title: A review of anaesthetic outcomes in patients with genetically confirmed mitochondrial disorders.
abstract: Mitochondrial disorders are a clinically and biochemically diverse group of disorders which may involve multiple organ systems. General anaesthesia (GA) poses a potential risk of decompensation in children with mitochondrial disorders, and there is little guidance for anaesthetists and other clinicians regarding the optimal anaesthetic agents and perioperative management to provide to patients with mitochondrial disease[15]. The aim of this review was to document adverse events and perioperative complications from GA in patients with genetically confirmed mitochondrial disorders. A retrospective chart review of patients with genetically confirmed mitochondrial disorders who had undergone GA was undertaken. The indication for GA, anaesthetic agents utilised, length of admission and post anaesthetic complications were documented and analysed. Twenty-six patients with genetically proven mitochondrial disease underwent 65 GAs. Thirty-four (52%), received propofol as their induction agent. Thirty-three (51%) patients received sevoflurane for the maintenance of anaesthesia, while 8 (12%) received isoflurane and 24 (37%) received propofol. The duration of most GAs was short with 57 (87%) lasting less than 1 h. Perioperative complications occurred in five patients while under GA including ST segment depression, hypotension and metabolic acidosis in one. All five patients were stabilised successfully and none required ICU admission as a consequence of their perioperative complications. The duration of hospital stay post GA was <24 h in 25 (38%) patients.
  
  
  CONCLUSIONS
  No relationship between choice of anaesthetic agent and subsequent perioperative complication was observed. It is likely that individual optimisation on a case-by-case basis is more important overall than choice of any one particular technique. What is Known: • General anaesthesia (GA) poses a potential risk of decompensation in children with mitochondrial disorders. • There is a great diversity in the anaesthetic approaches undertaken in this cohort, and little guidance exists for anaesthetists and other clinicians regarding the optimal anaesthetic agents and perioperative management to provide to patients with mitochondrial disease. What is New: • In this study of 26 patients with genetically confirmed mitochondrial disease who underwent 65 GAs, no relationship between choice of anaesthetic agent and subsequent perioperative complication was observed • It is likely that individual optimisation on a case-by-case basis is more important overall than choice of any one particular technique.
authors.affiliation: National Centre for Inherited Metabolic Disorders, Temple Street Children's University Hospital, Dublin, Ireland. smithai@tcd.ie.
authors.forename: A
authors.initials: A
authors.lastname: Smith
authors.affiliation: Department of Anaesthesia, Temple Street Children's University Hospital, Dublin, Ireland.
authors.forename: E
authors.initials: E
authors.lastname: Dunne
authors.affiliation: National Centre for Inherited Metabolic Disorders, Temple Street Children's University Hospital, Dublin, Ireland.
authors.forename: M
authors.initials: M
authors.lastname: Mannion
authors.affiliation: Department of Metabolic Medicine, Our Lady's Children's Hospital, Crumlin, Dublin, Ireland.
authors.forename: C
authors.initials: C
authors.lastname: O'Connor
authors.affiliation: National Centre for Inherited Metabolic Disorders, Temple Street Children's University Hospital, Dublin, Ireland.
authors.forename: I
authors.initials: I
authors.lastname: Knerr
authors.affiliation: National Centre for Inherited Metabolic Disorders, Temple Street Children's University Hospital, Dublin, Ireland.
authors.forename: A A
authors.initials: AA
authors.lastname: Monavari
authors.affiliation: National Centre for Inherited Metabolic Disorders, Temple Street Children's University Hospital, Dublin, Ireland.|Department of Metabolic Medicine, Our Lady's Children's Hospital, Crumlin, Dublin, Ireland.
authors.forename: J
authors.initials: J
authors.lastname: Hughes
authors.affiliation: Department of Anaesthesia, Temple Street Children's University Hospital, Dublin, Ireland.
authors.forename: N
authors.initials: N
authors.lastname: Eustace
authors.affiliation: National Centre for Inherited Metabolic Disorders, Temple Street Children's University Hospital, Dublin, Ireland.|Department of Metabolic Medicine, Our Lady's Children's Hospital, Crumlin, Dublin, Ireland.
authors.forename: E
authors.initials: E
authors.lastname: Crushell
mesh_terms: D000280:Administration, Inhalation; D061605:Administration, Intravenous; D000293:Adolescent; D000768:Anesthesia, General / Q000009:adverse effects*; D000778:Anesthetics, Dissociative / Q000008:administration & dosage / Q000009:adverse effects*; D018685:Anesthetics, Inhalation / Q000009:adverse effects*; D018686:Anesthetics, Intravenous / Q000008:administration & dosage / Q000009:adverse effects*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D007649:Ketamine / Q000008:administration & dosage / Q000009:adverse effects; D008297:Male; D008738:Methyl Ethers / Q000008:administration & dosage / Q000009:adverse effects; D028361:Mitochondrial Diseases / Q000150:complications* / Q000235:genetics*; D019990:Perioperative Care; D015742:Propofol / Q000008:administration & dosage / Q000009:adverse effects; D012189:Retrospective Studies; D000077149:Sevoflurane; D013874:Thiopental / Q000008:administration & dosage / Q000009:adverse effects
chemical_list: D000778:Anesthetics, Dissociative; D018685:Anesthetics, Inhalation; D018686:Anesthetics, Intravenous; D008738:Methyl Ethers; D000077149:Sevoflurane; D007649:Ketamine; D013874:Thiopental; D015742:Propofol
keywords: General anaesthesia; Metabolic disorder; Mitochondrial disorder; Paediatric anaesthesia