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September 2016

Unusual Retinal Vascular Proliferation in von Hippel-Lindau Disease

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

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.