ABSTRACT
Rett syndrome is a neurological disease that occurs only in females and it manifests with mental retardation, seizures, movement disorders, autistic behavior and abnormal breathing. A 19-year-old female with Rett syndrome underwent ophthalmologic surgery under general anesthesia at our institution. Airway control was difficult due to her limited mouth opening. We recommend that anesthesiologists should have proper knowledge about this disease and the patients to avoid the complications and problems that can be encountered during the perioperative period.
Subject(s)
Adult , Female , Humans , Young Adult , Airway Management , Anesthesia, General , Intellectual Disability , Mouth , Movement Disorders , Perioperative Period , Respiration , Rett Syndrome , SeizuresABSTRACT
A 6-year-old male patient who was suffering from a cold and a transient ischemic attack was scheduled to undergo encephalo-duro-arterio-synangiosis for treating his moyamoya disease. Acute brain edema occurred just after opening the dura mater. Head elevation, reduction of the head rotation and hyperventilation were done. The inhalational agents were discontinued and total intravenous anesthesia was started. The swelling was reduced after intravenously infusing mannitol. An abrupt return from hypocapnia to normocapnea during the induction of general anesthesia was thought to be the cause of the acute brain swelling. In conclusion, correction of hypocapnea needs to be performed gradually during the induction of anesthesia and when performing an operation for treating a patient with moyamoya disease.