What are the recommendations for anesthetic management in patients with mitochondrial disease undergoing surgery?

Anesthetic Management of Mitochondrial Disease

Patients with mitochondrial disease should receive trigger-free anesthesia with careful attention to fluid management using lactate-free solutions to prevent metabolic decompensation. 1

Preoperative Considerations

Risk Assessment

• Identify specific mitochondrial disorder type and affected organ systems
• Patients with Leigh's disease are at higher risk for complications 2
• Assess for pre-existing respiratory abnormalities, which increase risk of postoperative respiratory failure 2
• Evaluate cardiac function (cardiomyopathy and conduction abnormalities are common) 3

Preparation

• Minimize fasting time to prevent catabolism and lactic acidosis 1, 4
• Begin IV fluids during preoperative fasting period 1
• Standard premedication can be used (same as non-mitochondrial patients) 2

Anesthetic Machine Preparation

• Remove vaporizers before flushing anesthesia machine 2
• Change breathing circuit and soda lime to uncontaminated equipment 2
• Flush anesthesia machine with fresh gas flow of at least 10 L/min 2
• Follow manufacturer's recommendations for machine preparation time 2
• Consider using activated charcoal filters to reduce volatile anesthetic concentrations 2

Intraoperative Management

Anesthetic Technique

• Only trigger-free anesthesia should be used in all mitochondrial disease patients 2
• Total intravenous anesthesia (TIVA) is preferred over inhalational techniques 5
• Avoid prolonged propofol infusions due to risk of lactic acidosis 4, 6
• Use propofol cautiously and in limited amounts for induction 4
• Titrate all anesthetics incrementally while monitoring depth of anesthesia 4
• Succinylcholine is contraindicated due to risk of hyperkalemia 4
• Use non-depolarizing neuromuscular blockers judiciously 2

Fluid Management

• Use lactate-free IV fluids (5% dextrose-0.9% saline is preferred) 1
• Avoid Lactated Ringer's solution due to risk of worsening lactic acidosis 1
• Maintain normoglycemia throughout the perioperative period 1, 4
• Ensure adequate hydration to prevent catabolism 2, 1

Monitoring

• Standard monitoring plus continuous core temperature measurement 2
• Consider depth of anesthesia monitoring 2
• Monitor for signs of metabolic decompensation (acidosis, hyperlactatemia) 4
• Vigilant temperature management to prevent hypothermia or hyperthermia 4

Postoperative Care

• Standard recovery room care is appropriate 2
• Continue IV fluids until oral intake is adequate 1
• Monitor for delayed complications (respiratory failure, metabolic acidosis) 2
• Be vigilant for 24-48 hours postoperatively as metabolic decompensation may occur after apparent uneventful anesthesia 2

Special Considerations

• Outpatient surgery is acceptable if trigger-free anesthesia is used and national guidelines for ambulatory anesthesia are followed 2
• No specific pre- or postoperative blood tests are necessary 2
• Patients with mitochondrial disease are not at increased risk for malignant hyperthermia 4
• The inflammatory response to surgery may exacerbate mitochondrial dysfunction, particularly in the central nervous system 2

Common Pitfalls and Caveats

• Do not assume all mitochondrial disorders have the same anesthetic risk profile 1
• Avoid prolonged fasting which can precipitate metabolic crisis 1, 4
• Do not rely on Lactated Ringer's solution even in emergency situations 1
• Be aware that metabolic decompensation may occur hours to days after an apparently uneventful anesthetic 2
• Do not confuse mitochondrial disease management with malignant hyperthermia protocols 4