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Legg-Calve-Perthes Disease

Legg-Calve-Perthes disease - also called coxa plana­is an avascular necrosis of the femoral head. Vascular interruption leads eventually to a flattened femoral head. It's typically a unilateral condition but occurs bilaterally in 15% of patients. It's most common in boys ages 4 to 10 and tends to recur in families.

The disease usually runs its four-stage course in 3 to 4 years. In the first stage, vascular interruption causes necrosis of the femoral head (usually in several months to a year). In the second stage, which may take 1 to 3 years, a new blood supply causes bone resorption and deposition of new bone cells. Deformity may result from pressure on the weakened area. In the third stage, new bone replaces necrotic bone and the femoral head gradually reforms. This process takes 1 to 3 years. The final, or residual, stage involves healing and regeneration, which fixes the joint's shape.

Causes

The cause of Legg-Calvé-Perthes disease is unknown. It is four times more likely to occur in boys than girls.

Legg-Calvé-Perthes disease commonly affects first-born children and is typically seen in children 4 to 8 years of age. The majority of cases affect only one hip.

Signs and Symptoms

  • Knee pain
  • Persistent thigh or groin pain
  • Atrophy (wasting) of muscles in the upper thigh
  • Slight shortening of the leg, or legs of unequal length
  • Hip stiffness restricting movement in the hip
  • Difficulty walking, walking with a limp (which is often painless)
  • Limited range of motion

Diagnostic tests

Range-of-motion (ROM) tests help to differentiate between Legg-Calve-Perthes disease (restriction of only the abduction and rotation of the hip) and infection or arthritis (restriction of all motion).

X-rays of the hip confirm the diagnosis. Findings vary with the disease stage.

Magnetic resonance imaging and bone scan reveal classic involvement of the anterolateral portion of the femoral head and can aid early diagnosis.

Aspiration and culture of synovial fluid rule out joint sepsis.

Treatment

The goal of treatment is to retain the femoral head's normal shape. Typically, this is accomplished by containing the femoral head within the acetabulum to protect it from further stress and damage.

Methods of containing the femoral head include prolonged use of braces and casts and surgical correction. The use of braces and casts allows weight bearing while maintaining the femur in an abducted position to keep the head contained by the acetabulum. Conservative therapy lasts 2 to 4 years. Analgesics help relieve pain. Surgical containment involves osteotomy and subtrochanteric derotation, which returns the femoral head to its normal shape and full ROM. Proper placement of the epiphysis allows remolding with ambulation. Postoperatively, the patient requires a spica cast for about 2 months.

Surgical containment requires shorter treatment periods and less emphasis on compliance compared with prolonged bracing or casting. Which containment method works best is controversial. Prevention



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