Sturge-Weber-Syndrome with extreme ocular manifestation and rare association of upper airway angioma with anticipated difficult airway.

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.