Desflurane and remifentanil anesthesia in a child with citrin deficiency

Abstract

Rationale: Hyperammonemia, metabolic derangement, and/or the prolonged effects of anesthetics may lead to delayed emergence from general anesthesia as well as the onset of type 2 citrullinemia, even in compensated patients with citrin deficiency.Patient concern: A 5-year-old girl with citrin deficiency was scheduled for blepharoplasty under general anesthesia. She developed hyperammonemia with temporary interruption of medication for a few days before surgery. Diagnosis:The patient was genetically diagnosed as citrin deficiency with a mutation in the SLC25A13 gene via newborn screening for metabolic disorders. Her citrulline and ammonia levels were well-controlled with arginine medication and protein-rich diet. Her elevated ammonia level by temporary interruption of medication was corrected with resumption of arginine medication and proteinrich diet before surgery.Interventions: We used desflurane and remifentanil for general anesthesia to avoid hyperammonemia and delayed emergence. End-tidal desflurane concentration and anesthetic depth were carefully monitored to avoid excessive anesthesia.Outcomes: She recovered consciousness with slightly increased ammonia level immediately after anesthesia.Lessions: 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 proteinrich diet, and serial measurement of ammonia levels in the perioperative period are also important for avoiding hyperammonemiarelated neurological dysfunction.Abbreviations: BIS = bispectral index, CTLN2 = type 2 citrullinemia, NICCD = neonatal intrahepatic cholestasis caused by citrin deficiency.