Year : 2021

Volume : Volume 12

Issue : Issue 1

Address for correspondence :
Dr. Madhulika Sarvepalli
madhulikasarvepalli@gmail.com

Abstract

Background:  Encephalotrigeminal Angiomatosis is an infrequent congenital disorder of vasculature of eyes, face and brain. It manifests with glaucoma, port wine stain over the face and seizures. It is commonly known as Sturge-Webber Syndrome (SWS) and comes under the group of “Phakomatosis”. There are three classic variants of Sturge-Weber Syndrome based on involvement of the eye, skin and central nervous system. They are - Complete Trisymptomatic, Incomplete Bisymptomatic (either oculocutaneous or neurocutaneous) and Incomplete Monosymptomatic (only neural or cutaneous involvement). This is a case report of a 23-year-old male patient with loss of vision in the right eye, port wine strain involving the complete right side of face and bilaterally over the neck, hypertrophy of the face with history of seizures. The intraoral findings were hemangioma of the right of soft palate, over the entire right buccal mucosa, right side of upper and lower labial mucosa, with hypertrophy of the right side of maxilla and deranged occlusion. Various radiological and hematological investigations like IOPAR, OPG, CT, CECT, MRI were performed. All the radiological features are suggestive of Sturge-Webber Syndrome.

Keywords: Encephalotrigeminal Angiomatosis, trigeminal nerve, dentistry.