Helmi Hala A, Alsaad Rakan, Alkhiary Hattan, Alkatan Hind M
Department of Ophthalmology, McGill University, Montreal, Canada; Department of Ophthalmology, King Faisal Specialist Hospital and Research Center, Riyadh, Saudi Arabia.
Department of Ophthalmology, College of Medicine, King Saud University, Riyadh, Saudi Arabia.
Int J Surg Case Rep. 2025 Feb;127:110867. doi: 10.1016/j.ijscr.2025.110867. Epub 2025 Jan 12.
Retinal capillary hemangioma (RCH) is a benign vascular hamartoma that can occur sporadically or as a manifestation of Von Hippel-Lindau (VHL) disease. If left untreated, it results in adverse ocular complications depending on its location and eventual visual loss.
We present a 50-year-old man who was a known case of VHL with history of left eye vision loss in the left eye at the age of 30 years. He underwent resection of a brain hemangioblastoma 10 years earlier, which was complicated by left facial nerve palsy. He presented with acute left eye pain, redness, and eyelid swelling. He had large corneal infiltrate with hypopyon and obscured fundus view. He was treated medically as a case of left endophthalmitis with no improvement. The eye was eventually eviscerated. Histopathological examination revealed acute necrotizing keratitis, osseous metaplasia, and long-standing RCH.
Peripheral RCHs are the most encountered ocular vascular lesion in VHL disease. Other locations close to the optic nerve (juxtapapillary) result in complicated visual loss. The RCH in our VHL case was confirmed 20 years after the history of vision loss in the same eye. The patient also had confirmed surgically treated intracranial hemangioblastoma but didn't seek any ophthalmic care prior to his recent presentation with painful acute keratitis and endophthalmitis.
VHL has multiple organ involvement. Patients suspected or diagnosed with VHL should receive prompt health care counselling to ensure periodic eye examination for control of any intraocular vascular lesions to prevent visual loss.
视网膜毛细血管瘤（RCH）是一种良性血管错构瘤，可散发出现，或作为冯·希佩尔-林道（VHL）病的一种表现。如果不进行治疗，根据其位置和最终导致的视力丧失情况，会引发不良的眼部并发症。
我们报告一名50岁男性，他是已知的VHL病患者，30岁时左眼视力丧失。10年前他接受了脑成血管细胞瘤切除术，术后并发左侧面神经麻痹。他因左眼急性疼痛、发红和眼睑肿胀前来就诊。他有大面积角膜浸润伴前房积脓，眼底视不清。按照左眼眼内炎进行药物治疗后无改善。最终该眼被摘除。组织病理学检查显示急性坏死性角膜炎、骨化生和长期存在的RCH。
周边RCH是VHL病中最常见的眼部血管病变。靠近视神经（乳头旁）的其他部位的RCH会导致复杂的视力丧失。我们这位VHL病患者的RCH在同一眼视力丧失病史20年后才得到确诊。该患者还确诊接受过颅内成血管细胞瘤手术治疗，但在近期因疼痛性急性角膜炎和眼内炎就诊之前未寻求任何眼科护理。
VHL病会累及多个器官。疑似或确诊为VHL病的患者应接受及时的医疗咨询，以确保定期进行眼部检查，控制任何眼内血管病变，防止视力丧失。
