[Clinical characteristics of the vascular changes in pediatric patients with congenital retinoschisis].

Q3 Medicine
J Ma, J H Liu, S F Li, G D Deng, L Li, M Z Yuan, T Y Wang, H Lu
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引用次数: 0

Abstract

Objective: To investigate the clinical characteristics of abnormal vascular changes in the fundus of pediatric patients with congenital X-linked retinoschisis (XLRS). Methods: This retrospective case series study included pediatric patients (age≤18 years) diagnosed with XLRS at Beijing Tongren Hospital, Capital Medical University between December 2017 and November 2024. All eyes underwent wide-angle fundus photography with some patients additionally receiving fundus fluorescein angiography (FFA). The prevalence and patterns of vascular abnormalities were analyzed, along with their correlations with clinical outcomes. Statistical analyses employed Fisher's exact test, χ² test, or Mann-Whitney U test. Results: A total of 112 pediatric cases (218 eyes) were included, consisting of 110 right eyes and 108 left eyes. All patients were male, with a median age of 5.00 (4.00, 6.75) years (range: 9 months to 17 years). Abnormal vascular changes were detected in 139 eyes (63.8%) on fundus photography, including vascular sheathing (35.8%, 78/218), tortuosity (32.6%, 71/218), bridging vessels (29.4%, 64/218), exudation (9.2%, 20/218), and neovascularization (3.2%, 7/218). Among 85 FFA-evaluated eyes (44 patients), 96.5% (82/85) exhibited abnormalities, including nonperfusion areas (96.5%, 82/85), extra-macular leakage (94.1%, 80/85), and neovascularization (15.3%, 13/85). Vascular anomalies were observed in both schitic and non-schitic peripheral retina in 78.8% (67/85) of eyes, exclusively in schitic areas in 17.6% (15/85), and in non-schitic regions in 1.2% (1/85). One eye with exudative retinal detachment secondary to massive subretinal exudation achieved complete resolution following anti-VEGF therapy. Eyes with abnormal vascular changes demonstrated significantly higher rates of peripheral retinoschisis (100% vs. 58.2%), vitreous hemorrhage (33.1% vs. 7.6%), retinal detachment (24.5% vs. 6.3%) and tractional retinal detachment (20.1% vs. 2.5%) compared to unaffected eyes (all P<0.05). Worse median visual acuity was observed in eyes with peripheral retinoschisis [0.96 (0.70, 1.30) vs. 0.52 (0.46, 0.75)], vitreous hemorrhage [1.00 (0.70, 1.30) vs. 0.70 (0.52, 1.00)], retinal detachment [1.30 (1.00, 1.92) vs. 0.70 (0.52, 1.00)], abnormal vascular changes [1.00 (0.70, 1.30) vs. 0.70(0.52, 0.98)], vascular sheathing [1.00(0.70, 1.30) vs. 0.70(0.52, 1.00)], tortuous vessels [1.00 (0.70, 1.30) vs. 0.70 (0.52, 1.00)] compared to unaffected eyes (all P<0.05). Conclusions: Pediatric XLRS patients exhibit diverse fundus vascular abnormalities, predominantly vascular sheathing, tortuosity, and bridging vessels. Eyes with vascular abnormalities demonstrate significantly higher complication rates and worse visual acuity.

【儿童先天性视网膜裂血管病变的临床特点】。
目的:探讨小儿先天性x连锁视网膜裂(XLRS)患者眼底血管异常改变的临床特点。方法:回顾性病例系列研究纳入首都医科大学附属北京同仁医院2017年12月至2024年11月诊断为XLRS的儿童患者(年龄≤18岁)。所有患者均行广角眼底摄影,部分患者还行眼底荧光素血管造影(FFA)。分析了血管异常的患病率和模式,以及它们与临床结果的相关性。统计分析采用Fisher精确检验、χ 2检验或Mann-Whitney U检验。结果:共纳入患儿112例(218眼),其中右眼110眼,左眼108眼。所有患者均为男性,中位年龄为5.00(4.00,6.75)岁(范围:9个月至17岁)。眼底摄影检出异常血管改变139眼(63.8%),包括血管鞘(35.8%,78/218)、扭曲(32.6%,71/218)、桥血管(29.4%,64/218)、渗出(9.2%,20/218)和新生血管(3.2%,7/218)。在85只ffa评估的眼睛(44例)中,96.5%(82/85)出现异常,包括非灌注区(96.5%,82/85)、黄斑外渗漏(94.1%,80/85)和新生血管(15.3%,13/85)。在78.8%(67/85)的眼睛中,分裂性和非分裂性周围视网膜均有血管异常,分裂性区域有17.6%(15/85),非分裂性区域有1.2%(1/85)。在抗vegf治疗后,一只眼继发于大量视网膜下渗出的渗出性视网膜脱离得到完全解决。与未受影响的眼睛相比,有异常血管改变的眼睛的周围视网膜裂(100%比58.2%)、玻璃体出血(33.1%比7.6%)、视网膜脱离(24.5%比6.3%)和牵拉性视网膜脱离(20.1%比2.5%)的发生率显著高于正常眼睛(所有比例分别为0.52(0.46,0.75)、玻璃体出血[1.00(0.70,1.30)比0.70(0.52,1.00)]、视网膜脱离[1.30(1.00,1.92)比0.70(0.52,1.00)]、血管异常改变[1.00(0.70,1.30)比0.70(0.52,0.98)]。结论:儿童XLRS患者眼底血管异常表现多样,以血管鞘、弯曲和桥血管为主。有血管异常的眼睛表现出明显更高的并发症发生率和更差的视力。
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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来源期刊
中华眼科杂志
中华眼科杂志 Medicine-Ophthalmology
CiteScore
0.80
自引率
0.00%
发文量
12700
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