Flame-shaped epiretinal membranes: expanding the spectrum beyond neurofibromatosis type 2

IF 2.3 4区医学 Q1 OPHTHALMOLOGY

Canadian journal of ophthalmology. Journal canadien d'ophtalmologie Pub Date : 2026-08-01 Epub Date: 2026-03-05 DOI:10.1016/j.jcjo.2026.02.004

Meira Fogel Levin, Avner Hostovsky, Alessandro Feo, Melissa Yao, Ahmad Santina, Rania Estawro, Joo Yeon Jung, Neda Abraham, Stacy L. Pineles, David Sarraf

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引用次数: 0

Abstract

Objective

To characterize the spectral-domain optical coherence tomography (SD-OCT) features of flame-shaped epiretinal membranes (ERMs) in patients with and without neurofibromatosis type 2 (NF2).

Methods

This retrospective study included 9 patients (13 eyes) with flame-shaped ERMs from 3 tertiary centres. Multimodal imaging and clinical data were systematically analyzed.

Results

Six of 9 subjects with flame-shaped ERM were confirmed NF2 patients. Among the NF2 cohort of 12 eyes, SD-OCT revealed flame-shaped ERMs in 10 eyes (83%). Flame-shaped ERMs were bilateral in 4 patients (8 eyes) and unilateral in 2 patients (2 eyes). The fellow eyes of these 2 unilateral patients demonstrated combined hamartoma of the retina and retinal pigment epithelium (CHRRPE) and a retinal tuft, respectively. Isolated CHRRPE was noted in 3 eyes (25%) and a retinal tuft in 2 eyes (17%). Multiple retinal findings within the same eye occurred in 3 NF2 patients. By contrast, all 3 non-NF2 patients exhibited unilateral flame-shaped ERMs: 2 with CHRRPE and 1 isolated. OCT angiography performed in 4 patients revealed hyper-reflective foci anterior to the retinal nerve fibre layer in all 3 NF2 patients. During mean follow-up of 6.2 ± 3.4 years, findings remained stable in 78% of patients. One NF2 patient developed intraretinal cysts, while 1 non-NF2 patient underwent vitrectomy.

Conclusions

Flame-shaped ERMs are strongly associated with NF2, and bilateral retinal lesions were present in all NF2 patients. However, they can also occur unilaterally with or without CHRRPE in non-NF2 cases. Early recognition of flame-shaped ERMs enables timely and systematic NF2 evaluation.

查看原文本刊更多论文

火焰状视网膜前膜：扩展NF2以外的光谱。

目的：探讨2型神经纤维瘤病（NF2）患者火焰状视网膜前膜（ERMs）的光谱域光学相干断层扫描（SD-OCT）特征。方法：回顾性研究3个三级中心9例（13只眼）火焰状眼眼畸形。系统分析多模态影像及临床资料。结果：9例火焰状ERM患者中有6例为NF2患者。在12只眼的NF2队列中，SD-OCT在10只眼（83%）中显示火焰状erm。火焰状erm为双侧4例（8眼），单侧2例（2眼）。这2例单侧患者的同侧眼分别表现为视网膜和视网膜色素上皮（CHRRPE）合并错构瘤和视网膜簇状瘤。孤立性CHRRPE 3眼（25%），视网膜簇状2眼（17%）。3例NF2患者在同一只眼睛内出现多发性视网膜病变。相比之下，所有3例非nf2患者均表现为单侧火焰状erm: 2例伴有CHRRPE， 1例孤立。4例患者的OCT血管造影显示，所有3例NF2患者的视网膜神经纤维层前有高反射灶。在平均6.2±3.4年的随访中，78%的患者的结果保持稳定。1例NF2患者发生视网膜内囊肿，1例非NF2患者行玻璃体切除术。结论：火焰状erm与NF2密切相关，所有NF2患者均存在双侧视网膜病变。然而，在非nf2病例中，伴有或不伴有CHRRPE也可单侧发生。早期识别火焰状erm可以及时和系统地评估NF2。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Canadian journal of ophthalmology. Journal canadien d'ophtalmologie 医学-眼科学

CiteScore

3.20

自引率

4.80%

发文量

223

审稿时长

38 days

期刊介绍： Official journal of the Canadian Ophthalmological Society. The Canadian Journal of Ophthalmology (CJO) is the official journal of the Canadian Ophthalmological Society and is committed to timely publication of original, peer-reviewed ophthalmology and vision science articles.