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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.
Islam F, Hull S, Mansfield DC, Moore AT, Bird A. Unusual Retinal Vascular Proliferation in von Hippel-Lindau Disease. JAMA Ophthalmol. 2016;134(9):1073-1074. doi:10.1001/jamaophthalmol.2016.2046