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Unusual Retinal Vascular Proliferation in von Hippel-Lindau Disease

Farrah Islam, FCPS, FRCS1,2; Sarah Hull, MA, FRCOphth1,3; David C. Mansfield, PhD, FRCOphth4; Anthony T. Moore, MA, FRCS, FRCOphth1,3,5; Alan Bird, MD, FRCOphth3
[+] Author Affiliations
1Medical Retina Service, Moorfields Eye Hospital, London, United Kingdom
2Department of Vitreoretina, Al-Shifa Trust Eye Hospital, Rawalpindi, Pakistan
3UCL Institute of Ophthalmology, London, United Kingdom
4Department of Ophthalmology, Inverclyde Royal Hospital, Greenock, United Kingdom
5Department of Ophthalmology, University of California, San Francisco
JAMA Ophthalmol. 2016;134(9):1073-1074. doi:10.1001/jamaophthalmol.2016.2046.
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This case report describes a patient with a retinal vascular complex that most likely represents a developmental abnormality of the retinal vasculature, consistent with the role of von Hippel-Lindau disease in retinal vascular development.

Von Hippel-Lindau (VHL) disease is a rare, dominantly inherited, multisystem cancer syndrome.1 The most common ocular manifestation of VHL is multiple retinal capillary hemangioblastomas.2 It is also rarely seen with abnormal flat retinal vascular proliferation associated with fibrovascular tissue.3 We describe a patient with a retinal vascular complex that most likely represents a developmental abnormality of the retinal vasculature, consistent with the role of VHL disease in retinal vascular development. To our knowledge, this association has not been previously reported.

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Figure.
Results of Fundus Examination

A, Right eye. B, Fundus photograph of the left eye demonstrating an abnormal arrangement of retinal vessels radiating from the optic disc. C, Fundus fluorescein angiogram of the left eye highlighting the abnormal arrangement of retinal vessels with no evidence of vascular leakage or neovascularisation during the early to middle phase of the disease. There was fluorescein staining of the epiretinal membrane. D, Fundus fluorescein angiogram of the left eye during the late phase of the disease.

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