Retinal haemangioblastoma associated with peripheral non-perfusion: Widefield fluorescein angiography analysis of 41 cases

Aims To investigate the association of peripheral retinal non-perfusion with retinal haemangioblastoma. Methods 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. Results 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). Conclusion 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.