目的:描述和量化视网膜血管病变伴脑白质脑病(RVCL)对神经感觉视网膜的结构和功能后果。
方法:2021年12月至2022年12月的横断面描述性研究。
方法:从圣路易斯华盛顿大学RVCL研究中心招募的患有脑白质脑病的视网膜血管病变患者(n=9,18只眼)。
方法:视网膜血管病变伴脑白质病患者接受包括OCT在内的综合眼科评估,OCT血管造影(OCTA),超宽视野眼底成像,视网膜自发荧光,暗适应,视网膜电图(ERG),Goldmann动力学视野检查,和荧光素血管造影(FA)。
方法:从各种模式的综合表征,包括最佳矫正视力,来自OCT的中心子场厚度(μm),OCTA的中央凹无血管区(mm2),暗适应杆截距(秒),ERG中的锥体反应,血管异常的存在与否,泄漏,新生血管形成,和FA上的非灌注。
结果:本研究共纳入了9个个体的18只眼。最佳矫正视力范围为20/15至20/70。OCT的平均中心子场厚度为275.8μm(范围,217-488μm)。OCTA的平均中央凹无血管区(FAZ)为0.65(范围,0.18-1.76)mm2。在黑暗适应仪上,平均时间为5.02(范围,2.9-6.5)分钟,1名个体暗适应受损。视网膜电描记术显示4只眼有轻度视锥反应受损。在FA上,18只眼中有16只眼有黄斑和周围毛细血管未灌注的证据,18只眼中有5只眼有明显的血管渗漏和视网膜水肿.
结论:这项研究说明了疾病的表型谱,可能对辅助诊断具有临床价值。监测疾病进展,并进一步阐明RVCL的病理生理学,以帮助治疗的发展。
背景:专有或商业披露可以在本文末尾的脚注和披露中找到。
OBJECTIVE: To describe and quantify the structural and functional consequences of retinal vasculopathy with cerebral leukoencephalopathy (RVCL) on the neurosensory retina.
METHODS: Cross sectional descriptive study from December 2021 to December 2022.
METHODS: Retinal vasculopathy with cerebral leukoencephalopathy patients (n = 9, 18 eyes) recruited from the RVCL Research Center at Washington University in St. Louis.
METHODS: Retinal vasculopathy with cerebral leukoencephalopathy patients underwent comprehensive ophthalmological evaluation including OCT, OCT angiography (OCTA), ultrawidefield fundus imaging, retinal autofluorescence, dark adaptation, electroretinography (ERG), Goldmann kinetic perimetry, and fluorescein angiography (
FA).
METHODS: Comprehensive characterization from various modalities including best-corrected visual acuity, central subfield thickness (μm) from OCT, foveal avascular zone (mm2) from OCTA, dark adaptation rod intercept (seconds), cone response in ERG, and presence or absence of vascular abnormalities, leakage, neovascularization, and nonperfusion on
FA.
RESULTS: A total of 18 eyes from 9 individuals were included in this study. The best-corrected visual acuity ranged from 20/15 to 20/70. The mean central subfield thickness from OCT was 275.8 μm (range, 217-488 μm). The mean foveal avascular zone (FAZ) from OCTA was 0.65 (range, 0.18-1.76) mm2. On dark adaptometry, the mean time was 5.02 (range, 2.9-6.5) minutes, and 1 individual had impaired dark adaptation. Electroretinography demonstrated mild cone response impairment in 4 eyes. On
FA, there was evidence of macular and peripheral capillary nonperfusion in 16 of 18 eyes and notable areas of vascular leakage and retinal edema in 5 of the 18 eyes.
CONCLUSIONS: This study illustrates the phenotypic spectrum of disease and may be clinically valuable for aiding diagnosis, monitoring disease progression, and further elucidating the pathophysiology of RVCL to aid in the development of therapies.
BACKGROUND: Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.