Work

Macular Thinning and Microvasculature Abnormalities in Children with Sickle Cell Disease: A Longitudinal Analysis

Year: 2025

Type: article

Abstract: Purpose: To assess longitudinal changes in retinal thickness and vessel density (VD) in pediatric sickle cell disease (SCD).Design: Prospective cohort study Participants: Children (<18 years old) with HbSS and HbS Variant (HbSC and HbS thalassemia) genotypes were enrolled from a university-based retina subspecialty clinic from January 2017 to May 2019 and followed for at least two years.Methods: Participants received 3x3 and 6x6mm optical coherence tomography (OCT) and OCT angiography (OCTA) scans at baseline and at each follow up visit.Main Outcome Measures: Retinal thickness, superficial capillary plexus (SCP) and deep capillary plexus (DCP) VD were compared over time.Results: Children with HbSS (n=14) and HbS Variant (n=14) genotypes who had one or more follow ups were included in the study (total 56 eyes).For HbSS, rates of retinal thinning per year were significant in the inner retina in the nasal (-1.05um, p=0.002), inferior (-1.74um, p=0.003) and total parafovea (-0.87um, p=0.031), temporal (-0.3um, p=0.01), and total perifovea (-0.69um, p=0.02); in the middle retina in the superior perifovea (-1.02um, p=0.04); and in the total retina in the superior (-1.69um, p<0.001), nasal (-1.21um, p=0.01) and total perifovea (-1.26um, p=0.009).For HbS Variant, rate of retinal thinning was significant in the inner retina in the superior parafovea (-0.71um, p=0.002) only.VD did not change in the SCP in the HbSS group in any of the subfields studied but increased significantly in the SCP in the HbS Variant group in the nasal (0.54%, p=0.02) and inferior parafovea (0.52%, p=0.045), and superior (0.52%, p=0.03) and nasal perifovea (0.64%, p=0.004).VD in the DCP increased in the HbSS group in all the subfields studied (p<0.05) and in the HbS Variant group in the temporal parafovea (p=0.02). J o u r n a l P r e -p r o o fConclusions: Progressive retinal thinning, predominantly in the inner retinal layers, was particularly notable in children with HbSS disease.This was observed in conjunction with the lack of an increase in the SCP VD in the HbSS group, when compared to the HbS Variant group.These findings suggest that microstructural and microvasculature abnormalities in the macula start in childhood in SCD, especially for those with HbSS disease.

Source: Ophthalmology Science

DOI: 10.1016/j.xops.2025.100862