(1) Selçuk University Medical Faculty, Anesthesiology and Reanimation Department, Konya, Turkey
(2) Tepebaşı Teeth Hospital, Anesthesiology and Reanimation Department, Ankara, Turkey
* Corresponding author Email: email@example.com
Rett syndrome is a progressive developmental syndrome that occurs at the rate of 10,000–23,000 throughout the world and courses with autism, dementia, ataxia and loss of manual dexterity in girls. While children with Rett syndrome show a normal or near-normal development until the 6th to 18th months of their lives, they forget the skills they obtained with a rapid regression and then enter a long period of non-development. In this case report, a 26-year-old female patient undergoing operation for tooth extraction is presented.
26-year-old patient diagnosed with Rett syndrome was planned to undergo dental procedures under general anaesthesia. She had mental and motor developmental retardation and was not able to walk. Atrophia was present in all extremities with limitation in manual skills, bruxism and shouting episodes. For anaesthesia induction, propofol 3 mg kg-1, lidocain 1 mg kg-1, remifentanil 1 μg kg-1 were used at loading dose and tracheal intubation was performed with muscular relaxation by rocuronium 0.6 mg kg-1. In maintenance, propofol and remifentanil infusion was made. Overall 14 teeth underwent procedure without any complications. At the end of the procedure, propofol and remifentanil infusions were discontinued and patient extubated.
In patients with Rett syndrome, haemodynamic stabilisation should be maintained by vigilant monitorisation in the perioperative period due to the presence of malignant hyperthermia risk, apnoea attacks that may occur during respiration and sleep, difficulty in intubation and extubation, prolongation of QT in ECG and abnormal T waves, which is important for safe anaesthesia.