Dexmedetomidine infusion for sedation in a patient with myotonic dystrophy*

Myotonic dystrophy type 1 is the most common muscular dystrophy in adults. Anaesthetic management should take into consideration the numerous body systems affected, including the musculoskeletal; respiratory; cardiovascular; gastro-intestinal; and central nervous systems. A 42-year-old man with myotonic dystrophy presented for septoplasty and bilateral inferior turbinate reductions. He had severe upper and lower extremity myotonia and weakness, pulmonary impairment with non-obstructive patterns and first-degree atrioventricular block with reduced ejection fraction. He used bilevel positive airway pressure, a cough assist device and was paced 3% of the time with a single-chamber pacemaker. To reduce potential complications associated with opioid use and general anaesthetics, an opioid-free technique was planned using local anaesthetic infiltration and sedation with a dexmedetomidine infusion. The patient maintained spontaneous ventilation and haemodynamic stability, and had an uneventful postoperative course. Dexmedetomidine is a highly selective α2-adrenergic receptor agonist that has the ability to provide sedation, analgesia and anxiolysis with a stable haemodynamic profile. Avoiding both opioids and general anaesthetics in these patients may decrease the risk of peri-operative complications.