Von Hippel - Angioma of optic disc

These are fundus photos from a patient with von Hippel-Lindau syndrome (VHL). This inherited condition, caused by mutations of the VHL gene, is associated with a propensity to grow tumors within the retina, brain, spinal cord, kidney, adrenal glands, and other organs. The tumors in the retina and brain are benign, but malignant tumors can develop in the kidney. This patient has had numerous brain surgeries in the past for central nervous system hemangioblastomas. He presented with no visual complaints and had a visual acuity of 20/20 in both eyes. His fundus images reveal a new angioma of the optic disc in the left eye (white arrow). His right eye was normal. No treatment was administered and he was asked to return in 3 months for continued observation. If growth or exudation occurs, intravitreal injection of bevacizumab is a possible treatment. When angiomas occur in the retinal periphery, laser photocoagulation or cryotherapy are commonly used. Occasionally photodynamic therapy or brachytherapy are used as second line treatment.