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Legg-Calvé-Perthes disease is an osteochondrosis that involves idiopathic aseptic necrosis of the femoral capital epiphysis.
Legg-Calvé-Perthes disease has a maximum incidence at age 5 to 10 yr, is more common among boys, and is usually unilateral. About 10% of cases are familial. Characteristic symptoms are pain in the hip joint and gait disturbance (eg, limping); some children complain of pain in the knee. Onset is gradual, and progression is slow. Joint movements are limited, and thigh muscles may become wasted.
Diagnosis is suspected based on symptoms. X-rays are usually obtained and, if needed, an MRI is done to confirm the diagnosis and extent of the lesion. X-rays initially may not be diagnostic, because they can be normal or show minimal flattening. Later x-rays can show fragmentation of the femoral head, which contains areas of lucency and sclerosis.
In bilateral or familial cases, an x-ray skeletal survey to exclude hereditary skeletal disorders, particularly multiple epiphyseal dysplasia, is mandatory because prognosis and optimal management differ. Hypothyroidism, sickle cell anemia, and trauma must also be excluded.
Orthopedic treatment includes prolonged bed rest, mobile traction, slings, and abduction plaster casts and splints to contain the femoral head. Some experts advocate subtrochanteric osteotomy with internal fixation and early ambulation. Bisphosphonates have been effective in initial trials, but further studies are needed.
Without treatment, the course is usually prolonged but self-limited (usually 2 to 3 yr). When the disease eventually becomes quiescent, residual distortion of the femoral head and acetabulum predisposes to secondary degenerative osteoarthritis. With treatment, sequelae are less severe. Young children and children with less femoral head destruction when diagnosed have the best outcome.
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