Ocular Oncology Service, Wills Eye Hospital, Philadelphia, Pennsylvania, USA.
Ophthalmology, Mayo Clinic, Rochester, Minnesota, USA.
Br J Ophthalmol. 2020 Feb;104(2):167-172. doi: 10.1136/bjophthalmol-2019-314021. Epub 2019 May 16.
To investigate the association of peripheral retinal non-perfusion with retinal haemangioblastoma.
Medical and widefield fluorescein angiography records of patients diagnosed with retinal haemangioblastoma from 1990 to 2018 were reviewed for patient demographics, tumour features, fluorescein angiography features and characteristics of peripheral retinal non-perfusion.
There were 41 eyes of 40 patients with retinal haemangioblastoma imaged by widefield fluorescein angiography during this time period. Of 41 eyes, 14 (34%) had haemangioblastoma-associated peripheral retinal non-perfusion on fluorescein angiography. A comparison of eyes with versus without non-perfusion revealed younger mean age at presentation (28 vs 43 years old, p=0.05), increased prevalence of von Hippel-Lindau (VHL) disease (62% vs 22%, p=0.01), greater mean largest tumour basal diameter (3.7 vs 2.5 mm, p=0.04), greater tumour distance from optic nerve (8.4 vs 1.9 mm, p<0.01) and increased prevalence of vascular leakage from the tumour (86% vs 52%, p=0.03). After mean follow-up of 97 versus 71 months (p=0.52), eyes with non-perfusion were significantly more likely to develop neovascularisation (40% vs 0%, p<0.01) and experience a three-line or greater decrease in visual acuity (60% vs 11%, p<0.01).
Peripheral retinal non-perfusion can be associated with retinal haemangioblastoma, and could be more common with larger, more peripheral tumours in younger patients with VHL disease. Eyes with haemangioblastoma-associated peripheral non-perfusion could be more likely to develop neovascularisation and lose visual acuity.
研究视网膜周围无灌注与视网膜血管母细胞瘤的关系。
回顾 1990 年至 2018 年间经诊断患有视网膜血管母细胞瘤的患者的医疗和广角荧光素血管造影记录，以获取患者人口统计学、肿瘤特征、荧光素血管造影特征和周围视网膜无灌注特征的信息。
在此期间，通过广角荧光素血管造影对 40 例 41 只眼的视网膜血管母细胞瘤进行了成像。在这 41 只眼中，有 14 只（34%）眼在荧光素血管造影上存在与血管母细胞瘤相关的周围视网膜无灌注。对有和无无灌注的眼进行比较，发现无灌注眼的平均年龄较小（28 岁对 43 岁，p=0.05），von Hippel-Lindau（VHL）病的患病率较高（62%对 22%，p=0.01），肿瘤最大基底直径较大（3.7 毫米对 2.5 毫米，p=0.04），肿瘤距视神经的距离较远（8.4 毫米对 1.9 毫米，p<0.01），肿瘤的血管漏出率较高（86%对 52%，p=0.03）。在平均随访 97 个月与 71 个月（p=0.52）后，无灌注眼发生新生血管化的可能性明显更高（40%对 0%，p<0.01），视力下降超过 3 行的可能性也更高（60%对 11%，p<0.01）。
视网膜周围无灌注可与视网膜血管母细胞瘤相关，在患有 VHL 病的年轻患者中，更大、更外周的肿瘤可能更常见。与血管母细胞瘤相关的周围无灌注眼可能更容易发生新生血管化和视力丧失。
