ABSTRACT

Rett 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.

Keywords: Rett syndrome, malign hyperthermia, anesthesia