Abstract
PURPOSE: To evaluate macular abnormalities in sickle cell disease with optical coherence tomographic angiography and to determine associations with sickle cell retinopathy (SCR) and clinical and laboratory characteristics. METHODS: Complete ophthalmic examination was performed in consecutive patients with sickle cell disease (HbSS, HbSC, HbSβ 0 , or HbSβ + genotype), including fundoscopy and macular spectral-domain optical coherence tomography/optical coherence tomographic angiography scans. Sickle cell retinopathy stage was based on fundoscopic examination (without fluorescein angiography) instead of the Goldberg classification because fluorescein angiography was only used in case of tentative diagnosis. Medical/ophthalmological history and hematologic characteristics were retrieved from medical records. RESULTS: Two hundred and forty-nine eyes of 137 patients were analyzed. The mean age was 33.3 ± 12.4 years (range 15-70 years). Nonproliferative SCR was present in 57 eyes (22.9%) and proliferative SCR in 36 eyes (14.5%). Macular thinning was present in 100 eyes (40.2%) and was associated with lower foveal vessel density of the superficial capillary plexus and deep capillary plexus and with enlargement of the foveal avascular zone area, perimeter, and acircularity index. Age and female sex were associated with lower (para)foveal vessel density in the superficial capillary plexus and deep capillary plexus. No associations were found between SCR presence/severity and macular thinning or vessel density. CONCLUSION: Macular abnormalities were common but did not result in visual impairment. No relation with SCR presence/severity was found. Although optical coherence tomographic angiography imaging is suitable for detecting maculopathy, it appears to have no diagnostic value in identifying patients at risk for SCR.