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