Anesthesia Regimen for Children with Muscular Dystrophy
Use total intravenous anesthesia (TIVA) with propofol and remifentanil infusions, strictly avoiding succinylcholine and preferably avoiding volatile anesthetic agents in children with muscular dystrophy undergoing surgery.
Critical Contraindications
- Succinylcholine is absolutely contraindicated in all children with dystrophinopathies (Duchenne and Becker muscular dystrophy), as it can trigger acute rhabdomyolysis and hyperkalemic cardiac arrest 1
- Volatile anesthetic agents should be avoided due to risk of rhabdomyolysis, hyperkalemia, and hyperthermia, though this remains somewhat controversial in older children 1
Recommended Anesthetic Technique
Induction Protocol
- Propofol bolus (3 mg/kg) combined with remifentanil bolus (1-2 mcg/kg) for anesthetic induction 2, 3
- Add atropine (0.01-0.02 mg/kg) to prevent bradycardia from remifentanil 3
- Tracheal intubation can be achieved without neuromuscular blocking agents using adequate propofol and remifentanil dosing 2
Maintenance Protocol
- Propofol continuous infusion at 200-250 mcg/kg/min 2, 3
- Remifentanil continuous infusion at 0.2-0.3 mcg/kg/min 2, 3
- Adjust infusion rates based on surgical stimulation and hemodynamic response 4
If Muscle Relaxation is Required
- Use non-depolarizing agents (vecuronium) with extreme caution and only when absolutely necessary 5
- Monitor neuromuscular blockade continuously with train-of-four monitoring throughout the procedure 5
- Reverse with pyridostigmine and glycopyrrolate at procedure completion 5
Adjunctive Techniques
- Consider intrathecal morphine for major spinal procedures to provide postoperative analgesia and facilitate rapid emergence 2
- Regional anesthesia blocks should be utilized whenever feasible to reduce opioid and anesthetic requirements 4
Monitoring Requirements
- Standard ASA monitors plus processed EEG monitoring (BIS or entropy) to guide anesthetic depth, particularly if neuromuscular blockade is used 4
- Continuous temperature monitoring to detect malignant hyperthermia-like reactions 1
- Cardiac monitoring given high prevalence of cardiomyopathy in dystrophinopathies 1
Key Advantages of TIVA Approach
- Rapid recovery with remifentanil's ultra-short context-sensitive half-time 2, 3
- Spontaneous ventilation returns quickly (within 8-10 minutes of stopping infusions), critical in patients with baseline respiratory compromise 3
- Avoids triggering agents for rhabdomyolysis and malignant hyperthermia-like reactions 2, 1
- Controlled hypotension can be achieved when needed for spinal surgery 2
Critical Pitfalls to Avoid
- Never assume older age (>8 years) makes volatile agents safe - while most complications in one series occurred in older children, this may reflect the patient population rather than true safety 1
- Do not underestimate baseline comorbidities - respiratory failure and cardiac disease are common and contribute significantly to perioperative complications independent of anesthetic choice 1
- Avoid manual bolus techniques - use continuous infusions or target-controlled infusion systems for stable drug delivery 3, 4