Chen Jimmy S, Kalaw Fritz Gerald P, Nudleman Eric D, Scott Nathan L
Viterbi Family Department of Ophthalmology, Shiley Eye Institute, University of California, San Diego, La Jolla, California.
UCSD Health Department of Biomedical Informatics, University of California San Diego, La Jolla, California.
Ophthalmol Sci. 2024 Nov 22;5(2):100658. doi: 10.1016/j.xops.2024.100658. eCollection 2025 Mar-Apr.
To quantitatively assess the retinal vascular tortuosity of patients with sickle cell disease (SCD) and retinopathy (SCR) using an automated deep learning (DL)-based pipeline.
Cross-sectional study.
Patients diagnosed with SCD and screened for SCR at an academic eye center between January 2015 and November 2022 were identified using electronic health records. Eyes of unaffected matched patients (i.e., no history of SCD, hypertension, diabetes mellitus, or retinal occlusive disorder) served as controls.
For each patient, demographic data, sickle cell diagnosis, types and total number of sickle cell crises, SCD medications used, ocular and systemic comorbidities, and history of intraocular treatment were extracted. A previously published DL algorithm was used to calculate retinal microvascular tortuosity using ultrawidefield pseudocolor fundus imaging among patients with SCD vs. controls.
Cumulative tortuosity index (CTI).
Overall, 64 patients (119 eyes) with SCD and 57 age- and race-matched controls (106 eyes) were included. The majority of the patients with SCD were females (65.6%) and of Black or African descent (78.1%), with an average age of 35.1 ± 20.1 years. The mean number of crises per patient was 3.4 ± 5.2, and the patients took 0.7 ± 0.9 medications. The mean CTI for eyes with SCD was higher than controls (1.06 ± vs. 1.03 ± 0.02, < 0.001). On subgroup analysis, hemoglobin S, hemoglobin C, and HbS/beta-thalassemia variants had significantly higher CTIs compared with controls (1.07 vs. 1.03, < 0.001), but not with sickle cell trait variant (1.04 vs. 1.03 control, = .2). Univariable analysis showed a higher CTI in patients diagnosed with proliferative SCR, most significantly among those with sea-fan neovascularization (1.06 ± 0.02 vs. 1.04 ± 0.01, < 0.001) and those with >3 sickle cell crises (1.07 ± 0.02 vs. 1.05 ± 0.02, < 0.001).
A DL-based metric of cumulative vascular tortuosity associates with and may be a potential biomarker for SCD and SCR disease severity.
Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.
使用基于深度学习（DL）的自动化流程定量评估镰状细胞病（SCD）和视网膜病变（SCR）患者的视网膜血管迂曲度。
横断面研究。
通过电子健康记录识别出2015年1月至2022年11月期间在一家学术眼科中心被诊断为SCD并接受SCR筛查的患者。未受影响的匹配患者（即无SCD、高血压、糖尿病或视网膜阻塞性疾病病史）的眼睛作为对照。
提取每位患者的人口统计学数据、镰状细胞诊断、镰状细胞危象的类型和总数、使用的SCD药物、眼部和全身合并症以及眼内治疗史。使用先前发表的DL算法，通过超广角伪彩色眼底成像计算SCD患者与对照患者的视网膜微血管迂曲度。
累积迂曲指数（CTI）。
总体上，纳入了64例SCD患者（119只眼）和57例年龄及种族匹配的对照（106只眼）。大多数SCD患者为女性（65.6%），黑人或非裔（78.1%），平均年龄35.1±20.1岁。每位患者的平均危象次数为3.4±5.2次，患者服用0.7±0.9种药物。SCD患者眼睛的平均CTI高于对照组（1.06±对1.03±0.02，<0.001）。亚组分析显示，与对照组相比，血红蛋白S、血红蛋白C和HbS/β地中海贫血变异型患者的CTI显著更高（1.07对1.03，<0.001），但镰状细胞性状变异型患者与对照组无差异（1.04对1.03，=0.2）。单因素分析显示，诊断为增殖性SCR的患者CTI较高，在海扇状新生血管形成患者（1.06±0.02对1.04±0.01，<0.001）和镰状细胞危象>3次的患者（1.07±0.02对1.05±0.02，<0.001）中最为显著。
基于DL的累积血管迂曲度指标与SCD和SCR疾病严重程度相关，可能是一种潜在的生物标志物。
专有或商业披露信息可在本文末尾的脚注和披露中找到。
