CHOROIDAL VASCULARITY INDEX, RETINAL VASCULARITY, AND HEMOGLOBIN LEVELS IN PEDIATRIC SICKLE CELL MACULOPATHY.

Abstract

To evaluate the choroidal vascularity index (CVI) in pediatric patients with sickle cell disease (SCD) and its associations with retinal thickness and vasculature. This is a retrospective case series of children with sickle cell disease and healthy age-matched controls. The choroidal vascularity index was measured using an automated machine learning algorithm. Retinal assessments included total retinal thickness along with vessel density. A total of 184 eyes from 92 pediatric patients with sickle cell disease (55.4% male) were included. Eyes with low CVI had significantly lower median vessel density in the inferotemporal quadrant compared with eyes with higher CVI (14.8 vs. 22.2; P = 0.009). Eyes with lower CVI had higher median retinal thickness in the nasal perifovea (322 µ m vs. 316 µ m; P = 0.003) and the inferior perifovea (293 µ m vs. 290 µ m; P = 0.0028). Eyes with low CVI had significantly lower mean hemoglobin levels (9.3 vs. 10; P = 0.009). The mean CVI in the control group was 69%, which was significantly higher than the CVI in the studied population with a mean CVI of 54%, P < 0.001. In pediatric patients with sickle cell disease, there was a significant decrease in CVI when compared with healthy age-matched controls. Decreased CVI was associated with a loss of retinal vessel density in the inferotemporal macular quadrant as well as lower hemoglobin levels. These findings suggest a role of choroidal ischemia in the pathogenesis of sickle cell maculopathy and a potential for CVI to serve as a biomarker for systemic disease activity.