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Myotonic dystrophy is a neuromuscular condition inherited in an autosomal dominant fashion, and is most commonly diagnosed in the neonatal period. With improving levels of care, these patients are now presenting more commonly for anaesthesia. We review the clinical features of the condition, and then discuss the steps in the anaesthetic process, outlining the anaesthetic implications of myotonic dystrophy at each stage.
A 13-year-old boy with myotonic dystrophy underwent insertion of a percutaneous gastrostomy feeding tube under general anaesthesia. We used a laryngeal mask airway and a spontaneously breathing technique with propofol total intravenous anaesthesia. Postoperative vomiting and aspiration, 12 h after the procedure, subsequently required intubation and ventilation. We discuss the anaesthetic management of this case and review the features of the disease to be considered when contemplating anaesthesia in such patients.
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