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