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Mitochondrial Disease

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Background 

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  • ​Definition: group of disorders that affect the function of mitochondria (ATP powerhouse of the cell) resulting in a complex multisystem disease of varying severity

    •  disorders can affect any system with any symptom and any mode of inheritance

    •   associated with ↑ lactate 

  • Prevalence = 1: 4000, most manifest by 20 y/o

  • Common procedures:

    •  muscle biopsies, MRI/CT scans, endo/gastrostomy creations, strabismus sx

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Considerations

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  • ​Potential effects on multiple organ systems:

    • CVS: cardiomyopathy, conduction abnormalities 

    • Resp: central hypoventilation, respiratory muscle weakness

    • GU: renal insufficiency

    • GI: hepatic insufficiency

    • Endo: DM, hypoparathyroidism

    • Heme: anemia, thrombocytopenia

  • Ascertain degree of organ involvement especially cardio-resp:

    • PFTs, CXR, ABG, ECG, Echo if available

  • Ensure relevant multi-disciplinary consultants are involved

  • ↑ Risk of metabolic encephalopathy

  • Altered effects of anesthetic medications:

    • Propofol: avoid when possible (especially prolonged infusions) due to ↑ risk of PRIS

    • Succinylcholine: avoid due to ↑ hyperkalemia risk

    • NDMR: unpredictable response

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Goals

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  • Overarching goal: Minimize metabolic stress of surgery​

    • consider trending lactate as marker of metabolic stress

  • Avoid: 

    • Hypotension, hypoxia, hypoglycemia, hypothermia

    • Lactate containing solutions (Ringer's)

    • Propofol, succinylcholine 

    • Prolong periods of fasting

  • Good post-operative analgesia plan (regional, multi-modal analgesia when possible)

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References 

  1. Desai, V. (2021). Mitochondrial disease and anaesthesia : WFSA - resources. Mitochondrial Disease and Anaesthesia. Retrieved November 20, 2021, from https://resources.wfsahq.org/atotw/mitochondrial-disease-and-anaesthesia/. 

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