Legg-Calvé-Perthes Disease

Self-limited idiopathic osteonecrosis of the capital femoral epiphysis of the femoral head. Can be bilateral in 10% of patients.

Aseptic or Avascular Necrosis of the Femoral Head; Calvé Disease; Calvé-Perthes Disease; Legg Disease; Maydl Disease; Perthes-Calvé-Legg Disease; Perthes Disease; Perthes-Calvé-Legg-Waldenström Syndrome; Waldenström Syndrome.

First described by Karel Maydl in 1897.

1:1200 children younger than 15 years. Racial predominance in white children. Male predominance (4-5:1).

Not a genetic syndrome.

Unknown. The disease goes through four phases. (1) Interruption of the blood supply to the capital femoral epiphysis; the hip joint becomes inflamed, stiff, and painful as a result of bone infarctions, especially in the subchondral cortical bone, while articular cartilage continues to grow (several months up to 1 year). (2) Remodeling of the femoral head with occurrence of subchondral fracture (usually the result of normal physical activity, not direct trauma); the joint remains irritated and painful (1 to 3 years). (3) Rebuilding of the femoral head by new bone cells as a result of changes of the epiphyseal growth secondary to the subchondral fracture (1 to 3 years). (4) Normalization process in which normal bone cells replace the new bone cells (several years).

Established by radiographic findings (plain and frog-leg views). Five radiographic stages, from better (early stages) to worse (late stages): (1) smaller femoral head epiphysis and widening of articular space on affected side; (2) subchondral fracture; (3) bone resorption; (4) reossification of new bone; (5) healed stage. Technetium-99 bone scan helps delineate the extent of avascular changes, and dynamic arthrography allows assessment of the sphericity of the head of the femur.

Usually affects children between the ages of 3 and 12 years. Progressive hip and groin pain that may be referred to the thigh or the knee. Physical examination shows decreased range of motion (limited hip rotation and abduction), atrophy of thigh muscles (secondary to disuse), muscle spasm, and occasionally limb length inequality (because of collapse). Radiographs often show delayed bone age. Children older than 10 years are at high risk for developing osteoarthritis (coxa plana).

Careful mobilization of the patient to avoid favoring acute slipping of the femoral head.

No specific anesthetic considerations with this disorder.

Although there are no known pharmacological implications with this condition, special attention needed in that chronic use of corticosteroid in the initial phase of the disease requires supplementation intraoperatively.

Osgood-Schlatter Disease: Benign, self-limited knee condition associated with traction apophysitis in adolescents.

Pseudo-Achondroplastic Dysplasia: Rare inherited disorder characterized by skeletal malformations resulting in short-limbed dwarfism. Affected individuals present brachydactyly, genu varum, and genu valgum. In addition, they may have lumbar lordosis and kyphosis. Cases of pseudoachondroplastic dysplasia are the result of mutations of the COMP gene, indicating this disorder is allelic to some cases of multiple epiphyseal dysplasia. Pseudoachondroplastic dysplasia is inherited ...