ABSTRACT

Glutaric aciduria type 1 (GA-1) is an uncommon cerebral organic aciduria. The risk of acute encephalopathic crises and metabolic acidosis may present during anaesthetic and surgical or postsurgical periods. Few cases with GA-1 who underwent general anaesthesia have been reported previously, and there are no data on long-term anaesthetic management of patients with GA-1. We report the anaesthetic management of temporal tumour resection that lasted for 8 hours in a 16-year-old patient with GA1, and discuss the risks of malignant hyperthermia and propofol infusion syndrome.

Keywords: Glutaric aciduria type I, total intravenous anaesthesia, propofol infusion syndrome, malignant hyperthermia