An Anaesthetic Approach To A Case With Emery-Dreifuss Muscular Dystrophy

A 13-year-old girl with known Emery-Dreifuss Muscular Dystrophy (EDMD) admitted to the emergency service and diagnosed as appendicitis. On physical examination, contractures of her elbows, Achilles tendons and posterior cervical muscles were noticed. She showed muscular wasting. Preoperative airway examination revealed a difficulty for endotracheal intubation. Following the induction with fentanyl (1 μg/kg) and propofol (3 mg/kg), an attempt for laryngoscopy was done without using a neuromuscular blocking agent. Intubation seemed to be apparently very difficult and a laryngeal mask was inserted. Anaesthesia was maintained with desflurane 4-5 % and 67 % nitrous oxide in O2. Neuromuscular block was established with atracurium (0.5 mg/kg) after being sure that airway was secured with the laryngeal mask. The blood pressure, heart rate and oxygen saturation of the patient were stable all through the operation. The patient recovered from neuromuscular block without administration of anticholinesterase. In conclusion, this case report illustrates the potential difficulty of endotracheal intubation. Desflurane anaesthesia combined with atracurium offers an uneventful periand postoperative course.