Desflurane and remifentanil anesthesia in a child with citrin deficiency: A case report.

General anesthesia of the shortest duration with the least metabolized drugs using desflurane and remifentanil, would be beneficial for rapid emergence in surgical patients with citrin deficiency. Maintenance of nitrogen scavenging medication, a protein-rich diet, and serial measurement of ammonia levels in the perioperative period are also important for avoiding hyperammonemia-related neurological dysfunction.