Retinal Hemangioblastomas

von Hippel-Lindau (VHL) ocular disease is characterized by the retinal hemangioblastoma (formerly called retinal capillary hemangioma (RCH), retinal capillary hemangioblastoma, retinal angioma, or angiomatosis retinae), which was the initial finding described by the ophthalmologist, Eugen von Hippel, himself. It is also the most common tumor type seen in patients with VHL, with 80% of patients developing retinal hemangioblastomas (RHs) by age 80. Left untreated, or if treatment is delayed, they can cause significant visual morbidity and blindness. This chapter describes the pathophysiology, evaluation, diagnosis, and treatment of RHs. Other ophthalmic findings in patients with VHL, screening and surveillance guidelines, and special situations such as childhood and pregnancy, are covered as well.