Sickle cell retinopathy and systemic disease

Abstract: Sickle cell disease (SCD) is a widespread hemoglobinopathy that results in significant patient morbidity and mortality. Vascular occlusion can cause acute pain, acute chest syndrome, and avascular necrosis, while hemolysis and endothelial disruption can cause ischemic stroke, leg ulcers, pulmonary hypertension, and priapism. All ocular and orbital structures can be affected by SCD ischemic events, including orbital bone infarction, ischemic optic neuropathy, retinal artery occlusion, hyphema, secondary glaucoma, sickle cell maculopathy, and sickle cell retinopathy. Proliferative sickle cell retinopathy (PSR) is the most common cause of vision loss. Untreated PSR can lead to macular ischemia, vitreous hemorrhage, and tractional retinal detachment. Ophthalmic screening exams and multimodal imaging can lead to earlier detection of sickle cell retinopathy and improved patient outcomes. SCD patients undergoing vitreoretinal surgery may require coordination of care with hematologists to avoid ischemic complications. While hydroxyurea was the only United States Food and Drug Administration approved treatment for several decades, patients with SCD now have several more treatment options. Despite the United States screening all infants for SCD, there can be delays in diagnosis and treatment. This review article aims to provide an overview of sickle disease for the ophthalmologist, and to discuss emerging treatment options and current management of SCD ocular complications.