Sudden-onset blindness in sickle cell disease due to retinal artery occlusion

Abstract

Central retinal artery occlusion (CRAO) is a rare and potentially devastating cause of acute blindness in sickle cell disease (SCD) that is unique compared to classic sickle retinopathy. Few details related to this complication in SCD are known, including its risk factors, pathogenesis, presentation, treatment and outcomes. We present three patients with SCD and retinal artery occlusion. The overall variability in clinical presentation, treatment and prognosis reported in the literature underscores the need for a greater understanding of these factors as they relate to this complication in SCD.