与视网膜色素上皮先天性肥大相关的良性叶状核内层增生

Tarquin Boca, Salomon Y. Cohen, Elsabeth Hermouet, M. Srour, A. Miere
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引用次数: 0

摘要

报告两例伴有先天性视网膜色素上皮细胞肥大(CHRPE)的内核层(INL)视网膜内良性肿瘤。 对两例病例进行多模式成像,包括眼底彩色和自动荧光摄影、结构和全脸光谱域光学相干断层扫描(SD-OCT)、OCT 血管造影、荧光素和吲哚青绿血管造影。 在一次常规检查中,在两名分别为 27 岁和 32 岁的男性患者的一只眼睛中发现了 INL 视网膜内肿瘤,并伴有多个 CHRPE 病变。多模态成像显示,位于INL的白色多灶性分叶状病变呈拱形扩展,可诊断为良性分叶状核内层增生(BLIP)。 BLIP是一种位于INL的罕见良性肿瘤,具有独特的形态,迄今仅有4例报道。本病例证实BLIP与CHRPE有关,提示RPE和视网膜内层同时发育异常。
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Benign Lobular Inner Nuclear Layer Proliferation associated with Congenital Hypertrophy of the Retinal Pigment Epithelium
To report two cases of benign intraretinal tumors of the inner nuclear layer (INL) associated with congenital hypertrophy of the retinal pigment epithelium (CHRPE). Multimodal imaging of two cases, including fundus color and autofluorescence photography, structural and en face spectral-domain optical coherence tomography (SD-OCT), OCT-angiography, fluorescein and indocyanine green angiography. During a routine examination, intraretinal tumors of the INL associated with several CHRPE lesions were observed in one eye of two male patients, aged 27 and 32 years, respectively. Multimodal imaging showed white multifocal lobulated lesions with arching extensions, located in the INL, which allowed diagnosing benign lobular inner nuclear layer proliferation (BLIP). BLIP is a rare benign tumor located in the INL with a unique pattern and only 4 cases have been reported to date. The present two cases allowed confirming that BLIP is associated with CHRPE, suggesting a concomitant developmental anomaly of the RPE and the inner retina.
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