Touhami Sara, Rigo Sabrina, Faudi Emilien, March de Ribot Francesc, Bodaghi Bahram, Gaudric Alain
Ophthalmology Department, Sorbonne Université, Pitié-Salpêtrière University Hospital, Paris, France.
Department of Ophthalmology, University of Girone, Girone, Spain.
Ocul Immunol Inflamm. 2025 May 19:1-4. doi: 10.1080/09273948.2025.2505693.
To present a case of recurrent acute angioid streak retinopathy (AASR) and discuss its potential pathophysiological mechanisms based on multimodal imaging findings.
A 19-year-old woman with pseudoxanthoma elasticum (PXE) experienced three distinct episodes of posterior segment inflammation associated with angioid streaks (AS), previously identified as AASR. Each episode started with acute subretinal inflammation due to a disruption of the retinal pigment epithelium (RPE)/Bruch's membrane (BM) complex at an AS site, which appeared as a peri-AS whitening on fundus photography. About one week later, as the peri-AS lesions resolved, multifocal white dots characteristic of multiple evanescent white dot syndrome (MEWDS) appeared around the optic disc and at the posterior pole. Multimodal imaging, including blue autofluorescence, fluorescein angiography, indocyanine green angiography, optical coherence tomography (OCT), and OCT-angiography, confirmed these findings. While the peri-AS lesions resembled multifocal choroiditis, they differed in their extension along the AS and faster resolution. Each AASR episode resulted in a detectable AS enlargement on fundus autofluorescence. These findings suggested that AASR could involve a two-phase inflammatory process: an initial disruption of the RPE/BM complex at an AS site triggering localized choroiditis/outer retinitis, followed by secondary MEWDS and subsequent AS enlargement.
This case illustrates a recurring inflammatory pattern in PXE-associated AS, characterized by localized inflammation at AS sites with RPE/BM disruption, and secondary MEWDS. These findings highlight the pathophysiological mechanisms of AASR and its unique imaging features.
报告一例复发性急性血管样条纹性视网膜病变（AASR）病例，并基于多模态成像结果讨论其潜在的病理生理机制。
一名患有弹性假黄瘤（PXE）的19岁女性经历了三次与血管样条纹（AS）相关的后段炎症发作，之前被诊断为AASR。每次发作均始于AS部位视网膜色素上皮（RPE）/布鲁赫膜（BM）复合体破坏导致的急性视网膜下炎症，在眼底照片上表现为AS周围变白。大约一周后，随着AS周围病变消退，视盘周围和后极出现了多发性一过性白点综合征（MEWDS）特征性的多灶性白点。包括蓝色自发荧光、荧光素血管造影、吲哚菁绿血管造影、光学相干断层扫描（OCT）和OCT血管造影在内的多模态成像证实了这些发现。虽然AS周围病变类似于多灶性脉络膜炎，但它们在沿AS的扩展和更快的消退方面有所不同。每次AASR发作均导致眼底自发荧光下可检测到的AS扩大。这些发现表明，AASR可能涉及一个两阶段的炎症过程：AS部位RPE/BM复合体的初始破坏引发局部脉络膜炎/外层视网膜炎，随后继发MEWDS和AS扩大。
本病例说明了PXE相关AS中的一种复发性炎症模式，其特征为AS部位伴有RPE/BM破坏的局部炎症和继发性MEWDS。这些发现突出了AASR的病理生理机制及其独特的影像学特征。
