Peripheral Visual Fields in ABCA4 Stargardt Disease and Correlation With Disease Extent on Ultra-widefield Fundus Autofluorescence
Peripheral Visual Fields in ABCA4 Stargardt Disease and Correlation With Disease Extent on Ultra-widefield Fundus Autofluorescence
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DOI：
10.1016/j.ajo.2017.10.006
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发表时间：
2017-12-01
影响因子：
4.2
通讯作者：
Jayasundera, Thiran
中科院分区：
文献类型：
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作者：
Abalem, Maria Fernanda;Otte, Benjamin;Jayasundera, Thiran
目的：评价ABCA4型Stargardt病(STGD)患者超宽视野眼底自体荧光(UWF-Faf)的病变程度，并将其与功能预后指标进行相关性分析。设计：回顾性横断面研究。方法：地点：密歇根大学凯洛格眼科中心。研究人群：65名经临床诊断并证实ABCA4基因致病变异的患者。观察步骤：用Optos(200°)获取UWF-Faf图像，并将其分为3类。功能测试包括动态广域视野检查、视网膜电流图(FfERG)和视力(VA)。所有结果均参照UWF-Faf分类进行评价。主要观察指标：UWF-FAF的分类；I4E、III4E、IV4E等值线的面积；FERG模式；VA。结果：UWF-FAF的I型27例(41.5%)，I型17例(26.2%)。各等位线面积与病情严重程度呈负相关，所有等位线均能检测到UWF-Faf类型之间的差异(IV4e，P=.0013；II14e，P=.0003；I4e，P<；0.0001=3.93e(-8))。3种UWF-Faf类型之间的ffERG图形和VA也不同(P<；.001=6.61e(-6)和P<；.001=7.3e(-5))。结论：与局限于黄斑部疾病的患者相比，广泛性疾病患者表现出更多的周边视野狭窄，更多的ffERG功能障碍和更差的VA。UWF-Faf图像可为评估STGD患者的外周和中心视功能提供信息。(C)爱思唯尔公司出版的2017年。
PURPOSE: To evaluate the disease extent on ultra-widefield fundus autofluorescence (UWF-FAF) in patients with ABCA4 Stargardt disease (STGD) and correlate these data with functional outcome measures.DESIGN: Retrospective cross-sectional study.METHODS: SETTING: Kellogg Eye Center, University of Michigan. STUDY POPULATION: Sixty-five patients with clinical diagnosis and proven pathogenic variants in the ABCA4 gene. OBSERVATIONAL PROCEDURES: The UWF-FAF images were obtained using Optos (200 degrees) and classified into 3 types. Functional testing included kinetic widefield perimetry, electroretinogram (ffERG), and visual acuity (VA). All results were evaluated with respect to UWF-FAF classification. MAIN OUTCOME MEASURES: Classification of UWF-FAF; area comprising the I4e, III4e, and IV4e isopters; ffERG patterns; and VA.RESULTS: For UWF-FAF, 27 subjects (41.5%) were classified as type I, 17 (26.2%) as type. II, and 21 (32.4%) as type III. The area of each isopter correlated inversely with the extent of the disease and all isopters were able to detect differences among UWF-FAF types (IV4e, P = .0013; II14e, P = .0003; I4e, P < .0001 = 3.93e(-8)). ffERG patterns and VA were also different among the 3 UWF-FAF types (P < .001 = 6.61e(-6) and P < .001 = 7.3e(-5), respectively).CONCLUSION: Patients with widespread disease presented with more constriction of peripheral visual fields and had more dysfunction on ffERG and worse VA compared to patients with disease confined to the macula. UWF-FAF images may provide information for estimating peripheral and central visual function in STGD. (C) 2017 Published by Elsevier Inc.