Sickle cell retinopathy

Ten percent of North American blacks have one of four forms of sickle cell disease. Eight to nine percent have sickle cell trait (AS disease); One-half to one percent have SThal disease; Four-tenths percent have SS disease; One-tenth to three-tenths percent have SC disease. The SC and SThal forms present with the most frequent and severe ocular manifestations, including proliferative and non-proliferative retinopathies. Approximately 1% of the black population is at risk for serious ocular complications. The purpose of this paper is to familiarize the reader with the retinal signs, transmissibility, and pathophysiology of a high incidence disease. Early recognition and referral results in prolonged visual function if proper therapy is undertaken. A brief overview of ocular therapies is included.