How I Manage Sickle Cell Retinopathy

We describe our current approach to sickle cell retinopathy screening, evaluation, treatment, and surgical intervention.

Small and stable neovascular complexes in proliferative sickle cell retinopathy may be observed and monitored without treatment in select cases, given high rates of auto-infarction without visual consequence. Scatter laser photocoagulation remains the preferred first-line therapy for select high-risk cases of proliferative sickle cell retinopathy, including that observed in monocular patients, patients with demonstrated progression of neovascularization, or if close patient follow up for disease monitoring cannot be assured. Anti-vascular endothelial growth factor injections may be used as adjunctive therapy to laser in cases of persistent grown of sea fan neovascularization or vitreous hemorrhage. Surgical management of proliferative sickle cell retinopathy is complex, but overall outcomes have improved with modern vitreoretinal surgical techniques.

Sickle cell retinopathy care is focused on the identification and management of proliferative sickle cell retinopathy. Further research is needed to inform best practices in sickle cell retinopathy screening and treatment, to better understand which patients are at highest risk for vision loss, and to identify the impact of novel systemic therapies for sickle cell disease on these patient’s ophthalmic outcomes.