Department of Ophthalmology, West China Hospital, Sichuan University, Chengdu, China.
Department of Ophthalmology, First Hospital of China Medical University, Shenyang, China.
Curr Eye Res. 2021 Oct;46(10):1437-1448. doi: 10.1080/02713683.2021.1942073. Epub 2021 Aug 2.
Pachychoroid spectrum disease encompasses a set of macular disorders secondary to an abnormally thick choroid. However, the pathological process underlying pachychoroid spectrum disease and its overlap with age-related macular degeneration (AMD) remain unclear. This review aimed to understand the underlying pathology, classification, and phenotypes of pachychoroid spectrum disease.
This comprehensive literature review was performed based on a search of peer-reviewed published papers relevant to the current knowledge of pachychoroid disease spectrum.
Pachychoroid is primarily a bilateral phenomenon; the main pathological lesions include choriocapillaris attenuation and abnormally dilated pachyvessels. Chronic central serous chorioretinopathy (CSC) and pachychoroid neovasculopathy (PNV) show similar morphological changes and angiogenic cytokine levels. The subretinal fluid in PNV may not accurately indicate PNV activity. Besides, types 1 and 2 of choroidal neovascularization (CNV) may be involved in primary pachychoroidal disease. Both choroidal arteriosclerosis and higher hydrostatic pressure contribute to hyalinized choroidal arteries and aneurysmal dilatations, resulting in PNV progression to polypoidal choroidal vasculopathy (PCV). Thus, pachychoroid-related type 2 CNV and chronic CSC could be considered as PNV (IIIc) and as a precursor of PNV (IIIa), respectively. Tangled PCV on optical coherence tomography angiography that fails to develop aneurysms should be classified as a subtype of PNV or a forme fruste of PCV.
Multiple disorders of the pachychoroid spectrum are considered as a continuous disease process, ultimately stimulated by choroidal malfunction. PCV overlaps both AMD and pachychoroid disease, especially for thin-choroid and bilateral types. The terminology and classification of pachychoroid spectrum disease should be used cautiously.
脉络膜增厚谱疾病包括一组继发于异常增厚脉络膜的黄斑病变。然而，脉络膜增厚谱疾病的病理过程及其与年龄相关性黄斑变性（AMD）的重叠仍不清楚。本综述旨在了解脉络膜增厚谱疾病的潜在病理学、分类和表型。
本综述基于对当前脉络膜增厚疾病谱相关知识的同行评审已发表文献的全面检索。
脉络膜增厚主要是双侧现象；主要的病理病变包括脉络膜毛细血管萎缩和异常扩张的脉络膜。慢性中心性浆液性脉络膜视网膜病变（CSC）和脉络膜新生血管病变（PNV）表现出相似的形态学改变和血管生成细胞因子水平。PNV 中的视网膜下液可能无法准确反映 PNV 的活性。此外，1 型和 2 型脉络膜新生血管（CNV）可能与原发性脉络膜增厚疾病有关。脉络膜动脉硬化和较高的静水压力导致玻璃样变的脉络膜动脉和动脉瘤样扩张，导致 PNV 进展为息肉状脉络膜血管病变（PCV）。因此，脉络膜相关的 2 型 CNV 和慢性 CSC 可分别被认为是 PNV（IIIc）和 PNV（IIIa）的前身。OCTA 上出现的、未发展成动脉瘤的缠结性 PCV 应归类为 PNV 或 PCV 的顿挫型。
多种脉络膜增厚谱疾病被认为是一个连续的疾病过程，最终由脉络膜功能障碍引发。PCV 与 AMD 和脉络膜增厚疾病均有重叠，尤其是在薄脉络膜和双侧类型中。脉络膜增厚谱疾病的术语和分类应谨慎使用。
