Retinal Imaging Biomarkers and Correlation to Systemic Disease Activity in Pediatric Sickle Cell Disease

IF 3.2 Q1 OPHTHALMOLOGY

Ophthalmology science Pub Date : 2025-03-22 DOI:10.1016/j.xops.2025.100774

Sandra Hoyek MD , Celine Chaaya MD , Colin A. Lemire BS , Omar Halawa MD , Francisco Altamirano MD , Natasha M. Archer MD , Efren Gonzalez MD , Nimesh A. Patel MD

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Abstract

Purpose

To correlate retinal imaging findings with systemic disease activity in children with sickle cell disease (SCD).

Design

A retrospective consecutive series.

Subjects

Children with SCD aged ≤18 years who had an ophthalmic examination at Boston Children's Hospital between January 1998 and August 2022 were included.

Main Outcome Measures

Systemic findings included the number of hospitalizations, number of strokes, treatment with hydroxyurea, hemoglobin (Hgb), and fetal Hgb levels, and time-averaged mean velocity (TAMV) in the right middle cerebral artery (RMCA) and left middle cerebral artery (LMCA) on transcranial Doppler (TCD).

Methods

Total retinal thickness was measured on macular OCT (Spectralis OCT2, Heidelberg Engineering). Vessel density (VD) of the superficial capillary plexus (SCP) and deep capillary plexus (DCP) and superficial foveal avascular zone area were measured on 6 × 6-mm OCT angiography (OCTA) scans.

Results

Six hundred six eyes from 303 pediatric SCD patients (53% males) were included. OCT and OCTA images were acquired on 104 (17.2%) and 60 (9.9%) eyes at presentation and on 159 (26.2%) and 100 (16.5%) eyes at the final visit, respectively. When adjusting for race and age, retinal thinning on OCT was associated with a higher frequency of hospitalizations, a higher frequency of strokes, and treatment with hydroxyurea. Retinal thickness in the inferior and temporal macula was positively correlated with TAMV in RMCA and in LMCA. Foveal retinal thickness was positively correlated with Hgb level. Similarly, reduced VD in the SCP and DCP in the inferior temporal macula correlated with a higher number of hospitalizations and strokes. A higher VD of the DCP in the inferior-temporal macula positively correlated with TAMV in RMCA (ρ = 0.328, P = 0.3) and in LMCA (ρ = 0.342, P = 0.029). A higher Hgb level correlated with a higher prevalence (ρ = 0.237, P = 0.037) and severity (ρ = 0.299, P = 0.008) of peripheral retinopathy in HbSC, while it correlated with lower prevalence (ρ = −0.183, P = 0.004) and severity (ρ = −0.185, P = 0.004) of peripheral retinopathy in HbSS genotypes. Visual acuity did not correlate with TCD velocity, Hgb level, or number of hospitalizations in HbSS or HbSC genotypes.

Conclusions

OCT and OCTA findings are correlated with the severity of systemic disease in children with SCD. Imaging parameters were better correlated with key outcomes such as stroke and hospitalizations than visual acuity. The results suggest that quantitative measures on retinal imaging could be used as biomarkers to predict systemic disease risk and activity.

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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儿童镰状细胞病视网膜成像生物标志物及其与全身性疾病活动的相关性

目的探讨儿童镰状细胞病（SCD）视网膜影像学表现与全身性疾病活动的相关性。DesignA回顾性连续系列。纳入1998年1月至2022年8月在波士顿儿童医院接受眼科检查的年龄≤18岁的SCD儿童。主要结局指标：系统结果包括住院次数、卒中次数、羟基脲治疗、血红蛋白（Hgb）和胎儿Hgb水平，以及经颅多普勒（TCD）显示的右大脑中动脉（RMCA）和左大脑中动脉（LMCA）的时间平均平均流速（TAMV）。方法黄斑OCT （Spectralis OCT2, Heidelberg Engineering）测量视网膜总厚度。6 × 6 mm OCT血管造影（OCTA）扫描测量浅毛细血管丛（SCP）、深毛细血管丛（DCP）血管密度（VD）及浅中央凹无血管区面积。结果共纳入303例儿童SCD患者的660只眼，其中男性占53%。就诊时分别获得104只（17.2%）和60只（9.9%）眼的OCT和OCTA图像，末次就诊时分别获得159只（26.2%）和100只（16.5%）眼的OCT和OCTA图像。在调整种族和年龄后，OCT显示的视网膜变薄与更高的住院频率、更高的中风频率和羟基脲治疗相关。下黄斑和颞黄斑视网膜厚度与RMCA和LMCA的TAMV呈正相关。视网膜中央凹厚度与Hgb水平呈正相关。同样地，颞下黄斑的下颞黄斑的VD降低与住院和中风的高数量相关。颞下黄斑DCP较高的VD与RMCA （ρ = 0.328, P = 0.3）和LMCA （ρ = 0.342, P = 0.029）的TAMV呈正相关。Hgb水平越高，HbSC患者外周视网膜病变患病率（ρ = 0.237, P = 0.037）和严重程度（ρ = 0.299, P = 0.008）越高，HbSS基因型患者外周视网膜病变患病率（ρ = - 0.183, P = 0.004）和严重程度（ρ = - 0.185, P = 0.004）越低。在HbSS或HbSC基因型中，视力与TCD速度、Hgb水平或住院次数无关。结论SCD患儿的soct和OCTA表现与全身性疾病的严重程度相关。与视力相比，成像参数与中风和住院等关键结果的相关性更好。结果表明，视网膜成像的定量测量可以作为预测全身性疾病风险和活动的生物标志物。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。

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