Total intravenous anesthesia with propofol and remifentanil followed by planned sedation with propofol alone in Intensive care for a patient with glutaric acidemia type-1 undergoing complex thoracolumbar scoliosis surgery.

A 15-year-old boy with a severe phenotype of Glutaric Acidemia Type 1 (GA-1) presented for planned major thoracolumbar scoliosis correction under general anesthesia. Propofol is relatively contraindicated in patients with GA-1 according to latest guidance because of the risk of propofol infusion syndrome. Because of previous use of propofol in the patient when admitted to intensive care and the potential benefits as a sleep agent we decided to use propofol for his surgery and postoperative sedation. We report the successful use of propofol for general anesthesia and postoperative sedation on intensive care.