Sturge-Weber Syndrome Complicating with Ipsilateral Hemangioma-liked Nasal Polyps

Abstract

Sturge-Weber syndrome (SWS), also known as encephalotrigeminal angiomatosis, was reported by Schirmer, Sturge and Weber in succession in mid-19th century. And it is a neurocutaneous disease classically presenting with a facial portwine stain in the ophthalmic distribution of the trigeminal nerve, glaucoma and vascular eye abnormalities, and an ipsilateral occipital leptomeningeal angioma. It is possible to observe nose and sinus involvement in SWS [1]. And there already have been some cases about SWS accompanying with chronic rhinosinusitis [2], but to our knowledge, there are still no cases describing SWS combining with ipsilateral chronic rhinosinusitis and unusual nasal polyps. Here, we report a case of SWS which incorporated with ipsilateral chronic rhinosinusitis and nasal polyps-more precisely hemangioma-liked nasal polyps. A half year ago, a 40-year-old Chinese man known to have SWS visited our department complaining of progressive nasal obstruction, recurrent purulent nasal discharge and anosmia many years. He had been treated with no success using antibiotics, topical nasal decongestants and steroid nasal sprays. He had a history of left-sided epilepsy (but he had been seizure-free for many years without any antiepileptic therapy), headache, right buphthalmos and glaucoma which led his right eye to blindness, and progressive left hemiparesis. Fortunately, he was not severe mental disabled, and could look after himself and communicate with others basically. There was no family history of epilepsy, mental retardation or a #neurocutaneous syndrome.

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