Bilateral Optic Nerve Head Angiomas and Retrobulbar Haemangioblastomas in von Hippel-Lindau Disease

A 39-year-old man presented with progressive visual loss in both eyes. His past medical history was remarkable for biopsy-proven bilateral adrenal phaeochromocytomas. Ophthalmoscopy revealed an optic nerve head angioma and macular lipid exudates in the right eye and a regressed optic nerve head angioma in the left eye. A fluorescein angiogram revealed a lobulated pattern of leakage and venous staining from the optic nerve head angioma. Brain and orbital magnetic resonance imaging showed haemangioblastomas of the right intraorbital and left paraclinoid optic nerves and in the left cerebellopontine angle area. These findings were consistent with a clinical diagnosis of von Hippel-Lindau disease.