RESUMO
A case report of rare congenital disease of encephalo-trigeminal angiomatosis [Sturge-Weber Syndrome] [SWS], is presented to alert the anesthesiologist of its potential risk. The case was anesthetized for emergency orthopedic surgery. Anesthesia management is directed towards observing the clinical manifestations of localized superficial skin lesions, extensive systemic involvement, evaluating its associated anomalies and anticipating difficult intubation due to angiomas of the mouth and upper airway and be a ware of concurrent therapy. The authors reviewed the English-language literature and reported findings regarding the SWS pathophysiologic features, interventions, and outcomes, with emphasis on issues relevant to anesthesiologists. This patient tolerated balanced anesthesia well. Anesthesia should be planned to avoid trauma to the hemangiomata, increases in intraocular and intracranial pressure, and be cognizent of current anticonvulsant therapy