Ophthalmic Outcomes and Management of Retinopathy in Patients With Sickle Cell Disease: A Comprehensive Health Registry Study and Review of Management Strategies

BACKGROUND AND OBJECTIVE: The purpose of this study was to report the rates of ophthalmic complications and interventions in patients diagnosed with sickle cell disease (SCD) and sickle cell retinopathy (SCR), while reviewing current management strategies within the literature. PATIENTS AND METHODS: This retrospective cohort study included patients ≥ 18 years of age with the diagnosis of SCD and SCR. The electronic health record system TriNetX was utilized to identify patients diagnosed with SCD and SCR. The main outcome measures included rates of ophthalmic complications and interventions. RESULTS: We identified 1,249 patients with the diagnosis of both SCD and SCR. The mean age at diagnosis of SCR was 34.9 ± 14.8 years. The most common ocular complication was vitreous hemorrhage (VH) (19.5%) followed by cataracts (17.8%), rhegmatogenous retinal detachment (12.4%), and tractional retinal detachment (4.7%). The most common ocular intervention was retinal laser photocoagulation (13.6%). CONCLUSIONS: In a large, contemporary population-based study for SCD patients with SCR, the rates of preventable visually significant complications are high. Timely screening, diagnosis, and management strategies can help prevent these complications.