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doi:10.1378/chest.07-0458
(Chest. 2007; 132:1977-1986)
© 2007 American College of Chest Physicians
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American College of Chest Physicians Consensus Statement on the Respiratory and Related Management of Patients With Duchenne Muscular Dystrophy Undergoing Anesthesia or Sedation*

David J. Birnkrant, MD, FCCP; Howard B. Panitch, MD, FCCP; Joshua O. Benditt, MD, FCCP; Louis J. Boitano, RRT; Edward R. Carter, MD, FCCP; Valerie A. Cwik, MD; Jonathan D. Finder, MD; Susan T. Iannaccone, MD; Lawrence E. Jacobson, MD; Gary L. Kohn, MD, FCCP; Etsuro K. Motoyama, MD; Richard T. Moxley, MD; Mary K. Schroth, MD; Girish D. Sharma, MD, FCCP and Michael D. Sussman, MD

* From MetroHealth Medical Center, Case Western Reserve University (Dr. Birnkrant), Cleveland, OH; Children’s Hospital of Philadelphia (Dr. Panitch), University of Pennsylvania, Philadelphia, PA; University of Washington (Dr. Benditt and Mr. Boitano), Seattle, WA; Children’s Hospital (Dr. Carter), University of Washington, Seattle, WA; Muscular Dystrophy Association (Dr. Cwik), Tucson, AZ; University of Pittsburgh (Drs. Finder and Motoyama), Pittsburgh, PA; University of Texas Southwestern Medical School (Dr. Iannaccone), Dallas, TX; Shriner’s Hospital (Drs. Jacobson and Sussman), Portland, OR; University of Medicine and Dentistry of New Jersey (Dr. Kohn), Newark, NJ; University of Rochester (Dr. Moxley), Rochester, NY; University of Wisconsin (Dr. Schroth), Madison, WI; and Rush University School of Medicine (Dr. Sharma), Chicago, IL.

Correspondence to: David J. Birnkrant, MD, FCCP, Department of Pediatrics, MetroHealth Medical Center, 2500 MetroHealth Dr, Cleveland, OH 44109-1900; e-mail: dbirnkrant{at}metrohealth.org

Abstract

This statement on the management of patients with Duchenne muscular dystrophy (DMD) undergoing procedural sedation or general anesthesia represents the consensus opinion of a multidisciplinary panel convened under the auspices of the American College of Chest Physicians. Expert recommendations on this subject are needed for several reasons. First, patients with DMD have an increased risk of complications when they undergo sedation or general anesthesia. In addition, due to improved cardiopulmonary therapies, patients with DMD are experiencing an unprecedented duration of survival. As a result, it is more common for them to require procedures involving sedation or general anesthesia. The risks related to anesthesia and sedation for DMD patients include potentially fatal reactions to inhaled anesthetics and certain muscle relaxants, upper airway obstruction, hypoventilation, atelectasis, congestive heart failure, cardiac dysrhythmias, respiratory failure, and difficulty weaning from mechanical ventilation. This statement includes advice regarding the highly interrelated areas of respiratory, cardiac, GI, and anesthetic management of patients with DMD undergoing general anesthesia or procedural sedation. The statement is intended to aid clinicians involved in the care of patients with DMD and to be a resource for other stakeholders in this field, including patients and their families. It is an up-to-date summary of medical literature regarding this topic and identifies areas in need of future research.

Key Words: anesthesia • consensus statement • Duchenne muscular dystrophy • mechanical insufflation-exsufflation • neuromuscular diseases • noninvasive ventilation • sedation






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