Myotonic Dystrophy and Anesthetic Challenges: A Case Report and Review

Abstract

Providing anesthesia to patients with myotonic dystrophy (DM) can be very challenging due to the multisystemic effects of the disease and extreme sensitivity of these patients to sedatives, opioids, and anesthetic agents. Other factors such as hypothermia, shivering, or mechanical or electric stimulation during surgery can precipitate myotonia which is difficult to abolish and can lead to further complications. Generally, local or regional anesthesia is preferred to avoid the complications associated with general anesthesia in this group. However there are several case reports of successful use of general anesthesia (with or without volatile agents and with or without opioids). These general anesthetic cases led to postoperative admission to the regular floor or ICU. We present a case of a woman with a history of DM who underwent robotic assisted laparoscopic hysterectomy under general anesthesia and was discharged home on the same day.

Anesthetic management for a patient with myotonic dystrophy with remimazolam

“…Myotonic dystrophy is a rare but serious inherited disorder that may pose substantial problems for anesthetic management including the prolonged recovery after anesthesia and post-operative respiratory failure [ 1 ]. Therefore, we planned the anesthesia for this patient to avoid the delayed recovery from anesthesia safely.…”

Dexmedetomidine infusion for sedation in a patient with myotonic dystrophy*

“…Finally, smooth muscle involvement slows peristalsis in the gastro-intestinal tract, which increases the risk of aspiration and also leads to prolonged postoperative ileus. [3,4] Patients with myotonic dystrophy are exquisitely sensitive to drugs that cause respiratory depression, including intravenous and volatile anaesthetic agents; opioids; benzodiazepines; and neuromuscular blocking drugs [3]. Early onset of respiratory muscle weakness in the disease process results in alveolar hypoventilation and poor cough [3].…”

Use of sugammadex in patients with neuromuscular disorders: a systematic review of case reports

“…The dose of rocuronium have been very variable with these studies. Reduced doses (< 0.6 mg/kg) of rocuronium have been administered to aid intubation in majority of the cases in our review 3 [13,20,[43][44][45][46][47][48][49]. With these cases, the reversal times to TOF ratio of 0.9 with 2 mg/kg sugammadex ranged from 2 min [20,45,46] to 5 min [13,44,48].…”

“…Hence, monitoring TOF ratio as well as the recovery of first twitch height to baseline at both the muscles is recommended. reported delayed neuromuscular recovery time of 10 min [43,49], but no explanations were given. It is possible that slight underdosing of sugammadex could have contributed to the delay with Mangla et al [49].…”

“…reported delayed neuromuscular recovery time of 10 min [43,49], but no explanations were given. It is possible that slight underdosing of sugammadex could have contributed to the delay with Mangla et al [49]. Three authors had used 1 mg/kg rocuronium to aid intubation, possibly related to their rapid sequence induction [50][51][52].…”