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[富克斯角膜内皮营养不良的发病机制与诊断评估]

[Pathogenesis and diagnostic evaluation of Fuchs' endothelial corneal dystrophy].

作者信息

Wacker K, Reinhard T, Maier P

机构信息

Klinik für Augenheilkunde, Universitätsklinikum Freiburg, Killianstr. 5, 79106, Freiburg, Deutschland.

出版信息

Ophthalmologe. 2019 Mar;116(3):221-227. doi: 10.1007/s00347-018-0799-5.

Abstract

BACKGROUND

Following new treatment options for Fuchs' endothelial corneal dystrophy, our understanding of optical and structural changes in the patient's cornea is also starting to improve.

OBJECTIVE

To provide an update on clinical evaluation of Fuchs' dystrophy.

MATERIAL AND METHODS

Standardized approaches to evaluate patients with Fuchs dystrophy in routine practice and research are discussed, accounting for the progressive and partially irreversible structural changes in all corneal layers.

RESULTS

Early structural changes in the cornea can be detected before clinical edema becomes visible on slit-lamp exam. Optical limitations resulting from these structural changes can be quantified not only with high-contrast acuity but also with glare or contrast sensitivity tests. Characteristic vision-related limitations of patients with Fuchs dystrophy can, e.g., be assessed with V-FUCHS, a Fuchs dystrophy-specific "Visual Function and Corneal Health Status" instrument for patient-reported outcomes.

CONCLUSION

Clinical grading of Fuchs dystrophy in an edematous and a non-edematous stage is outdated. Better therapy options and our improved understanding of progressive changes in the entire cornea require a standardized assessment of optical and structural changes and patient-reported limitations.

摘要

背景

随着针对富克斯角膜内皮营养不良的新治疗方案的出现,我们对患者角膜光学和结构变化的理解也开始有所提高。

目的

提供富克斯角膜营养不良临床评估的最新情况。

材料与方法

讨论了在常规临床实践和研究中评估富克斯角膜营养不良患者的标准化方法,同时考虑到角膜各层进行性且部分不可逆的结构变化。

结果

在裂隙灯检查可见临床水肿之前,就可以检测到角膜的早期结构变化。这些结构变化导致的光学限制不仅可以通过高对比度视力进行量化,还可以通过眩光或对比敏感度测试进行量化。例如,富克斯角膜营养不良患者与视力相关的特征性限制可以使用V-FUCHS进行评估,这是一种针对富克斯角膜营养不良的、用于患者报告结局的“视觉功能和角膜健康状况”专用工具。

结论

对富克斯角膜营养不良处于水肿期和非水肿期进行临床分级已过时。更好的治疗方案以及我们对整个角膜进行性变化的深入理解,需要对光学和结构变化以及患者报告的限制进行标准化评估。

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