The Retina Reference

Sturge-Weber Syndrome

Sturge-Weber syndrome is a neurological condition that affects the development of blood vessels in the skin, eye, and brain. Patients with this disorder typically are born with a distinctive port-wine stain (pink/red stain) on the forehead, scalp, and or eyelids. Defects and vision loss usually occur on the same side as the skin lesion. Vascular brain lesions, diffuse choroidal hemangiomas, and unilateral secondary glaucoma are associated with this condition. This 59-year-old patient with Sturge-Weber Syndrome has a port-wine stain on his left face extending from his forehead towards his nose (white arrows). His vision at initial visit was 20/20 on the right and 20/400 on the left. He has glaucoma of the left eye and a diffuse choroidal hemangioma of the left eye (yellow arrows). The choroidal hemangioma was associated with overlying pigment epithelial and subretinal scarring (green arrow) associated with secondary choroidal neovascularization treated with photodynamic therapy without success in preserving visual acuity.