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

IF 4.6 Q1 OPHTHALMOLOGY

Ophthalmology science Pub Date : 2025-06-24 DOI:10.1016/j.xops.2025.100862

Sally S. Ong MD , Ann Nampomba MS , Sara Rahman BS , Loka Thangamathesvaran MD , Grace Reilly MD , Jianqiao Ma ScM , Jay Vaidya PhD , Sophie Lanzkron MD, MHS , Adrienne W. Scott MD

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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 (P = 0.002, 0.003, and 0.03 respectively), temporal and total perifovea (P = 0.01 and 0.02); in the middle retina in the superior perifovea (P = 0.04); and in the total retina in the superior, nasal, and total perifovea (P < 0.001, = 0.01, and 0.009). For HbS variant, the rate of retinal thinning was significant in the inner retina in the superior parafovea (P = 0.002) only. Vessel density did not change in the SCP in HbSS subjects in any of the subfields studied but increased significantly in the SCP in HbS variant subjects in the nasal and inferior parafovea (P = 0.02 and 0.045) and superior and nasal perifovea (P = 0.03 and 0.004). Vessel density 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).

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 Disclosure(s)

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

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镰状细胞病儿童黄斑变薄和微血管异常：一项纵向分析

目的探讨儿童镰状细胞病（SCD）视网膜厚度和血管密度（VD）的纵向变化。设计前瞻性队列研究。2017年至2019年，从一所大学视网膜亚专科诊所招募了HbSS和HbS变体（HbSC和HbS地中海贫血）基因型的儿童（18岁），随访时间≥2年。方法受试者在基线和每次随访时接受3 × 3和6 × 6 mm OCT和OCT血管造影扫描。主要观察指标视网膜厚度、浅毛细血管丛（SCP）和深毛细血管丛（DCP） VD随时间的变化进行比较。结果纳入HbSS （n = 14）和HbS变异（n = 14）基因型儿童，随访≥1次（共56眼）。对于HbSS，每年视网膜变薄率在鼻、下和总旁凹内视网膜（P分别= 0.002、0.003和0.03）、颞和总旁凹内视网膜（P = 0.01和0.02）显著；上窝周视网膜中部（P = 0.04）；在视网膜上、鼻和总窝周(P <；0.001, = 0.01和0.009)。对于HbS变异型，视网膜变薄率仅在上副中央眼的内视网膜显著（P = 0.002）。HbSS患者在所有子领域的血管密度均未发生变化，但HbS变异患者的鼻和下鼻尖旁窝（P = 0.02和0.045）以及上鼻尖和鼻周围窝的血管密度显著增加（P = 0.03和0.004）。HbSS组DCP血管密度在研究的所有子区均增加(P <；HbS变异体组颞旁凸区差异有统计学意义（P = 0.02）。结论进展性视网膜变薄，主要发生在视网膜内层，在HbSS患儿中尤为显著。与HbS变异组相比，HbSS组的SCP VD没有增加。这些发现表明，SCD患者的黄斑微结构和微血管异常始于儿童期，特别是那些患有HbSS疾病的患者。财务披露专有或商业披露可在本文末尾的脚注和披露中找到。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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