Sturge weber syndrome: A case report & review of literature

Sturge Weber Syndrome, also known as encephalo trigeminal hemangiomatosis, is an uncommon congenital condition. It is characterized by hemangiomatous lesions of the face over one or more branches of the trigeminal nerve, calcifications of the leptomeninges over the cerebral cortex, and convulsions affecting the limbs. The primary defect is a developmental insult affecting precursors of tissues that originate in the promesencephalic and mesencephalic neural crest. The PORTWINE NEVI are usually the first component of the syndrome to be observed. Intraorally, the angiomatous lesions also involve the gingiva and palate. Treatment of the disorder is essentially a neurosurgical problem, although convulsions can sometimes be controlled by anticonvulsant drugs. This paper deals with a rare case of Sturge Weber Syndrome with characteristic neurological and orofacial features.