Macular neurodegenerative and vascular changes on OCTA in sickle cell disease are not related to its ocular and systemic complications.

Purpose: To evaluate macular abnormalities in sickle cell disease (SCD) with OCT-Angiography (OCTA) and to determine associations with sickle cell retinopathy (SCR) and clinical and laboratory characteristics.

Methods: Complete ophthalmic examination was performed in consecutive SCD patients (HbSS, HbSC, HbSβ0 or HbSβ+ genotype), including fundoscopy and macular SD-OCT/OCTA scans. SCR stage was based on fundoscopic examination (without fluorescein angiography) instead of the Goldberg classification, since fluorescein angiography was only used in case of tentative diagnosis. Medical/ophthalmological history and hematologic characteristics were retrieved from medical records.

Results: 249 eyes of 137 patients were analyzed. The mean age was 33.3 ± 12.4 years (range 15-70). Non-proliferative 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 (VD) of the superficial capillary plexus (SCP) and deep capillary plexus (DCP) and with enlargement of the foveal avascular zone (FAZ) area, perimeter and acircularity index (AI). Age and female sex were associated with lower (para)foveal VD in the SCP and DCP. No associations were found between SCR presence/severity and macular thinning or VD.

Conclusion: Macular abnormalities were common, but did not result in visual impairment. No relation with SCR presence/severity was found. While OCTA-imaging is suitable for detecting maculopathy, it appears to have no diagnostic value in identifying patients at risk for SCR.