视网膜和视网膜色素上皮周边部与黄斑部联合错构瘤：影像学特征。
Peripapillary Versus Macular Combined Hamartoma of the Retina and Retinal Pigment Epithelium: Imaging Characteristics.
机构信息
Smt. Kanuri Santhamma Centre for Vitreo-Retinal Diseases.
Westmead Hospital, Sydney, New South Wales, Australia; Faculty of Medicine and Health Sciences, Macquarie University, Sydney, New South Wales, Australia; Save Sight Institute, University of Sydney, Sydney, New South Wales, Australia.
出版信息
Am J Ophthalmol. 2019 Apr;200:263-269. doi: 10.1016/j.ajo.2019.01.016. Epub 2019 Jan 26.
PURPOSE
To compare clinical, optical coherence tomography (OCT), and fundus autofluorescence (FAF) characteristics of peripapillary vs macular variants of combined hamartoma of the retina and retinal pigment epithelium (combined hamartoma).
DESIGN
Retrospective observational, comparative case series.
METHODS
Setting: Multicenter collaborative study.
STUDY POPULATION
Fifty eyes with a clinical diagnosis of combined hamartoma. Observational Analysis: A comparative analysis of color fundus photographs (CFPs), OCT, and FAF was performed for peripapillary and macular variants of combined hamartoma.
MAIN OUTCOME MEASURES
Pigmentation and OCT features of macular and peripapillary combined hamartoma.
RESULTS
The review of imaging from 50 eyes of 49 patients diagnosed with combined hamartoma identified 18 (36%) peripapillary lesions, 27 (54%) macular lesions, and 5 (10%) peripheral lesions. A comparative analysis of peripapillary vs macular combined hamartoma identified differences in the following features: lesion pigmentation on CFPs corresponding to hypoautofluorescent FAF (88% vs 0%, P < .001) and OCT features of full-thickness involvement (88% vs 3%, P < .001), preretinal fibrosis (27% vs 81%, P < .001), maxi peaks (5% vs 88%, P < .001), intraretinal cystoid spaces (72% vs 40%, P < .038), outer plexiform layer involvement (5% vs 96%, P < .001), ellipsoid zone disruption (83% vs 3%, P < .001), RPE disruption (77% vs 3%, P < .001), and choroidal neovascularization (16% vs 0%, P = .028).
CONCLUSIONS
This comparative analysis identified a higher frequency of pigmentation with hypoautofluorescence, full-thickness retinal involvement, intraretinal cystoid spaces, ellipsoid zone disruption, RPE disruption, and choroidal neovascularization in peripapillary combined hamartoma. These findings suggest that lesions occurring near or at the optic nerve are associated with a more severe degree of pigmentary changes and retinal disruption than those located in the macula.
目的
比较视网膜和脉络膜色素上皮联合错构瘤（CHRPE）的视盘周围和黄斑变异型的临床、光学相干断层扫描（OCT）和眼底自发荧光（FAF）特征。
设计
回顾性观察性比较病例系列。
方法
地点：多中心合作研究。
研究人群
50 只临床诊断为 CHRPE 的眼睛。观察分析：对 CHRPE 的视盘周围和黄斑变异型进行彩色眼底照片（CFPs）、OCT 和 FAF 的对比分析。
主要观察指标
黄斑和视盘周围 CHRPE 的色素沉着和 OCT 特征。
结果
对 49 例 CHRPE 患者的 50 只眼的影像学检查发现 18 只（36%）视盘周围病变，27 只（54%）黄斑病变和 5 只（10%）周边病变。对视盘周围与黄斑 CHRPE 进行比较分析，发现以下特征存在差异：CFPs 上的病变色素沉着对应于低自发荧光 FAF（88%对 0%，P<.001）和 OCT 全层受累特征（88%对 3%，P<.001），视网膜前纤维化（27%对 81%，P<.001），最大峰值（5%对 88%，P<.001），视网膜内囊样间隙（72%对 40%，P<.038），外丛状层受累（5%对 96%，P<.001），椭圆体带破坏（83%对 3%，P<.001），RPE 破坏（77%对 3%，P<.001）和脉络膜新生血管（16%对 0%，P=.028）。
结论
本对比分析发现，视盘周围 CHRPE 色素沉着伴低自发荧光、全层视网膜受累、视网膜内囊样间隙、椭圆体带破坏、RPE 破坏和脉络膜新生血管的频率更高。这些发现表明，发生在视神经附近或视神经上的病变与黄斑病变相比，色素沉着和视网膜破坏的程度更严重。
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