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脉络膜增厚疾病谱的当前观点。一篇综述。

CURRENT VIEW OF THE SPECTRUM OF PACHYCHOROID DISEASES. A REVIEW.

出版信息

Cesk Slov Oftalmol. 2023 Spring;79(4):163-166. doi: 10.31348/2023/9.

Abstract

INTRODUCTION

The term "pachychoroid" (greek pachy- [παχύ] - thick) was first used by Warrow et al. in 2013. It is defined as an abnormal and permanent increase in choroidal thickness ≥ 300 μm, which is caused by dilatation of the choroidal vessels of the Haller's layer, thinning of the Sattler's layer and the choriocapillaris layer.

METHODOLOGY

Literary research focused on the current view of pachychoroid spectrum diseases, including clarification of the pathophysiological theories of the formation of "pachychoroid".

RESULTS

It is assumed that "pachychoroid" disease has an autosomal dominant type of heredity. Depending on the further activity of various exogenous and/or endogenous factors, pachychoroid diseases may appear. According to the current knowledge, the spectrum of pachychoroid disease covers six clinical entities: pachychoroid pigment epitheliopathy, central serous chorioretinopathy, pachychoroid neovasculopathy, polypoid choroidal vasculopathy, focal choroidal excavation and peripapillary pachychoroid syndrome. In this study, we describe the clinical symptoms and objective findings of focal choroidal excavation and peripapillary pachychoroid syndrome. The current pathophysiological theory of pachychoroid diseases is based on impaired venous outflow from the choroid ("venous overload choroidopathy") and thickening of the sclera in the eyes of affected patients.

CONCLUSION

Pachychoroid diseases should be included in the differential diagnosis of characteristic features observed during multimodal imaging analysis of choroidal changes.

摘要

简介

“厚脉络膜”(希腊语 pachy- [παχύ]-厚)一词最早由 Warrow 等人于 2013 年使用。它被定义为脉络膜厚度异常且永久性增加≥300μm,这是由 Haller 层脉络膜血管扩张、Sattler 层和脉络膜毛细血管层变薄引起的。

方法

文献研究集中于厚脉络膜谱疾病的当前观点,包括阐明“厚脉络膜”形成的病理生理理论。

结果

据推测,“厚脉络膜”疾病具有常染色体显性遗传类型。根据各种外源性和/或内源性因素的进一步活动,可能会出现厚脉络膜疾病。根据目前的知识,厚脉络膜疾病的谱涵盖六个临床实体:厚脉络膜色素上皮病变、中心性浆液性脉络膜视网膜病变、厚脉络膜新生血管病变、息肉样脉络膜血管病变、局灶性脉络膜凹陷和视盘周围厚脉络膜综合征。在本研究中,我们描述了局灶性脉络膜凹陷和视盘周围厚脉络膜综合征的临床症状和客观发现。目前厚脉络膜疾病的病理生理理论基于脉络膜静脉流出受阻(“静脉超负荷脉络膜病变”)和受影响患者巩膜增厚。

结论

厚脉络膜疾病应包括在脉络膜变化的多模态成像分析中观察到的特征性表现的鉴别诊断中。

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