Anesthetic Management of a case with Rett Syndrome

Abstract

ÖZETRett sendromu, özellikle kızları etkileyen, ilerleyici genetik bir bozukluktur. Sıklıkla X'e bağlı bir gen olan MECP2'deki bir mutasyon nedeniyle olur. Kızlardaki mental retardasyonun nedenleri arasında Down sendromundan sonra ikinci sıradadır. Solunum bozuklukları, EEG ve EKG anormallikleri, spastisite, ileri evrelerde kas yıkımı ve distoni, periferik motor bozukluk, skolyoz preanestezik hazırlık ve anestezi uygulaması özen ve hassasiyet gerektirmektedir. Biz bu olgu sunumunda Rett sendromunda anestezik yaklaşımı tartışmak istedik.Anahtar kelimeler: Rett sendromu, malign hipertermi, anestezi SUMMARY Anesthetic Management of a case with Rett SyndromeRett syndrome (RS) is a progressive genetic disorder affecting mainly females. It is usually caused by a mutation in X-linked gene MECP2. After Down syndrome, Rett syndrome is the second leading cause of mental retardation seen in girls. Respiration disorders, EEG and EKG abnormalities spasticity, advanced muscle wasting and dystonia, peripheral motor dysfunction, scoliosos require precision, and attention in preoperative preparation and anesthetic management. We want to evaluate the anesthetic management of Rett Syndrome in this case presentation.

Anesthetic approach to a patient with Rett syndrome during tooth extraction

“…It is the second leading cause of mental retardation among girls following Down syndrome 2 . While children with RS show a normal or near-normal development until the 6th 18th months of their lives, they forget the skills they obtained with a rapid regression and then enter a long period of non-development.…”