Abstract
PURPOSE: The purpose of this study was to investigate the correlation between defects in automatic perimetry and macular temporal thinning in optical coherent tomography (OCT) on patients with sickle cell disease (SCD) to define the impact of its maculopathy on visual function. METHODS: This single site retrospective cross-sectional study was conducted in a referral center for rare disease from January to July 2020. Fifty-eight patients with SCD were referred for an ophthalmological examination including automatic perimetry and OCT. The correlation between macular thickness and visual field defect in each Early Treatment Diabetic Retinopathy Study (ETDRS) quadrants was calculated. RESULTS: Visual acuity was normal in 104 eyes (94.5%). Thirty-three eyes (30.0%) had a defect of visual field in at least one ETDRS quadrant. There was a significant correlation between macular thinning on OCT and decrease of the mean defect in visual field in the temporal quadrant (P < 0.001). When comparing sickle cell genotype groups, macular thickness was significantly reduced in temporal outer quadrants in patients with the SS genotype, and in the inner and outer temporal quadrants in other genotypes. CONCLUSIONS: Despite normal visual acuity, macular temporal thinning in patients with SCD is associated with visual field defects in SS genotypes. This points to a primitive vascular dysfunction rather than neurogenic mechanism in occurrence of SCD maculopathy.