Structure–Function Correlation With Optical Coherence Tomography–Derived Macular Thickness and Macular Microperimetry in Children, Adolescents, and Young Adults With Sickle Cell Disease

Purpose: To evaluate the relationship between macular retinal thickness on optical coherence tomography (OCT) and retinal sensitivity on microperimetry in children, adolescents, and young adults with sickle cell disease. Methods: This cross-sectional study included 63 eyes of 34 patients with sickle cell disease. Data included demographics, macular retinal thickness determined by OCT, and retinal sensitivity on Macular Integrity Assessment (CenterVue) using a 68-point, 10-degree grid under mesopic conditions. Total retinal thickness was measured in the central, parafoveal, and perifoveal regions. The relationship between OCT macular thickness and retinal sensitivity on mesopic microperimetry was assessed in specific macular regions, including differences by peripheral sickle cell retinopathy status. Results: The median age of the 34 patients (63 eyes) was 14.6 years, and the median best-corrected visual acuity (BCVA) at the time of testing was 0 logMAR (Snellen 20/20). Of the 63 eyes, 55 (87.3%) had ≤30% fixation loss and thus were considered for the primary analysis. In multivariate regression analyses adjusted for patient age, BCVA, sickle cell genotype, sex, and race and ethnicity, decreased retinal sensitivity measured by microperimetry was associated with a decrease in OCT macular thickness (β = 0.06, 95% CI, 0.015–0.104; P = .009). Moreover, eyes with peripheral sickle cell retinopathy had lower temporal perifoveal retinal sensitivity on mesopic microperimetry compared with eyes without retinopathy (β = −6.3, 95% CI, −10.9 to −1.7; P = .008). BCVA at the time of testing was not associated with decreased macular sensitivity ( P = .817) or with decreased OCT macular thickness ( P = .086). Conclusions: Children, adolescents, and young adult patients with sickle cell maculopathy demonstrated reduced visual function, which was correlated with the degree of retinal thinning. Eyes with peripheral sickle cell retinopathy had lower temporal macular sensitivity compared with eyes without peripheral retinopathy. There was no difference in visual acuity between the groups, suggesting that functional tests are required to assess vision in sickle cell disease comprehensively.