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

PURPOSE: To evaluate the choroidal vascularity index (CVI) in pediatric patients with sickle cell disease (SCD) and its associations with retinal thickness and vasculature. METHODS: 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. RESULTS: 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. CONCLUSION: 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.