SHORT-TERM TRANSFUSION THERAPY FOR AVASCULAR NECROSIS OF THE HIPS IN CHILDREN WITH SICKLE-CELL DISEASE

Avascular necrosis (AVN) of the hips is a complication of sickle cell disease that may progress to complete destruction of the joint requiring surgical total hip replacement. While current management of AVN includes maneuvers to limit weight bearing, osteotomy, and arthroplasty, these approaches do not always prevent progressive bone destruction. Purpose: To evaluate the role of a short-term transfusion regimen for AVN of the hips in children with sickle cell disease. Patients and Methods: Eight children with sickle cell disease and AVN of the hips were identified who received short-term transfusion therapy. Retrospective chart review was performed to determine the effects of transfusion on clinical signs and symptoms. Hip X-rays were also retrospectively reviewed, to identify radiographic changes associated with the transfusion program. Results: Although transfusion therapy usually was associated with a reduction in hip pain, it rarely restored normal range of motion or improved the radiologic appearance of the affected hip. Conclusion: A short-term transfusion regimen is of little benefit for the management of AVN in sickle cell disease.