THE USE OF SUGAMMADEX IN A PEDIATRIC PATIENT WITH TAY SACHS SYNDROME

Özet

Tay Sachs is an infrequently seen neurodegenerative, lysosomal sphingolipid storage disease. The disease has infantil, juvenil and adult forms. In the juvenile form, the patient may be normal until the age of 2 years, motor skills are lost around the age of 4 years. Sugammadex (Bridion; MSD, Oss, The Netherlands), is a cyclodextrin ring agent used in recent years for the recovery of neuromuscular block provided by rocuronium and vecuronium and to accelerate recovery. In this case report we aimed to present the use of sugammadex in the anesthesia approach to a patient diagnosed with Tay Sachs undergoing rigid bronchoscopy.

Abstract

Tay Sachs nadir görülen bir nörodejeneratif, lizozomal sfingolipid depolama hastal›¤›d›r. Hastal›¤›n infantil, juvenil ve eriflkin formlar› vard›r. Juvenil formunda hasta 2 yafl›na kadar normal olabilirken, motor becerileri 4 yafl civar›nda kaybolur. Sugammadeks (Bridion; MSD, Oss, Hollanda), son y›llarda rokuronyum ve vekuronyum taraf›ndan sa¤lanan nöromüsküler blo¤un geri dönmesini sa¤lamak ve h›zland›rmak için kullan›lan siklodekstrin halkal› bir ajand›r. Bu olguda rijid bronkoskopi yap›lan Tay Sachs sendromlu hastan›n anestezi yönetiminde sugammadeks kullan›m›n› sunmay› amaçlad›k.