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.
- fluorescein angiography
- non-perfusion von Hippel-Lindau
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Contributors Data collection and analysis was performed by LAD, DAA-L and MDY. Drafting of the manuscript by LAD. Critical revision of the manuscript by DAA-L, MDY, JSP, TWO and CLS. Conception of the project by JSP, TWO and CLS.
Funding Support provided in part by the Eye Tumor Research Foundation, Philadelphia, Pennsylvania, USA (CLS), an unrestricted grant from the Research to Prevent Blindness, Inc (LAD, JSP and TWO) and the Heed Ophthalmic Foundation (LAD). CLS, MD, has had full access to all the data in the study and takes responsibility for the integrity of the data and the accuracy of the data analysis.
Disclaimer The funders had no role in the design and conduct of the study, in the collection, analysis and interpretation of the data, and in the preparation, review or approval of the manuscript.
Competing interests None declared.
Patient consent for publication Not required.
Ethics approval Institutional Review Board/Ethics Committee approval was obtained from the Wills Eye Hospital (IRB #18-716E). The study adhered to the tenets of the Declaration of Helsinki and the Health Insurance Portability and Accountability Act (HIPAA).
Provenance and peer review Not commissioned; externally peer reviewed.
Data availability statement All data relevant to the study are included in the article or uploaded as supplementary information.
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