Macular telangiectasia type 2.
Macular telangiectasia type 2.
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DOI：
10.1016/j.preteyeres.2012.11.002
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发表时间：
2013-05
影响因子：
17.8
通讯作者：
Scholl HP
中科院分区：
文献类型：
--
作者：
Charbel Issa P;Gillies MC;Chew EY;Bird AC;Heeren TF;Peto T;Holz FG;Scholl HP
黄斑毛细血管扩张症2型是一种原因不明的双侧疾病，具有黄斑毛细血管网和感觉神经萎缩的特征性改变。其患病率可能被低估，最近显示在40岁及以上人群中高达0.1%。生物显微镜检查可显示视网膜透明度降低、结晶沉积物、轻度毛细血管扩张、小静脉变钝、视网膜色素斑块、中央凹萎缩和新生血管复合体。血管造影显示早期主要在小凹颞侧的毛细血管扩张，晚期可见弥漫性高荧光。高分辨率光学相干断层扫描（OCT）可以揭示感光细胞内段-外段边界的破坏，内部或外部视网膜水平的低反射腔，以及后期视网膜的萎缩。2型黄斑毛细血管扩张症显示出中央视网膜中黄斑色素的独特消耗，并且最近的治疗试验显示，这种消耗的区域在口服补充剂后不能重新积累叶黄素和玉米黄质。已经有各种疗效有限或无效的治疗方法。最近用阻断血管内皮生长因子（VEGF）的化合物进行的临床试验已经确立了VEGF在疾病的病理生理学中的作用，但至少对于非新生血管疾病阶段没有显示出显著的功效。结构-功能相关性的最新进展可能有助于为未来的临床试验开发替代结局指标。本文就黄斑毛细血管扩张症2型的流行病学、遗传学、临床表现、分期及鉴别诊断等方面的研究进展作一综述。使用视网膜成像的结果进行了讨论，包括荧光素血管造影，OCT，自适应光学成像，共焦扫描激光检眼镜，眼底自发荧光，因为是使用视觉功能测试，包括视敏度和眼底控制的微视野检查的结果。我们提供了一个非新生血管和新生血管疾病阶段的治疗方法的概述，并提供了未来的方向，包括动物模型和潜在的治疗方法的角度。
Macular telangiectasia type 2 is a bilateral disease of unknown cause with characteristic alterations of the macular capillary network and neurosensory atrophy. Its prevalence may be underestimated and has recently been shown to be as high as 0.1% in persons 40 years and older. Biomicroscopy may show reduced retinal transparency, crystalline deposits, mildly ectatic capillaries, blunted venules, retinal pigment plaques, foveal atrophy, and neovascular complexes. Fluorescein angiography shows telangiectatic capillaries predominantly temporal to the foveola in the early phase and a diffuse hyperfluorescence in the late phase. High-resolution optical coherence tomography (OCT) may reveal disruption of the photoreceptor inner segment–outer segment border, hyporeflective cavities at the level of the inner or outer retina, and atrophy of the retina in later stages. Macular telangiectasia type 2 shows a unique depletion of the macular pigment in the central retina and recent therapeutic trials showed that such depleted areas cannot re-accumulate lutein and zeaxanthin after oral supplementation. There have been various therapeutic approaches with limited or no efficacy. Recent clinical trials with compounds that block vascular endothelial growth factor (VEGF) have established the role of VEGF in the pathophysiology of the disease, but have not shown significant efficacy, at least for the nonneovascular disease stages. Recent progress in structure–function correlation may help to develop surrogate outcome measures for future clinical trials. In this review article, we summarize the current knowledge on macular telangiectasia type 2, including the epidemiology, the genetics, the clinical findings, the staging and the differential diagnosis of the disease. Findings using retinal imaging are discussed, including fluorescein angiography, OCT, adaptive optics imaging, confocal scanning laser ophthalmoscopy, and fundus autofluorescence, as are the findings using visual function testing including visual acuity and fundus-controlled microperimetry. We provide an overview of the therapeutic approaches for both non-neovascular and neovascular disease stages and provide a perspective of future directions including animal models and potential therapeutic approaches.
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影响因子：
3.4
作者：
Bok, D;Yasumura, D;Lavail, MM
通讯作者：
Lavail, MM
DOI：
10.1097/iae.0b013e3181b9f11e
发表时间：
2010-02-01
影响因子：
3.3
作者：
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DOI：
10.1007/s00417-007-0633-2
发表时间：
2007-11-01
影响因子：
2.7
作者：
Arevalo, J. Fernando;Sanchez, Juan G.;Garcia-Amaris, Rafael
通讯作者：
Garcia-Amaris, Rafael
影响因子：
1.7
作者：
Cakir, M.;Kapran, Z.;Perente, I.
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