Sturge-Weber-Syndrome with Extreme Ocular Manifestation and Rare Association of Upper Airway Angioma with Anticipated Difficult Airway

Author: Hon Seng Wong ; Ropilah Abdul Rahman ; Swee Ying Choo ; Nurlia Yahya
Publication Type:Case Reports
From: The Medical Journal of Malaysia 2012;67(4):435-437
CountryMalaysia
Language:English
Abstract: We report a rare case of an 18 year old girl with Sturge- Weber syndrome, she had extensive facial port wine stains, right bupthalmos and advanced glaucoma involving both eyes. She underwent right eye glaucoma drainage device surgery under general anaesthesia, and had a difficult intubation due to extensive angiomatous like soft tissue swelling at her upper airway. This report highlights the importance of being aware of the need for continuous follow-up in Sturge-Weber syndrome patients as this syndrome can lead to blindness due to advance glaucoma and the awareness of possible difficult intubation for this group of patients.