Duchenne muscular dystrophy
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Duchenne Muscular Dystrophy (DMD) is an X linked recessive, progressive myopathy resulting form a dystrophin gene mutation. Despite the rarity of this disease it represents a significant anesthetic challenge for both pediatric and adult patients.
Anesthetic implications
Preoperative optimization
Intraoperative management
Postoperative management
Related surgical procedures
Pathophysiology
X linked recessive, progressive myopathy resulting form a dystrophin gene mutation. Located on chromosome Xp21. Typically diagnosed during the ages of 3 to 5 and most commonly in males. Survival of these patients is generally only 20 to 30 years, and they ultimately succumb to ventilatory failure or heart failure.
Signs and symptoms
Typically patients present with progressive proximal muscle weakness at a young age, first manifested as weakness and growth delay. This eventually develops into diaphragmatic weakness and subsequent respiratory distress.
Patients develop florid respiratory distress and cardiomyopathy in advanced stage of the disease.
The patients have associated difficult airway anatomy in the form of macroglossia and limited mobility of mandible and cervical spine. They also can develop contractures of bilateral upper and lower extremities.
These patients are at an increased risk of developing extreme hyperthermia, rhabdomyolysis and hyperkalemic cardiac arrest when exposed to halogenated inhalational anesthetics and depolarizing muscle relaxants