Duchenne Muscular Dystrophy
Background
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X-linked recessive degenerative disease of skeletal & smooth muscle that usually first manifests in males of 2-5 years of age
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Becker muscle dystrophy is essentially a milder form of Duchenne
Considerations
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Possible difficult airway if macroglossia
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Aspiration risk: bulbar weakness, ↓ gastric motility
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Succinylcholine/volatile anesthetics contraindicated due to rhabdomyolysis/hyperkalemia risk, use total IV anesthesia
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Pulmonary:
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Possible obstructive sleep apnea:
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Sensitivity to sedatives/hypnotics
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Pulmonary hypertension/RV failure
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Restrictive lung disease from scoliosis & respiratory muscle weakness
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Risk of perioperative respiratory failure
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Impaired cough reflex (atelectasis, recurrent aspirations)
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Cardiovascular:
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Dilated cardiomyopathy:
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Tall R waves in precordial leads, ↑ R:S, deep Q in I, aVL, V5-6
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Mitral regurgitation common (due to papillary muscle involvement from LV dilation)
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Conduction defects & arrhythmias common (atrial, SVT, AV nodal)
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CNS: mild cognitive impairment is common
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Medications: ACE inhibitors, beta-blockers, steroids (may need stress dose)
Conflicts
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RSI vs. succinylcholine
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Prolonged postoperative ventilation
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Advanced directive discussions