Legg-Calvé-Perthes syndrome

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, though it also affects small dogs. more...

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 also known as Perthes disease, 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).

Cause

The direct cause is a reduction in blood flow to the joint, though what causes this is unknown. 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.

Signs and symptoms

Symptoms are hip or groin pain, exacerbated by hip/leg movement. There is a reduced range of motion at the hip joint and a painful gait. There may be atrophy of thigh muscles from disuse and an inequality of leg length. In some cases, some activity can cause severe irritation or inflammation of the damaged area including standing, walking, running, kneeling, or stooping repeatedly for an extended period of time.

The first signs are complaints of soreness from the child, which are often dismissed as growing pains, and limping or other guarding of the joint, particularly when tired. The pain is usually in the hip, though can also be felt in the knee (so-called 'referred pain'). It is predominantly a disease of boys (around 4 in 5 cases), and is generally diagnosed between 5 and 12 years of age. Typically the disease is only seen in one hip, though appearance in both hips is not uncommon (about 10-15% of cases).

Diagnosis

X-Rays of the hip joint are absolutely necessary. A bone scan may be useful in helping determine the extent of the avascular changes. A hip aspiration may be performed if there is suspicion of a septic arthritis.

Diagnosis is made predominantly by X-ray study, together with physical examination (MRIs have also been found useful for judging the extent of the deformity). Sufferers typically have limited range of motion in their hip, particularly when rotating the joint.

Treatment

The goal of treatment is to avoid severe degenerative arthritis.

Treatment involves bedrest to take weight off the joint. It may require traction, leg braces, a plaster cast and physiotherapy. Surgery is only rarely necessary.

Orthopedic assessment is crucial. Younger children have a better prognosis than older children.

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