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脉络膜增厚性疾病。

Pachychoroid disease.

机构信息

Singapore National Eye Center, Singapore, Singapore.

Singapore Eye Research Institute, Singapore, Singapore.

出版信息

Eye (Lond). 2019 Jan;33(1):14-33. doi: 10.1038/s41433-018-0158-4. Epub 2018 Jul 11.

Abstract

Pachychoroid is a relatively novel concept describing a phenotype characterized by attenuation of the choriocapillaris overlying dilated choroidal veins, and associated with progressive retinal pigment epithelium dysfunction and neovascularization. The emphasis in defining pachychoroid-related disorders has shifted away from simply an abnormally thick choroid (pachychoroid) toward a detailed morphological definition of a pathologic state (pachychoroid disease) with functional implications, which will be discussed in this review. Several clinical manifestations have been described to reside within the pachychoroid disease spectrum, including central serous chorioretinopathy, pachychoroid pigment epitheliopathy, pachychoroid neovasculopathy, polypoidal choroidal vasculopathy/aneurysmal type 1 neovascularization, focal choroidal excavation, peripapillary pachychoroid syndrome. These conditions all exhibit the characteristic choroidal alterations and are believed to represent different manifestations of a common pathogenic process. This review is based on both the current literature and the clinical experience of our individual authors, with an emphasis on the clinical and imaging features, management considerations, as well as current understanding of pathogenesis of these disorders within the context of the recent findings related to pachychoroid disease.

摘要

脉络膜增厚是一个相对较新的概念,用于描述一种表现为脉络膜毛细血管层在扩张的脉络膜静脉上方变薄,并伴有进行性视网膜色素上皮功能障碍和新生血管形成的表型。在定义与脉络膜增厚相关的疾病时,重点已经从单纯的异常增厚的脉络膜(脉络膜增厚)转移到具有功能意义的病理性状态(脉络膜增厚性疾病)的详细形态定义,这将在本综述中进行讨论。已经描述了几种临床表现位于脉络膜增厚性疾病谱内,包括中心性浆液性脉络膜视网膜病变、脉络膜色素上皮增厚性病变、脉络膜新生血管病变、息肉状脉络膜血管病变/1 型动脉瘤样新生血管形成、局限性脉络膜凹陷、视盘周围脉络膜增厚综合征。这些疾病都表现出典型的脉络膜改变,被认为代表了同一致病过程的不同表现。本综述基于当前文献和我们每位作者的临床经验,重点介绍了这些疾病的临床和影像学特征、管理注意事项以及发病机制的最新认识,同时也考虑到了与脉络膜增厚性疾病相关的最新发现。

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