Supplementary Material for: Full-Spectrum Pachychoroid Manifestation in One Eye: A Case Report

Introduction:
The pachychoroid spectrum refers to a group of chorioretinal disorders including pachychoroid pigment epitheliopathy (PPE), pachychoroid neovasculopathy (PNV), polypoidal choroidal vasculopathy (PCV), and central serous chorioretinopathy (CSC). These conditions are thought to represent progressive stages, beginning with subclinical RPE changes in PPE, advancing to serous retinal detachment in CSC, followed by choroidal neovascularization in PNV, and culminating in aneurysmal dilation of vessels in PCV. We present a rare case of the pachychoroid spectrum in which lesions in all four stages were simultaneously observed in a single, fovea-sparing eye.
Case presentation:
An 85-year-old man presented with a one-month history of a visual disturbance in his left eye. Examination revealed all four stages of pachychoroid disease in the same eye: choroidal neovascular membrane (CNV) with subretinal hemorrhage (pachychoroid neovascularization, PNV), multiple retinal pigment epithelium (RPE) defects (pachychoroid pigment epitheliopathy, PPE), and findings consistent with central serous chorioretinopathy (CSC) and polypoidal choroidal vasculopathy (PCV). He was treated with a combination of anti-VEGF injections and focal laser therapy with the lesions stabilizing after 2 years.
Conclusion:
Although it has features similar to age-related macular degeneration, pachychoroid spectrum is a distinct disease entity, with a slower onset and greater response to initial therapy. It may necessitate therapies otherwise not used for other causes of neovascularization like focal laser treatment and verteporforin photodynamic therapy (vPDT). It is a unique pathologic process presenting with varying stages/lesions that have distinct morphological features but are thought to be a part of the same spectrum.

引言：厚脉络膜谱（pachychoroid spectrum）指一组脉络膜视网膜疾病，包括厚脉络膜色素上皮病变（pachychoroid pigment epitheliopathy, PPE）、厚脉络膜新生血管病变（pachychoroid neovasculopathy, PNV）、息肉状脉络膜血管病变（polypoidal choroidal vasculopathy, PCV）以及中心性浆液性脉络膜视网膜病变（central serous chorioretinopathy, CSC）。此类病症被认为代表了一系列进展阶段：起始于PPE中的亚临床视网膜色素上皮（retinal pigment epithelium, RPE）改变，进展为CSC中的浆液性视网膜脱离，继而出现PNV中的脉络膜新生血管，最终以PCV中的血管瘤样扩张收尾。本研究报告1例罕见的厚脉络膜谱病例，在单个未累及黄斑中心凹的眼球中同时观察到全部四个阶段的病变。

病例报告：1例85岁男性患者，因左眼视物模糊1个月就诊。眼科检查显示同一患眼存在厚脉络膜疾病的全部四个阶段病变：伴视网膜下出血的脉络膜新生血管膜（choroidal neovascular membrane, CNV，即厚脉络膜新生血管病变PNV）、多发视网膜色素上皮（RPE）缺损（即厚脉络膜色素上皮病变PPE），以及符合中心性浆液性脉络膜视网膜病变（CSC）与息肉状脉络膜血管病变（PCV）的体征。患者接受抗血管内皮生长因子（anti-VEGF）注射联合局部激光治疗，2年后病变趋于稳定。

结论：尽管厚脉络膜谱与年龄相关性黄斑变性存在相似特征，但其为独立的疾病实体，起病更为缓慢，对初始治疗的应答更佳。其治疗可能需要采用其他用于新生血管性疾病的手段，如局部激光治疗及维替泊芬光动力疗法（verteporfin photodynamic therapy, vPDT）。该疾病属于独特的病理过程，表现为具有不同形态学特征的多阶段/多病变，但被认为同属一个疾病谱的组成部分。