Familial Bilateral Osteochondritis Dissecans of the Femoral Head

Abstract

Osteochondritis dissecans is a pathologic fragmentation of a segment of subchondral bone and its overlying cartilage. Although the exact etiology is unclear, one commonly accepted theory is a focal interruption in blood supply to the periarticular bone1. Osteochondritis dissecans lesions are most commonly seen in the distal part of the femur, the distal part of the humerus, and the talus. Involvement of the femoral head is rare and typically is associated with skeletal dysplasias or Legg-Calve-Perthes disease. Idiopathic osteochondritis dissecans of the femoral head is extremely rare and is typically unilateral2. We present three cases of bilateral idiopathic femoral head osteochondritis dissecans that occurred in multiple members of the same family over three generations. The pattern of transmission is presented with a pedigree diagram. The patients were informed that data concerning the case would be submitted for publication, and they consented. Case 1. In 1981, an eleven-year-old Hispanic male without a relevant medical or surgical history presented after eighteen months of worsening pain in the lateral part of the left hip; the pain radiated to the knee and was aggravated by running. The patient did not report antecedent trauma, fevers, chills, or earlier symptoms. He did not report morning stiffness or other joint complaints. The family history was notable in that a great-grandfather reportedly had “hip problems” but was otherwise healthy. On examination, the patient was in the seventy-fifth percentile for weight and the fortieth percentile for height. There was decreased motion of the left hip, most marked in rotation and abduction (flexion, 100°; extension, −10°; abduction, 30°; internal rotation, 0°; and external rotation, 10°). Anterior groin pain was elicited with internal and external rotation maneuvers, and lateral hip pain was reported with extremes of flexion. Laboratory results demonstrated a normal hemoglobin level (133 g/L) …