Skip to Main Content

At a glance

Self-limited idiopathic osteonecrosis of the capital femoral epiphysis of the femoral head. Can be bilateral in 10% of patients. It is a childhood hip disorder initiated by a disruption of blood flow to the ball of the femur called the femoral head. Due to the lack of blood flow, the bone dies (osteonecrosis or avascular necrosis) and stops growing. Over time, healing occurs by new blood vessels infiltrating the dead bone and removing the necrotic bone, which leads to a loss of bone mass and a weakening of the femoral head. The bone loss leads to some degree of collapse and deformity of the femoral head and sometimes secondary changes to the shape of the hip socket. It is also referred to as idiopathic avascular osteonecrosis of the capital femoral epiphysis of the femoral head since the cause of the interruption of the blood supply of the head of the femur in the hip joint is unknown. The condition is most commonly found in children between the ages of 4 and 8, but it can occur in children between the ages of 2 and 15.

Synonyms

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.

History

The first clinical description was provided by Karel Maydl in 1897. The name of this medical condition originates from Drs Arthur Legg (1874-1939), an American orthopedic surgeon, and Jacques Calvé (1868-1943), a French orthopedic surgeon, and Georg Perthes (1869-1927), a German orthopedic surgeon.

Incidence

The estimated annual incidence varies between 0.45 and 21 cases/100,000 children. The life time risk of a child developing the disease is estimated at one per 1,200 individuals before the age of 15 years. There is racial predominance in Caucasian children. It is five times more common in boys and involves both hips in 8 to 24% of affected children. The disease is usually diagnosed among children less than 15 years of age, with a peak onset between 5 and 8 years of age.

Genetic inheritance

Not a genetic syndrome.

Pathophysiology

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 (1-3 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 (1-3 years). (4) Normalization process in ...

Pop-up div Successfully Displayed

This div only appears when the trigger link is hovered over. Otherwise it is hidden from view.