AI Summary & Key Takeaways
AI InsightIn this article, pre-anaesthetic preparations and general anaesthesia management of a patient with Morquio syndrome for whom decompression of craniocervical junction and surgical correction of C1-2 subluxation because of odontoid hypoplasia was planned is reported
📌 3 Key Takeaways
Abstract
Morquio syndrome is a subtype of mucopolysaccharidoses that is characterised by intracellular keratine sulphate accumulation and is inherited in an auto- somal recessive manner. This accumulation causes anatomical distortion in the upper airway and therefore results in difficult or impossible endotracheal intubation. In this article, pre-anaesthetic preparations and general anaesthesia management of a patient with Morquio syndrome for whom decompression of craniocervical junction and surgical correction of C1-2 subluxation because of odontoid hypoplasia was planned is reported
Özet
Morquio sendromu hücre içi keratin sülfat birikimiyle karakterize, otozo- mal resesif kalıtımla ortaya çıkan bir mukopolisakkaridoz alt tipidir. Bu birikim üst hava yollarında anatomik değişikliklere yol açarak zor veya imkansız endotrakeal entübasyona neden olmaktadır. Bu bildiride, Mor- quio sendromu olan, odontoid hipoplazi nedeniyle kranioservikal bileşke dekompresyonu ve C1-2 subluksasyonunun fiksasyonu planlanan bir has- tada yaptığımız preanestezik hazırlık ve genel anestezi yönetimi anlatıl- maktadır.