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

Publication/Presentation Date

1-1-2025

Abstract

PURPOSE: To assess longitudinal changes in retinal thickness and vessel density (VD) in pediatric sickle cell disease (SCD).

DESIGN: A 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 2017 to 2019 and followed for ≥2 years.

METHODS: Participants received 3 × 3 and 6 × 6 mm OCT and OCT angiography 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 with ≥1 follow-up 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, inferior, and total parafovea (

CONCLUSIONS: 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 with 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.

FINANCIAL DISCLOSURES: Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.

Volume

5

Issue

6

First Page

100862

Last Page

100862

ISSN

2666-9145

Disciplines

Business Administration, Management, and Operations | Health and Medical Administration | Management Sciences and Quantitative Methods

PubMedID

40755744

Department(s)

Administration and Leadership

Document Type

Article

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