Legg-Calvé-Perthes syndrome

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Legg-Calvé-Perthes syndrome is a degenerative disease of the hip joint, where a loss of bone mass leads to some degree of collapse of the hip joint, that is, to deformity of the ball of the femur and the surface of the hip socket. The disease is typically found in young children and small dogs, and it can lead to osteoarthritis in adults.

It is the idiopathic avascular osteonecrosis of the capital femoral epiphysis of the femoral head. It is caused by an interruption to the blood supply of the head of the femur close to the hip joint. It is equivalent to adult avascular necrosis.

It is also known as Perthes disease,^[1] ischemic necrosis of the hip, coxa plana, osteochondritis and avascular necrosis of the femoral head, or Legg-Perthes Disease or Legg-Calve-Perthes Disease (LCPD).

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

The direct cause is a reduction in blood flow to the joint, though what causes this is unknown. It is thought that the artery of the ligamentum teres femoris closes too early, not allowing time for the medial circumflex femoral artery to take over. Genetics does not appear to be a determining factor, though it may be involved. When the disease is genetic in origin, it typically runs along the male line. Some evidence suggests that parental smoking may be a factor, though this is not yet proven, or more recently that a deficiency of some blood factors used to disperse blood clots may lead to blockages in the vessels supplying the joint, but that, too, has not been proven.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

Perthes is a relatively unusual condition, with only 4 per 100,000 children being affected.

It is most commonly seen in persons aged 3-12 years, with a median of 6 years of age.

Males are affected 4-5 times more often than females, suggesting a partial sex-linked genetic inheritance of the the syndrome. 1 in 100 male children of adults with Legg-Calvé-Perthes syndrome also exhibit the syndrome.

Caucasians are affected more frequently than other races.

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]

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Osteoarthritis and coxa magna may develop later in life. Early recognition and proper treatment of Legg-Calve-Perthes disease may minimize this complication.

Children younger than 6 or 7 have the best prognosis since they have time for the dead bone to revascularize and remodel. Children that have been diagnosed with Perthes’ Disease after the age of 10 are at a very high risk of developing osteoarthritis and coxa magna.

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