Dexmedetomidine infusion for sedation in a patient with myotonic dystrophy *

Summary 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.