title: Avascular necrosis of the femoral head in sickle cell syndrome: a report of 5 cases.
abstract: The course and management of avascular necrosis of the femoral head (AVNFH) in six hips of 5 sickle cell syndrome patients (3 with Hb SS, 1 with Hb SC and 1 with Hb S/beta+-thalassaemia) are described. Two patients (aged 13 and 17 years) presented with Perthes- and osteochondritis dessicans-type lesions. These hips progressed to roller-bearing-type joints with good function and no pain following conservative management of weight restriction and rest. Three patients (aged 14, 22 and 30 years at original presentation) suffered whole-head necrosis. Initially, these 3 patients had four hip joints replaced, two cemented-stemmed types, one cemented double-cup and one uncemented hemi-arthroplasty. All four joints failed and were revised 21-61 months after the original operation. One of the revision hips has now failed and is awaiting further surgery. These results demonstrate that it is very difficult to achieve a successful hip arthroplasty in the sickle cell syndrome patient.
mesh_terms: D000328:Adult; D000755:Anemia, Sickle Cell / Q000150:complications*; D001803:Blood Transfusion; D005260:Female; D005271:Femur Head Necrosis / Q000150:complications* / Q000000981:diagnostic imaging / Q000601:surgery; D006622:Hip Prosthesis; D006801:Humans; D008297:Male; D011300:Preoperative Care; D011859:Radiography; D012086:Reoperation; D012805:Sickle Cell Trait / Q000150:complications* / Q000628:therapy; D013577:Syndrome