Anesthetic management of a Rett syndrome patient with apnea and epilepsy: a case report

Abstract

Rett syndrome, which is a progressive, central nervous system disease that is caused by a gene mutation, is known to present with various symptoms. This case is that of a 15-year-old girl who was diagnosed with Rett syndrome at the age of 2 years. Laryngotracheal isolation under general anesthesia was planned due to recurrent aspiration pneumonia. Since the patient’s nutritional status and control of convulsions were good, this was deemed an appropriate time for the surgery. Following careful preoperative evaluation of her airway, we performed oral endotracheal intubation using a video laryngoscope after rapid induction. Since postoperative pain control was important to prevent apneic attacks and convulsions, we used a multimodal analgesic regimen including carefully titrated fentanyl, acetaminophen, nonsteroidal anti-inflammatory drug, and wound infiltration with a local anesthetic. Postoperatively, the patient returned to the intensive care unit under spontaneous ventilation and followed a good course. Patients with Rett syndrome present several symptoms. Thus, several points must be considered during the preoperative evaluation, anesthetic management, and postoperative care of these patients.