PEDBASE.org - The Pediatric Database - Detailed information of LEGG-CALVE-PERTHES DISEASE

LEGG-CALVE-PERTHES DISEASE

DEFINITION:

A disease of the hip characterized by avascular necrosis of the femoral head resulting in a limp, hip pain, and decreased range of motion of the affected hip.

EPIDEMIOLOGY:

incidence: 1/20,000
age of onset:
- 2 years -> adolescence, peak between 4 - 8 years (before SCFE)
risk factors:
- sex: M > F (4:1)
- culture: Japanese, Eskimo, Central European (white)
associations:
- low birth weight
- delayed bone age (2-3 years)
- short stature
- abnornal birth presentation
- 3rd - 6th child
- older parents (constitutional growth delay)

PATHOGENESIS:

1. Etiology

temporary interruption of blood flow to the femoral head plus a subchrondral fracture leading to avascular necrosis
usually idiopathic but may be related to certain triggers:
- vascular occlusion - sickle cell, transient synovitis
- venous obstruction - deep vein thromboses
- trauma
site of occurence:
- left > right > bilateral (56:34:10%)

CLINICAL FEATURES:

1. Limp

1. Initially

acute or insidious onset
painless and occurring intermittently after activity

2. Later

painful and constant associated with:
- pain in groin, inner thigh, and/or knee (20%)
- decreased range of motion (abduction, extension, internal rotation) of the hip
- antalgic gait

2. Muscular Changes

muscle spasm
proximal thigh atrophy
Trendelenburg gait
- due to gluteus medius weakness, the pelvis on the unsupported side descends

3. Others

short stature (constitutional delay of growth)

INVESTIGATIONS:

1. Imaging Studies

1. Skeletal X-Rays

AP and frogleg lateral views of the hips
variable findings in hip:
- 5 stages
- bulging of joint capsule
- increased joint space (cresent sign)
- increased density of the femoral head
- lateral flattening of the epiphysis
- partial or complete collapse of the head
- sublexing and pathological fractures
delayed bone age

2. Bone Scan

decreased uptake within the femoral head

3. Others

MRI, CT, arthrography

MANAGEMENT:

1. Maintain Range of Motion

bed rest, traction, physical therapy

2. Bracing

containment of Femoral Head (retain normal shape)
abduction orthosis for at least 1 year
Atlanta Scottish Rite Hospital brace

3. Surgery

varus osteotomy of proximal femur
Salter innominate osteotomy
no advantage of surgery over bracing

4. Positive Prognosticators

male
good range of motion
early age of detection
- if < 6 years: no arthritis (less extensive disease)
- if > 6 years: 100% arthritis

Pediatric Database - LEGG-CALVE-PERTHES DISEASE

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