RESUMO
BACKGROUND: Sturge-Weber syndrome is a sporadic phacomatoses with angiomas involving the leptomeninges and skin of the face, typically in the ophthalmic and maxillary distributions of the trigeminal nerve. Glaucoma is a common ocular manifestation. Presentation is typically at birth with a facial angioma. METHOD: Case note of a patient with a diagnosis of Sturge-Weber syndrome was used and the relevant literature reviewed. RESULT: A 56 year old woman with pain, photophobia in the left eye of 6 months duration. She had lost vision in the eye about 20 years earlier and was born with a dark patch on the left side of her face. She had no previous history of convulsion. On examination, she had a port-wine stain involving the left side of her face. Examination of the left eye revealed a visual acuity of no light perception with episcleral haemangioma. There was a relative afferent pupillary defect and fundoscopy revealed a pale pathologically cupped disc with tortuous retinal vessels. The intraocularpressure was elevated. CONCLUSION: Sturge-Weber syndrome is a rare phacomatoses which may present with ocular complications such as glaucoma. If glaucoma is left untreated decreased vision and blindness result. People of any age therefore with port-wine stain in the ophthalmic distribution of the trigeminal nerve should have yearly eye examination and measurement of intraocular pressure, regardless of whether they have symptoms or not.