What are the AAN/AANEM guidelines management of surgical complications of congenital muscular dystrophy?

Updated: Aug 17, 2020
  • Author: Twee T Do, MD; Chief Editor: Jeffrey D Thomson, MD  more...
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The AAN/AANEM guidelines recommend that before children undergo any surgical interventions and general anesthesia, physicians should discuss the potential increased risk of complications with patients' families, because these factors may affect decision-making regarding consent to certain elective procedures (level B). When children undergo procedures involving sedation or general anesthesia, physicians should monitor longer than usual in the immediate postoperative period to diagnose and treat respiratory, nutritional, mobility, and gastrointestinal mobility complications (level B). [77]

The International Standard of Care Committee recommends postoperative respiratory management by an experienced team including physical therapists to improve outcome. Nutritional aspects should be considered to ensure that maintenance of weight is achieved. Additional considerations for postoperative management and care include [76] :

  • Intensive pulmonary treatment in the following 6 months (insufflation techniques, prolongation of mechanical ventilation) may be needed
  • Self-feeding can be more difficult initially
  • Wheelchairs may need to be adapted to the changed shape of the child
  • Management of transfers, including the hoist and slings, must be considered
  • All aspects of postoperative activities of daily living should be addressed preoperatively by an occupational or physical therapist
  • Bracing may still be required after surgery, and it requires an experienced orthotist
  • Support of the head may also be needed
  • Pain management is needed
  • Long-term follow-up by spinal surgeons is needed given the child’s changing status
  • Increasing hyperextension of the neck is common in this group of disorders and needs to be monitored; strategies must be developed to alleviate the concomitant problems of function
  • Extension of the spinal surgery into the neck may be required

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