Anesthesia Considerations 

Duchenne Muscular Dystrophy 

 

 

Background 

 

• X-linked recessive degenerative disease of skeletal & smooth muscle that usually first manifests in males of 2-5 years of age

• Becker muscle dystrophy is essentially a milder form of Duchenne 

 

 

Considerations

 

• Possible difficult airway if macroglossia

• Aspiration risk: bulbar weakness, ↓ gastric motility

• Succinylcholine/volatile anesthetics contraindicated due to rhabdomyolysis/hyperkalemia risk, use total IV anesthesia​

• Pulmonary: 

  • Possible obstructive sleep apnea:

    • Sensitivity to sedatives/hypnotics

    • Pulmonary hypertension/RV failure 

  • Restrictive lung disease from scoliosis & respiratory muscle weakness

  • Risk of perioperative respiratory failure

  • Impaired cough reflex (atelectasis, recurrent aspirations)

• Cardiovascular:

  • Dilated cardiomyopathy:

    • Tall R waves in precordial leads, ↑ R:S, deep Q in I, aVL, V5-6

  • Mitral regurgitation common (due to papillary muscle involvement from LV dilation) 

  • Conduction defects & arrhythmias common (atrial, SVT, AV nodal)

• CNS: mild cognitive impairment is common

• Medications: ACE inhibitors, beta-blockers, steroids (may need stress dose) 

 

 

Conflicts

 

• RSI vs. succinylcholine

• Prolonged postoperative ventilation

• Advanced directive discussions