Journal of Inborn Errors of Metabolism and Screening (May 2017)

Mitochondrial Disease and Anesthesia

  • Vincent C. Hsieh MD,
  • Elliot J. Krane MD,
  • Philip G. Morgan MD

DOI
https://doi.org/10.1177/2326409817707770
Journal volume & issue
Vol. 5

Abstract

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It is increasingly common for children with mitochondrial disease to undergo surgery and anesthesia. Although many different anesthetics have been used successfully for these patients, serious, unexpected complications have occurred during and following anesthetic exposure. This has led to the widespread opinion among anesthesiologists that mitochondrial patients are at increased risk from the stress of surgery and anesthesia. Defects in function of the mitochondrial electron transport chain can lead to striking hypersensitivity to volatile anesthetics in children. Despite this striking finding, the connection between mitochondrial function and response to anesthetics is unknown. We review here the anesthetic considerations for patients with mitochondrial defects. In addition, we present an approach to anesthetic care of these patients at our institutions.