Perthes disease

By
Dr. Abdul Karim
Postgraduate Resident Orthopedic Surgery
PGMI/LGH. LAHORE; PAKISTAN.
 FIRST DESCRIBED
BY LEGG, AND
WALDENSTORM
IN 1909, AND BY
PERTHES AND
CALVE IN 1910
DEFINITION

Legg-Calvé-Perthes
disease (LCPD) is the
name given to
idiopathic
osteonecrosis of the
capital femoral
epiphysis in a child.
 Increased Joint
space
Normal Smaller head
joint Denser head
Epidemiology
 Prevalence:
Transient
synovitis
5.3% 2.8% 6.0%
SCFE 8.5%

Infection 77.4%

Perthes'
disease

Other
Epidemiology
 Race: Caucasians are affected
more frequently than persons of
other races.
 Sex: Males are affected 4-5 times
more often than females.
 Age: LCPD most commonly is seen in
persons aged 3-12 years, with a
median age of 7 years.
BLOOD SUPPLY
Causes
 Exact cause
unknown.
 Proposed
theories.
 Inherited protein C
and/or S
deficiency.
 Venous thrombosis
 Arterial occlusion
 Raised intra
osseous pressure
Causes
 Proposed theories.
 Excessive femoral antiversion.
 Synovitis.
 Generalized skeletal disorder.
 Arterial anomalies.
Causes
Pathophysiology
 The capital femoral epiphysis always is involved. In 15-
20% of patients with LCPD, involvement is bilateral.

 The blood supply to the capital femoral epiphysis is

interrupted.

 Bone infarction occurs, especially in the subchondral

cortical bone, while articular cartilage continues to
grow. (Articular cartilage grows because its nutrients
come from the synovial fluid.)

 Revascularization occurs, and new bone ossification

starts.
Pathophysiology
 At this point, a percentage of patients
develop LCPD, while other patients have
normal bone growth and development.

 LCPD is present when a subchondral

fracture occurs. This is usually the result of
normal physical activity, not direct trauma
to the area

 Changes to the epiphyseal growth plate

occur secondary to the subchondral
fracture.
Pathogenesis
 Avascular necrosis
 Temporary cessation of growth
 Revascularization from periphery
 Resumption of ossification and trauma
 Pathological fracture
 Resorption of underlying bone
 Replacement of biologically plastic
bone
 Sublaxation
 Deformity
Clinical
History: Symptoms usually have been
present for weeks.
 Hip or groin pain, which may be

referred to the thigh

 Mild or intermittent pain in anterior

thigh or knee
 Limp

 Usually no history of trauma

Clinical
Physical:
 Painful gait

 Decreased range of motion (ROM),

particularly with internal rotation and
abduction
 Atrophy of thigh muscles secondary
to disuse
 Muscle spasm

 Leg length inequality due to collapse

Clinical
 Short stature: Children with LCPD
often have delayed bone age.
 Roll test
 With patient lying in the supine position,
the examiner rolls the hip of the affected
extremity into external and internal
rotation.
 This test should invoke guarding or
spasm, especially with internal rotation.
Differentials
 Unilateral  Bilateral
 Septic hip  Hypothyroidism
 Toxic synovitis  Multiple epiphyseal
 Slipped femoral dysplasia
capital epiphysis  Spondyloepiphyseal
 Spondyloepiphyseal dysplasia
dysplasia  Sickle cell disease
 Metaphyseal
dysplasia
 Lymphoma
Workup
Lab Studies:
 CBC

 Erythrocyte sedimentation rate - May

be elevated if infection present

Workup
Imaging Studies:
 Plain x-rays of the hip are extremely useful in
establishing the diagnosis.
 Frog leg views of the affected hip are very helpful.
 Plain radiographs have a sensitivity of 97% and a
specificity of 78% in the detection of LCPD
 Multiple radiographic classification systems exist,
based on the extent of abnormality of the capital
femoral epiphysis.
 Waldenstrom, Catterall, Salter and Thompson, and
Herring are the 4 most common classification
systems.
 No agreement has been reached as to the best
classification system.
Radiographic stages
 Five radiographic stages can be seen
by plain x-ray. In sequence, they are
as follows:
Radiographic stages

1. Cessation of growth at
the capital femoral
epiphysis; smaller
femoral head epiphysis
and widening of
articular space on
affected side.
Radiographic stages

2. Subchondral
fracture; linear
radiolucency within
the femoral head
epiphysis
Radiographic stages
3. Resorption of bone
Radiographic stages
4. Re-ossification of new bone
Radiographic stages
5. Healed stage
 Catterall classification
 Catterall Group I: Involvement only of the anterior
epiphysis (therefore seen only on the frog lateral film)
 Catterall Group II: Central segment fragmentation
and collapse. However the lateral rim is intact and thus
protects the central involved area.
 Catterall Group III: The lateral head is also involved
or fragmented and only the medial portion is spared.
The loss of lateral support worsens the prognosis.
 Catterall Group IV: The entire head is involved.
 Catterall's classification has a significant inter and intra
observer error.
Catterall classification
 Groups I and II had a good
prognosis (in 90%) and required no
intervention.
 Groups III and IV had a poor
prognosis (in 90 %) and required
treatment.
 The classification is applied to the
frog lateral and AP film during the
fragmentation phase
Salter and Thompson Classification
 Salter and Thompson recognized that
Catterall's first two groups and second two
groups were distinct and therefore
proposed a two part classification.
 Salter & Thompson Group A: Less than
1/2 head involved.
 Salter & Thompson Group B: More than
1/2 head involved.
 Again the main difference between these
two groups is the integrity of the lateral
pillar.
(Herring) Lateral Pillar
Classification
 Lateral Pillar Group A: There is no loss in height of
the lateral 1/3 of the head and minimal density
change. Fragmentation occurs in the central segment
of the head.
 Lateral Pillar Group B: There is lucency and loss of
height in the lateral pillar but not more that 50% of
the original (contralateral) pillar height. there may be
some lateral extrusion of the head.
 Lateral Pillar Group C: There is greater than 50%
loss in the height of the lateral pillar. The lateral pillar
is lower than the central segment early on.
Intraobserver and interobserver reliability of
Catterall, Herring, Salter-Thompson and Stulberg
classification systems in Perthes.

 Conclusions: The results of our study

suggest the use of Catterall and Salter-
Thompson systems prior to treatment and
the Stulberg system at the end of the
treatment at skeletal maturity. However,
evaluation of the patients during the
treatment period is still a dilemma and
necessitates a new more reliable
classification system.
 Journal of Pediatric Orthopaedics B. 13(3):166-169, May 2004.
Agus, Haluk a; Kalenderer, Onder a; Eryanlmaz, Gurkan
b[latin dotless i]; Ozcalabi, Isa Turkay a
Unilateral Perthes with entire head involvement and fragmentation. The
reossification phase has not yet begun.
Unilateral Perthes disease with widening of the medial joint space, blurring of
the physis, increased density of the head and lucency between the medial and
central 1/3's of the head corresponding to early fragmentation phase.
Unilateral Perthes in the
reossification phase with
a visible subchondral line
similar to Waldenstrom's
sign. However
Waldenstrom's
subchondral fracture is
seen very early in the
disease process, before
fragmentation. In this
case the lateral pillar has
maintained some
integrity.
“Head at risk signs”
1. Gage's sign. a V shaped lucency in
the lateral epiphysis.
2. lateral calcification (lateral to the
epiphysis) (implies loss of lateral
support)
3. lateral subluxation of the
head.(implies loss of lateral support)
4. A horizontal growth plate.(implies
a growth arrest phenomenon and
deformity)
Workup
 Technetium 99 bone
scan - Helpful in
delineating the extent
of avascular changes
before they are
evident on plain
radiographs.
 The sensitivity of
radionuclide scanning
in the diagnosis of
LPD is 98%, and the
specificity is 95%.
 Dynamic arthrography
- Assesses sphericity
of the head of the
femur.
Ultrasonography in transient
synovitis and early Perthes' disease

 Ultrasonography may provide significant

diagnostic clues to differentiate early
Perthes' from transient synovitis.

T Futami, Y Kasahara, S Suzuki, S Ushikubo, and T Tsuchiya

Journal of Bone and Joint Surgery - British Volume, Vol 73-B, Issue 4, 635-
639
CT Scan
 Staging
determined by
using plain
radiographic
findings is
upgraded in 30%
of patients.
 Not as sensitive as
nuclear medicine
or MRI.
 CT may be used for
follow-up imaging
in patients with
LPD.
MRI
 It allows more precise
localization of
involvement than
conventional
radiography.
 MRI is preferred for
evaluating the
position, form, and
size of the femoral
head and surrounding
soft tissues.
 MRI is as sensitive as
isotopic bone
scanning.
Outcome variables
 Age
 Extent of involvement
 Duration
 Remodeling potential
 Premature physeal closure
 Type of treatment
 Stage of disease at treatment.
Treatment
 Goals of treatment
 Achieve and maintain ROM
 Relieve weight bearing
 Containment of the femoral epiphysis
within the confines of the acetabulum
 Traction
Rational behind "containment"
 Salter has demonstrated the" biologic
plasticity" of the femoral head in pigs
following a vascular insult.
 Containment of the head within the
acetabulum is reported to encourage
spherical remodelling during the
reossification and subsequent phases.
 However if there is total head involvement
and the lateral pillar collapses then the effect
of containment is probably less.
 Therefore it seems that the extent of
involvement of the head is the critical factor
and containment simply optimizes the
situation.
Medications
 Medical treatment does not stop or reverse
the bony changes.
 Appropriate analgesic medication should be
given.
 Nonsteroidal anti-inflammatory drugs
 Ibuprofen
 Adult dose:
 200-400 mg PO q4-6h; not to exceed 3.2 g/d.
 Pediatric dose:
 6 months to 12 years: 20-40 mg/kg/d PO divided
tid or qid; start at lower end of dosing range and
titrate upward; not to exceed 2.4 g/d
>12 years: Administer as in adults.
Non surgical containment
Scotish Rite abduction brace
Japenes modification of
petrie abduction cast
Surgical containment
Greater trochanteric
overgrowth
 The trochanteric overgrowth can be
dramatic on radiographs but several studies
have shown that a Trendelenberg gait does
not always occur.
 If it does occur, and is significant, then
trochanteric advancement may improve the
gait.
 An alternative is to perform a trochanteric
arrest at an earlier date but this assumes
that the first statement will not apply to the
particular child.
RECONSTRUCTIVE SURGERY
 INDICATIONS:
 Hinge abduction. valgus subtrochanteric

osteotomy.
 Malformed femoral head
in late group III or residual
group IV. Garceau’s cheilectomy.
 Coxa magna. shelf augmentation
 A large malformed
femoral head with lateral
sublaxation. Chiari’s pelvic
osteotomy.
 Capital femoral physeal
arrest. trochanteric
advancement or
arrest.
A STUDY AT CINCINNATI
INSTITUTE
 Hinge abduction and joint stiffness in
perthes disease:
 Effect of medial soft tissue release and
petrie casting prior to femoral head
containment.
 Hypothesis:
 Correct hinge abduction
 Improve motion
 Normalization of femoral head and acetabulum
relationships

C.T Mehlman, D.O.MPH D.R.Roy M.D A.H.Crawford M.D

CONCLUSION
 Medial capsulotomy and dynamic
positiong effectively corrects hinged
abduction.
 ROM was improved in all parameters.
 High degree of patient and parent
satisfaction.
SUMMARY
 For patients less that 6 years old the
prognosis is good for the majority.
 If they are stiff or painful they
respond to bed rest, traction and pain
relieving anti-inflammatory
medication.
 There is no evidence that abduction
splints or surgical intervention is
warranted in the majority of these
younger patients.
SUMMARY
 For patients between 6 and 8 years but
with a bone age less than 6 and an intact
lateral pillar (Herring A and B) the
prognosis is similar to that for the first
group and observation is as good as
surgical intervention for the majority.
 If they have bone ages greater than 6
years and Herring lateral pillar classification
B then "containment" of the head within
the acetabulum seems to be warranted.
 This may be done by abduction bracing,
femoral varus osteotomy or a pelvic
osteotomy.
SUMMARY
 If they are between 6 and 8 and are in
lateral pillar group C then the result of
intervention are equivocal.
 Children presenting with Perthes disease at
age 9 or older often have lateral pillar B or
C and a poor prognosis.
 The trend is towards early containment of
these hips although stiffness can be a
problem following early pelvic (Salter's)
osteotomy.
Follow-up
 Initially, close follow-up is required to
determine the extent of necrosis.
 Once the healing phase has been
entered, follow-up can be every 6
months.
 Long-term follow-up is necessary to
determine the final outcome.
Complications

Femoral Pelvic
 Shortening  Lenghtening
 stiffness  Stiffness
 Malrotation  Chondrolysis
 Limp  Failure of
containment
 Positive
trendelenburg
Prognosis
 The younger the age of onset of
LCPD, the better the prognosis.
 Children older than 10 years have a
very high risk of developing
osteoarthritis.
 Most patients have a favorable
outcome.
 Prognosis is proportional to the
degree of radiologic involvement.