Vitelliform macular dystrophy
Vitelliform macular dystrophy
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DOI：
10.1016/j.ophtha.2006.03.023
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发表时间：
2006-08-01
期刊：
影响因子：
13.7
通讯作者：
Freund, K. Bailey
中科院分区：
文献类型：
--
作者：
Spaide, Richard F.;Noble, Kenneth;Freund, K. Bailey
目的：研究并整合一组不同阶段卵黄状黄斑营养不良2型患者的摄影、血管造影和断层扫描结果（VMD 2;也称为Best's disease），并利用这些信息提出疾病发病机制。设计：回顾性观察病例系列。参与者：作者在私人诊所转诊的9名连续患者。方法：对VMD 2患者进行常规眼底和自体荧光照相、荧光素血管造影、眼底照相和光学相干断层扫描（OCT）检查。主要结果：综合眼部影像学表现。该物质具有高度自发荧光，OCT显示位于视网膜外表面。晚期病变的特征为较大病变，中央清除黄色物质，并在病变外缘沉积自发荧光视网膜下物质。早期和晚期病变都有视网膜下液体成分，OCT检测无反射性。血管造影显示透射缺陷，提示晚期渗漏，与慢性中心性浆液性脉络膜视网膜病变（CSC）非常相似。与CSC中所见相似，VMD 2患者的外层视网膜上有物质积聚，其可以代表与视网膜下液相关的脱落的光感受器外节。(c)2006年，美国眼科学会。
Purpose: To investigate and integrate the photographic, angiographic, and tomographic findings from a group of patients with various stages of vitelliform macular dystrophy type 2 (VMD2; also known as Best's disease) and use this information to propose mechanisms of disease pathogenesis.Design: Retrospective observational case series.Participants: Nine consecutive patients seen in a private practice referral setting by the authors.Methods: Patients with VMD2 were imaged with conventional fundus and autofluorescence photography, fluorescein angiography, fundus photography, and optical coherence tomography (OCT).Main Outcome Measures: The integrated ocular imaging findings.Results: Early stage lesions were smaller and had accumulation of yellowish material in the central macula. This material was highly autofluorescent and appeared to be located on the outer retinal surface by OCT. Later stages were characterized by larger lesions with central clearing of the yellowish material and deposition of autofluorescent subretinal material at the outer borders of the lesion. Both early and late lesions had a subretinal fluid component with no reflectivity as detected by OCT. Fluorescein angiography showed transmission defects with a suggestion of late leakage, much like that seen in chronic central serous chorioretinopathy (CSC).Conclusions: Similar to that seen in CSC, patients with VMD2 have an accumulation of material on the outer retina, which may represent shed photoreceptor outer segments in association with subretinal fluid. (c) 2006 by the American Academy of Ophthalmology.