Abstract
Sickle cell disease is characterized by intravascular hemolysis, vaso-occlusion, and defective oxygen transport, leading to significant systemic morbidity. Ocular complications result from hypoxia, though it is unclear how systemic morbidity correlates with incidence or progression of sickle cell related ophthalmic disease. Patients generally have good vision, but often manifest macular and peripheral retinal ischemic changes. The peripheral retinal changes may best be diagnosed and followed by ultra-wide field fundus imaging and fluorescein angiography. OCT angiography is a novel method of imaging macular ischemic changes and may be useful in the evaluation of sickle cell maculopathy. Retinal ischemia and neovascularization may be managed with laser photocoagulation. Anti-VEGF therapy may play an adjunctive role in facilitating regression of retinal neovascularization and vitreous hemorrhage. Further study is needed to create evidence-based guidelines for screening and prompt referral for possible treatment of sickle cell patients at risk for ocular ischemia prior to development of ischemic and neovascular complications and subsequent vision loss.
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