Anesthetic management of a patient with Rett Syndrome and distal humerus fracture: A case report and literature review

a b s t r a c t Rett Syndrome (RS) is a neurological disorder that almost exclusively affects females. RS is characterized by severe mental retardation following a period of apparent normal development in early childhood. The main clinical features include autism, spasticity, microcephaly, scoliosis, stereotyping, abnormal respiratory control and seizures. Patients with RS have been reported to be extremely sensitive to sedatives and exhibit a slow recovery from anesthesia. We describe and discuss the anesthetic management of a 26-year-old woman with RS and distal fracture of the humerus that underwent reduction and fixation with Kirschner’s wires under regional anesthesia and sedation. Intraoperatively, the patient experienced respiratory depression following the administration of 1 mg of I.V. midazolam. Our experience in this case led us to conclude and suggest that benzodiazepines should not be used in RS and in the absence of contraindications the technique of choice should be regional anesthesia.