Bilateral optic nerve head hemangioma in Von Hippel Lindau disease: Report of a case with severe visual loss

Abstract: A 22 year-old woman presented with gradual visual loss in her right eye since 1990. Medical and family histories were unremarkable. Her visual acuity was 20/80 and 20/20 in right and left eyes respectively. Slit lamp examination was quite normal with no relative afferent papillary defect.Fundi of both eyes revealed subretinal lesion with optic nerve head involvement and subretinal fluid in papillomacular bundle with macular pucker in right eye. Whole body MRI revealed a large hemangioma in the spinal canal. In 1998 the patient ahsd significant visual loss at both eyes. This is the first report of Von hippel disease with bilateral optic nerve hemangioma in Iran.