title: A retrospective review of anesthesia and perioperative care in children with medium-chain acyl-CoA dehydrogenase deficiency.
abstract: BACKGROUND
  Medium-chain acyl-CoA dehydrogenase deficiency is the most common genetically determined disorder of mitochondrial fatty acid oxidation. Decompensation can result in hypoglycemia, seizures, coma, and death but may be prevented by ensuring glycogen stores do not become depleted. Perioperative care is of interest as surgery, fasting, and infection may all trigger decompensation and the safety of anesthetic agents has been questioned. Current guidelines from the British Inherited Metabolic Disease Group advise on administering fluid containing 10% glucose during the perioperative period.
  
  
  OBJECTIVE
  To review the management of anesthesia and perioperative care for children with medium-chain acyl-CoA dehydrogenase deficiency and determine the frequency and nature of any complications.
  
  
  METHODS
  A retrospective review of case notes of children with medium-chain acyl-CoA dehydrogenase deficiency undergoing anesthesia between 1997 and 2014.
  
  
  RESULTS
  Fourteen patients underwent 21 episodes of anesthesia. In 20 episodes, the patient received a glucose-containing fluid during their perioperative fast, of which eight received fluid containing 10% dextrose throughout the entire perioperative period. No episodes of hypoglycemia or decompensation occurred, but perioperative hyperglycemia occurred in five episodes. A propofol bolus was administered at induction in 16 episodes and volatile agents were administered for maintenance of anesthesia in all episodes without any observed complications. In one episode, delayed offset of atracurium was reported.
  
  
  CONCLUSIONS
  Perioperative metabolic decompensation and hypoglycemia appear to be uncommon in children who are well and receive glucose supplementation. Hyperglycemia may occur as a consequence of surgery and glucose supplementation. Propofol boluses and volatile anesthetic agents were used without any apparent complications. Prolonged action of atracurium was reported in one case, suggesting that nondepolarizing muscle relaxants may have delayed offset in this patient group. We do not recommend any particular approach to anesthesia but would advise administering glucose supplementation according to current guidelines, frequent monitoring of blood glucose perioperatively, and monitoring of neuromuscular blockade.
authors.affiliation: Department of Paediatric Anaesthesia, Royal Manchester Children's Hospital, Central Manchester University Hospitals NHS Foundation Trust, Manchester, UK.
authors.forename: Claire
authors.initials: C
authors.lastname: Allen
authors.affiliation: Department of Paediatric Anaesthesia, Royal Manchester Children's Hospital, Central Manchester University Hospitals NHS Foundation Trust, Manchester, UK.
authors.forename: Russell
authors.initials: R
authors.lastname: Perkins
authors.affiliation: Willink Biochemical Genetics Unit, Manchester Centre for Genomic Medicine, Central Manchester University Hospitals NHS Foundation Trust, Manchester, UK.
authors.forename: Bernd
authors.initials: B
authors.lastname: Schwahn
mesh_terms: D042964:Acyl-CoA Dehydrogenase / Q000172:deficiency*; D000293:Adolescent; D000758:Anesthesia / Q000379:methods*; D002648:Child; D002675:Child, Preschool; D005260:Female; D005947:Glucose / Q000008:administration & dosage; D006801:Humans; D006993:Hypnotics and Sedatives; D007223:Infant; D008052:Lipid Metabolism, Inborn Errors / Q000601:surgery*; D008297:Male; D019990:Perioperative Care / Q000379:methods*; D015742:Propofol; D012189:Retrospective Studies
chemical_list: D006993:Hypnotics and Sedatives; D042964:Acyl-CoA Dehydrogenase; D005947:Glucose; D015742:Propofol
keywords: anesthesia; blood glucose; child; humans; inborn errors; lipid metabolism; medium-chain acyl-CoA dehydrogenase