Orthopedic aspects of sickle cell anemia and allied hemoglobinopathies.

The study of 23 cases of hemoglobinopathies in Nigeria using electrophoresis, showed that 16 cases were HB-SS, 5 cases HB-SC, and 2 cases HB-S Th. There were infarcts of long bones, which were usually multiple in the diaphyses in 17 cases, septic arthritis of big joints in 4 patients and aseptic necrosis of the hip in one, and in the hip and knee in one. Infection was proved by culture in only the 4 joints and 7 of the affected long bones, 8 of the causative organisms were Salmonella and 3 were Staphylococci. Only 2 of the infected cases needed sequestrectomy and the other 9 responded to simpler methods like aspiration and incision of abscess U.L.A. without resulting in a sinus formation. The aseptic necrosis in the hip may simulate Perthe's disease and dysbaric osteonecrosis, the massive periosteal reaction may simulate Caffey's disease and hypervitaminosis A, and give rise to difficulties in diagnosis when hemoglobinopathies are not suspected.