Perthes Disease

Idiopathic osteochondritis of the femoral head

Aetiology

  • Usually occurs between the ages of 4 to 9
  • More common in boys (around 5:1), particularly very active boys of short stature

Pathophysiology

  • The femoral head transiently loses its blood supply resulting in necrosis with subsequent abnormal growth
  • The femoral head may fracture and collapse
  • Subsequent remodeling occurs however the shape of the femoral head and congruence of the joint is determined by age of onset (with older children faring worse) and the amount of collapse
  • An incongruent joint will lead to early onset of arthritis and severe cases may require hip replacement in adolescence or early adulthood

Clinical presentation

Symptoms

  • Affected children present with pain and a limp
  • Most cases are unilateral and bilateral cases may represent an underlying skeletal dysplasia or a thrombophilia

Signs

  • Loss of internal rotation is usually the first clinical sign followed by loss of abduction and later on a positive Trendellenburg test from gluteal weakness

Investigations

  • X-ray - may be normal
  • MRI or bone scan can be used to identify pathology

Management

  • No specific treatment other than regular xray observation and avoidance of physical activity
  • Approximately 50% of cases do well
  • In some cases the femoral head becomes aspherical, flattened and widened
    • The lever arm of the abductor muscles is altered resulting in weakness (Trendellenburg positive)
  • Occasionally the femoral head may sublux (partially dislocate) requiring an osteotomy of the femur or acetabulum