Intraoral hemangiomas in Sturge-Weber syndrome

Abstract

Sturge-Weber syndrome is a rare congenital disease also called as encephalo-trigeminal-angiomatosis, caused by persistence of transitory primordial arterio-venous connections of fetal intracranial vasculature. It is characterized by vascular malformations with capillary venous angiomas that involve face, choroid of eye, and leptomeninges. The main clinical features of this syndrome are port-wine stains, glaucoma, convulsions and angiomas of the airway. Anesthesia management is directed towards keeping the intracranial and intraocular pressures normal, avoiding trauma to hemangiomas, and anticipating difficult airway. We discuss the case of a 13-year-old boy with intraoral hemangiomas resulting in difficulty in speech. Patient was scheduled for digital subtraction angiography (DSA) and sclerotherapy of hemangiomas under general anesthesia. The anesthetic considerations during the peri-operative management, including airway problems, multiple changes in patient position during DSA procedure, precautions during use of radio-contrast media and sclerosing agents are highlighted.