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A review of anaesthetic outcomes in patients with genetically confirmed mitochondrial disorders

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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.

Conclusion: 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.

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Abbreviations

ASA:

American Society of Anaesthesiology

ATP:

Adenosine triphosphate

CVC:

Central venous catheter

GA:

General anaesthesia

NEC:

Necrotising enterocolitis

PEG:

Percutaneous endoscopic gastrostomy

TIVA:

Total intravenous anaesthesia

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Authors’ contributions

Dr. AS had primary responsibility for overall content and manuscript preparation. She was involved with the study design, research collection, research interpretation and manuscript preparation.

Dr. ED had was involved with the study design, research collection, research interpretation and manuscript preparation.

Dr. MM was involved in the collection and interpretation of data.

Dr. CO was involved in the collection and interpretation of data.

Dr. IK was involved with the data interpretation and manuscript preparation.

Dr. AM was involved with the data interpretation and manuscript preparation.

Dr. JH was involved with the data interpretation and manuscript preparation.

Dr. NE involved with the data interpretation and manuscript preparation.

Dr. EC was the supervisor for this study. She was involved with the overall content, study design, manuscript preparation, research collection and research interpretation.

Author information

Authors and Affiliations

  1. National Centre for Inherited Metabolic Disorders, Temple Street Children’s University Hospital, Dublin, Ireland

    A. Smith, M. Mannion, I. Knerr, A. A. Monavari, J. Hughes & E. Crushell

  2. Department of Anaesthesia, Temple Street Children’s University Hospital, Dublin, Ireland

    E. Dunne & N. Eustace

  3. Department of Metabolic Medicine, Our Lady’s Children’s Hospital, Crumlin, Dublin, Ireland

    C. O’Connor, J. Hughes & E. Crushell

Authors
  1. A. Smith
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  2. E. Dunne
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  3. M. Mannion
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  4. C. O’Connor
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  5. I. Knerr
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  6. A. A. Monavari
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  7. J. Hughes
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  8. N. Eustace
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  9. E. Crushell
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Corresponding author

Correspondence to A. Smith.

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Funding statement

This research received no specific grant from any funding agency in the public, commercial or not-for-profit sectors.

Conflict of interest

The authors declare that they have no conflict of interest.

Ethical approval

Ethical approval was sought and granted from the Department of Research, Temple Street Children’s University Hospital, Dublin. This article does not contain any studies with human participants or animals performed by any of the authors.

Additional information

Communicated by Beat Steinmann

Revisions received: 5 November 2016; 10 November 2016

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Smith, A., Dunne, E., Mannion, M. et al. A review of anaesthetic outcomes in patients with genetically confirmed mitochondrial disorders. Eur J Pediatr 176, 83–88 (2017). https://doi.org/10.1007/s00431-016-2813-8

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  • DOI: https://doi.org/10.1007/s00431-016-2813-8

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