Case Report

Myotonic Dystrophy and Anesthetic Challenges: A Case Report and Review

Table 3

Anesthetic drugs and use in myotonic dystrophy patients.

DrugUse in DMAdverse effects/complicationsOther effects

PropofolDemonstrated to be safe in many reported cases [2, 14, 15]Can induce myotonia [16, 17]
Prolonged recovery after targeted controlled infusions. [11]
Sensitivity is altered. Less than 1mg/kg has been shown to be enough to induce general anesthesia and intubation by Speedy et al. [16]

ThiopentoneHas been used in multiple case reports/series without any adverse event [18].Shown to result in unexpected apnea/prolonged respiratory depression [3, 13]Prolonged apnea due to thiopentone seems to be idiosyncratic.

EtomidateUse has been described before [19] Continuous infusion not recommended due to steroid suppression [2, 20].

Volatile AnestheticsHas been reported to be used with success [21]Concern with deep inhalation anesthesia leading to shivering and compromised cardiac statusSome reluctance to use initially due to association with malignant hyperthermia (MH). But recent literature showed DM patients are no more susceptible to MH than general population. [22, 23]

Muscle relaxantsShorter and intermediate acting nondepolarizing agents are safe to be used.
Atracurium has been described to be used without reversal agent or prolonged muscle weakness [24].
Depolarizing muscle relaxants has been shown to induce myotonia leading to inability to ventilate and maintain the airway [3, 25].
Longer acting nondepolarizing agents have been shown to add to the weakness/respiratory depression. [25].
Avoidance of muscle relaxants, if possible, is recommended.

Reversal agentsSugammadex has been reported to be safe and has been used multiple times in recent past [26].Incomplete reversal and postoperative breathing difficulties have been noted in multiple case reports where neostigmine was used [18]Avoid use of anticholinesterases for reversal [27], exact mechanism not known.

DexmedetomidineSuccessful use of dexmedetomidine also has been reported to provide opioid free anesthesia in many recent case reports [8]none reported

OpioidsUse of shorter acting opioids is recommended for intraoperative period.Higher risk of respiratory depression.
Higher risk of postoperative ileus.
Avoid opioids in preoperative and postoperative period.

Total intravenous anesthesiaUse of propofol and remifentanil infusion have been reported before in cases of DM [13, 28]Remifentanil has been linked to postoperative hyperalgesia [29].