Orofacial manifestation of Sturge-Weber syndrome: A case report with review

Sturge-Weber syndrome (SWS), sometimes referred to as encephalotrigeminal angiomatosis, is a rare congenital neurological and skin disorder. It is one of the phakomatoses which is often associated with port-wine stains of the face, glaucoma, seizures, mental retardation, and ipsilateral leptomeningeal angioma. It is characterized by proliferation of arteries of the brain, resulting in multiple angiomas that occur on the same side due to arteriovenous malformations. Normally, only one side of the head is affected and mainly results from the errors in both the mesodermal and ectodermal development. Here, we report a rare case of SWS having oral and facial manifestations, where an 8-year-old boy had actually come for treatment of swollen gums, and on examination, we found that he had typical signs and symptoms of this rare developmental anomaly. The main purpose of this publication is to make the clinician aware of the oral manifestations that are seen in the patients with SWS and also to lay stress on the importance of radiographs in the diagnosis of this syndrome