Anesthesia Guidelines for Myotonic Dystrophy

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PRACTICAL RECOMMENDATIONS FOR SURGERY AND ANESTHESIA

Note:  The information below was provided by anesthesiologist, Brownell Payne, M.D. in Los Angeles, CA and neurologist, Tetsuo Ashizawa M.D., Chairman of Neurology at University of Florida in Gainsville, FL.

Important general points to remember are as follows:

  1. Complications are not proportional to the severity of the disease; they often arise in mildly affected patients.
  2. Even though the operation may be uneventful, serious, even fatal problems may occur in the post-operative period; especially when opiates have been given as analgesia.

Pre-operative:

  1. Cardiological assessment - ECG essential, 24 hour tape if any indication of arrhythmia from ECG or history
  2. Respiratory assessment: a) FEV1 and FVC both lying and standing. b) Chest x-ray, noting elevation of diaphragm(s) or areas of atelectasis c) arterial blood gases
  3. Pre-medication avoidance of opiates, caution with benzodiazepines

Peri-operative:

  1. Tendency to temporomandibular dislocation - care needed in manipulating jaw
  2. Induction: preferably gaseous; avoid hypnotic agents with slow metabolism such as thiopentone; adverse reactions have also been reported with propofol; lower doses are likely to be required
  3. Relaxation: a) Avoid suxamethonium b) Short acting non-depolarising muscle relaxants are best used and may be needed in smaller doses; recovery from these may be prolonged
  4. Neuromuscular monitoring is helpful
  5. ECG monitoring essential – risk of arrhythmias, capnograph
  6. Reversal – neostigmine may induce depolarization blockade
  7. To avoid postoperative shivering maintain normothermia; monitor core temperature
  8. Avoid K+ containing fluids

Post-operative (first 24 hours)

  1. Ensure respiration is fully re-established
  2. Cardiac monitoring *
  3. Respiratory monitoring – pulse oximetry, supplemented by ABG *
  4. Use of a high dependency bed is preferable *
  5. Early chest physiotherapy – these patients are especially prone to post-operative chest infections and atelectasis
  6. Minimal use of opiates, analgesia; instead, explore other methods of analgesia, eg. local anesthetic blocks or non-steroidal anti-flammatory agents and paracetamol

Summary:  Most problems occur when the disorder is unrecognized or when problems are not anticipated; it is worth considering whether regional anesthesia is a viable alternative or even if the surgical procedure is really necessary.

*The extent to which these precautions are taken will depend on the length and nature of the procedure.

REMEMBER: Problematic medications for DM patients:

  • Quinine, procainamide, tocainide (especially for those with cardiac problems)
  • General anesthesia: avoid thiopentone, suxamethonium, propofol, neostigmine, and halothane;  use fluranes, non-deporalizing blockers, spinal anestheasia, etc.
  • Liquid paraffin
  • Neuroleptics (including metoclopramide)
  • Opiates
  • Benzodiazepines

8/06