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POSTERIOR POLAR ANNULAR CHOROIDAL DYSTROPHY: A CASE SERIES

机译：后极环形脉络膜营养不良型：案例系列

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摘要

Purpose: To describe the multimodal imaging findings of posterior polar annular choroidal dystrophy (PPACD). Methods: Retrospective case series of 2 patients diagnosed with PPACD. Spectral-domain optical coherence tomography, fundus autofluorescence, and fluorescein angiog-raphy findings of PPACD are described. Electroretinography results are also presented. Results: Both patients presented with bilateral peripapillary atrophy extending to involve the temporal arcades in an annular, foveal sparing pattern. Both cases demonstrated outer retinal atrophy with foveal sparing on spectral-domain optical coherence tomography, and fluorescein angiography illustrated corresponding window defects with late staining. Fundus autofluorescence showed hypoautofluorescence in the center of atrophic areas but hyperautofluorescence at the leading edge. Electroretinography findings included cone loss with rod preservation. Inflammatory and infectious workup was unremarkable in both cases. Conclusion: This report describes the multimodal imaging findings of a rare and poorly described chorioretinal disorder, PPACD, and will serve to guide clinicians regarding the evaluation and management of this elusive condition.
机译：目的：描述后极环形脉络膜营养不良（PPACD）的多峰成像发现。方法：回顾性案例系列2例诊断PPACD患者。描述了光谱域光学相干断层扫描，眼底自体荧光和PPACD的荧光素angiog-raphy-raphy发现。还提出了电气识别结果。结果：两种患者均呈现双侧围毛绒萎缩，延伸以涉及环形，温度备件图案中的时间拱形。两种病例都展示了在光谱结构域光学相干断层扫描上的外视网膜萎缩，并且荧光素血管造影显示了具有晚期染色的相应窗口缺陷。眼底自发荧光在萎缩地区的中心显示出低荧光，但前沿的高荧光。电动图造影调查结果包括带杆保存的锥体损失。两种情况下炎症和传染性疗法都未解开。结论：本报告描述了稀有和难以描述的泡沫变性疾病，PPACD的多模式成像结果，并将用于指导临床医生关于这种难以捉摸的情况的评估和管理。

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