Sturge-Weber-Syndrome with extreme ocular manifestation and rare association of upper airway angioma with anticipated difficult airway.
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.
Universiti Kebangsaan Malaysia Medical Centre, Department of Opthalmology, Universiti Kebangsaan Malaysia, Jalan Yaacob Latif, Cheras, Kuala Lumpur 56000, Malaysia. whs1975@gmail.com
Hon Seng
HS
Wong
Ropilah
R
Abdul Rahman
Swee Ying
SY
Choo
Nurlia
N
Yahya
D000293:Adolescent; D005260:Female; D006801:Humans; D006871:Hydrophthalmos / Q000150:complications / Q000601:surgery*; D007442:Intubation, Intratracheal; D012142:Respiratory Tract Neoplasms / Q000150:complications*; D013341:Sturge-Weber Syndrome / Q000150:complications*