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黄斑旁脉络膜毛细血管萎缩

Paramacular Choriocapillaris Atrophy.

作者信息

Bućan Ivona, Bućan Kajo

机构信息

Eye Clinic, University Hospital Centre Split, 21000 Split, Croatia.

Department of Opthalmology, School of Medicine, University of Split, 21000 Split, Croatia.

出版信息

Biomedicines. 2023 Jul 24;11(7):2074. doi: 10.3390/biomedicines11072074.

Abstract

In this paper, a review of a rare case of paramacular choriocapillaris atrophy with a foveal-sparing phenotype is carried out. The 73-year-old patient stated that they had impaired vision and photophobia in both eyes during a regular ophthalmological examination, denying visual field defects and night blindness. A complete ophthalmological examination (best-corrected visual acuity, applanation tonometry, and biomicroscopy of anterior and posterior segments) and diagnostic tests, including fundus autofluorescence, fluorescein angiography, optical coherence tomography with angiography, computerized perimetry, and electroretinography, were carried out. The underlying genetic pattern is unclear, which points to paramacular choriocapillaris atrophy. According to recent research on histology, pathologies categorized as regional choroidal dystrophies are caused by alterations at the level of the retinal pigment epithelium. Despite the unresolved etiopathogenetic mechanism of foveal sparing in central choroidal and retinal dystrophies, a highly variable disease phenotype with spared fovea and central visual acuity present in a variety of heterogeneous dystrophies supports a disease-independent mechanism that allows the survival of foveal cones. The related preservation of BCVA has implications for individual prognosis and influences how treatment trials for choroidal and retinal dystrophies are designed.

摘要

本文对一例罕见的具有黄斑中心凹保留表型的黄斑旁脉络膜毛细血管萎缩病例进行了综述。这位73岁的患者表示,在一次常规眼科检查中,他们双眼视力受损且畏光,否认有视野缺损和夜盲症。进行了全面的眼科检查(最佳矫正视力、压平眼压测量以及眼前段和后段的生物显微镜检查)和诊断测试,包括眼底自发荧光、荧光素血管造影、光学相干断层扫描血管造影、电脑视野计检查和视网膜电图检查。潜在的遗传模式尚不清楚,这表明是黄斑旁脉络膜毛细血管萎缩。根据最近关于组织学的研究,归类为区域性脉络膜营养不良的病变是由视网膜色素上皮水平的改变引起的。尽管中央脉络膜和视网膜营养不良中黄斑中心凹保留的病因发病机制尚未解决,但在各种异质性营养不良中存在黄斑中心凹和中央视力保留的高度可变疾病表型,这支持了一种与疾病无关的机制,该机制使黄斑中心凹视锥细胞得以存活。最佳矫正视力的相关保留对个体预后有影响,并影响脉络膜和视网膜营养不良治疗试验的设计方式。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/f3a1/10377535/e57852b60426/biomedicines-11-02074-g001.jpg

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