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急性区域性隐匿性外层视网膜病变(AZOOR)一例的结构、功能及细胞分析

Structural, Functional, and Cellular Analysis of a Case of Acute Zonal Occult Outer Retinopathy (AZOOR).

作者信息

Iuliano Mattia, Lombardo Marco, Falsini Benedetto, Sebastiani Jacopo, D'Ambrosio Michele, Martelli Francesco, Cusumano Andrea

机构信息

Ophthalmology Unit, Department of Experimental Medicine, University of Rome Tor Vergata, 00133 Rome, Italy.

Macula & Genoma Foundation USA, New York, NY 10017, USA.

出版信息

Biomedicines. 2025 Jun 21;13(7):1521. doi: 10.3390/biomedicines13071521.

DOI:10.3390/biomedicines13071521
PMID:40722597
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC12292167/
Abstract

Adaptive optics transscleral flood illumination (AO-TFI) enables in vivo, non-invasive, high-resolution imaging of retinal pigment epithelium (RPE) and photoreceptor (PR) cells, paving the way for a new potential characterization of retinal diseases. This study aimed to analyze RPE and PR cells in a case of acute zonal occult outer retinopathy (AZOOR) using AO-TFI. A patient affected by AZOOR underwent a comprehensive eye examination, perimetry, electroretinography (ERG), autofluorescence, and optical coherence tomography (OCT) during the acute phase (T0). After three years (T1), OCT angiography (OCTA) and AO-TFI were also performed. Voronoi analysis was utilized to quantify RPE and PR cells. At T0, OCT revealed interruptions in the ellipsoid zone (EZ) of the right eye, while the structure of the left eye appeared normal. Perimetry and ERG were abnormal in both eyes. At T1, OCT indicated recovery of the EZ in the right eye, while thinning of the ONL persisted. Perimetry and mfERG values remained below normative limits. OCTA exhibited globally reduced vessel density in the inner retina of the right eye. AO-TFI demonstrated reduced PR density in affected areas despite preserved EZ, while RPE cell density appeared unaffected. AO-TFI enabled a detailed visualization and quantification of macular RPE and PR cells, providing valuable insights into the pathophysiology of AZOOR.

摘要

自适应光学经巩膜泛光照明(AO-TFI)能够对视网膜色素上皮(RPE)和光感受器(PR)细胞进行活体、非侵入性、高分辨率成像,为视网膜疾病的新潜在特征描述铺平了道路。本研究旨在使用AO-TFI分析一例急性区域性隐匿性外层视网膜病变(AZOOR)患者的RPE和PR细胞。一名受AZOOR影响的患者在急性期(T0)接受了全面的眼部检查、视野检查、视网膜电图(ERG)、自发荧光和光学相干断层扫描(OCT)。三年后(T1),还进行了OCT血管造影(OCTA)和AO-TFI检查。利用Voronoi分析对RPE和PR细胞进行量化。在T0时,OCT显示右眼椭圆体带(EZ)中断,而左眼结构正常。双眼的视野检查和ERG均异常。在T1时,OCT显示右眼EZ恢复,而外核层(ONL)变薄持续存在。视野检查和多焦ERG值仍低于正常范围。OCTA显示右眼内视网膜的血管密度整体降低。尽管EZ保留,但AO-TFI显示受影响区域的PR密度降低,而RPE细胞密度似乎未受影响。AO-TFI能够详细可视化和量化黄斑区的RPE和PR细胞,为AZOOR的病理生理学提供了有价值的见解。

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A Case of an Acute Zonal Occult Outer Retinopathy Variant Characterized With an Insidious Peripheral Onset and Centripetal Progression.一例以隐匿性周边发病和向心性进展为特征的急性区域性隐匿性外层视网膜病变变异型病例。
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