Recent advances in the treatment of proliferative sickle cell retinopathy.

Sickle cell eye disease is characterized by microvascular occlusion that affects the peripheral retinal vasculature and results in retinal ischemia and the development of proliferative sickle cell retinopathy. Complications of proliferative sickle cell retinopathy such as vitreous hemorrhage and retinal detachment are the most common causes of visual loss in this disease. Prevention of this visual loss is therefore contingent on the prevention or early detection and treatment of proliferative sickle cell retinopathy. Most effort has been directed toward therapy for established proliferative sickle cell retinopathy, and several therapeutic trials have been reported. Much less is known about the risk factors for development of proliferative sickle cell retinopathy and its natural history, in particular the true incidence of permanent visual loss. Visual loss is believed to be predominantly a complication of perfused proliferative sickle cell retinopathy, and its autoinfarction renders such visual loss less likely. To better define the place of treatment, identification of the determinants of autoinfarction is required, but identification of patients whose proliferative sickle cell retinopathy is likely to proceed to visual loss is of greater importance.