Myotonic Dystrophy and Anesthetic Challenges: A Case Report and Review

Table 5

Summary of anesthesia management in patient with myotonic dystrophy.

Preoperative	Assess the type and duration of surgery. If it is emergent or upper abdominal surgery, expected duration longer than 1 hour, have posted bed available in ICU, patient may require post-op mechanical ventilation; Assess patients muscle weakness by using MIRS score or Gilliam’s grading. If it is of higher grade, careful postoperative monitoring is required.; Check patient’s glucose levels; Twelve lead EKG and echocardiography because of higher risk of conduction defects.; Consider pulmonary function testing if there are cardiorespiratory symptoms.; Interrogation of cardiac rhythm management devices, if any.; Avoid premedication with opioids.; Consider antiaspiration prophylaxis with sodium citrate, H2 antagonist, metoclopramide.
Intraoperative	Choice of anesthesia local or regional if possible; If general, avoid muscle relaxants if the surgery does not require them.; If muscle relaxants are given, consider reversal.; Maintain normothermia using thermal blankets, fluid warmers, and higher operating room temperature.; Avoid shivering; Invasive blood pressure monitoring, neuromuscular block monitoring, and temperature monitoring along with standard ASA monitors.; Rapid sequence induction because of pharyngeal weakness and possibility of aspiration.; Avoid drugs like Succinylcholine and anticholinesterases like Neostigmine.; Have pacemaker/defibrillator in room if patient has conduction abnormalities.; Consider Sugammadex for reversal; Regional for postoperative pain control.
Postoperative	Continuous pulse oximetry and EKG monitoring for prolonged period.; Consider postoperative ventilation in patient with high risk factors of developing pulmonary complications.; Restrict use of opioids to minimal; Consider multimodal pain management.; Aggressive pulmonary toilet with incentive spirometry, chest physiotherapy, and cough assistant devices.