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Duchenne muscular dystrophy anaesthesia — FRCA Final MCQ

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HardPaediatricDuchenne muscular dystrophy anaesthesiaFRCA Final

A 12-year-old with Duchenne muscular dystrophy requires urgent fracture fixation. He has cardiomyopathy with ejection fraction 38%, weak cough and previous prolonged ventilation. The orthopaedic team request general anaesthesia. What is the most appropriate anaesthetic technique?

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Correct answer: E — Total intravenous anaesthesia avoiding suxamethonium and volatile agents

Duchenne muscular dystrophy is associated with life-threatening hyperkalaemic reactions to suxamethonium and rhabdomyolysis-like reactions with volatile agents. TIVA with careful respiratory and cardiac planning is usually preferred, with postoperative ventilatory support anticipated. Heavy sedation without airway control is unsafe in a child with weak cough and cardiomyopathy. Preoperative discussion should include ICU and cardiology input.

Reference: Association of Anaesthetists neuromuscular disease guidance; APAGBI paediatric anaesthesia guidance