Abstract T MP110: Central Retinal Artery Occlusion in Patients with Sickle Cell Disease

Objective: To determine the rate of and risk factors for incident central retinal artery occlusion (CRAO) among patients with sickle cell disease enrolled in a large cohort with longitudinal follow-up. Background: Sickle cell disease increases the risk of ischemic stroke among women but the risk of CRAO is not studied. Design/Methods: A total of 4085 patients from newborns to 77 years-old, were enrolled in Phase 1 of Cooperative Study of Sickle Cell Disease from 23 centers across the US. Participants underwent a baseline examination for assessment of demographics, prior medical history, lab assessments, and clinical data. Post baseline data included routine follow-up examinations, measures of organ damage, and collection of acute and chronic complications. The risk factors for CRAO were identified using Cox Proportional Hazards analysis. Results: A total of 9 (0.002%) of 4085 patients with sickle cell disease developed CRAO over a mean follow-up of 5.2 (95% CI 0.9 - 11.3) months, with an estimated incidence of 0.02 per 100 patient years. The incidence of CRAO was 0.03 per 100 patient years and 0.05 per 100 patient years in men and women, respectively. The history of previous stroke (2.6% versus 0.0%, p=0.7) and the proportion of patients diagnosed with large arterial occlusive disease (55.6% versus 7.7%, p=<0.001) was greater in those who developed CRAO compared to those who did not develop CRAO. History of exchange transfusions (2.7 % versus 22.2 %, p=0.02) and cigarette smoking (13.7 % versus 22.2 %, p =0.4) were more common among patients who developed CRAO. The hematocrit (%) (mean ± SD) was similar between patients who did or did not develop CRAO (29.5 ± 9.6 versus 27.3 ± 5.6, p=0.3). At completion of follow-up, 22.2% (n=2) patients with CRAO reported disability compared with 11.1% (n=455) patients without CRAO (p=0.7) Conclusions: The incidence of CRAO is relatively high among patients with sickle cell disease and was associated with disability.