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视乳头旁视网膜毛细血管瘤的治疗困境

Therapeutic Dilemma of a Juxtapapillary Retinal Capillary Hemangioma.

作者信息

Mohamad Nurul Munirah, Shokri Siti Sarah, Sukaimy Fatimah Suhaila, Wan Muda Wan Norliza, Ahmad Tajudin Liza Sharmini

机构信息

Department of Ophthalmology and Visual Science, Universiti Sains Malaysia School of Medical Sciences, Kubang Kerian, MYS.

Department of Ophthalmology and Visual Science, Hospital Tengku Ampuan Afzan, Kuantan, MYS.

出版信息

Cureus. 2024 Dec 29;16(12):e76598. doi: 10.7759/cureus.76598. eCollection 2024 Dec.

Abstract

A juxtapapillary retinal capillary hemangioma (JRCH) is a rare vascular hamartoma located on the optic nerve head or adjacent region. While often associated with von Hippel-Lindau (VHL) disease, JRCHs can also occur as an isolated condition, presenting unique therapeutic challenges and risks of visual impairment. We report a case of a 50-year-old Malay gentleman with diabetes mellitus who presented with a non-progressive superior visual field defect in his left eye for three months. Fundus examination revealed a raised reddish mass adjacent to the nasal optic disc, accompanied by macular exudates. Optical coherence tomography confirmed cystoid macular oedema, while fundus fluorescence angiography revealed feeder and draining vessels with late leakage and adjacent small vessel vasculitis. Systemic evaluation found no evidence of VHL or other abnormalities. Despite treatment with photodynamic therapy and intravitreal anti-vascular endothelial growth factor (anti-VEGF) injections, the patient's vision progressively deteriorated. This case highlights the diagnostic and therapeutic challenges associated with JRCH and emphasizes the need for early recognition and tailored interventions to mitigate significant visual impairment.

摘要

视乳头旁视网膜毛细血管瘤(JRCH)是一种罕见的血管错构瘤,位于视神经乳头或相邻区域。虽然JRCH常与冯·希佩尔-林道(VHL)病相关,但也可作为一种孤立病症出现,带来独特的治疗挑战和视力损害风险。我们报告一例50岁患有糖尿病的马来男性患者,其左眼出现非进行性上半视野缺损3个月。眼底检查发现鼻侧视盘旁有一隆起的红色肿物,并伴有黄斑渗出。光学相干断层扫描证实存在黄斑囊样水肿,而眼底荧光血管造影显示有供养血管和引流血管,晚期有渗漏及相邻小血管血管炎。全身评估未发现VHL或其他异常证据。尽管接受了光动力疗法和玻璃体内抗血管内皮生长因子(抗VEGF)注射治疗,患者视力仍逐渐恶化。该病例突出了与JRCH相关的诊断和治疗挑战,并强调需要早期识别和采取针对性干预措施以减轻严重的视力损害。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8ad9/11775493/9528b47a5f02/cureus-0016-00000076598-i01.jpg

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