What anesthetic regimen is recommended for a patient with limb girdle muscle dystrophy (LGMD) undergoing surgery?

Anesthetic Management for Limb Girdle Muscular Dystrophy

Primary Recommendation

Use total intravenous anesthesia (TIVA) with propofol and remifentanil or fentanyl, and absolutely avoid succinylcholine and volatile anesthetics due to the risk of rhabdomyolysis and hyperkalemic cardiac arrest. 1, 2

Critical Drug Contraindications

Succinylcholine is absolutely contraindicated in LGMD patients due to the risk of:

• Acute rhabdomyolysis with massive potassium release 1, 3
• Hyperkalemic cardiac arrest that can occur immediately after injection 1, 3
• This applies even to previously undiagnosed cases 1

Volatile anesthetics (isoflurane, sevoflurane, desflurane) should be avoided or used with extreme caution given the similar risk profile to other muscular dystrophies, including potential for rhabdomyolysis 1, 2

Recommended Anesthetic Technique

For induction:

• Propofol 0.5 mg/kg followed by 10 mg every 10 seconds 2, 4
• Remifentanil bolus 1 mcg/kg 2

For maintenance:

• Propofol infusion (typically 100 mcg/kg/min, titrated to effect) 1, 4
• Remifentanil infusion at 0.25-0.5 mcg/kg/min 2, 5
• This TIVA approach provides rapid awakening and minimal respiratory depression 4

If muscle relaxation is required:

• Non-depolarizing agents such as rocuronium or vecuronium can be used cautiously 1, 4
• Mandatory quantitative neuromuscular monitoring throughout 6
• Consider sugammadex for reversal to eliminate residual neuromuscular blockade 6

Essential Preoperative Assessment

Pulmonary function testing is mandatory:

• Measure forced vital capacity (FVC), maximum inspiratory pressure (MIP), maximum expiratory pressure (MEP), and peak cough flow (PCF) 1, 3
• Patients with FVC <50% predicted require assisted ventilation during all phases of anesthesia 1, 3
• Patients with FVC <30% predicted are at particularly high risk and need ICU-level postoperative care 7

Cardiac evaluation is mandatory:

• Obtain cardiology consultation before any anesthetic 1, 3
• LGMD can cause cardiomyopathy and conduction abnormalities similar to other muscular dystrophies 7
• Assess for dilated cardiomyopathy and conduction defects 3

Nutritional assessment:

• Optimize nutritional status preoperatively 7
• Plan for enteral feeding if oral intake will be delayed >24-48 hours postoperatively 7

Intraoperative Monitoring

Continuous monitoring requirements:

• SpO₂ monitoring continuously throughout the procedure 1, 3
• End-tidal or blood CO₂ monitoring 1, 3
• Quantitative neuromuscular monitoring if muscle relaxants are used 6
• Cardiac monitoring for dysrhythmias and conduction abnormalities 7

Postoperative Management

Respiratory support:

• Continue SpO₂ monitoring for minimum 24 hours postoperatively 1, 3
• Use supplemental oxygen cautiously, as it can mask hypoventilation 7
• Monitor blood or end-tidal CO₂ levels whenever possible 7
• For patients with FVC <50% predicted, consider extubating directly to noninvasive positive pressure ventilation (NPPV) 7
• Delay extubation until respiratory secretions are well controlled and SpO₂ is normal or at baseline 7

Airway clearance:

• Use manually assisted cough and mechanical insufflation-exsufflation (MI-E) postoperatively in patients with impaired cough (PCF <270 L/min or MEP <60 cm H₂O) 7

Pain management:

• Optimize postoperative pain control, but monitor closely for sedation and hypoventilation 7
• Consider regional anesthesia techniques (e.g., intra-articular local anesthetic cocktails) to minimize opioid requirements 2
• If sedation or hypoventilation occurs, delay extubation for 24-48 hours or use NPPV 7

Cardiac monitoring:

• Obtain cardiology consultation and closely monitor cardiac and fluid status postoperatively 7
• Monitor for congestive heart failure and dysrhythmias 7
• Careful attention to fluid balance, especially after IV fluid boluses or blood transfusions 7

Gastrointestinal management:

• Initiate bowel regimens to prevent constipation 7
• Consider prokinetic GI medications 7
• Consider gastric decompression with nasogastric tube if GI dysmotility is present 7
• Start parenteral nutrition or enteral feeding via small-diameter tube if oral feeding is delayed for 24-48 hours 7

Level of Care

ICU admission criteria:

• Have an ICU bed available for postoperative care, especially for patients with FVC <50% predicted 1, 3
• Patients with significant baseline cardiopulmonary compromise require ICU-level monitoring for at least 24-48 hours 1, 3

Critical Pitfalls to Avoid

• Never use succinylcholine under any circumstances 1, 3
• Do not rely solely on clinical assessment for neuromuscular blockade reversal—use quantitative monitoring 6
• Avoid excessive supplemental oxygen that can mask hypoventilation 7
• Do not underestimate the risk of postoperative respiratory failure—these patients can deteriorate rapidly 7
• Recognize that LGMD patients may have limited mandibular and cervical spine mobility, making airway management more challenging 7