5: A- developmental dysplasia of the hip

Leg length discrepancy in a child may result from fracture, dislocation, bone infection, vascular malformation, or
any process that may interfere with bone growth. It is seen most often in developmental dysplasia of the hip,
which is found in 1 in 5,000 children by the age of 18 months.

Although abnormal hip abduction on physical examination in early infancy is the usual finding associated with hip
dysplasia, older infants and children, especially those who walk, may present with waddling gait. One reason for
the change in nomenclature from "congenital" to "developmental" hip dysplasia is that a subpopulation of children
seems to present in later infancy or toddlerhood with hip dysplasia despite no findings on physical examination.
Development of the femoral head and acetabulum are intimately related, and normal adult hip joints depend on
further growth of these structures. Hip dysplasia may occur in utero, perinatally, or during infancy. Asymmetric
thigh or gluteal folds (Item C5A), better observed when the child is prone; apparent limb length discrepancy, as
described for the girl in the vignette; and restricted motion, especially abduction, are significant, albeit not
pathognomonic signs.

Developmental dysplasia usually is identified with abnormal Ortolani (Item C5B) and Barlow (Item C5C)
maneuvers in early infancy and abnormal abduction until about 3 to 4 months of age. Older infants and children
may present with waddling gaits.

Leg length discrepancy should prompt ultrasonographic evaluation (Item C5D) of the hip in the infant younger
than 12 weeks of age. Plain radiographs (Item C5E) with anteroposterior and frogleg views of the hip in the older
infant and toddler can reveal hip dysplasia, healed fractures, ossification, and other abnormalities.

Failure to identify and treat developmental dysplasia of the hip may result in degenerative changes of the hip and
arthritis.

A number of other disorders may cause abnormal gait. Rickets normally presents with bowing of the legs, along
with a "waddling" gait, and may be accompanied by other findings, such as swelling of the wrists or ankles or a
"rachitic rosary" (enlargement of the costochondral junctions). Legg-Calvé-Perthes disease (Item C5F) may cause
pain and a limp, but typically occurs in children 2 to 12 years of age (mean age, 7 years). Slipped capital femoral
epiphysis (Item C5G) is a disease of the older, heavier preadolescent and is not seen in the toddler/preschool age
group. In contrast to these conditions, femoral anteversion (internal femoral torsion) causes intoeing, not a
"waddling" gait or leg-length discrepancy. Typically, children present at 2 years of age or older and have
increased internal rotation of the hips when examined in the prone position.

112: E- measurement of the pressures in the muscle compartments of the lower leg is the diagnostic
test of choice
Worsening pain and neurovascular compromise following an extremity fracture suggest the possibility of
compartment syndrome, a potentially limb-threatening complication. Compartment syndrome results when
increased tissue pressures (typically related to muscle and soft-tissue edema) within closed osteofascial
compartments (Item C112A) cause decreased muscle perfusion and ischemia. Most commonly seen after
extremity fractures, compartment syndrome also may complicate burns, snakebite, and crush injuries. The lower
leg is affected most commonly, with tibial fractures accounting for 40% of fracture-related cases.

Compartment syndrome should be suspected in patients who have fractures and complain of worsening pain or
pain out of proportion to the injury, such as the boy described in the vignette. If compartment syndrome is
suspected, immediate surgical consultation is indicated, and compartment pressures should be measured. Normal
muscle compartment pressure is typically less than 10 to 12 mm Hg. Subtracting the measured
intracompartmental pressure from the diastolic blood pressure provides a measure of the compartmental
perfusion pressure. A compartmental perfusion pressure of 30 mm Hg or less is indicative of ischemic
compromise and should prompt consideration of surgical intervention to relieve the compartment pressure.

With the exception of pain, most of the classically described clinical features of compartment syndrome—"the 5
Ps" (pain, pallor, paresthesias, paralysis, pulselessness)—are late findings. It also is unusual for the muscle group
to feel "tight" despite rising pressures. A high index of clinical suspicion should prompt the clinician to evaluate
high-risk patients before neurovascular compromise is evident. Release of intracompartmental pressure within 6
to 12 hours of diagnosis is critical to a favorable outcome.

Initial treatment of compartment syndrome involves removal of constricting dressings, splints, or casts and
maintenance of the extremity at "heart level" to maximize perfusion and minimize further dependent edema.
Fasciotomies of the affected compartments are performed to release the intracompartmental pressure and
restore muscle perfusion. Untreated compartment syndrome results in scarring of the affected muscle, with
contracture formation and peripheral neuropathy of the local nerves leading to severe disability.

Anticoagulant therapy is indicated for treatment of deep venous thrombosis, which can cause pain in the affected
extremity but does not lead to neurologic deficits or arterial compromise, as described in the vignette.
195: C- femoral anteversion
Children who present with "intoeing" usually have one of three problems. In early infancy, the condition may
result from metatarsus adductus (Item C195A); in toddlerhood, tibial torsion may be evident; and in early
childhood, femoral anteversion is common.

The history and physical findings for the child described in the vignette are consistent with a diagnosis of femoral
anteversion (internal femoral torsion). Most children who have femoral anteversion are between 3 and 5 years of
age. To assess for femoral anteversion, the child is placed in the prone position and the femurs rotated internally
and externally with the knees flexed (Item C195B). Normally, children have 45 degrees of internal and 45
degrees of external rotation; in those who have femoral anteversion, internal rotation is increased (with a
reduction in external rotation). Of note, all infants have physiologic femoral anteversion. Children who have
femoral anteversion often sit in the "W" position with the knees flexed and legs behind them(Item C195C).
Sitting in this position may aggravate the femoral anteversion, and some orthopedic surgeons discourage it. The
natural course of femoral anteversion is spontaneous regression between the ages of 8 and 10 years.

Osgood-Schlatter disease (Item C195D) occurs in adolescents, produces pain over the tibial tuberosities, and is
not associated with intoeing. Limp may result if the patient is experiencing pain. Bowed legs, which may be
physiologic or pathologic (eg, due to Blount disease (Item C195E), do not necessarily cause intoeing.
Equinovarus deformity (Item C195F) generally is identified in infancy, presents with a limp, and typically is
unilateral. Waddling gait generally results from hip instability or proximal limb girdle muscle weakness; limp is
not a common association.

Metatarsus adductus represents an

incurving of the forefoot. Normally,
a line bisecting the heel passes between
the second and third toes (left).
In metatarsus adductus, the line bisecting
the heel passes more laterally (right).

210: B- bilateral anteroposterior and frog leg radiographs of the hips

Slipped capital femoral epiphysis (SCFE) occurs when the epiphysis of the femoral head "slips" in relation to the
shaft of the femur. SCFE occurs most commonly in obese preteens and adolescents, although 30% of affected
patients are not overweight. In its early stages, SCFE presents as pain, typically referred via the obturator nerve
to the knee or distal thigh, as reported for the boy in the vignette. Such pain can prompt mistaken imaging of the
knee. Clinically, many affected children walk with a limp or complain of pain with ambulation or sports
participation. The clinician may observe external rotation of the hip when the patient walks and pain with and
limitation of internal rotation of the hip during physical examination.

Bilateral anteroposterior and frogleg view radiographs (Item C210A) of the hips are recommended to diagnose
SCFE because the condition often is or ultimately becomes bilateral. Ultrasonography of the hip is unlikely to be
helpful in the evaluation of an adolescent who has chronic pain and limp. Although the boy described in the
vignette is complaining of knee pain, physical examination of the knee yields normal results, and he has pain with
hip motion. Thus, it is unlikely that the knee is the source of the pain, and radiographs or magnetic resonance
imaging of this area are not indicated. Ultrasonography is not a helpful study in evaluating the knee.

224: E- unicameral bone cysts occur most commonly in the humerus and femur
Two primary types of bone cysts can be seen in the pediatric population. Unicameral bone cysts, or simple bone
cysts, are most common before skeletal maturity. These fluid-filled cysts are found adjacent to the epiphyseal
plate, usually at the proximal humerus and the proximal femur (Item C224A).

Pathologic fractures are common, often after minor trauma, which is usually when they are diagnosed because
most children are asymptomatic before the trauma. Simple bone cysts typically resolve after skeletal maturity.
However, many experts advocate treating them, especially large cysts in the high-stress area of the proximal
femur, to prevent further pathologic fractures. The treatment consists of allowing the first pathologic fracture to
heal if present, followed by intralesional injection of steroids or, less commonly, autogenous bone marrow. These
lesions are not considered precancerous.

Aneurysmal bone cysts usually are seen in the teenage years. These cavitary lesions arise within the metaphysis,
and an associated periosteal reaction may be seen (Item C224B).

They occur primarily in long bones, especially the femur and tibia, but spine lesions also are common. Most
affected children complain of pain with or without swelling. The lesions can grow rapidly, and spinal lesions
(which are more common in the posterior vertebrae than in the vertebral body) can spread to adjacent vertebrae
and cause significant cord or nerve root compression. Because aneurysmal bone cysts may be associated with
underlying bone tumors, referral to orthopedics for further evaluation and management is indicated. Management
might include curettage with bone grafting.

239: C- developmental dysplasia of the hip

Developmental dysplasia of the hip (DDH) occurs when the femoral head and acetabulum have an abnormal
relationship. The spectrum of the abnormality includes frank dislocation, subluxation (partial dislocation), and
capsular instability. DDH is more common in girls, infants who have a family history of DDH, infants born in the
breech presentation, and infants born with other conditions seen with intrauterine crowding, such as congenital
torticollis and clubfeet.

The diagnosis commonly is made at birth but may be delayed until after the child is walking. A thorough
examination of the hips at birth, using the Ortolani and Barlow maneuvers, is critical for early diagnosis and
treatment. The diagnostic procedure of choice is the physical examination (Ortolani and Barlow maneuvers). If
signs are inconclusive and radiologic evaluation is considered, the best test for infants younger than 4 months of
age is ultrasonography of the hips. The entire hip, including the cartilage and acetabulum, can be seen with
ultrasonography, and hip stability can be assessed (Item C239A). Ultrasonography allows dynamic evaluation
(Item C239B) of the hip, is noninvasive, and does not require sedation. The ability to diagnose DDH via
ultrasonography improves with the experience of the technician. After 4 to 6 months of age, plain radiographs
(Item C239C) of the hips are more reliable for diagnosis. Treatment consists of placing the child in a Pavlik
harness to hold the hips in a position of abduction, flexion, and external rotation.

Blount disease is abnormal growth of the medial portion of the proximal tibial epiphysis and is a cause of bowing
of the legs in children. Brodie abscess is a circumscribed area of osteomyelitis. Legg-Calvé-Perthes disease is
idiopathic avascular necrosis of the femoral head (Item C239D), and slipped capital femoral epiphysis is
displacement of the femoral head from the femoral neck (Item C239E). All of these conditions are diagnosed by
plain radiographs rather than ultrasonography.

Legg Calve Perthes SCFE

89: A- follow up the patient in 6 months

Kyphosis refers to the convex alignment in the sagittal plane of the thoracic spine. The normal range of convexity
is 20 to 40 degrees. Those who have an increased convex alignment have a clinical sign of round back that may
be flexible (postural) or structural. A structural, nonflexible, round back is called idiopathic kyphosis or
Scheuermann disease.

A round back commonly is noticed during observations of adolescent posture. During the physical examination,
the round back is viewed best from the side with the patient in the forward bending position. Affected patients
who have a flexible kyphosis can correct the posture actively with hyperextension in both the standing and prone
positions. Although the posture often is a concern of parents, such flexible kyphosis has no associated adverse
health consequences.

Scheuermann disease, as exhibited by the patient described in the vignette, is a fixed kyphosis (Item C89A) that
develops around the time of puberty, most commonly in the thoracic region, and affects 7% to 8% of the
population. Affected patients cannot correct the round back appearance with active hyperextension. Other
findings on physical examination include an accentuated dorsal kyphosis with a sharp contour (or steep apex), a
compensatory lumbar lordosis with a protuberant abdomen, and less frequently, a compensatory lordosis of the
cervical spine. Neurologic examination findings generally are normal. A definitive diagnosis of Scheuermann
disease of the thoracic spine is based on an increased kyphotic angle and the pathognomonic finding on the
standing lateral radiograph of anterior wedging (Item C89B) of at least 5 degrees in three or more adjacent
vertebrae. The standard radiographic evaluation should include both standing anteroposterior and lateral
radiographs of the entire spine. Patients who have Scheuermann kyphosis also may have spondylolisthesis and
scoliosis, which are treated as separate conditions.

Treatment of Scheuermann disease depends on several factors, including the age of the patient, degree of
skeletal maturity, degree of deformity, progression of the deformity, pain in the apical region of the kyphosis,
and cosmetic and psychosocial factors. Neurologic complications and cardiopulmonary complaints are rare.
Symptoms, including pain, usually are associated with more severe, often short-segment deformities that are
greater than 90 degrees. Fortunately, Scheuermann disease is primarily a cosmetic problem for most patients.
Treatment decisions are individualized in consultation with an orthopedic surgeon; there are no absolute
guidelines.

The patient described in the vignette is asymptomatic and skeletally mature (Risser stage 4 [indicating near-
complete fusion of the iliac crest apophysis]) (Item C89C) and has a curve that is modest and cosmetically
acceptable. Therefore, he can be managed with education and guidance and followed without treatment. The use
of the Milwaukee brace early in the growth spurt may be recommended in adolescents who have kyphotic
deformities of at least 45 degrees and often greater than 60 degrees. The bracing is continued until skeletal
maturity is attained. However, bracing therapy remains controversial; long-term benefits are debated by experts.
Detractors believe that the gains from bracing are slowly lost with subsequent long-term progression.

A physical therapy program probably will not alter the natural progression of the disease, but a program that
incorporates thoracic extension exercises and emphasizes general conditioning may aid in reducing symptoms. At
present, surgical intervention usually is reserved for patients who have a mature skeleton and a severe, painful
deformity. More options may become available as advances are made in microsurgical techniques, instruments,
and materials for the treatment of spinal conditions. Pulmonary function tests are not indicated for a patient who
has an asymptomatic thoracic kyphosis of 50 degrees. Restrictive lung disease is uncommon even in patients who
have severe curves of 90 degrees or more.

Scheuermann disease is not associated with renal abnormalities and, therefore, ultrasonography is not indicated.
Human leukocyte antigen B-27 is associated with spondyloarthritis and Reiter syndrome, not Scheuermann
disease.

105: B- Osgood-Schlatter disease

The chronic knee pain and swelling and tenderness to palpation over the area of the tibial tuberosity described for
the young adolescent in the vignette are consistent with Osgood-Schlatter disease, an overuse injury that is a
common cause of chronic anterior knee pain in young athletes. During adolescence, the tibial tuberosity develops
as a specialized growth center called an apophysis. The tuberosity is the site of attachment of the quadriceps
muscle via the patellar tendon. Repetitive traction stress applied to the apophysis results in inflammation or small
fractures, with resultant pain and swelling. Pain is aggravated by activities that involve quadriceps contraction
(eg, running and jumping) or by kneeling on the tuberosity. The disease may be bilateral, although unilateral
presentations or predominance of pain are more common. The onset of Osgood-Schlatter disease often coincides
with the period of rapid adolescent growth and poses a chronic, intermittent problem for several months or
occasionally years before abating. After symptoms resolve, permanent prominence of the tuberosity may remain.
The diagnosis generally is made clinically, although a radiograph (Item C105A) may be obtained when symptoms
or history are strikingly atypical or unilateral to rule out a bony neoplasm or complication (eg, avulsion of the
tibial tubercle or a loose ossicle in the patellar tendon).

Prepatellar bursitis, a cause of anterior knee pain, presents with swelling overlying the patella that is visible and
palpable. The clinician usually can demonstrate full extension of the knee. The bursitis commonly is the result of
direct trauma and friction or repetitive flexion and extension overuse that presents as an abrupt and rapid
swelling. The mainstay of therapy is rest and the use of anti-inflammatory medication. Protective pads are
recommended for use in sports that are associated with knee friction or trauma (eg, volleyball, wrestling).
Patients who have very large swelling or recurrent episodes may need to be referred to an orthopedist for
evaluation that may include aspiration, analysis, and culture of the fluid. The differential diagnosis includes septic
bursitis that is commonly caused by Staphylococcus aureus.

The term "jumper's knee" is given to knee extensor tendonitis (patellar and quadriceps tendonitis), which affects
older and more skeletally mature adolescents. The associated pain is localized to the superior or inferior pole of
the patella. Pain at the inferior pole may be caused by a partial tear of deep layers of the patellar tendon.
Sinding-Larsen-Johansson disease also is associated with tenderness at the inferior pole of the patella rather than
over the anterior tibial tuberosity, as in Osgood-Schlatter disease. Similar to Osgood-Schlatter disease, it is
believed to be an apophysitis caused by repetitive stress injuries at the junction of the patella and the patellar
tendon during early adolescent growth.

Patellofemoral syndrome is another common cause of chronic anterior knee pain. Physical examination findings
consistent with this diagnosis are tenderness to palpation of the underside of the patella medially and laterally,
crepitus with flexion and extension, and pain with patellar compression.

165: B- Klippel-Feil syndrome

The Klippel-Feil syndrome involves the fusion of cervical vertebrae (Item C165A) and occurs in approximately 1
in 42,000 births, with a 65% female predominance. It is usually a sporadic event. Due to neck immobility,
affected individuals are at risk of cervical spine injury. Associated defects may include deafness (conduction or
sensorineural, occurring in up to 30% of patients), congenital heart defects (usually ventricular septal defect), rib
defects, hemivertebrae, Sprengel anomaly (elevation of the scapula), scoliosis, and renal anomalies.

Noonan and Turner syndrome may be associated with a "webbed" neck, but the spine is not involved. Sandifer
syndrome refers to the tonic extension of the neck associated with gastroesophageal reflux and is functional
rather than anatomic. Cleidocranial dysostosis presents with absence of the clavicles, yielding a "droopy chest"
rather than the elevated scapula and shoulders that appear in Klippel-Feil syndrome.
166: C- magnetic resonance imaging of the brain and cervical spine
A head tilt commonly is produced by muscular torticollis, but the examiner always should consider a number of
other disorders that can produce a twisted neck.

Muscular torticollis results from bleeding within the sternocleidomastoid muscle shortly after birth. Swelling can
be palpated within the muscle. The head is tipped toward the injured muscle, and the chin is rotated toward the
other. In older children, muscular torticollis can result from minor trauma or inflammation of the cervical
muscles. However, a high index of suspicion should be maintained for other processes producing head tilt.

Neurologic processes causing head tilt are perhaps the most worrisome. Posterior fossa or cervical cord tumor
(Item C166A) should be considered, along with syringomyelia, dystonia (from drug or dystonia musculorum
deformans), or visual disturbance (trochlear nerve palsy more commonly than abducens or oculomotor nerve
paresis). Congenital disorders such as hemivertebrae and Klippel-Feil syndrome can masquerade as muscular
torticollis in the newborn. Other causes of head tilt include atlantoaxial rotary subluxation, lymphadenitis,
Sandifer syndrome (gastroesophageal reflux), spasmus nutans, and paroxysmal torticollis.

Paroxysmal torticollis is a migraine variant with onset in the first year after birth. Infants experience recurrent
attacks of head tilt for minutes, sometimes with pallor, vomiting, or agitation. Episodes cease by age 2 to 3
years. No treatment is indicated, although affected children often develop migraines later in childhood.

The 12-month-old boy described in the vignette is on the borderline of being delayed in cruising. Although his
periodic withdrawal of arms and legs could be interpreted as due to reflux, such behavior also is seen with
infantile spasms or cervical spine pain. The increased deep tendon reflexes point to an upper motor neuron
process. Magnetic resonance imaging of the brain and cervical spine is warranted, particularly to exclude a
tumor; radiographs would visualize only the bony anatomy. A pH probe would be useful only if gastroesophageal
reflux were more likely. Audiometry can exclude hearing loss causing a head tilt, and electroencephalography can
exclude seizures with head posturing.