Management of osteonecrosis in sickle-cell anemia and its genetic variants.

Osteonecrosis secondary to sickling disorders differs from most other forms of osteonecrosis, because vaso-occlusive episodes generally involve many other organs. For this reason optimum management and care of these patients requires a team approach. The spectrum of clinical symptoms and bone involvement varies widely from one patient to another, even among patients with the same abnormal hemoglobin patterns. Although the efficacy of conservative therapeutic measures has not been definitely established, these may be all that can be offered because of the patient's other problems. Before contemplating surgery, the surgical and anesthetic risks should be evaluated and measures taken to prevent complications. The varus osteotomy, Chiari pelvic osteotomy, prosthetic or total hip replacement, and the Neer shoulder prosthesis may benefit a limited number of carefully selected patients.