Systemic Treatment Reduces Von-Hippel-Lindau–Associated Retinal Capillary Hemangioblastoma

A 24-year-old man with Von-Hippel-Lindau syndrome and central nervous system (CNS) hemangioblastomas was found to have multiple leaking retinal capillary hemangioblastomas in the superotemporal periphery of the left eye (A). Systemic treatment was initiated with Belzutifan, a hypoxia-inducible factor-2α inhibitor. There was marked improvement in the engorgement and tortuosity of the feeding arterioles and draining veins, and a reduction in the number and size of the retinal capillary and CNS hemangioblastomas after 6 months of treatment (B) (Magnified version of Fig A-B is available online at www.aaojournal.org).