforced recumbency.
condylar’; type B fractures are simply shear fractures of
one of the condyles; and type C fractures have supra- Surgery Operative treatment with internal fixation
condylar and intercondylar fissures (Figure 29.32). can enable accurate fracture reduction, especially of
Gastrocnemius, arising from the posterior surface of the the joint surface, and early movement. If the neces-
distal femur, will tend to pull the distal segment into sary facilities and skill are available, this is the treat-
extension, thus risking injury to the popliteal artery. ment of choice. For the elderly, early mobilization is
so important that internal fixation is almost obliga-
tory. Sometimes the hold on osteoporotic bone is
Clinical features poor (despite modern implant designs) or the patient
The knee is swollen because of a haemarthrosis – this may be old and frail, making early mobilization diffi-
can be severe enough to cause blistering later. Move- cult or risky, but nursing in bed is made easier and
ment is too painful to be attempted. The tibial pulses knee movements can be started sooner.
Several different devices are available:
1. Locked intramedullary nails which are introduced
retrograde through the intercondylar notch –
these are suitable for the type A and simpler type
C fractures
2. Plates that are applied to the lateral surface of the
femur: traditional angled blade-plates or 95 degree
condylar screw-plates. They are suitable for type A
and the simpler type C fractures. For severely
comminuted type C fractures, the newer plate
designs with locking screws appear to offer an
(a) (b) (c)
advantage over other implants; they provide
29.32 The AO classification of supracondylar
adequate stability, even in the presence of
fractures (a) Type A fractures do not involve the joint
surface; (b) type B fractures involve the joint surface (one osteoporotic bone, but (as with compression
condyle) but leave the supracondylar region intact; (c) type plates) unprotected weightbearing is best avoided
870 C fractures have supracondylar and condylar components. until union is assured.
3. Simple lag screws – these suffice for type B fractures Knee movements are started soon after operation, if 29
and are inserted in parallel, with the screw heads wound healing allows. This limits adhesions forming
buried within the articular cartilage to avoid within the knee joint.
abrading the opposing joint surface. They are also
used to hold the femoral condyles together in type
Complications
C fractures before intramedullary nails or lateral

plates are used to hold the main supracondylar EARLY
break (Figure 29.33). Arterial damage There is a small but definite risk of
arterial damage and distal ischaemia. Careful assess-
ment of the leg and peripheral pulses is essential, even
if the x-ray shows only minimal displacement.
LATE
Joint stiffness Knee stiffness – probably due to scarring
from the injury and the operation – is almost inevitable.
A long period of exercise is needed in all cases, and even
then full movement is rarely regained. For marked
stiffness, arthroscopic division of adhesions in the joint
(a) (b)
or even a quadricepsplasty may be needed.
Malunion Internal fixation of these fractures is diffi-
cult and malunion – usually varus and recurvatum – is
not uncommon. Corrective osteotomy may be
needed for patients who are still physically active.
Non-union Modern surgical techniques of internal
fixation recognize the importance of minimizing
damage to the soft tissues around the fracture; where
possible, only those parts that are essential for fracture
reduction are exposed. The knee joint may need to be
(c) (d)
opened for reduction of articular fragments but the
29.33 Femoral condyle fractures – treatment (a) A metaphyseal area is left untouched in order to pre-
single condylar fracture can be reduced open and held with
Kirschner wires preparatory to (b) inserting compression serve its vitality. If these precautions are taken, non-
screws. (c) T- or Y-shaped fractures are best fixed with a union is unlikely. If non-union does occur,
dynamic condylar screw and plate (d). autogenous bone grafts and a revision of internal fix-
(a)
(b)
(e) (f) (g)
(c) (d)
29.34 Supracondylar fractures (a–c) These fractures can sometimes be treated successfully by traction through the
upper tibia. (d–g) If the bone is not too osteoporotic, internal fixation is often preferable and the patient can get out of
bed sooner: a dynamic condylar screw and plate for a Type A fracture (d) and a combination of lag screws and a lateral
side plate for more complex fracture patterns (e,f,g). 871
29 ation will be needed – particularly if there are signs forward displacement of the epiphysis, the popliteal
that the fixation is working loose or has failed. artery may be obstructed by the lower femur.
Knee stiffness is another threat. Unless great care is ex-
ercised during mobilization, the ultimate range of move-
Treatment
ment at the knee may be less than that at the fracture!
The fracture can usually be perfectly reduced manu-
ally, but further x-ray checks will be needed over the

next few weeks to ensure that reduction is maintained.
FRACTURE-SEPARATION OF DISTAL Occasionally open reduction is needed; a flap of
FEMORAL EPIPHYSIS periosteum may be trapped in the fracture line. Salter–
Harris types 3 and 4 should be accurately reduced and
In the childhood or adolescent equivalent of a supra- fixed. If there is a tendency to redisplacement, the
condylar fracture, the lower femoral epiphysis may be fragments may be stabilized with percutaneous
displaced – either to one side (usually laterally) by Kirschner wires or lag screws driven across the meta-
forced angulation of the straight knee or forwards by physeal spike. The limb is immobilized in plaster and
a hyperextension injury. Although not nearly as com- the patient is allowed partial weightbearing on
mon as physeal fractures at the elbow or ankle, this crutches. The cast can be removed after 6–8 weeks
injury is important because of its potential for causing and physiotherapy started.
abnormal growth and deformity of the knee.
The fracture is usually a Salter–Harris type 2 lesion Complications
– i.e. physeal separation with a large triangular meta-
physeal bone fragment (Figure 29.35). Although this EARLY
type of fracture usually has a good prognosis, asym- Vascular injury There is danger of gangrene unless
metrical growth arrest is not uncommon and the child the hyperextension injury is reduced without delay.
may end up with a valgus or varus deformity. All
grades of injury, but especially Salter–Harris types 3 LATE
and 4, may result in femoral shortening. Nearly 70 per Physeal arrest Damage to the physis is not uncommon
cent of the femur’s length is derived from the distal and residual deformity may require corrective
physis, so an early arrest can present a major problem. osteotomy at the end of the growth period. Small
areas of tethering across the growth plate can some-
times be successfully removed and normal growth
Clinical features restored. Shortening, if it is marked, can be treated by
The knee is swollen and perhaps deformed. The femoral lengthening.
pulses in the foot should be palpated because, with
Barnes R, Brown JT, Garden RS, et al. Subcapital fractures

of the femur. A prospective review. J Bone Joint Surg
1976; 58B: 2–24.
Bonnaire FA, Weber AT. The influence of haemarthrosis
on the development of femoral head necrosis following
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Suppl 3: C33–40.
Dimon JH III, Hughston JC. Unstable Intertrochanteric
Fractures of the Hip. J Bone Joint Surg 1967; 49A: 440–
50.
Garden RS. Low angle fixation in fractures of the femoral
neck. J Bone Joint Surg 1961; 43B: 647–63.
Harper WM, Barnes MR, Gregg PJ. Femoral head blood
flow in femoral neck fractures. An analysis using intra-
(a) (b) osseous pressure measurement. J Bone Joint Surg 1991;
73B: 73–5.
29.35 Fracture-separation of the epiphysis These
fractures are not difficult to reduce and can usually be held Hughes LO, Beaty JH. Fractures of the head and neck of
adequately in plaster, but they must be watched carefully the femur in children. J Bone Joint Surg 1994; 76A: 283–
872 for several weeks. 92.
Kaplan K, Miyamoto R, Levine BR, et al. Surgical Man- vance of biochemical markers. J Trauma 2001; 50(6): 29
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parison of reduction and fixation, bipolar hemiarthro- fractures: a systematic review of 2422 cases. J Orthop

plasty, and total hip arthroplasty. Treatment of displaced Trauma 2006; 20(9): 648–54.
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Joint Surg 2006; 88A: 249–60. lowing traumatic hip dislocation. Clin Orthop Relat Res
Kyle RF. Fractures of the Proximal Part of the Femur. J Bone 1979; 140: 255–61.
Joint Surg 1994; 76A: 924–50. Thompson VP, Epstein VP. Traumatic dislocation of the
Masson M, Parker MJ, Fleischer S. Internal fixation versus hip. J Bone Joint Surg 1951; 33A: 746–78.
arthroplasty for intracapsular proximal femoral fractures Tornetta P III, Mostafavi HR. Hip Dislocation: Current Treat-
in adults. Cochrane Database of Systematic Reviews 2003; ment Regimens. J Am Acad Orthop Surg 1997; 5(1): 27–36.
(2): CD001708. Winquist RA, Hansen ST Jnr, Clawson DK. Closed
Pape HC, van Griensven M, Rice J, et al. Major second- intramedullary nailing of femoral fractures. A report of
ary surgery in blunt trauma patients and perioperative five hundred and twenty cases. J Bone Joint Surg 1984;
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873
Injuries of the knee
and leg 30
Selvadurai Nayagam
ACUTE KNEE LIGAMENT INJURIES

Posterior
The bony structure of the knee joint is inherently
oblique
unstable; were it not for the strong capsule, intra- and ligament
extra-articular ligaments and controlling muscles, the including the
knee would not be able to function effectively as a superficial arm
mechanism for support, balance and thrust.
Valgus stresses are resisted by the superficial and
deep layers of the medial collateral ligament (MCL),
semimembranosus tendon and its expansions, the Superficial Semimembranosus
medial including
tough posteromedial part of the capsule (referred to
collateral capsular,
as the posterior oblique ligament) as well as the cruci- ligament anterior and
ate ligaments (Fig. 30.1a). inferior arms
Depending on the position of the knee, some will
Gastrocnemius
act as primary and others as secondary stabilizers. At (a)
(a)
30 degrees of flexion, the MCL is the primary
stabilizer.
The main checks to varus angulation are the iliotib-
ial tract and the lateral collateral ligament (LCL).
Lateral
Structures forming the posterolateral corner of the gastrocnemius
knee also make an important contribution to stability; tendon Iliotibial
they comprise the popliteus tendon, the capsule and tract
the arcuate ligament – a condensation of fibres lying
posterior to the LCL and running from the fibula Popliteus tendon
over popliteus tendon to the posterior capsule (Fig.
30.1b). The iliotibial band and LCL are the primary
stabilizers to a varus stress between full extension and Popliteo-
30 degrees of flexion; however, as flexion increases, fibular
the LCL relaxes and the posterolateral structures ligament
come into play to provide additional stability.
The cruciate ligaments provide both anteroposte- Fibular
collateral
rior and rotary stability; they also help to resist exces- ligament
sive valgus and varus angulation. Both cruciate
ligaments have a double bundle structure and some (b)
(b)
fibres of each bundle are taut in all positions of the
knee (Petersen and Zantop, 2007). The anterior cru- 30.1 Extracapsular restraints to valgus and varus
ciate has anteromedial and posterolateral bundles, stresses on the knee (a) Restraints on valgus stresses:
whereas the posterior cruciate has anterolateral and the deep and superficial parts of the medial collateral
ligament, semimembranosus and the posterior oblique
posteromedial bundles. Anterior displacement of the ligament. (b) Extracapsular restraints on varus stresses:
tibia (as in the anterior drawer test) is resisted by the lateral collateral ligament, popliteus tendon,
anteromedial bundle of the anterior cruciate ligament popliteofibular ligament and the capsule.
30 (ACL) whilst the posterolateral part tightens as the detectable when the knee is extended, then it is likely
knee extends. Posterior displacement is prevented by the expansions of the semimembranosus tendon, cap-
the posterior cruciate ligament (PCL), specifically by sule and ACL are also damaged.
the anterolateral bundle when the knee is in near 90 Forces that push the tibia into varus will damage
degree flexion and by the posteromedial bundle when the lateral structures, but these forces are relatively
the knee is straight (Fig. 30.2). uncommon; as with medial injuries, the cruciate liga-
Injuries of the knee ligaments are common, partic- ments are at risk if there is a twisting component, and
ularly in sporting pursuits but also in road accidents, a clinically detectable opening on varus stressing in an
where they may be associated with fractures or dislo- extended knee suggests that there is, in addition to a
cations. They vary in severity from a simple sprain to rupture of the LCL, capsular and cruciate damage.
complete rupture. It is important to recognize that Cruciate ligament injuries occur in isolation or in
these injuries are seldom ‘unidirectional’; they often combination with damage to other structures. The
involve more than one structure and it is therefore ACL is the more commonly affected. Solitary cruciate
useful to refer to them in functional terms (e.g. ligament injuries result in instability in the sagittal
‘anteromedial instability’) as well as anatomical terms plane, i.e. the tibia can be pushed backwards or pulled
(e.g. ‘torn MCL and ACL’). forwards in relation to the femoral condyles. If there
is accompanying damage to a collateral ligament or
the capsule, then the direction of instability is often
Mechanism of injury and pathological oblique and there may be a problem in controlling
rotation. These oblique plane and rotatory instabili-
anatomy
ties are complex; in essence, one of the cruciate liga-
Most ligament injuries occur while the knee is bent, ments is ruptured and there is also laxity in one part
i.e. when the capsule and ligaments are relaxed and of the capsule – this causes movement of the tibia on
the femur is allowed to rotate on the tibia. The dam- the femur, usually around an axis of the remaining
aging force may be a straight thrust (e.g. a dashboard intact capsule or other supporting ligament. Thus, in
injury forcing the tibia backwards) or, more com- the more common anterolateral instability, where the
monly, a combined rotation and thrust as in a football ACL, lateral capsule and LCL are injured, the lateral
tackle. The medial structures are most often affected plateau of the tibia can be made to sublux anteriorly
but if the injury involves a twist in addition to a val- when the tibia is rotated internally. If this is done with
gus force, the ACL also may be damaged. This twist- the knee fully extended whilst maintaining a valgus
ing force in a weightbearing knee often tears the force, and the knee is then gradually flexed, a palpable
medial meniscus, causing the well-recognized triad of reduction of this subluxation is felt at 20–30 degrees.
MCL, ACL and medial meniscal injury described by This is the basis of the pivot shift test; it is thought the
O’Donoghue. A solitary MCL injury, if sufficiently tibia rotates around the axis of an intact MCL.
severe, can be shown to cause the knee joint to ‘open’ The common rotational instability patterns are
on the medial side when the joint is flexed to 30 summarized in Table 30.1, showing the likely liga-
degrees a valgus stress is applied, but if this is still ments involved and the clinical tests for assessment.
PL
AM
AL
PM
(a) (b)
30.2 Dual-bundle structure of the anterior and posterior cruciate ligaments (a) The anteromedial (AM) bundle of
an anterior cruciate ligament is taut in 90° of knee flexion whereas the posterolateral (PL) bundle tightens in extension.
(b) In contrast, it is the anterolateral (AL) bundle of the posterior cruciate ligament that is tight in 90° flexion and the
876 posteromedial (PM) bundle tightens in extension (and therefore resists hyperextension).
Table 30.1 Rotational instabilities of the knee
30
Type of instability Test Positive Probable
result structures
damaged
Anterolateral rotatory Perform an anterior The tibia subluxes ACL
instability drawer test but with forward to an equal LCL
Injuries of the knee and leg

the foot internally or greater extent as Lateral aspect of
rotated 30 degrees when the foot is in a knee capsule
neutral position
Perform the pivot The tibia is subluxed
shift manoeuvre when the knee is
extended and felt to
reduce as it is gradually
flexed
Anteromedial rotatory Perform an anterior The tibia subluxes forward ACL

instability drawer test but with MCL
the foot externally Posteromedial aspect of knee
rotated 15 degrees capsule (including the posterior
oblique ligament and expansions
of semimembranosus
Posterolateral rotatory Perform a reverse The tibia subluxes PCL

instability (external rotation) pivot posteriorly in the extended Popliteus tendon
shift manoeuvre knee but is felt to reduce Arcuate ligament
as flexion is gradually
increased
Pick up the foot by The knee hyperextends and
grasping the medial forefoot tibia externally rotates. The
tibia appears to be in varus
ACL, anterior cruciate ligament; LCL, lateral collateral ligament, MCL, medial collateral ligament; PCL, posterior cruciate ligament.
Clinical features but the sharply defined tender spot of a partial tear
(usually medial and 2.5 cm above the joint line) con-
The patient gives a history of a twisting or wrenching trasts with the diffuse tenderness of a complete one.
injury and may even claim to have heard a ‘pop’ as the The entire limb should be examined for other injuries
tissues snapped. The knee is painful and (usually) and for vascular or nerve damage.
swollen – and, in contrast to meniscal injury, the The most important aspect of the examination is to
swelling appears almost immediately. Tenderness is test for joint stability. Partial tears permit no abnormal
most acute over the torn ligament, and stressing one movement, but the attempt causes pain. Complete
or other side of the joint may produce excruciating tears permit abnormal movement, which sometimes is
pain. The knee may be too painful to permit deep almost painless. To distinguish between the two is
palpation or much movement. critical because their treatment is different; if there is
For all the apparent consistency, the findings can be doubt, examination under anaesthesia is mandatory.
somewhat perverse: thus, with a complete tear the Sideways tilting (varus/valgus) is examined, first
patient may have little or no pain, whereas with a par- with the knee at 30 degree of flexion and then with
tial tear the knee is painful. Swelling also is worse with the knee straight. Movement is compared with the
partial tears, because haemorrhage remains confined normal side. If the knee angulates only in slight flex-
within the joint; with complete tears the ruptured ion, there is probably an isolated tear of the collateral
capsule permits leakage and diffusion. With a partial ligaments; if it angulates in full extension, there is
tear attempted movement is always painful; the abnor- almost certainly rupture of the capsule and cruciate
mal movement of a complete tear is often painless or ligaments as well.
prevented by spasm. Anteroposterior stability is assessed first by placing
Abrasions suggest the site of impact, but bruising is the knees at 90 degrees with the feet resting on the
more important and indicates the site of damage. The couch and looking from the side for posterior sag of
doughy feel of a haemarthrosis distinguishes ligament the proximal tibia; when present, this is a reliable sign
injuries from the fluctuant feel of the synovial effusion of posterior cruciate damage. Next, the drawer test is
of a meniscus injury. Tenderness localizes the lesion, carried out in the usual way; a positive drawer sign is 877
30 diagnostic of a tear, but a negative test does not healing has occurred and knee motion recovered, is
exclude one. The Lachman test is more reliable; for reconstruction of cruciate ligament tears in those
anteroposterior glide is tested with the knee flexed individuals who would benefit, and to deal with other
15–20 degrees. Rotational stability arising from acute internal injuries such as meniscal tears.
injuries can usually be tested only under anaesthesia.
Treatment
Imaging
SPRAINS AND PARTIAL TEARS
Plain x-rays may show that the ligament has avulsed a The intact fibres splint the torn ones and spontaneous
small piece of bone: healing will occur. The hazard is adhesions, so active
exercise is prescribed from the start, facilitated by
• from the medial edge of the femur by the medial
aspirating a tense effusion, applying ice-packs to the
ligament
knee and, sometimes, by injecting local anaesthetic
• from the fibula by the lateral ligament
into the tender area. Weightbearing is permitted but
• from the tip of the fibula, probably by a postero-
the knee is protected from rotational or angulatory
lateral corner injury
strain by a heavily padded bandage or a functional
• from the tibial spine by the anterior cruciate liga-
brace. A complete plaster cast is unnecessary and dis-
ment
advantageous; it inhibits movement and prevents
• from the back of the upper tibia by the posterior
weekly reassessment – an important precaution if the
cruciate
occasional error is to be avoided. With a dedicated
• from the near edge of the lateral tibial condyle by
exercise programme, the patient can usually return to
the iliotibial tract or capsule (a Segond fracture,
sports training by 6–8 weeks.
which is often associated with anterior cruciate lig-
ament and meniscal injuries).
COMPLETE TEARS
Stress films (if necessary under anaesthesia) show Isolated tears of the MCL, i.e. where the knee is stable
whether the joint hinges open on one side (Fig. 30.3). in full extension, usually heal well enough to permit
Magnetic resonance imaging (MRI) is helpful in near-normal function. Operative repair is unnecessary.
distinguishing partial from complete ligament tears. A long cast-brace is worn for 6 weeks and thereafter
This may also reveal ‘bone bruising’, a hitherto poorly graded exercises are encouraged.
recognized source of pain. Isolated tears of the LCL are rare. If the diagnosis is
certain, these can be treated conservatively as for
MCL tears. If the fibular styloid is avulsed, the injury
Arthroscopy is probably more severe and involves part of the pos-
With severe tears of the collateral ligaments and cap- terolateral capsule and arcuate complex. Examination
sule, arthroscopy should not be attempted; fluid for posterolateral instability should be done and, if
extravasation will hamper diagnosis and may compli- confirmed, these injuries may benefit from repair. In
cate further procedures. The main indication for contrast, a fibular head fracture indicates an avulsion
arthroscopy, which is usually conducted after capsular of the LCL as a solitary injury.
Isolated tears of the ACL should, in theory, be
treated by early operative reconstruction. Indeed,
such are the pressures on professional sportspersons
that this is often demanded. Operation may also be
indicated for non-professionals if the tibial spine is
avulsed; the bone fragment, with the attached ACL, is
replaced and fixed under arthroscopic control and the
knee is braced for 6 weeks. In all other cases it is more
(a) prudent to follow the conservative regime described
earlier; the cast-brace is worn only until symptoms
subside and thereafter movement and muscle-
strengthening exercises are encouraged. About half of
these patients regain sufficiently good function not to
need further treatment. The remainder complain of
varying degrees of instability; late assessment will
(b) identify those who are likely to benefit from ligament
30.3 Stress x-rays Stress films show: (a) complete tear reconstruction.
of medial ligament, left knee; (b) complete tear of lateral Isolated tears of the PCL are treated conservatively.
878 ligament. In both, the anterior cruciate also was torn. Most patients end up with little or no loss of function.
CHRONIC LIGAMENTOUS 30
INSTABILITY
Instability (‘giving way’) of the knee may be obvious

soon after the acute injury has healed, or it may only

become apparent much later. It is usually progressive
(a partial meniscectomy for a meniscal tear is likely to
make it worse and create new tears) but, except in
people engaged in strenuous sport, dancing or certain
work activities, the disability is often tolerated without
complaint. In more severe and longstanding cases,
osteoarthritis may eventually supervene.
30.4 Apley’s test The knee is flexed to 90° and rotated
while applying first a compression force and then a Functional pathology
distraction force. Pain and/or clicking on compression is
suggestive of a meniscal lesion. Unstable tibiofemoral relationships may result in
abnormal sideways tilt (varus or valgus), excessive
glide (forwards, backwards or even in an oblique
direction), unnatural rotation (internal or external),
However, some experience instability whilst walking or combinations of these.
up stairs and are sufficiently disabled to warrant late Seldom is only one ligament at fault. As described
reconstruction. at the beginning of this chapter, stability is normally
Combined injuries may result in significant loss of maintained by both primary and secondary stabilizers
function. With concurrent ACL and collateral liga- (not to mention the dynamic forces of surrounding
ment injury, reconstruction of the ACL often obviates muscles). In different positions, different structures
the need for collateral ligament treatment; however, come into play as primary stabilizers. Therefore, when
early operation carries the risk of postoperative joint testing for medial and lateral stability, valgus and
fibrosis, so it is wiser to start treatment with joint sup- varus stresses should be applied with the knee first in 30
port and physiotherapy in order to restore a good degrees of flexion and then in full extension.
range of movement before following on with ACL Abnormal translation or rotation of the tibia on the
reconstruction. A similar approach is adopted for femur is even more complex. A positive anterior
combined injuries involving the PCL, but here all drawer sign is the result of a torn ACL, but a solitary
damaged structures will need to be repaired. cruciate injury is unusual. More commonly there is
anterolateral rotatory instability where, in addition to
a torn ACL, the lateral capsule and LCL are torn or
Complications
‘stretched’. In this instance, not only will the anterior
Adhesions If the knee with a partial ligament tear is drawer test be positive, but the lateral tibial condyle
not actively exercised, torn fibres stick to intact fibres can be made to sublux forwards as the tibia rotates
and to bone. The knee ‘gives way’ with catches of pain; abnormally around an axis through the medial
localized tenderness is present and there is pain on condyles; this is the basis of the pivot shift phenomenon
medial or lateral rotation. The obvious confusion with (Galway and MacIntosh, 1980).
a torn meniscus can be resolved by the grinding test A positive posterior tibial sag and drawer sign means
(Fig. 30.4) or, better still, by MRI. Physiotherapy will that the posterior cruciate ligament is torn (Fig. 30.5).
resolve the problem caused by adhesions and rarely is Soon after injury, however, this sign is difficult to
manipulation under anaesthesia needed. elicit unless the ligaments of the arcuate complex and
popliteus also are torn. Chronic deficiency of the
Ossification in the ligament (Pellegrini–Stieda’s disease)
arcuate ligament complex causes a type of posterol-
Occasionally, an abduction injury is followed by
ateral rotatory instability that is a counterpart of the
ossification near the upper attachment of the medial
pivot shift phenomenon (Bahk and Cosgarea, 2006;
ligament. This is usually discovered as a chance finding
Ranawat et al., 2008). Complete tears of all the pos-
in x-rays of the knee and carries no prognostic
terior structures also allow the knee to hyperextend.
significance.
Instability The knee may continue to give way. The
Clinical features
instability tends to get worse and the repeated injury
predisposes to osteoarthritis. This important subject is The patient complains of a feeling of insecurity and of
discussed under a separate heading later. giving way. With collateral ligament instability the 879
30 30.5 Cruciate ligaments (a) Viewed from the
side, any backwards displacement of the upper
tibia is plainly visible and can be confirmed by
(b) pushing the tibia backwards.
(a) (b)
cause is obvious even to the patient, but with antero- for increased tilting into varus or valgus (at 0 and 30
lateral rotatory instability the symptoms are more sub- degrees knee flexion), followed by the drawer tests
tle – the knee suddenly gives way as the patient pivots and the more specific Lachman test (see later), and
on the affected side (effectively causing a pivot shift to finally to perform special tests for rotational instabil-
occur). Some patients describe this jerking sensation ity.
by grinding the knuckles of clenched fists upon each Start by watching the patient walk and noting knee
other. The explanation is that, with the knee just short posture and movement in the stance phase. Then ask
of full extension, the lateral tibial condyle slips for- the patient to stand on one leg – those with severe
ward (subluxes); then, as the knee is flexed, the ili- instabilities may not be able to achieve this task,
otibial band pulls the condyle back into the reduced whereas others who do may demonstrate the prob-
position with a ‘clunk’. For a sportsman, ‘cutting’ is lem.
particularly troublesome. Locking is not a feature of Hyperextension is tested with the patient supine
instability and always suggests an associated meniscal and the knee straight; with the patient relaxed, lift
tear. each heel in turn. Repeat the test, but this time grasp
In the less common posterior cruciate insufficiency, the medial forefoot – if the tibia sags posteriorly and
symptoms are mild unless the arcuate ligament com- externally rotates, this suggests that both posterior
plex also is torn or stretched; instability is sometimes cruciate and posterolateral capsule are torn (postero-
felt only on climbing stairs. lateral rotatory instability).
The joint looks normal apart from slight wasting; To test stability in the coronal plane, the patient’s
there is rarely tenderness but excessive movement in ankle is tucked under the examiner’s armpit whilst
one or more directions can usually be demonstrated. both hands support the knee by straddling it on either
Comparison with the normal knee is essential. A use- side (Fig. 30.6a).
ful routine is to observe gait and knee posture in The examiner is then able to control both knee flex-
standing, then to examine for hyperextension, then ion and the amount of varus or valgus thrust applied;
Quadriceps
contraction
(a)
30.6 Testing collateral ligaments (a) Side-to-side stabil-

ity of the knee can be checked by holding the foot
between the upper arm and body and moving the joint
between supporting hands. This method is useful if the leg
is large. (b) The quadriceps active test. Note the position of
the examiner’s hands in supporting the thigh and resisting
knee extension by the ankle. At 90° of knee flexion, a pos-
terior sag caused by a damaged posterior cruciate ligament
880 is corrected when the quadriceps contracts. (b)
perform the test first with the knee straight and then instability. Likewise, a positive drawer sign with the 30
flexed at 30 degrees. This manner of performing varus knee in external rotation (about 15 degrees) suggests
and valgus stressing enables even large limbs to be anteromedial rotatory instability (Slocum and Larson,
held and examined. 1968).
Next, place the knees at 90 degrees with the soles
Dial test The leg is dangled over the edge of the
of the feet flat on the couch and the heels lined up;
couch. The examiner steadies the distal femur with one

the quadriceps should be relaxed. Looking from the
hand and holds the heel firmly in the other. The knee
side, note if there is any posterior sag of the upper
is flexed at 30 degrees. External rotation is applied
tibia by checking the levels of the tibial tuberosities on
through the heel and the position of the tibial
each leg – a posterior sag is a sure sign of posterior
tuberosity is noted. If external rotation is greater by 15
cruciate laxity. Then support the patient’s thigh in this
degrees as compared to the other side, a posterolateral
position to ensure the hamstring muscles are relaxed,
corner injury is suspected. If the test is repeated with
and use the other hand to grasp the patient’s ankle
the knee flexed further to 90 degrees and the external
(Fig. 30.6b). Ask the patient to slide the foot slowly
rotation is noted to increase, a posterior cruciate injury
down the couch while resisting this movement by
is likely too (LaPrade and Wentorf, 2002).
holding on to the ankle as the quadriceps contracts,
the posterior sag is pulled up and the proximal tibia Pivot shift test The examiner supports the knee in
shifts forward. This is the quadriceps active test extension with the tibia internally rotated (the sub-
(Daniel et al., 1988). luxed position – the lateral tibial condyle is drawn in
Again with the knees flexed at 90 degrees and both front of the femoral condyle); the knee is then gradu-
feet resting on the couch (it is useful to sit across the ally flexed while a valgus stress is applied. In a positive
couch to prevent the feet sliding forward), grasp the test, as the knee reaches 20 or 30 degrees, there is a
upper tibia with both hands, and making sure the sudden jerk as the tibial condyle slips backwards and
hamstrings are relaxed, test for anterior and posterior reduces. The valgus stress compresses the lateral
laxity (the drawer sign). A more reliable test for ante- femoral condyle against the tibia and, through a jam-
rior cruciate laxity is to examine for anterior–posterior ming effect, amplifies the sudden ‘jerk’ when the
displacement with the knee flexed to 20 degrees (the condyle drops back. Another way to show this is
Lachman test). Hold the calf with one hand and the MacIntosh’s test (Fig. 30.8). A positive pivot shift test
thigh with the other, and try to displace the joint indicates anterolateral rotatory instability. A modifica-
backwards and forwards. tion of this test can be used to diagnose posterolateral
Rotational stability can be tested in several ways: rotatory instability; the tibia is held in external rota-
tion while the knee is extended and, similarly, a valgus
Modified drawer test The anterior drawer test is per- stress is applied as the knee is gradually flexed – a char-
formed with the tibia in 30 degrees of internal rota- acteristic ‘clunk’ signals the change from a subluxed
tion; if positive, it suggests anterolateral rotatory to a reduced position (the reverse pivot shift).
30.7 Tests for cruciate ligament

instability (a) Drawer test: Wth the knee
at 90° and the hamstrings relaxed, grasp
the top of the patients leg and try to shift
it forwards and backwards. (b) Note that
there is some anterior shift when the tibia
is pulled forwards (slight anterior cruciate
laxity. (c) Lachman test: This is more
sensitive than the drawer test. Note the
position of the knee and the examiner’s
hands.
(a)
(b) (c) 881

30
(a) (b)
(a) 30.9 Torn knee ligaments – MRI (a) Coronal T2-
weighted image showing a medial collateral ligament tear
with surrounding oedema and joint effusion. (b) Sagittal
T2-weighted image showing an intrasubstance tear of the
anterior cruciate ligament with a large joint effusion.
other lesions, such as meniscal tears or cartilage dam-

age, are suspected; (3) surgical treatment is antici-
pated. Partial meniscectomy and removal of loose
cartilage tags can be performed at the same time.
(b)
Treatment
Most patients with chronic instability have reasonably
good function and will not require an operation. The
first approach should always be a supervised, disci-
plined and progressively vigorous exercise programme
to strengthen the quadriceps and the hamstrings. At
the end of 6 months the patient should be re-
examined.
The indications for operation are:
1. Recurrent locking, with MRI or arthroscopic con-
(c)
firmation of a meniscal tear (arthroscopic menis-
cectomy alone may alleviate the patient’s
30.8 Cruciate ligament tears – MacIntosh’s test (a) symptoms, though this may later lead to increased
The leg is lifted with the knee straight. (b) The fibula is instability);
pushed forwards – if the anterior cruciate is torn the
lateral tibial condyle is now subluxed forwards. (c) It is 2. intolerable symptoms of giving way;
held forwards while the knee is flexed; at 30–40° the 3. suboptimal function in a sportsperson or others
condyle reduces with a jerk. This may be painful and an with similarly demanding occupations (even in
alternative method is to lift the straight leg by holding it this group, some patients will accept the use of a
with both hands just above the ankle, rotating the leg knee brace for specific activities that are known to
inwards, then flexing the knee. The jerk is often visible and
usually painless. cause trouble);
4. ligament injuries in adolescents (the long-term
effects of chronic instability in this group are
more marked).
Imaging
Partial tears of the anterior cruciate ligament are
MRI is a reliable method of diagnosing cruciate ligament more problematic and there is still much controversy
and meniscal injuries, providing almost 100 per cent about the need for surgery in these cases. The deci-
sensitivity and over 90 per cent accuracy (Fig. 30.9). sion should be based on an assessment of the patient’s
symptoms and functional capacity rather than the
appearance of the ligament. Young adults with
Arthroscopy
chronic anterior cruciate insufficiency and proven
Arthroscopy is indicated if: (1) the diagnosis, or the partial tears show diminished activity and run the risk
882 extent of the ligament injury, remains in doubt; (2) of developing secondary problems such as meniscal
lesions, cartilage damage, increasing instability and (usually from the patellar tendon or from hamstring 30
(eventually) secondary osteoarthritis. With careful fol- tendons) or by an allograft. Some surgeons advocate
low-up and reassessment, those most at risk can replicating the dual bundle arrangement of the origi-
usually be identified and advised to undergo recon- nal ligament. The ideal synthetic graft has yet to be
structive surgery. developed. Postoperative care will depend on the fix-
ation of the new ligament; in many cases a short

period of splintage can be followed by regular physio-
Operative treatment therapy to avoid joint stiffness and improve muscle
Medial collateral ligament insufficiency seldom causes control. Many patients return to sports within 6
much disability unless there is an associated anterior months.
cruciate tear. However, if valgus instability is marked, The treatment of combined injuries in which the
and particularly if it is progressive, ligament recon- PCL is involved is changing; until recently, it was
struction, by advancing the proximal or distal end of thought that most of these patients had good func-
the ligament, restoring the tension of the posterome- tion and therefore did not need reconstructive sur-
dial capsule and reinforcing the medial structures with gery. Newer studies have shown that there is an
the semimembranosus tendon, is justified. increased risk of osteoarthritis (especially of the
Isolated lateral instability is uncommon and symp- medial compartment) and this is seen as an indication
toms are rarely troublesome enough to warrant sur- for PCL reconstruction in patients who have more
gery. If operative reconstruction is attempted, it than 10–15 mm of posterior tibial translation in the
should follow the lines described earlier. drawer test. Unlike injuries involving the ACL, com-
Isolated PCL insufficiency rarely causes loss of func- bination injuries involving the PCL require all dam-
tion. Conservative treatment (mainly quadriceps aged structures to be repaired.
strengthening exercises) will usually suffice.
Isolated ACL insufficiency is uncommon and can
usually be managed by physiotherapy. Splints or
braces may be used to speed the return to weight- FRACTURED TIBIAL SPINE
bearing. Patients seeking to resume competitive sport
may need something more; reconstructive surgery Severe valgus or varus stress, or twisting injuries, may
involves replacing the torn ACL with an autologous damage the knee ligaments and fracture the tibial
graft, usually a strip of patellar tendon with bone spine. This is, in fact, a type of traction injury – the
attachments at either end or with hamstring tendons. adolescent variant of a cruciate ligament tear.
Combined injuries such as anterolateral or antero-
medial rotatory instability are the commonest reasons
for reconstructive surgery. When the ACL is damaged
together with either the medial or lateral collateral lig- The detached bone fragment may remain almost
ament, reconstruction of the ACL alone often suf- undisplaced, held in position by the soft tissues; it may
fices. The torn ACL is replaced by an autograft be partially displaced, the anterior end lifted away on
(a) (b) (c) (d)
30.10 Tibial spine fracture (a,b) This young man injured his knee while playing football; x-rays showed a large,
displaced avulsion fracture of the tibial spine. (c) An undisplaced tibial spine fracture. (d) Posterior fractures, with avulsion
of the posterior cruciate ligament, are often missed. 883
30 a posterior hinge, or it may be completely detached
DISLOCATION OF KNEE
and displaced. Because its articular surface is covered
with cartilage – invisible on x-ray – the image seen on
x-ray is smaller than the actual fragment. The knee can be dislocated only by considerable vio-
lence, as in a road accident. The cruciate ligaments
and one or both lateral ligaments are torn.
Clinical features
The patient – usually an older child or adolescent – Clinical features

presents with a swollen, immobile knee. The joint
feels tense, tender and ‘doughy’ and aspiration will Rupture of the joint capsule produces a leak of the
reveal a haemarthrosis. Examination under anaesthe- haemarthrosis, leading to severe bruising and swelling.
sia may show that extension is blocked. There may This may be the only clue on inspection, especially if the
also be associated ligament injuries; always test for dislocated joint has reduced spontaneously. Otherwise,
varus and valgus stability and cruciate laxity. the diagnosis is straightforward as there is gross defor-
mity (Fig. 30.11). The circulation in the foot must be
X-ray The fracture is not always obvious and a small examined because the popliteal artery may be torn or
posterior fracture may be missed unless the x-rays are obstructed. Repeated examination is necessary as com-
carefully examined. The fragment – often including partment syndrome is also a risk. Common peroneal
part of the intercondylar eminence – may be nerve injury occurs in nearly 20 per cent of cases; distal
undisplaced, tilted upwards or completely detached sensation and movement should be tested.
(Fig. 30.10).
X-ray In addition to the dislocation, the films
occasionally reveal a fracture of the tibial spine or
Treatment posterior part of the plateau (cruciate ligament
Under anaesthesia the joint is aspirated and gently avulsion), avulsion of the fibular styloid or avulsion of
manipulated into full extension. Often the fragment a fragment from the near the edge of the lateral tibial
falls back into position and the x-ray shows that the condyle (the Segond fracture).
fracture is reduced. As long as the knee extends fully, Arteriograpy is not essential if the clinical assess-
small amounts of fragment elevation can be accepted. ment of the circulation is normal. The ankle/brachial
If there is a block to full extension or if the bone frag- arterial pressure index (ratio of systolic pressure at the
ment remains displaced, operative reduction is essen- ankle relative to systolic pressure at the elbow) is a
tial. The fragment – often larger than suspected – is useful measure and should not be less than 0.9, but if
restored to its bed and anchored by small screws, there is any doubt an arteriogram should be obtained
taking care to avoid the physis. (Robertson et al., 2006).
After either closed or open reduction, a long plaster
cylinder is applied with the knee almost straight; it is
worn for 6 weeks and then movements are encour-
Treatment
aged. Reduction under anaesthesia is urgent; this is usually
The outcome is usually good and full movement achieved by pulling directly in the line of the leg, but
regained; there may be some residual laxity on exam- hyperextension must be avoided because of the dan-
ination, but this rarely causes symptoms. ger to the popliteal vessels. If reduction is achieved,
(a) (b) (c) (d)
884 30.11 Dislocations of the knee (a,b) Posterolateral dislocation; (c,d) anteromedial dislocation.
30.12 Knee dislocation and vascular 30
trauma (a,b) This patient was admitted
with a dislocated knee. After reduction
(c) the x-ray looked satisfactory, but the
circulation did not. (d) An arteriogram
showed vascular cut-off just above the knee;
had this not been recognized and treated,

amputation might have been necessary.
(a) (b) (c) (d)
the limb is rested on a back-splint and the circulation Stiffness Loss of movement, due to prolonged
is checked repeatedly during the 48 hours. Because of immobilization, is a common problem and may be
swelling, a plaster cylinder is dangerous. even more troublesome than instability. Even with
A vascular injury will need immediate repair and the early surgical reconstruction, normal knee function is
limb is then more conveniently splinted with an ante- elusive.
rior external fixator (Fig. 30.12). If possible, repair or
reconstruction of the capsule and collateral ligaments
should be undertaken at the same time – this may
involve simple suture or reattachment of the avulsed ACUTE INJURIES OF EXTENSOR
portions to bone – in order to enable early movement APPARATUS
of the knee with the support of a hinged knee brace.
If the direct repair is tenuous, augmentation using
Disruption of the extensor apparatus may occur: in
tendon grafts may be needed.
the quadriceps tendon, at the attachment of the
In general, early reconstruction of the torn liga-
quadriceps tendon to the proximal surface of the
ments followed by protected movement of the joint
patella, through the patella and retinacular expan-
reduces the severity of joint stiffness. The cruciate lig-
sions, at the junction of the patella and the patellar lig-
aments can be reconstructed after knee movement has
ament, in the patellar ligament or at the insertion of
recovered, usually some 6–12 months later. Pro-
the patellar ligament to the tibial tubercle. (Note: The
longed cast immobilization (usually 12 weeks) is no
patellar ligament is often called the patellar tendon).
longer recommended as it has been shown to be less
In all but direct fractures of the patella, the mecha-
good at preserving knee function.
nism of injury is the same: sudden resisted extension
of the knee or (essentially the same thing) sudden pas-
Complications sive flexion of the knee while the quadriceps is con-
tracting. The patient gives a history of stumbling on a
EARLY
stair, catching the foot while running, or kicking hard
Arterial damage Popliteal artery damage occurs in
at a muddy football.
nearly 20 per cent of patients and needs immediate
The lesion tends to occur at progressively higher
repair. Delay and an extended warm ischaemic period
levels with increasing age: adolescents suffer avulsion
can result in amputation.
fractures of the tibial tubercle; young adult sports-
Nerve injury The lateral popliteal nerve may be injured. people tear the patellar ligament, middle-aged adults
Spontaneous recovery is possible if the nerve is not fracture their patellae; and older people (as well as
completely disrupted – about 20 per cent of patients those whose tissues are weakened by chronic illness or
can be expected to improve. If nerve conduction steroid medication) suffer acute tears of the quadri-
studies or clinical examination shows no sign of ceps tendon.
recovery, a transfer of tibialis posterior tendon through
the interosseous membrane to the lateral cuneiform
may help restore ankle dorsiflexion. RUPTURE OF QUADRICEPS TENDON
LATE The patient is usually elderly, may have a history of
Joint instability Anteroposterior glide or a lateral diabetes or rheumatoid disease, or may have been
wobble often remains but, provided the quadriceps treated with corticosteroids. Occasionally acute rup-
muscle is sufficiently powerful, the disability is not ture is seen in a young athlete. The typical injury is
severe. followed by tearing pain and giving way of the knee. 885
30 There is bruising and local tenderness; sometimes a ‘Chronic’ ruptures (usually the result of delayed
gap can be felt proximal to the patella. Active knee presentations or missed diagnoses) are difficult to
extension is either impossible (suggesting a complete repair because the ends have retracted. The gap can
rupture) or weak (partial rupture). The diagnosis can often be made smaller by closing the medial and
be confirmed by MRI. lateral ends, and the remaining central gap is then
covered by a full-thickness V-flap turned down from
the proximal quadriceps tendon (Codivilla). A pull-

Treatment
out or cerclage wire protects the repair.
The results of acute repairs are good, with most
Partial tears Non-operative treatment will suffice: a
patients regaining full power, a good range of move-
plaster cylinder is applied for 6 weeks, followed by
ment and little or no extensor lag. Late repairs are less
physiotherapy that concentrates on restoring knee
predictable.
flexion and quadriceps strength.
Complete tears Early operation is needed, or else the
ruptured fibres will retract and repair will be more RUPTURE OF PATELLAR LIGAMENT
difficult. End-to-end suturing can be reinforced by
turning down a partial-thickness triangular flap of This is an uncommon injury; it is usually seen in
quadriceps tendon proximal to the repair (Scuderi). If young athletes and the tear is almost always at the
the tendon has been avulsed from the proximal pole of proximal or distal attachment of the ligament. There
the patella, it should be re-attached to a trough created may be a previous history of ‘tendinitis’ and local
at that site using pull-through sutures. Postoperatively injection of corticosteroid.
the knee is held in extension in hinged brace. Early The patient gives a history of sudden pain on forced
supervised movement through the brace is important extension of the knee, followed by bruising, swelling
to prevent adhesions; limits to the amount of flexion and tenderness at the lower edge of the patella or
can be controlled through the brace and increased as more distally.
the repair heals over the next 12 weeks (Fig. 30.13). X-rays may show a high-riding patella and a tell-tale
flake of bone torn from the proximal or distal attach-
ment of the ligament.
MRI will help to distinguish a partial from a com-
plete tear.
Treatment
ACUTE TEARS
Partial tears can be treated by applying a plaster
cylinder. Complete tears need operative repair or re-
attachment to bone. Tension on the suture line can be
lessened by inserting a temporary pull-out wire to
(a) keep the distance between the inferior pole and
attachment to the tibial tuberosity constant. Immobi-
lization in full extension may precipitate stiffness – it
is, after all, a joint injury – and it may be better to sup-
port the knee in a hinged brace with limits to the
amount of flexion permitted. This range can be grad-
ually increased after 6 weeks.
Early repair of acute ruptures gives excellent results.
Late repairs are less successful and the patient may be
left with a permanent extension lag.
LATE CASES
(b)
Late cases are difficult to manage because of proximal re-
traction of the patella. A two-stage operation may be
30.13 Repairing ruptures of the quadriceps tendon needed: first to release the contracted tissues and apply
(a) Acute ruptures can usually be sutured and reinforced
traction directly to the patella, then at a later stage to re-
with a partial-thickness flap of the quadriceps tendon
(Scuderi). When the patient presents late (b), the retracted pair the patellar ligament and reinforce it with grafts of
ends may have to be bridged by a full-thickness V-shaped tendon from gracilis or semitendinosus. Here, again, a
886 flap (Codivilla). tension-relieving pull-out wire is helpful. Postoperatively
a hinged brace is used to hold the knee in extension with hammer or by an indirect traction force that pulls the 30
supervised knee movement and limits to the amount of bone apart (and often tears the extensor expansions as
flexion until the repair is healed, usually at 12 weeks. well).
Direct injury – usually a fall onto the knee or a blow
against the dashboard of a car – causes either an undis-
FRACTURES OF TIBIAL TUBERCLE placed crack or else a comminuted (‘stellate’) fracture

without severe damage to the extensor expansions.
Fracture or avulsion of the tibial tubercle usually Indirect injury occurs, typically, when someone
occurs as a sports injury in young people. If the knee catches the foot against a solid obstacle and, to avoid
is suddenly forced into flexion while the quadriceps is falling, contracts the quadriceps muscle forcefully.
contracting, a fragment of the tubercle – or some- This is a transverse fracture with a gap between the
times the entire apophysis – may be wrenched from fragments.
the bone. The diagnosis is suggested by the history.
The area over the tubercle is swollen and tender;
active extension causes pain.
Clinical features
The lateral x-ray shows the fracture. Sometimes the Following one of the typical injuries, the knee
patella is abnormally high, having lost part of its distal becomes swollen and painful. There may be an abra-
attachment. sion or bruising over the front of the joint. The patella
An incomplete fracture can be treated by applying a is tender and sometimes a gap can be felt.
long-leg cast with the knee in extension for 6 weeks. Active knee extension should be tested. If the
Complete separation requires open reduction and fix- patient can lift the straight leg, the quadriceps mech-
ation with lag screws; a cast or hinged brace is applied anism is still intact. If this manoeuvre is too painful,
for 6 weeks. active extension can be tested with the patient lying
on his side.
Osgood–Schlatter disease Repetitive strain on the
If there is an effusion, aspiration may reveal the
patellar ligament may give rise to a painful, tender
presence of blood and fat droplets.
swelling over the tibial tubercle. The condition is fairly
common in adolescents who are keen on sport. X-ray The x-ray may show one or more fine fracture
Treatment consists of restricting sports activities until lines without displacement, multiple fracture lines with
the symptoms subside (see page 576). irregular displacement or a transverse fracture with a
gap between the fragments (Fig. 30.14). Comparative
x-rays of the opposite knee may help to distinguish
normal from abnormal appearances in undisplaced
FRACTURED PATELLA fractures.
Patellar fractures are classified as transverse, longi-
The patella is a sesamoid bone in continuity with the tudinal, polar or comminuted (stellate). Any of these
quadriceps tendon and the patellar ligament (also may be either undisplaced or displaced. Separation of
called the patellar tendon). There are additional inser- the fragments is significant if it is sufficient to create a
tions from the vastus medialis and lateralis into the step on the articular surface of the patella or, in the
medial and lateral edges of the patella. The extensor case of a transverse fracture, if the gap is more than
‘strap’ is completed by the medial and lateral extensor 3 mm wide.
retinacula (or quadriceps expansions), which bypass A fracture line running obliquely across the super-
the patella and insert into the proximal tibia. olateral corner of the patella should not be confused
The mechanical function of the patella is to hold the with the smooth, regular line of a (normal) bipartite
entire extensor ‘strap’ away from the centre of rotation patella. Check the opposite knee; bipartite patella is
of the knee, thereby lengthening the anterior lever arm often bilateral.
and increasing the efficiency of the quadriceps.
The key to the management of patellar fractures is the Treatment
state of the entire extensor mechanism. If the extensor
retinacula are intact, active knee extension is still poss- Undisplaced or minimally displaced fractures If there is a
ible, even if the patella itself is fractured. haemarthrosis it should be aspirated. The extensor
mechanism is intact and treatment is mainly protective.
A plaster cylinder holding the knee straight should be
Mechanism of injury and pathological worn for 3–4 weeks, and during this time quadriceps
anatomy exercises are to be practised every day.
The patella may be fractured, either by a direct force Comminuted (stellate) fracture The extensor expansions
that cracks the bone like a tile under the blow of a are intact and the patient may be able to lift the leg. 887
30
(a) (b) (c) (d)
30.14 Fractured patella – stellate (a,b) A fracture with little or no displacement can be treated conservatively by a
posterior slab of plaster that is removed several times a day for gentle active exercises. (c,d) With severe comminutions,
patellectomy is arguably the best treatment, although some surgeons would consider preserving as many useful fragments
as possible.
However, the undersurface of the patella is irregular the knee is regained; either may be removed every day
and there is a serious risk of damage to the to permit active knee-flexion exercises.
patellofemoral joint. For this reason some people
advocate patellectomy, whatever the degree of
displacement. To others it seems reasonable to preserve
Outcome
the patella if the fragments are not severely displaced Patients usually regain good function but, depending
(or to remove only those fragments that obviously on the severity of the injury, there is a significant inci-
distort the articular surface); a hinged brace is used in dence of late patellofemoral osteoarthritis.
extension but unlocked several times daily for exercises
to mould the fragments into position and to maintain
mobility.
DISLOCATION OF PATELLA
Displaced transverse fracture The lateral expansions are
torn and the entire extensor mechanism is disrupted.
Because the knee is normally angled in slight valgus,
Operation is essential.
there is a natural tendency for the patella to pull
Through a longitudinal incision the fracture is
towards the lateral side when the quadriceps muscle
exposed and the patella repaired by the tension-band
contracts. Lateral deviation of the patella during knee
principle. The fragments are reduced and transfixed
extension is prevented by a number of factors: the
with two stiff K-wires; flexible wire is then looped
patella is seated in the intercondylar groove, which has
tightly around the protruding K-wires and over the
a high lateral ‘embankment’; the force of extensor
front of the patella (Fig. 30.15). The tears in the
muscle contraction pulls it firmly into the groove; and
extensor expansions are then repaired. A plaster back-
the extensor retinacula and patellofemoral ligaments
slab or hinged brace is worn until active extension of
guide it centrally as it tracks along the intercondylar
runway. The most important static check-rein on the
medial side is the medial patellofemoral ligament, a
more or less distinct structure extending from the
superomedial border of the patella towards the medial
femoral condyle deep to vastus medialis (Conlan et
al., 1993). Additional restraint is provided by the
medial patellomeniscal and patellotibial ligaments and
the associated medial retinacular fibres. In the normal
knee, considerable force is required to wrench the
patella out of its track. However, if the intercondylar
groove is unusually shallow, or the patella seated
higher than usual, or the ligaments are abnormally lax,
dislocation is not that difficult.
(a) (b)
30.15 Fractured patella – transverse The separated Mechanism of injury

fragments (a) are transfixed by K-wires; (b) malleable wire
is then looped around the protruding ends of the K-wires While the knee is flexed and the quadriceps muscle
888 and tightened over the front of the patella. relaxed, the patella may be forced laterally by direct
violence; this is rare. More often traumatic dislocation MRI may reveal a soft-tissue lesion (e.g. disruption 30
is due to indirect force: sudden, severe contraction of of the medial patellofemoral ligament) as well as artic-
the quadriceps muscle while the knee is stretched in val- ular cartilage and/or bone damage.
gus and external rotation. Typically this occurs in field
sports when a runner dodges to one side. The patella
Treatment
dislocates laterally and the medial patellofemoral liga-

ment and retinacular fibres may be torn. Predisposing In most cases the patella can be pushed back into
factors are anatomical variations such as genu valgum, place without much difficulty and anaesthesia is not
tibial torsion, high-riding patella (patella alta) and a always necessary; the exception is an intra-articular
shallow intercondylar groove, as well as patellar hyper- (intercondylar) dislocation, which may need open
mobility due to generalized ligamentous laxity or lo- reduction.
calized muscle weakness. If there are no signs of soft tissue rupture – i.e.
there is minimal swelling, no bruising and little ten-
derness – cast splintage alone will usually suffice. The
Clinical features knee is aspirated and then immobilized in almost full
In a ‘first-time’ dislocation the patient may experience extension; a small pad along the lateral edge of the
a tearing sensation and a feeling that the knee has gone patella may help to keep the medial soft tissues
‘out of joint’; when running, he or she may collapse and relaxed. The cast is retained for 2 or 3 weeks and the
fall to the ground. Often the patella springs back into patient then undergoes a long period (2–3 months) of
position spontaneously; however, if it remains unre- quadriceps strengthening exercises.
duced there is an obvious (if somewhat misleading) The same approach has been advocated for more
deformity: the displaced patella, seated on the lateral severe forms of dislocation. However, if there is much
side of the knee, is not easily noticed but the uncovered bruising, swelling and tenderness medially, the
medial femoral condyle is unduly prominent and may patellofemoral ligaments and retinacular tissues are
be mistaken for the patella. Neither active nor passive probably torn and immediate operative repair will
movement is possible (Fig. 30.16). In the rare intra- reduce the likelihood of later recurrent dislocation.
articular (downward) dislocation the patella is stuck be-
tween the condyles and there is a marked prominence OPERATIVE TREATMENT
on the front of the knee. The area is approached through a medial incision. If
If the dislocation has reduced spontaneously, the the patellofemoral ligament is avulsed from the femur,
knee may be swollen and there may be bruising and it is reattached with suitable anchors. Mid-substance
tenderness on the medial side. If there is fluid in the tears of the ligaments are sutured directly. At the same
joint, aspiration may show that it is bloodstained; the time, if the lateral retinaculum is tight it is released.
presence of fat droplets suggests a concurrent osteo- Osteochondral fragments are removed – unless they
chondral fracture. are single, large and amenable to reattachment. Post-
With recurrent dislocation the symptoms and signs operatively a padded cylinder cast is applied with the
are much less marked, though still unpleasant. After knee in extension; this can be renewed when the
spontaneous reduction the knee looks normal, but the swelling has subsided. A hinged brace is substituted,
apprehension test is positive. which provides control for weightbearing and allows
knee movement. Quadriceps exercises are encour-
aged.
Imaging
Anteroposterior, lateral and tangential (‘skyline’)
Complications
x-ray views are needed. In an unreduced dislocation,
the patella is seen to be laterally displaced and tilted or Recurrent dislocation Patients treated non-operatively
rotated. In 5 per cent of cases there is an associated for a first-time dislocation have a 15–20 per cent
osteochondral fracture. chance of suffering further dislocations. This depends
30.16 Dislocation of the patella (a)

The right patella has dislocated laterally;
the flattened appearance is typical.
(b,c) Anteroposterior and lateral films of
traumatic dislocation of the patella.
(a) (b) (c) 889

30 also on whether there are other predisposing adjacent ridge on the tibial plateau. The condition is
abnormalities, and prevention consists of dealing with described in Chapter 6.
all these conditions (the subjects of recurrent
dislocation, subluxation, chronic patellar instability and
patellar mal-tracking are dealt with in Chapter 20).
TIBIAL PLATEAU FRACTURES
Mechanism of injury
OSTEOCHONDRAL INJURIES
Fractures of the tibial plateau are caused by a varus or
valgus force combined with axial loading (a pure val-
Osteochondral fractures and osteochondritis dissecans
gus force is more likely to rupture the ligaments).
are similar injuries of the articular cartilage and sub-
This is sometimes the result of a car striking a pedes-
chondral bone. The knee joint is a common site for
trian (hence the term ‘bumper fracture’); more often
both conditions. The lesion is usually located on one of
it is due to a fall from a height in which the knee is
the femoral condyles, the intercondylar groove or the
forced into valgus or varus. The tibial condyle is
medial facet of the patella, and is thought to be due to
crushed or split by the opposing femoral condyle,
the patella striking the opposed articular surface.
which remains intact.
OSTEOCHONDRAL FRACTURES Pathological anatomy

The fracture pattern and degree of displacement
The patient gives a history of patellar dislocation or a
depend on the type and direction of force as well as
blow to the front of the knee. The joint is swollen and
the quality of the bone at the upper end of the tibia.
aspiration yields blood-stained fluid mixed with fat
A useful classification is that of Schatzker (Fig. 30.17):
globules.
Standard anteroposterior and lateral x-rays seldom Type 1 – a vertical split of the lateral condyle This is a frac-
show the abnormality; if the diagnosis is suspected, ture through dense bone, usually in younger people. It
tunnel and patellar skyline views are needed, and even may be virtually undisplaced, or the condylar fragment
then the fracture may be hard to see because the dam- may be pushed inferiorly and tilted; the damaged lateral
aged area consists largely of articular cartilage. MRI or meniscus may be trapped in the crevice.
arthroscopy will be more helpful.
Type 2 – a vertical split of the lateral condyle combined with
depression of an adjacent loadbearing part of the condyle
Treatment The wedge fragment, which varies in size from a
portion of the rim to a sizeable part of the lateral
Small fragments should be removed as they may cause
condyle, is displaced laterally; the joint is widened and,
symptoms. Larger fragments, and especially those
if the fracture is not reduced, may later develop a valgus
from loadbearing areas, can be reattached with screws
deformity.
(counter-sunk or ‘headless’ small fragment screws).
Postoperatively a long-leg cast is applied for 2 weeks Type 3 – depression of the articular surface with an intact
before movement is allowed. condylar rim Unlike type 2, the split to the edge of the
Sometimes a large area of cartilage damage, or even plateau is absent. The depressed fragments may be
a crater, is discovered on the anterior intercondylar wedged firmly into the subchondral bone. The joint is
surface. In the past this was treated by trimming any usually stable and may tolerate early movement.
ragged parts and drilling through the crater to stimu-
Type 4 – fracture of the medial tibial condyle Two types of
late an inflammatory response (‘micro-fracturing’).
fracture are seen: (1) a depressed, crush fracture of
More recently, cartilage transplantation into these
osteoporotic bone in an elderly person (a low-energy
defects has shown promising results.
lesion), and (2) a high-energy fracture resulting in a
condylar split that runs obliquely from the
intercondylar eminence to the medial cortex. The
OSTEOCHONDRITIS DISSECANS momentary varus angulation may be severe enough to
cause a rupture of the lateral collateral ligament and a
Teenagers and young adults who complain of inter-
traction injury of the peroneal nerve. The severity of
mittent pain in the knee are sometimes found to have
these injuries should not be underestimated.
developed a small segment of osteochondral necrosis,
usually on the lateral aspect of the medial femoral Type 5 – fracture of both condyles Both condyles are split
condyle. This is probably a traumatic lesion, caused by but there is a column of the metaphysis wedged in
890 repetitive contact with the overlying patella or an between that remains in continuity with the tibial shaft.
Type 6 – combined condylar and subcondylar fractures 30
This is a high-energy injury that may result in severe
comminution. Unlike type 5 fractures, the tibial shaft
is effectively disconnected from the tibial condyles.
Clinical features

The knee is swollen and may be deformed. Bruising is
usually extensive and the tissues feel ‘doughy’ because
of haemarthrosis. Examining the knee may suggest
medial or lateral instability but this is usually painful
(a) (b) (c) and adds little to the x-ray diagnosis. More impor-
tantly, the leg and foot should be carefully examined
for signs of vascular or neurological injury. Traction
injury of the peroneal or tibial nerves is not uncom-
mon and it is important to establish whether this is
present at the time of admission and before operation.
Imaging
Anteroposterior, lateral and oblique x-rays will usually
show the fracture, but the amount of comminution or
plateau depression may not be appreciated without
computer tomography (CT). This provides information
on the location of the main fracture lines, the site and
size of the portion of condyle that is depressed and the
position of major parts of articular surface that have
been displaced. Software-generated re-assembly of the
axial images can provide sagittal and coronal views that
(d) (e) (f)
aid in surgical planning (Fig. 30.18). It is important not
30.17 Tibial plateau fractures (a) Type 1 – simple split to miss a posterior condylar component in high-energy
of the lateral condyle. (b) Type 2 – a split of the lateral fractures because this may require a separate postero-
condyle with a more central area of depression. (c) Type 3
medial or posterolateral exposure for internal fixation.
– depression of the lateral condyle with an intact rim.
(d) Type 4 – a fracture of the medial condyle. (e) Type 5 – With a crushed lateral condyle the medial ligament is
fractures of both condyles, but with the central portion of often intact, but with a crushed medial condyle the
the metaphysis still connected to the tibial shaft. (f) Type 6 lateral ligament is often torn.
– combined condylar and subcondylar fractures; effectively
a disconnection of the shaft from the metaphysis.
Treatment
Treatment by traction is simple and often produces a
well-functioning knee, but residual angulation is not
(a) (b) (c) (d)
30.18 Tibial plateau fractures – imaging (a) X-rays provide information about the position of the main fracture lines
and areas of articular surface depression. (b,c) CT reconstructions reveal the extent and direction of displacements, vital
information for planning the operation. (d) The postoperative x-ray shows that perfect reduction has been achieved. 891
30 uncommon (Apley, 1979). On the other hand, obses- out every day. As soon as the fracture is ‘sticky’ (usually
sional surgery to restore the shattered surface may at 3–4 weeks), the traction pin is removed, a hinged
produce a good x-ray appearance – and a stiff knee, cast-brace is applied and the patient is allowed up on
especially if the operation is followed by prolonged crutches. Full weightbearing is deferred for another 6
immobilization (Fig. 30.19). weeks.
In younger patients, and more so in those with a
Type 1 fractures Undisplaced type 1 fractures can be

central depression of more than 5 mm, open reduc-
treated conservatively. The haemarthrosis is aspirated
tion with elevation of the plateau and internal fixation
and a compression bandage is applied. The limb is
is often preferred. A midline incision offers good
rested on a continuous passive motion (CPM) machine
exposure – together with a limited transverse arthro-
and knee movements are begun. As soon as the acute
tomy beneath the lateral meniscus; the joint is seen to
pain and swelling have subsided (usually within 1
allow a check on the quality of reduction. Bone graft
week), a hinged cast-brace is fitted and the patient is
or a similar substitute is needed to support the ele-
allowed up; however, weightbearing is not allowed for
vated fragments. Small 3.5 mm screws placed in paral-
another 3 weeks. Thereafter, partial weightbearing is
lel just beneath the subchondral bone hold up the
permitted but full weightbearing is delayed until the
elevated fragments well (these are sometimes referred
fracture has healed (usually around 8 weeks).
to as ‘raft’ screws, describing the arrangement of par-
Displaced fractures should be treated by open
allel screws, Fig. 30.20).
reduction and internal fixation.
Alternatively cannulated screws can be used. The
The condylar surface is examined and trapped frag-
wedge of lateral condyle is then fixed with a buttress
ments are released or removed. The aim is for an accu-
plate – newer designs of contoured and angle-stable
rate reduction; two lag screws or a buttress plate are
plates (using screws that lock into the plate) are avail-
usually sufficient for fixation.
able but are not always necessary – and early knee
Type 2 fractures If depression is slight (less than 5 mm) movement is encouraged after surgery (Fig. 30.21). A
and the knee is not unstable, or if the patient is old and CPM machine can help with the regime of passive
frail or osteoporotic, the fracture is treated closed with exercise to complement the active work; at 2 weeks
the aim of regaining mobility and function rather than the patient is allowed up in a cast-brace, which is
anatomical restitution. After aspiration and retained until the fracture has united.
compression bandaging, skeletal traction is applied via
Type 3 fractures The principles of treatment are similar
a threaded pin passed through the tibia 7 cm below the
to those applying to type 2 fractures. However, the fact
fracture. An attempt is made to squeeze the condyle
that the lateral rim of the condyle is intact means that
into shape; the knee is then flexed and extended several
the knee is usually stable and a satisfactory outcome is
times to ‘mould’ the upper tibia on the opposing
more predictable. The depressed fragments may need
femoral condyle. The leg is cradled on pillows and,
to be elevated through a window in the metaphysis;
with 5 kg traction in place, active exercises are carried
reduction should be checked by x-ray or arthroscopy.
The elevated fragments are supported with bone grafts
and the whole segment is fixed in position with ‘raft’
screws. Postoperatively, exercises are begun as soon as
possible and the patient is allowed up in a cast-brace,
which is retained until the fracture has united.
Type 4 fracture of the medial condyle Osteoporotic crush
fractures of the medial plateau are difficult to reduce;
in the long term the patient is likely to be left with
some degree of varus deformity. The principles of
treatment are the same as for type 2 fractures of the
lateral plateau.
Medial condylar split fractures usually occur in
younger people and are caused by high-energy
trauma. The fracture itself is often more complex than
is appreciated at first sight; there may be a second,
posterior split in the coronal plane that cannot be
(a) (b) fixed through the standard anterior approach. Good
lateral x-rays or CT are needed to define the fracture
3.19 Tibial plateau fractures – fixation (a) Tomography
showed significant depression and some lateral pattern. There is often an underlying ligament injury
displacement of the lateral condyle. (b) Open reduction on the lateral side. Stable fixation of the medial side,
892 and internal fixation with a buttress plate. along the lines described for the type 2 fracture will
30

(b) (c)
30.20 Raft screws (a–c) These small 3.5 mm cortical screws are inserted
just beneath the subchondral surface and form a ‘raft’ above which the
elevated fragments of the plateau are supported. In types 2, 5 or 6
(a) injuries, they need to be supplemented by a buttress plate.
(a) (b) (c) (d) (e)
30.21 Tibial plateau fractures – fixation (a) Two or three lag screws may be sufficient for simple split fractures (type
1), though (b) a buttress plate and screws may be more secure. (c) Depression of more than 5 mm in a type 3 fracture can
be treated by elevation from below and (d) supported by bone grafts and fixation. (e) Type 2 fractures require a combina-
tion of both techniques – direct reduction, elevation of depressed areas, bone grafting and buttress plate fixation.
then allow an assessment of the ligament injury. If the supporting soft tissues, thus increasing the risk of
joint is unstable after fracture fixation, the torn struc- wound breakdown and delayed union or non-union.
tures on the lateral side may need repair. New strategies involve spanning the knee joint with
an external fixator, thereby providing provisional sta-
Types 5 and 6 fractures These are severe injuries that bility, and waiting for the soft-tissue conditions to
carry the added risk of a compartment syndrome. A improve – sometimes as long as 2–3 weeks. Then a
simple bicondylar fracture, in an elderly patient, can double incision approach (anterior and posteromedial
often be reduced by traction and the patient then usually) is made, which provides access to the main
treated as for a type 2 injury – some residual angulation fracture fragments and limits the amount of sub-
may follow (Fig. 30.22). However, it is more usual to periosteal elevation carried out if both condyles are
consider stable internal fixation and early joint approached through a single anterior incision only.
movement for these injuries, but surgery is not without Buttress plates placed in a submuscular fashion are
significant risk. The danger is that the wide exposure used (Fig. 30.23). An alternative method is to per-
necessary to gain access to both condyles may strip the form the articular reduction through a limited surgi- 893
30
(b)
(a) (c) (d)
30.22 Complex plateau fractures – non-operative treatment (a) Even in this complex bicondylar fracture,
non-operative treatment (b,c) with a low-traction pin makes early movement possible. (d) 10 days later the x-ray shows
reasonably good reduction and the functional result was excellent.
cal exposure (this can often be done percutaneously) underestimated; operative treatment should be under-
and to stabilize the metaphysis to the diaphysis using taken only if the full range of implants and the neces-
a circular external fixator (Fig. 30.24). This approach sary expertise are available.
is less risky and can produce better results (Canadian The standard approach to the lateral part of the
Orthopaedic Trauma Society, 2006). joint is through a longitudinal parapatellar incision.
The aim is to preserve the meniscus while fully expos-
Principles in reduction and fixationTraction is used to ing the fractured plateau; this is best done by entering
achieve reduction; many of the fragments that have the joint through a transverse capsular incision
soft-tissue attachments will reduce spontaneously beneath the meniscus. If exposure of the medial com-
(ligamentotaxis). This is done by applying bone partment is needed, a separate posteromedial incision
distractors across the knee joint or by traction on a and approach is made. Dividing the patellar ligament
traction table. in a Z-fashion – whilst giving good access across the
If open reduction is needed or intended, the oper- entire joint – limits the extent of knee flexion exercises
ation should be carefully planned. High-quality imag- after surgery, even if the ligament is repaired.
ing is needed to define the fracture pattern accurately. A single large fragment may be re-positioned and
The difficulty of fixing plateau fractures should not be held with lag screws and washers; a buttress plate is
(a) (b) (c)
30.23 Complex tibial plateau fractures – internal fixation Soft tissue trauma in high-energy complex fractures of the
tibial plateau usually makes it unsafe to undertake extensive open surgery early on. Provisional stabilization by a spanning
external fixator allows the swelling to reduce and the patient to rest comfortably (a). When conditions improve, and this
may take as long as 2 weeks, open surgery can be undertaken. In this example two buttress plates were used to shore up
894 the lateral and posteromedial aspects of the tibial plateau (b,c).
30.24 Complex tibial plateau fractures – 30
external fixation Rather than expose the
joint formally in order to reduce the frac-
ture, this can be done percutaneously, albeit
with x-ray control, and the articular frag-
ments held with multiple screws (a,b). The
tibial metaphysis is then held to the shaft

using a circular external fixator (c).
(a) (b) (c)
added for security. Comminuted, depressed fractures developing a stiff knee. This is prevented by avoiding
must be elevated by pushing the fragmented mass prolonged immobilization and encouraging movement
upwards from below; the osteoarticular surface is then as early as possible.
supported by packing the subchondral area with
Deformity Some residual valgus or varus deformity is
cortico-cancellous grafts (obtained from the iliac
quite common – either because the fracture was
crest) and held in place by inserting ‘raft’ screws and
incompletely reduced or because, although ade-
a suitably contoured buttress plate. Unless it is torn,
quately reduced, the fracture became re-displaced
the meniscus should be preserved and sutured back in
during treatment. Fortunately, moderate deformity is
place when the capsule is repaired.
compatible with good function, although constant
Displaced fractures with splits in both the sagittal
overloading of one compartment may predispose to
and the coronal plane may be impossible to reduce
osteoarthritis in later life.
and fix through the anterior approach; a second, pos-
teromedial or posterolateral approach is the answer. Osteoarthritis If, at the end of treatment, there is
Extensive exposure and manipulation of highly marked depression of the plateau, or deformity of the
comminuted fractures can sometimes be self- knee or ligamentous instability, secondary
defeating. These injuries may be better treated by osteoarthritis is likely to develop after 5 or 10 years.
percutaneous manipulation of the fragments (under This may eventually require reconstructive surgery.
traction) and circular-frame external fixation.
Stability is all-important; no matter which method is
used, fixation must be secure enough to permit early
joint movement. There is little point in ending up with
a pleasing x-ray and a stiff knee.
FRACTURE-SEPARATION OF
Postoperatively the limb is elevated and splinted PROXIMAL TIBIAL EPIPHYSIS
until swelling subsides; movements are begun as soon
as possible and active exercises are encouraged. The This uncommon injury is usually caused by a severe
patient is allowed up as swelling subsides, and at the hyperextension and valgus strain. The epiphysis dis-
end of 6 weeks the patient can partial weightbear with places forwards and laterally, often taking a small frag-
crutches; full weightbearing is resumed when healing ment of the metaphysis with it (a Salter–Harris type 2
is complete, usually after 12–16 weeks. injury). There is a risk of popliteal artery damage
where the vessel is stretched across the step at the
back of the tibia.
Complications
EARLY Clinical features
Compartment syndrome – With closed types 4 and 5
The knee is tensely swollen and extremely tender. If
fractures there is considerable bleeding and swelling
the epiphysis is displaced, there may be a valgus or
of the leg – and a risk of developing a compartment
hyperextension deformity. All movements are resisted.
syndrome. The leg and foot should be examined
The swelling may extend into the calf and a careful
repeatedly for signs.
watch for compartment syndrome, particularly if the
fracture was caused by hyperextension, is important.
LATE
Joint stiffness With severely comminuted fractures, and X-ray Salter–Harris type 1 and 2 injuries may be
after complex operations, there is a considerable risk of undisplaced and difficult to define on x-ray; a few small 895
30 bone fragments near the epiphysis may be the only
FRACTURE OF PROXIMAL END OF
clue. In the more serious injuries the entire upper tibial
epiphysis may be tilted forwards or sideways. The FIBULA
fracture is categorized by the direction of displacement,
so there are hyperextension, flexion, varus or valgus Fracture of the proximal end of the fibula may be
types. caused by either direct injury or an indirect twisting
injury of the lower limb. Beware: an isolated fracture

Treatment of the proximal fibula is rare; it may be merely the
most visible part of a more extensive rotational injury
Under anaesthesia, closed manipulative reduction can of the leg involving a serious fracture or ligament
usually be achieved. The direction of tilt may suggest injury of the ankle (the Maisonneuve fracture) or a
the mechanism of injury; the fragment can be reduced major disruption of the posterolateral corner of the
by gentle traction and manipulation in a direction knee. Always x-ray the ankle and check for knee stabil-
opposite to that of the fracturing force. Fixation using ity!
smooth K-wires or screws may be needed if the frac- The fracture itself is of little consequence and it
ture is unstable. Occasionally, when the entire tibial requires no treatment. However, associated injuries
epiphysis cannot be accurately reduced by closed are frequent and they may result in prolonged dis-
manipulation, it is repositioned at operation and held ability.
by a screw (Figure 30.25). The rare Salter–Harris type
3 or 4 fractures also may need open reduction and
fixation. Complications
Following reduction, whether closed or open, a Associated injuries Associated lesions, which should be
long-leg cast is applied. For the usual hyperextension looked for in every case, are: (1) the ankle injury
injury the knee is held flexed at 30 degrees; for the mentioned earlier; (2) peroneal nerve injury; (3) lateral
less common flexion and varus injuries the knee is collateral ligament injury – more usually a disruption of
kept straight. The cast is worn for 6–8 weeks, with this ligament and the posterolateral corner – especially
partial weightbearing from the outset. Knee move- if the fibula styloid is avulsed; (4) peroneal nerve
ment quickly returns when the cast is removed. entrapment – an occasional late complication. Each of
these conditions requires specific treatment.
Complications
Epiphyseal fractures in young children sometimes
result in angular deformity of the proximal tibia. This
may later require operative correction.
DISLOCATION OF PROXIMAL TIBIO-
With the higher grades of injury there is a risk of FIBULAR JOINT
complete growth arrest at the proximal tibia. If the
predicted leg length discrepancy is greater than A blow or twisting injury may cause subluxation or
2.5 cm, tibial lengthening (or epiphyseodesis of the dislocation of the proximal tibio-fibular joint. Isolated
opposite limb) may be needed. injuries are rare; they usually occur in parachuting or
30.25 Fracture-separation of proximal tibial

epiphysis (a) This hyperextension type of fracture
needs urgent reduction because the popliteal vessels are
endangered. (b) A flexion type of fracture-separation,
but essentially a Salter–Harris type 4 pattern; in this case
reduction was held with internal fixation (c).
896 (a) (b) (c)

similar activities. Occasionally the condition is habit- 1. The state of the soft tissues – The risk of 30
ual and associated with generalized ligamentous laxity. complications and the progress to fracture healing
The fibular head displaces upwards, and either are directly related to the amount and type of
anterolaterally or posteromedially. There is usually soft-tissue damage. Closed fractures are best
pain and local tenderness; the abnormal contour over described using Tscherne’s (Oestern and Tscherne,
the lateral aspect of the knee is best seen when the two 1984) method; for open injuries, Gustilo’s grading

knees are flexed to 90 degrees on the examination (Table 30.2) is more useful (Gustilo et al., 1984).
couch. Always check for peroneal nerve injury. The incidence of tissue breakdown and/or
infection ranges from 1 per cent for Gustilo type I
X-ray In the normal anteroposterior x-ray of the knee
to 30 per cent for type IIIC.
the fibular head overlaps the lateral tibial condyle; in a
2. The severity of the bone injury – High-energy
dislocation the fibular head stands clear of the tibia,
fractures are more damaging and take longer to
and in the lateral view the fibular head is displaced
heal than low-energy fractures; this is regardless of
either forwards or backwards.
whether the fracture is open or closed. Low-
Manual reduction is carried out by flexing the knee
energy breaks are typically closed or Gustilo I or
to 90 degrees (to relax the lateral collateral ligament)
II, and spiral. High-energy fractures are usually
and pressing upon the fibular head; reductions are
caused by direct trauma and tend to be open
usually stable and a plaster cylinder is applied for 4
(Gustilo III A–C), transverse or comminuted.
weeks. Recurrent subluxation may call for excision of
3. Stability of the fracture – Consider whether it will
the fibular head.
displace if weightbearing is allowed. Long oblique
fractures tend to shorten; those with a butterfly
fragment tend to angulate towards the butterfly.
FRACTURES OF TIBIA AND FIBULA Severely comminuted fractures are the least stable
of all, and the most likely to need mechanical
fixation.
Because of its subcutaneous position, the tibia is more
4. Degree of contamination – In open fractures this is
commonly fractured, and more often sustains an open
an important additional variable.
fracture, than any other long bone.
TSCHERNE’S CLASSIFICATION OF SKIN

Mechanism of injury
LESIONS IN CLOSED FRACTURES
A twisting force causes a spiral fracture of both leg
bones at different levels; an angulatory force produces IC1 No skin lesion
transverse or short oblique fractures, usually at the
IC2 No skin laceration but contusion
same level.
Indirect injury is usually low energy; with a spiral or IC3 Circumscribed degloving
long oblique fracture one of the bone fragments may
IC4 Extensive, closed degloving
pierce the skin from within.
Direct injury crushes or splits the skin over the frac- IC5 Necrosis from contusion
ture; this is usually a high-energy injury and the most
common cause is a motorcycle accident.
Clinical features
Pathological anatomy The limb should be carefully examined for signs of
The behaviour of these injuries – and therefore the soft-tissue damage: bruising, severe swelling, crushing
choice of treatment – depends on the following factors: or tenting of the skin, an open wound, circulatory
Table 30.2 Gustilo’s classification of open fractures
Grade Wound Soft-tissue injury Bone injury

I <1 cm long Minimal Simple low-energy fractures
II >1 cm long Moderate, some muscle damage Moderate comminution
IIIA Usually >1 cm long Severe deep contusion; High-energy fracture patterns;
+ compartment syndrome comminuted but soft-tissue cover possible
IIIB Usually >10 cm long Severe loss of soft-tissue cover Requires soft-tissue reconstruction for cover
IIIC Usually >10 cm long As IIIB, with need for vascular repair Requires soft-tissue reconstruction for cover
897
30 changes, weak or absent pulses, diminution or loss of fractures with minimal soft-tissue damage (including
sensation and inability to move the toes. Any defor- open injuries like Gustilo I) are likely to heal with a
mity should be noted before splinting the limb. minimum of trouble; they can be treated conserva-
Always be on the alert for signs of an impending com- tively unless there is a definite indication for surgery
partment syndrome. (see later). Fractures associated with severe soft-tissue
damage (whether open or closed) and unstable frac-
X-ray The entire length of the tibia and fibula, as well

ture patterns need much more careful attention if
as the knee and ankle joints, must be seen. The type of
complications are to be avoided.
fracture, its level and the degree of angulation and
displacement are recorded. Rotational deformity can
LOW-ENERGY FRACTURES
be gauged by comparing the width of the tibio-fibular
Most low-energy fractures, including Gustilo I
interspace above and below the fracture.
injuries after attention to the wounds, can be treated
Spiral fractures without comminution are low-
by non-operative methods.
energy injuries. Transverse, short oblique and com-
If the fracture is undisplaced or minimally displaced,
minuted fractures, especially if displaced or associated
a full-length cast from upper thigh to metatarsal necks
with a fibular fracture at a similar level, are high-
is applied with the knee slightly flexed and the ankle
energy injuries.
at a right angle (Fig. 30.26). Displacement of the
fibular fracture, unless it involves the ankle joint, is
unimportant and can be ignored.
Management
If the fracture is displaced, it is reduced under gen-
The main objectives are: (1) to limit soft-tissue dam- eral anaesthesia with x-ray control. Apposition need
age and preserve (or restore, in the case of open frac- not be complete but alignment must be near-perfect
tures) skin cover; (2) to prevent – or at least recognize (no more than 7 degrees of angulation) and rotation
– a compartment syndrome; (3) to obtain and hold absolutely perfect. A full-length cast is applied as for
fracture alignment; (4) to start early weightbearing undisplaced fractures (note, however, that if placing
(loading promotes healing); (5) to start joint move- the ankle at 0 degrees causes the fracture to displace,
ments as soon as possible. a few degrees of equinus are acceptable). The position
The first step is to gain a clear idea of the character is checked by x-ray; minor degrees of angulation can
of the injury – what some have called the ‘fracture still be corrected by making a transverse cut in the
personality’ – which is a combination of the soft tissue plaster and wedging it into a better position.
condition and fracture pattern. Uncomminuted, spiral The limb is elevated and the patient is kept under
(a) (b)
(c) (d)
30.26 Fractured tibia and fibula – closed treatment (1) Reduction is facilitated by bending the knee over the end of
the table, with the normal leg alongside for comparison (a). The surgeon holds the position while an assistant applies
plaster from the knee downwards (b). When the plaster has set, the leg is lifted and the above-knee plaster completed
(c); note that the foot is plantigrade, the knee slightly bent, and the plaster moulded round the patella. A rockered boot is
898 fitted for walking (d).
observation for 48–72 hours. If there is excessive is reduced and fixed at surgery. Indeed, many surgeons 30
swelling, the cast is split. Patients are usually allowed would hold that unstable fractures are better treated
up (and home) on the second or third day, bearing by skeletal fixation from the outset.
minimal weight with the aid of crutches. The imme-
diate application of plaster may be unwise if skin via- Closed intramedullary nailing This is the method of
bility is doubtful, in which case a few days on skeletal choice for internal fixation. The fracture is reduced

traction is useful as a preliminary measure (Fig. under x-ray control and image intensification. The
30.27). proximal end of the tibia is exposed; a guide-wire is
After 2 weeks the position is checked by x-ray. A passed down the medullary canal and the canal is
change from an above- to a below-the-knee cast is reamed. A nail of appropriate size and shape is then
possible around 4–6 weeks, when the fracture becomes introduced from the proximal end across the fracture
‘sticky’. The cast is retained (or renewed if it becomes site. Transverse locking screws are inserted at the
loose) until the fracture unites, which is around 8 proximal and distal ends (Fig. 30.28). Postoperatively,
weeks in children but seldom under 12 weeks in adults. partial weightbearing is started as soon as possible,
progressing to full weightbearing when this is
Exercise From the start, the patient is taught to comfortable.
exercise the muscles of the foot, ankle and knee. When For diaphyseal fractures, union can be expected in
he gets up, an overboot with a rocker sole is fitted and over 95 per cent of cases. However, the method is less
he is taught to walk correctly. When the plaster is suitable for fractures near the bone ends.
removed, a crepe bandage or elasticated support is
applied and the patient is told that he may either Plate fixation Plating is best for metaphyseal fractures
elevate and exercise the limb or walk correctly on it, that are unsuitable for nailing. It is also sometimes used
but he must not let it dangle idly. for unstable tibial shaft fractures in children. Previously,
the disadvantages of plate fixation included the need
Functional bracing With stable fractures the full-length
to expose the fracture site and, in so doing, stripping
cast may be changed after 4–6 weeks to a functional
the soft tissues around the fracture. This may increase
below-knee brace that is carefully moulded to bear
the risk of introducing infection and delaying union.
upon the upper tibia and patellar tendon. This liberates
Newer techniques of plating overcome these
the knee and allows full weightbearing (Sarmiento and
disadvantages. The plate is slid across the fracture
Latta, 2006). A snug fit is important and the fastening
through proximal and distal ‘access incisions’ on the
straps will need to be tightened as the swelling
anterolateral aspect of the tibia and then fixed to the
subsides.
bone only at these levels. This method of ‘submuscular’
Indications for skeletal fixation If follow-up x-rays show plating preserves the soft tissues around the fracture
unsatisfactory fracture alignment, and wedging fails to site better than conventional open plating, and
correct this, the plaster is abandoned and the fracture provides a relative stability that appears to hasten
(a)
(b) (c) (d)
30.27 Fractured tibia and fibula – closed treatment (2) (a) Skeletal traction is used to reduce overlap, and also as
provisional treatment when skin viability is doubtful. Plaster is applied 10–14 days later (b), using the technique shown in
Figure 30.26, except that the skeletal pin is retained until the plaster has set. Examples of spiral and transverse fractures
treated in this way are shown in (c) and (d). 899
30 Partial weightbearing is permitted from the start and
the external fixator can be replaced by a functional
brace once there are signs of union (although, with
modern fixators, this is usually unnecessary because
fracture loading can be controlled and adjusted in the
fixator).
HIGH-ENERGY FRACTURES
Initially, the most important consideration is the via-
bility of the damaged soft tissues and underlying
bone. Tissues around the fracture should be disturbed
as little as possible and open operations should be
avoided unless there is already an open wound.
Transverse fractures are usually stable after reduc-
tion; they can be treated ‘closed’, provided a careful
watch is kept for symptoms and signs of complications
(excessive pain, swelling, tightness or sensory
change).
Comminuted and segmental fractures, those associ-
ated with bone loss, and indeed any high-energy frac-
(a) (b) (c) ture that is inherently unstable, require early surgical
stabilization. For closed fractures, external fixation
30.28 Fractured tibia and fibula – intramedullary
nailing Closed intramedullary nailing is now the preferred
and closed nailing are equally suitable; in both cases
treatment for unstable tibial fractures. This series of x-rays the tissues around the fracture are left undisturbed
shows the fracture before (a) and after (b,c) nailing. (Fig. 30.29). For open fractures, the use of internal
Active movements and partial weightbearing were started fixation has to be accompanied by judicious and
soon after operation. expert debridement and prompt cover of the exposed
bone and implant; alternatively, external fixation can
be safer if these pre-requisites cannot be met.
union. Even so, full weightbearing will need to be
In cases of bone loss, small defects can be treated by
deferred until some callus formation is evident on x-
delayed bone grafting; larger defects will need either
ray, usually at 6–8 weeks.
bone transport or compression-distraction (acute
External fixation This is an alternative to closed nailing; shortening to close the defect, with subsequent
it avoids exposure of the fracture site and allows further lengthening at a different level) with an external
adjustments to be made if this should be needed. fixator (Chapter 12).
30.29 Fixation (a–d)

This method of fixation
offers the benefit of mul-
tilevel stability and can be
carried out with little
additional damage to the
soft tissues around the
injury.
900 (a) (b) (c) (d)

OPEN FRACTURES nization. In general the aim should be to close the 30
A suitable mantra for the treatment of open tibial frac- wound in the first 3–5 days.
tures is: It is important to stabilize the fracture. For Gustilo
I, II and IIIA injuries, locked intramedullary nailing is
• antibiotics
permissible as definitive wound cover is usually possi-
• debridement
ble at the time of debridement. For more severe

• stabilization
grades of open tibial fracture, internal fixation should
• prompt soft-tissue cover
be performed only at the time of definitive soft tissue
• rehabilitation.
cover. If this is not feasible at the time of primary
Antibiotics are started immediately. A first- or debridement, the fracture should be stabilized tem-
second-generation cephalosporin is suitable for porarily with a spanning external fixator. Exchange of
Gustilo grades I–IIIA wounds but more severe grades the fixator for an intramedullary nail can be done at
may benefit from Gram-negative cover as well (an the point when definitive soft tissue cover is carried
aminoglycoside such as gentamicin is often used). out – ideally within 5 days of the injury. Alternatively,
With an adequate debridement, the antibiotics are definitive fracture management can be carried out
continued for 24 hours in a grade 1 fracture and 72 using external fixation.
hours in more severe grades. However, the evidence Severe grades of open fractures should, whenever
for prolonged antibiotic use is lacking and, not sur- possible, be managed from the outset under the com-
prisingly, most infections from delayed closure of bined care of an orthopaedic surgeon and a plastic
open tibial wounds tend to be by nosocomial surgeon.
hospital-acquired bacteria. These can be multiresistant
organisms that are not covered by standard anti-
Postoperative management
biotics, thus good debridement of the fracture and
prompt cover remain the strongest defence against Swelling is common after tibial fractures; even after skele-
infection. tal fixation the soft tissues continue to swell for several
The wound should be photographed on first days. The limb should be elevated and frequent checks
inspection in the emergency department using a made for signs of compartment syndrome (see later).
Polaroid or digital camera, and then covered with a After intramedullary nailing of a transverse or short
sterile dressing. The photograph can then be printed oblique fracture, weightbearing can be started within
for inclusion in the patient’s case notes to serve as a a few days and increased to full weight when this is
record and prevent further disturbance to the wound. comfortable. If the fracture is comminuted or seg-
Adequate debridement is possible only if the origi- mental, meaning that almost the entire load will be
nal wound is extended. However, excise as little skin taken by the nail initially, only partial weightbearing is
as possible and discuss wound extensions with a plas- permitted until some callus is seen on x-ray.
tic surgeon, especially if there appears to be a need for With plate fixation, additional support with a cast
local or free skin or muscle flaps. Ideally the debride- may be needed if partial weightbearing is to start soon
ment should be performed jointly with the plastic sur- after surgery; otherwise weightbearing is delayed for 6
geon. All dead and foreign material is removed; this weeks. Unlike fractures treated with intramedullary
includes bone without significant soft-tissue attach- nails, callus formation is not seen as rapidly and this
ments. Tissue of doubtful viability may be left for a may give a poor signal for increasing the amount of
second look in 48 hours. The wound and fracture site weightbearing.
are then washed out with large quantities of normal Patients with fractures stabilized with external fixa-
saline. tors can usually weightbear early unless there is major
Gustilo grade I injuries can be closed primarily – bone loss. Weightbearing through the fractured tibia is
being a low-energy injury with a small wound, closure increased when callus is visible on x-ray; the fixator is
should be possible without skin tension – and the frac- later ‘dynamized’ to allow greater load transfer through
ture then treated as for closed injuries. More severe the bone and help the callus bridge to mature. This
wounds should, ideally, be closed at primary surgery does away with the need for exchanging the external
as long as the debridement has been thorough and the fixator for a functional brace. However, if the pin sites
skills of a plastic surgeon are at hand. If there is tissue are in poor condition or there is loosening of the hold
of doubtful viability that requires another look, or a on the tibia, a change to functional bracing is helpful.
local flap cover deemed to be inappropriate, a second
planned operation is needed. This allows further
Early complications
debridement and, hopefully, sufficient time to plan
cover by free tissue transfer. Temporary cover of the VASCULAR INJURY
exposed bone by using antibiotic beads sealed with an Fractures of the proximal half of the tibia may damage
impervious plastic film can help reduce bacterial colo- the popliteal artery. This is an emergency of the first 901
30 order, requiring exploration and repair. Damage to
one of the two major tibial vessels may also occur and
go unnoticed if there is no critical ischaemia.
COMPARTMENT SYNDROME
Tibial fractures – both open and closed – are among
the commonest causes of compartment syndrome in

the leg. The combination of tissue oedema and bleed-
ing (oozing) causes swelling in the muscle compart-
ments and this may precipitate ischaemia. Additional
risk factors are proximal tibial fractures, severe crush
injury, a long ischaemic period before revasculariza-
tion (in type IIIC open fractures), a long delay to
treatment, haemorrhagic shock, difficult and pro-
longed operation and a fracture fixed in distraction.
The diagnosis is usually suspected on clinical
grounds. Warning symptoms are increasing pain, a (a) (b)
feeling of tightness or ‘bursting’ in the leg and numb-
ness in the leg or foot. These complaints should
always be taken seriously and followed by careful and
repeated examination for pain provoked by muscle
stretching and loss of sensibility and/or muscle
strength.
Heightened awareness is all! The diagnosis can be
confirmed by measuring the compartment pressures
in the leg. Indeed, so important is the need for early
diagnosis that some surgeons advocate the use of
continuous compartment pressure monitoring for all
tibial fractures (McQueen et al., 1996). This deals (c)
admirably with patients who are unconscious or unco-
operative, and those with multiple injuries. It also 30.30 Compartment syndrome (a) With a fracture at
serves as an ‘early warning system’ in less problematic this level the surgeon should be constantly on the alert for
symptoms and signs of a compartment syndrome. This
cases. A split-tip 20-gauge catheter is introduced into patient was treated in plaster. Pain became intense and
the anterior compartment of the leg and the pressure when the plaster was split (which should have been done
is measured close to the level of the fracture (Heck- immediately after its application), the leg was swollen and
man et al., 1994). A differential pressure (ΔP) – the blistered (b). Tibial compartment decompression
difference between diastolic pressure and compart- (c) requires fasciotomies of all the compartments in the
leg.
ment pressure – of less than 30 mmHg (4.00 kPA) is
regarded as critical and an indication for compartment
decompression. Ideally the pressure should be meas-
cial peroneal nerve. A second, similar incision is made
ured in all four compartments but this is often imprac-
just posterior to the posteromedial border of the tibia;
tical; however, if the clinical features suggest a
the fascial covering of the superficial posterior com-
compartment syndrome and the anterior compart-
partment is split. The deep posterior compartment is
ment pressure is normal or borderline, pressures
identified just above the ankle (where its fascial cover-
should be measured in the other compartments.
ing is absent) and traced proximally; the muscle bulk of
Fasciotomy and decompression Once the diagnosis is the superficial compartment needs to be retracted
made, decompression should be carried out with the posteriorly, exposing the fascial envelope of the deep
minimum delay – and that means decompression of all posterior compartment, which is likewise split down its
four compartments at the first operation. This is best and entire length. Segmental arteries that perforate the fas-
most safely accomplished through two incisions, one cia from the posterior tibial artery should be preserved
anterolateral and one posteromedial. The anterolat- for possible use in local skin flaps (Fig. 30.31). The in-
eral incision is made about 2–3 cm lateral to the crest cisions are left open, a well-padded dressing is applied
of the tibia and extends from the level of the tibial and the leg is splinted with the ankle in the neutral
tuberosity to just above the ankle (Fig. 30.30). The fas- position. The fracture is treated as a grade III open
cia is split along the length of the anterior and lateral injury requiring a spanning external fixator and prompt
902 compartments taking care not to damage the superfi- return for wound closure or skin grafting.
30

(a) (b) (c)
30.31 Fasciotomies for compartment decompression (a) The first incision is usually anterolateral, giving access to the
anterior and lateral compartments. But this is not enough. The superficial and deep posterior compartments also must be
opened; their position is shown in (b), a cross-section of the leg. This requires a second incision (b,c), which is made a
finger’s breadth behind the posteromedial border of the tibia; care must be taken not to damage the deep perforators of
the posterior tibial artery. Note that the two incisions should be placed at least 7 cm apart so as to ensure a sufficient skin
bridge without risk of sloughing.
Outcome Compartment decompression within 6 hours deformity, apart from being unsightly, can be disabling
of the onset of symptoms (or critical pressure meas- because the knee and ankle no longer move in the same
urement) should result in full recovery. Delayed plane.
decompression carries the risk of permanent dysfunc- Angulation should be prevented at all stages; any-
tion, the extent of which varies from mild sensory and thing more than 7 degrees in either plane is unac-
motor loss to severe muscle and nerve damage, joint ceptable. Angulation in the sagittal plane, especially if
contractures and trophic changes in the foot. accompanied by a stiff equinus ankle, produces a
marked increase in sheer forces at the fracture site dur-
INFECTION ing walking; this may result in either refracture or
Open fractures are always at risk; even a small perfo- non-union.
ration should be treated with respect and debride- Varus or valgus angulation will alter the axis of
ment carried out before the wound is closed. loading through the knee or ankle, causing increased
If the diagnosis is suspected, wound swabs and blood stress in some part of the joint. This is often cited as a
samples should be taken and antibiotic treatment cause of secondary osteoarthritis; however while this
started forthwith, using a ‘best guess’ intravenous may be true for angular deformities close to the joint,
preparation; once the laboratory results are obtained, long-term studies have failed to show that it applies to
a more suitable antibiotic may be substituted. moderate deformities in the middle third of the bone.
With established infection, skeletal fixation should Rotational alignment should be near-perfect (as
not be abandoned if the system is stable; infection compared with the opposite leg). This may be difficult
control and fracture union are more likely if fixation is to achieve with closed methods, but it should be pos-
secure. However, if there is a loose implant it should sible with locked intramedullary nailing.
be removed and replaced by external fixation. Late deformity, if marked, should be corrected by
tibial osteotomy.
Late complications Delayed union High-energy fractures are slow to unite
Malunion Slight shortening (up to 1.5 cm) is usually and liable to non-union or fatigue failure if a nail has
of little consequence, but rotation and angulation been used. If there is insufficient contact at the fracture 903
30 site, either through bone loss or comminution,
FRACTURE OF TIBIA ALONE
‘prophylactic’ bone grafting as soon as the soft tissues
have healed is recommended (Watson, 1994). If there
is a failure of union to progress on x-ray by 6 months, A direct injury, such as a kick or blow with a club, may
secondary intervention should be considered. The first cause a transverse or slightly oblique fracture of the
nail is removed, the canal reamed and a larger nail re- tibia alone at the site of impact. In children, the frac-
inserted. If the fibula has united before the tibia, it ture is usually caused by an indirect injury; the fibula
should be osteotomized so as to allow better is intact or may show plastic deformation.
apposition and compression of the tibial fragments. Local bruising and swelling are usually evident, but
knee and ankle movements are possible. Transverse or
Non-union This may follow bone loss or deep slightly oblique fractures are easy to spot on x-ray
infection, but a common cause is faulty treatment. even if displacement is slight. The child with a spiral
Either the risks and consequences of delayed union fracture may be able to stand on the leg, and as the
have not been recognized, or splintage has been fracture may be almost invisible in an anteroposterior
discontinued too soon, or the patient with a recently film, the injury can be missed unless two views are
united fracture has walked with a stiff equinus ankle. obtained; a few days later an angry mother brings the
Hypertrophic non-union can be treated by intra- child back with a lump that proves to be callus!
medullary nailing (or exchange nailing) or compression
plating. Atrophic non-union needs bone grafting in
addition. If the fibula has united, a small segment Treatment
should be excised so as to permit compression of the If the fracture is displaced, reduction should be
tibial fragments. Intractable cases will respond to attempted. An above-knee plaster is applied as with a
nothing except radical Ilizarov techniques (Fig. 30.32). fracture of both bones; first a split plaster and then,
Joint stiffness Prolonged cast immobilization is liable to when swelling has subsided, a complete one. A frac-
cause stiffness of the ankle and foot, which may persist ture of the tibia alone takes just as long to unite as if
for 12 months or longer in spite of active exercises. This both bones were broken, so at least 12 weeks is
can be avoided by changing to a functional brace as needed for consolidation and sometimes much longer.
soon as it is safe to do so, usually by 4–6 weeks. The child with a spiral fracture, however, can be safely
released after 6 weeks; and with a mid-shaft transverse
Osteoporosis Osteoporosis of the distal fragment is so fracture the surgeon may (if he or she is a skilled plas-
common with all forms of treatment as to be regarded terer and reduction is perfect) replace the above-knee
as a ‘normal’ consequence of tibial fractures. Axial plaster by a short plaster gaiter.
loading of the tibia is important and weightbearing
should be re-established as soon as possible. After
prolonged external fixation, special care should be Complications
taken to prevent a distal stress fracture.
Delayed union Isolated tibial fractures, especially in the
Regional complex pain syndrome With distal third lower third, may be slow to join and the temptation is
fractures, this is not uncommon. Exercises should be to discard splintage too soon. Even slight displacement
encouraged throughout the period of treatment. The and loss of contact at the fracture level may delay
management of the established condition is discussed union, so internal fixation is often preferred as primary
in Chapter 10. treatment. This fracture also has a tendency to drift
30.32 Fractured tibia and

tibula – late complications
(a) Hypertrophic non-union:
the exuberant callus formation
and frustrated healing process
are typical. (b) Atrophic
non-union: there is very little
sign of biological activity at
the fracture site. (c) Malunion:
treated, in this case, by
gradual correction in an
Ilizarov fixator (d,e).
904 (a) (b) (c) (d) (e)

into varus in the later stages of healing; sometimes a Bahk MS, Cosgarea AJ. Physical examination and imaging 30
fibular osteotomy is needed to allow correction of the of the lateral collateral ligament and posterolateral corner
deformity at surgery. of the knee. Sports Med Arthrosc 2006; 14: 12–19.
Canadian Orthopaedic Trauma Society. Open reduction
and internal fixation compared with circular fixator appli-
cation for bicondylar tibial plateau fractures. Results of a
FRACTURE OF FIBULA ALONE

multicenter, prospective, randomized clinical trial. J Bone
Joint Surg 2006; 88A: 2613–23.
Isolated spiral fractures should be regarded with sus- Conlan T, Garth WP, Lemons JE. Evaluation of the medial
picion: they are often associated with other injuries soft-tissue restraints of the extensor mechanism of the
and it is wise to obtain x-rays of the ankle and knee. knee. J Bone Joint Surg 1993; 75A: 682–93.
A transverse or short oblique fracture may be due Daniel DM, Stone ML, Barnett P, Sachs R. Use of the
to a direct blow. There is local tenderness, but the quadriceps active test to diagnose posterior cruciate-liga-
patient is able to stand and to move the knee and ment disruption and measure posterior laxity of the knee.
ankle. Pain can usually be controlled by analgesic J Bone Joint Surg 1988; 70A: 386–91.
medication and the patient will need no more than an Galway HR, MacIntosh DL. The lateral pivot shift: a symp-
elastic bandage, from knee to toes, for 2 or 3 weeks. tom and sign of anterior cruciate ligament insufficiency.
In the occasional case where pain is more severe, a Clin Orthop Relat Res 1980; 147: 45–50.
below-knee walking cast may be necessary. Gustilo RB, Mendoza RM, Williams DN. Problems in the
Pathological fractures sometimes occur in patients management of type III (severe) open fractures: a new
with osteomyelitis or bone tumours. Treatment is that classification of type III open fractures. J Trauma 1984;
of the underlying condition. 24: 742–6.
Heckman MM, Whitesides TE Jr, Grewe SR, Rooks MD.
Compartment pressure in association with closed tibial
fractures. The relationship between tissue pressure, com-
FATIGUE FRACTURES
partment, and the distance from the site of the fracture.
J Bone Joint Surg 1994; 76A: 1285–92.
Repetitive stress may cause a fatigue fracture of the tibia
LaPrade RF, Wentorf F. Diagnosis and treatment of pos-
(usually in the upper half of the bone) or the fibula
terolateral knee injuries. Clin Orthop Relat Res 2002;
(most often in the lower third). This injury is seen in
402: 110–21.
army recruits, mountaineers, runners and ballet
McQueen MM, Christie J, Court-Brown CM. Acute com-
dancers, who complain of pain in the leg. There is
partment syndrome in tibial diaphyseal fractures. J Bone
local tenderness and slight swelling. The condition
Joint Surg 1996; 78B: 95–8.
may be mistaken for a chronic compartment syndrome.
O’Donoghue D. Surgical treatment of fresh injuries to the
X-ray For the first 4 weeks there may be nothing major ligaments of the knee. J Bone Joint Surg 1950;
abnormal about the x-ray, but a bone scan shows 32A: 721–38.
increased activity. After some weeks periosteal new bone Oestern H, Tscherne H. Pathophysiology and classification of
may be seen, with a small transverse defect in the cortex. soft tissue injuries associated with fractures. In: Tscherne
There is a danger that these appearances may be H, Gotzen L (Eds) Fractures with Soft Tissue Injuries.
mistaken for those of an osteosarcoma, with tragic Springer Verlag, Berlin, 1984.
consequences. If the diagnosis of stress fracture is kept Petersen WMD, Zantop TMD. Anatomy of the anterior
in mind, such mistakes are unlikely. cruciate ligament with regard to its two bundles. Clin
Ranawat A, Baker CL 3rd, Henry S, Harner CD. Postero-
Treatment lateral corner injury of the knee: evaluation and manage-
The patient is told to avoid the stressful activity. Usu- ment. J Am Acad Orthop Surg 2008; 16: 506–18.
ally after 8–10 weeks the symptoms settle down. A Robertson A, Nutton RW, Keating JF. Dislocation of the
short leg gaiter can be applied for comfort during knee. J Bone Joint Surg 2006; 88B: 706–11.
weightbearing. Sarmiento A, Latta L. The evolution of functional bracing
of fractures. J Bone Joint Surg 2006; 88B: 141–8.
Slocum DB, Larson RL. Rotatory instability of the knee: Its
REFERENCES AND FURTHER READING pathogenesis and a clinical test to demonstrate its pres-
ence. J Bone Joint Surg 1968; 50A: 211–25.
Apley AG. Fractures of the tibial plateau. Orthop Clin Watson JT. Treatment of unstable fractures of the shaft of
North Am 1979; 10: 61–74. the tibia. J Bone Joint Surg 1994; 76A: 1575–84.
905
Injuries of the ankle
and foot 31
Gavin Bowyer
In more than 75 per cent of cases it is the lateral lig-

INTRODUCTION ament complex that is injured, in particular the ante-
rior talofibular and calcaneofibular ligaments. Medial
The foot and ankle act to both support and propel the ligament injuries are usually associated with a fracture
body and are well adapted for these roles. During run- or joint injury.
ning and jumping, loads well in excess of 10 times A sudden twist of the ankle momentarily tenses the
body weight are transmitted through the ankle and structures around the joint. This may amount to no
foot. If this loading is excessive, or excessively more than a painful wrenching of the soft tissues –
repeated, it can lead to foot and ankle injuries. what is commonly called a sprained ankle. If more
The ankle is a close-fitting hinge-like joint of which severe force is applied, the ligaments may be strained
the two parts interlock like a mortise (the box formed to the point of rupture. With a partial tear, most of
by the distal ends of the tibia and fibula) and tenon the ligament remains intact and, once it has healed, it
(the upward projecting talus). The mortise bones are is able to support the weight of the body. With a com-
held together as a syndesmosis by the distal (inferior) plete tear, the ligament may still heal but it never
tibiofibular and interosseous ligaments, and the talus regains its original form and the joint will probably be
is prevented from slipping out of the mortise by the unstable.
medial and lateral collateral ligaments and joint cap-
sule. The peroneal tendons provide additional stabil-
ity. Functional anatomy
The ankle moves only in one plane (flexion/
extension), but with a complex axis of rotation, actually The lateral collateral ligaments consist of the anterior
rolling forward as the talus goes into plantar flexion; talofibular, the posterior talofibular and (between
sideways movement is prevented by the malleolar but- them) the calcaneofibular ligaments. The anterior
tresses and the collateral ligaments, but the bony con- talofibular ligament (ATFL) runs almost horizontally
straint lessens as the ankle flexes. If the talus is forced to from the anterior edge of the lateral malleolus to the
tilt or rotate, something must give: the ligaments, the neck of the talus; it is relaxed in dorsiflexion and tense
malleoli or both. Movements of the talus into internal in plantarflexion. In plantarflexion the ligament essen-
or external rotation come about from a rotatory force tially changes its orientation from horizontal with
upon the foot, or more commonly inversion/supination respect to the floor, to almost vertical. Thus the liga-
of the foot, which, through the orientation of the sub- ment at greatest stretch, and most vulnerable, with
talar joint, causes external rotation of the talus. When- the foot plantar-flexed is the ATFL – hence the
ever a fracture of the malleolus is seen, it is important propensity for ATFL injury with the plantar-flexed,
to ask about the associated ligament injury. inverting, foot (down a pot-hole, off a kerb, etc). The
calcaneofibular ligament stretches from the tip of the
lateral malleolus to the posterolateral part of the cal-
caneum, thus it helps also to stabilize the subtalar
ANKLE LIGAMENT INJURIES joint. Maximum tension is produced by inversion and
dorsiflexion of the ankle. The posterior talofibular lig-
Ankle sprains are the most common of all sports- ament runs from the posterior border of the lateral
related injuries, accounting for over 25 per cent of malleolus to the posterior part of the talus.
cases. They are probably even more common in The medial collateral (deltoid) ligament consists of
pedestrians and country walkers who stumble on stair- superficial and deep portions. The superficial fibres
ways, pavements and potholes. spread like a fan from the medial malleolus as far
31 31.1 Ankle ligament injuries
(a) Schematic diagram showing the
mortise-and-tenon articulation and
Anterior
Posterior talofibular
main ligaments of the ankle.
talofibular (b) The three components of the
lateral collateral ligament. (c) The
commonest injury is a partial tear of
one or other component of the

lateral ligament. Following a com-
Calcaneofibular plete tear, the talus may be
(a) (b) (c) displaced in the ankle mortise; the
tibiofibular ligament may have
ruptured as well, shown here in
somewhat exaggerated form.
(d) Stress x-ray showing talar tilt.
(e,f) X-rays demonstrating
anteroposterior instability. Pulling
the foot forward under the tibia
causes the talus to shift appreciably
at the ankle joint; this is usually
seen after recurrent sprains.
(d) (e) (f)
anteriorly as the navicular and inferiorly to the calca- to a fracture. If the patient is able to walk, and bruis-
neum and talus. Its chief function is to resist eversion ing is only faint and slow to appear, it is probably a
of the hindfoot. The deep portion is intra-articular, sprain; if bruising is marked and the patient unable to
running directly from the medial malleolus to the put any weight on the foot, this suggests a more
medial surface of the talus. Its principal effect is to severe injury. Tenderness is maximal just distal and
prevent external rotation of the talus. The combined slightly anterior to the lateral malleolus. The slightest
action of restraining eversion and external rotation attempt at passive inversion of the ankle is extremely
makes the deltoid ligament the major stabilizer of the painful. It is impossible to test for abnormal mobility
ankle. without using local or general anaesthesia.
The distal tibiofibular joint is held by four ligaments: With all ankle injuries it is essential to examine the
anterior, posterior, inferior transverse and the entire leg and foot; undisplaced fractures of the fibula
interosseous ‘ligament’, which is really a thickened part or the tarsal bones, or even the fifth metatarsal bone
of the interosseous membrane. This strong ligament are easily missed and injuries of the distal tibiofibular
complex still permits some movement at the tibio- joint and the peroneal tendon sheath cause features
fibular joint during flexion and extension of the ankle. that mimic those of a lateral ligament strain.
Pathology
Imaging
The common ‘twisted ankle’ is due to unbalanced
loading with the ankle inverted and plantarflexed. About 15 per cent of ankle sprains reaching the Emer-
First the anterior talofibular and then the calcane- gency Department are associated with an ankle frac-
ofibular ligament is strained; sometimes the talocal- ture. This complication can be excluded by obtaining
caneal ligaments also are injured. If fibres are torn an x-ray, but there are doubts as to whether all
there is bleeding into the soft tissues. The tip of the patients with ankle injuries should be subjected to
malleolus may be avulsed and in some cases the per- x-ray examination. Almost 2 decades ago The Ottawa
oneal tendons are injured. There may be a small frac- Ankle Rules were developed to assist in making this
ture of an adjacent tarsal bone or (on the lateral side) decision. X-ray examination is called for if there is:
the base of the fifth metatarsal. (1) pain around the malleolus; (2) inability to take
weight on the ankle immediately after the injury;
(3) inability to take four steps in the Emergency
Department; (4) bone tenderness at the posterior
ACUTE INJURY OF LATERAL LIGAMENTS edge or tip of the medial or lateral malleolus or the
base of the fifth metatarsal bone.
Clinical features If x-ray examination is considered necessary, antero-
A history of a twisting injury followed by pain and posterior, lateral and ‘mortise’ (30-degree oblique)
908 swelling could suggest anything from a minor sprain views of the ankle should be obtained. Localized soft
tissue swelling and, in some cases, a small avulsion effective in many cases, allowing a return to full func- 31
fracture of the tip of the lateral malleolus or the an- tion and sports.
terolateral surface of the talus may be the only corrob-
orative signs of a lateral ligament injury. However, it is
important to exclude other injuries, such as an undis- RECURRENT LATERAL INSTABILITY
placed fibular fracture or diastasis of the tibiofibular syn-
Injuries of the ankle and foot

desmosis. If tenderness extends onto the foot, or if Recurrent sprains are potentially associated with
swelling is so severe that the area cannot be properly ex- added cartilage damage, and warrant careful investi-
amined, additional x-rays of the foot are essential. gation by MRI, arthroscopy and examination under
Persistent inability to weightbear over 1 week or anaesthesia.
longer should call for re-examination and review of all
the initial ‘negative’ x-rays. For patients who have had
Clinical features
persistent pain, swelling, instability and impaired
function over 6 weeks or longer, despite appropriate The patient gives a history of a ‘sprained ankle’ that
early treatment, magnetic resonance imaging (MRI) never quite seems to recover and is followed by recur-
or computed tomography (CT) will be required to rent ‘giving way’ or a feeling of instability when walk-
assess the extent of soft tissue injury or subtle bony ing on uneven surfaces. This is said to occur in about
changes. 20 per cent of cases after acute lateral collateral liga-
ment tears (Colville, 1994).
The ankle looks normal and passive movements are
Treatment full, however stress tests for abnormal lateral ligament
Initial treatment consists of rest, ice, compression and laxity may show either excessive talar tilting in the
elevation (RICE), which is continued for 1–3 weeks sagittal plane or anterior displacement (an anterior
depending on the severity of the injury and the drawer sign) in the coronal plane. In the chronic
response to treatment. Cold compresses should be phase these tests are painless and can be performed
applied for about 20 minutes every 2 hours, and after either manually or with the use of special mechanical
any activity that exacerbates the symptoms. stress devices. Both ankles are tested, so as to allow
More recently the acronym has been extended to comparison of the abnormal with the normal side.
‘PRICE’ by adding protection (crutches, splint or
Talar tilt test With the ankle held in the neutral position,
brace) and still further to ‘PRICER’, adding rehabili-
the examiner stabilizes the tibia by grasping the leg
tation (supported return to function). The principles
with one hand above the ankle; the other hand is then
remain the same – a phased approach, to support the
used to force the heel into maximum inversion. The
injured part during the first few weeks and then allow
range of movement can be estimated clinically and com-
early mobilization and a supported return to function.
pared with that of the normal ankle. The exact degree
An advice leaflet for patients is probably helpful.
of talar tilt can also be measured by x-rays, which should
The use of non-steroidal anti-inflammatory drugs
be taken with the ankles in 30 degrees of internal rota-
(NSAIDs) in the acute phase can be helpful, with the
tion (mortise views); 15 degrees of talar tilt (or 5
usual contraindications and caveats. There is evidence
degrees more than in the normal ankle) is regarded as
that in acute injuries topical non-steroidal anti-inflam-
abnormal. Inversion laxity suggests injury to both the
matory (NSAI) gels or creams might be as beneficial
calcaneofibular and anterior talofibular ligaments.
as oral preparations, probably with a better risk pro-
file. Anterior drawer test The patient should be sitting with
Functional treatment, i.e. ‘protected mobilization’, the knee flexed to 90 degrees and the ankle in 10
leads to earlier recovery of all grades of injury – with- degrees of plantarflexion. The lower leg is stabilized
out jeopardizing stability – than either rigid immobi- with one hand while the other hand forces the patient’s
lization or early operative treatment. heel forward under the tibia. In a positive test the talus
can be felt sliding forwards and backwards. The
OPERATIVE TREATMENT position of the talus is verified by lateral x-rays; anterior
If the ankle does not start to settle within 1 or 2 weeks displacement of 10 mm (or 5 mm more than on the
of starting RICE, further review and investigation are normal side) indicates abnormal laxity of the anterior
called for. Persistent problems at 12 weeks after injury, talofibular ligament. With an isolated tear of the
despite physiotherapy, may signal the need for opera- anterior talofibular ligament, the anterior drawer test
tive treatment. Residual complaints of ankle pain and may be positive in the absence of abnormal talar tilt.
stiffness, a sensation of instability or giving way and (Note: A positive anterior drawer test can sometimes
intermittent swelling are suggestive of cartilage be obtained in normal, asymptomatic individuals; the
damage or impinging scar tissue within the ankle. finding should always be considered in conjunction
Arthroscopic repair or ligament substitution is now with other symptoms and signs). 909
31 31.2 Recurrent lateral instability – special tests
(a) Anterior drawer test: When the heel is drawn
forwards under the tibia, the abnormally lax ligaments
allow the talus to displace anteriorly. (b) Talar tilt test:
Forcibly inverting the ankle causes the talus to tilt
abnormally in the mortise. For both tests comparison
with the normal side is important.
(a) (b)
Treatment debridement of the impinging tissue within the ankle

joint, followed by physiotherapy.
Recurrent ‘giving way’ can sometimes be prevented Various operations for mechanical stabilization are
by modifying shoe-wear, raising the outer side of the described; they fall mainly into two groups: (1) those
heel and extending it laterally. More effectively, the that aim to repair or tighten the ligaments, (2) those
secondary dynamic ankle stabilizers, the peronei, can that are designed to construct a ‘check-rein’ against
be strengthened and brought into play by specific the unstable movement. The Broström–Karlsson or
physiotherapy regimes. Ankle exercises to strengthen Gould operation is an example of the first type: the
the peroneal muscles are helpful, and a light brace can anterior talofibular and calcaneofibular ligaments are
be worn during stressful activities. exposed and repaired, usually by an overlapping –
If, in spite of these measures, the patient continues or ‘double-breasting’ – technique (Karlsson et al.,
to experience mechanical instability (true giving way) 1988). In the second type of operation a substitute
during everyday activities, reconstruction of the lat- ligament is constructed by using peroneus brevis to
eral ligament should be considered. More commonly act as a tenodesis and prevent sudden movements into
the persisting problem will be functional instability, in varus (Chrisman and Snook, 1969). The disadvan-
which the patient does not trust the ankle, and there tages of the non-anatomic reconstructions are that
are recurrent episodes in which the patient has rapidly they sacrifice or partially sacrifice the secondary stabi-
or suddenly to unload the ankle, probably because of lizers, the peroneal tendons.
inhibitory feedback from the injured ankle. Postoperatively the ankle is immobilized in eversion
Most patients with functional instability can be for 2 weeks; a below-knee cast is then applied for
improved and returned to sport by arthroscopic another 4 weeks, during which time the patient can
(a) (b)
(a) (b)
31.3 Recurrent lateral instability – operative treatment (a) The lax anterior talofibular and calcaneofibular ligaments
can be reinforced by a double-breasting technique (the Boström–Karlsson operation). (b) Another way of augmenting the
lateral ligament is to re-route part of the peroneus brevis tendon so that is acts as a check-rein (tenodesis) (The Chrisman
910 operation).
bear weight. Thereafter, a removable brace is worn 31
and exercises are encouraged. The brace can usually
be discarded after 3 months but it may need to be
used from time to time for sports activities.
Injuries of the ankle and foot

DELTOID LIGAMENT TEARS
Rupture of the deltoid ligament is usually associated
with either a fracture of the distal end of the fibula or
tearing of the distal tibiofibular ligaments (or both). The
effect is to destabilize the talus and allow it to move into
eversion and external rotation. The diagnosis is made by
(b)
x-ray: there is widening of the medial joint space in the
mortise view; sometimes the talus is tilted, and diasta- (a)
sis of the tibiofibular joint may be obvious.

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Apley’s

Selvadurai Nayagam BSc MChOrth

© 2010 Solomon, Warwick, Nayagam

British Library Cataloguing in Publication Data

Library of Congress Cataloging-in-Publication Data

ISBN-13 978 0 340 942 055

Commissioning Editor: Gavin Jamieson

Cover image © Linda Bucklin/stockphoto.com

Typeset in 10 on 12pt Galliard by Phoenix Photosetting, Chatham, Kent

PART 1: GENERAL ORTHOPAEDICS

PART 2: REGIONAL ORTHOPAEDICS

PART 3: FRACTURES AND JOINT INJURIES

Epilogue: Global Orthopaedics 935

Principal Authors Timothy William Roy Briggs MD(Res)

Selvadurai Nayagam BSc, MChOrth FRCSOrth

Honorary Consultant, North Bristol Trust, UK

ACA angulation correction axis CRP C-reactive protein

OPG osteoprotegerin SONK ‘spontaneous’ osteonecrosis of the

orthopaedics it is useful to ask the patient to point to

Provocative movement One of the most telling clues

(a) (b) (c)

(d) (e) (f)

(a) (b) (c) (d)

6–9 months Able to sit up

Cortical and cerebellar function 9–12 months Crawling and standing up

A staggering gait may imply an unstable knee – or a 9–18 months Walking

(a) (b) (c)

The map is not the territory

PLAIN FILM RADIOGRAPHY

THE PATIENT 1.13 The radiographic image X-ray of an anatomical

(a) (b) (c) (d) (e)

decreased; osteophytes at the joint margin.

THE JOINTS types of arthropathy. In rheumatoid arthritis and psori-

(a) (b) (c)

are deliberately blurred out. When several ‘cuts’ are

New multi-slice CT scanners provide images of MAGNETIC RESONANCE IMAGING (MRI)

1.21 MRI A case of septic arthritis of the ankle, suspected

fractures). It is also one of the only techniques to give

Gallium-67 ( 67Ga) Gallium-67 concentrates in inﬂam-

DXA Results Summary:

Region Area BMC BMD T - score PR(%) Z - score AM(%)

cussed under the relevant conditions.

Normal Clear yellow High Few – As for plasma –

Septic arthritis Purulent Low + – Glucose low +

Rheumatoid arthritis Cloudy Low ++ – – –

Gout Cloudy Normal ++ Urate – –

Pseudogout Cloudy Normal + Pyrophosphate – –

Osteoarthritis Clear yellow High Few Often+ – –

coccus (Streptococcus pyogenes) which is found in

Aetiology and pathogenesis

Acute haematogenous osteomyelitis shows a charac- Sinus

otics is essential. Surgical debridement of necrotic

This condition is no longer rare, and in some coun-

Imaging months. If the diagnosis is in doubt, an open biopsy

organisms which can be tested for antibiotic sensitiv-

42 (a) (b) (c) (d)

(a) (b) (c) (d)

is indicative of an effusion, which may be echo-free

2.9 Syphilis (a–c) Congenital

(a) (c) (d) 47