Chest
Volume 132, Issue 6, December 2007, Pages 1977-1986
Journal home page for Chest

Special Feature
Consensus Statement
American College of Chest Physicians Consensus Statement on the Respiratory and Related Management of Patients With Duchenne Muscular Dystrophy Undergoing Anesthesia or Sedation

https://doi.org/10.1378/chest.07-0458Get rights and content

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.

Section snippets

Executive Summary

Duchenne muscular dystrophy (DMD) is a progressive neuromuscular disease transmitted by X-linked inheritance with an incidence of approximately 1 in 3,500 live male births. DMD affects the muscles of respiration and is associated with dilated cardiomyopathy, which often leads to death from cardiopulmonary causes. Patients with DMD are especially vulnerable to the adverse physiologic effects of general anesthesia and procedural sedation, prompting the need for expert recommendations on this

Background

DMD is a progressive neuromuscular disease transmitted by X-linked inheritance with an incidence of approximately 1 in 3,500 live male births. DMD affects the muscles of respiration and is associated with dilated cardiomyopathy, which often leads to death from cardiopulmonary causes. With current medical management, including the use of nocturnally assisted ventilation, mean survival now approximates 25 years.1 Patients with DMD are especially vulnerable to the adverse physiologic effects of

A. Pulmonary Assessment

DMD is characterized by weakness of the diaphragm, intercostal muscles, and the accessory muscles of respiration, resulting in restrictive pulmonary impairment and a progressive decrease in total lung capacity and vital capacity.1011 These abnormalities lead to hypoventilation and impaired cough, which predisposes to atelectasis and respiratory failure. Before patients with DMD receive general anesthesia or procedural sedation, they should undergo measurement of Spo2 in room air, and

A. Choice of Pharmacologic Agents for General Anesthesia

Patients with DMD are at increased risk for extreme hyperthermic events and rhabdomyolysis when they are exposed to certain anesthetics, especially inhaled agents such as halothane, isofluorane, and servoflurane. Such episodes can cause hyperkalemia and sudden death from cardiac arrest.234 These events mimic malignant hyperthermia, but DMD and malignant hyperthermia are genetically distinct diseases. Succinylcholine, a depolarizing muscle relaxant that can disrupt unstable cell membranes, has

A. Respiratory Support

Extubation directly to NPPV should be considered for DMD patients with baseline FVC < 50% of predicted, and should be strongly considered for those with FVC < 30% of predicted who have been endotracheally intubated for general anesthesia or procedural sedation. Extubation directly to NPPV should also be considered for any patient using NPPV preoperatively (see Section II, D, above).2744 Continuous use of NPPV can then be weaned as tolerated,18 except in patients who require NPPV 24 h/d at

IV. Areas in Need of Further Study

The area explored in this consensus statement is characterized by a lack of prospective, randomized studies. Examples of specific studies needed to develop evidence-based guidelines for the care of DMD patients undergoing general anesthesia or procedural sedation include the following: prospective studies that identify the baseline pulmonary function parameters that predict an increased risk of postoperative respiratory complications for DMD patients undergoing different types of procedures;

Acknowledgments

We thank the leadership and staff of the ACCP and the Muscular Dystrophy Association for their support of this project.

References (48)

  • GN Schmidt et al.

    Acute heart failure during spinal surgery in a boy with Duchenne muscular dystrophy

    Br J Anaesth

    (2003)
  • E Servera et al.

    Respiratory muscle aids during an episode of aspiration in a patient with Duchenne muscular dystrophy

    Arch Bronconeumol

    (2005)
  • P Morris

    Duchenne muscular dystrophy: a challenge for the anesthetist

    Paediatr Anaesth

    (1997)
  • JD Finder et al.

    Respiratory care of the patient with Duchenne muscular dystrophy: an ATS consensus statement

    Am J Respir Crit Care Med

    (2004)
  • DJ Birnkrant

    New challenges in the management of prolonged survivors of pediatric neuromuscular diseases: a pulmonologist's perspective

    Pediatr Pulmonol

    (2006)
  • DJ Birnkrant et al.

    Noninvasive ventilation during gastrostomy tube placement in patients with severe Duchenne muscular dystrophy: case reports and review of the literature

    Pediatr Pulmonol

    (2006)
  • A Daftary et al.

    Effect of long-term steroids on cough efficiency and respiratory muscle strength in patients with Duchenne muscular dystrophy

    Pediatrics

    (2007)
  • WM King et al.

    Orthopedic outcomes of long-term daily corticosteroid treatment in Duchenne muscular dystrophy

    Neurology

    (2007)
  • RW Zickler et al.

    A simplified open gastrostomy under local anesthesia

    Am Surg

    (2001)
  • D Gozal

    Pulmonary manifestations of neuromuscular disease with special reference to Duchenne muscular dystrophy and spinal muscular atrophy

    Pediatr Pulmonol

    (2000)
  • LM Gauld et al.

    Prediction of childhood pulmonary function using ulna length

    Am J Respir Crit Care Med

    (2003)
  • JG Jenkins et al.

    Evaluation of pulmonary function in muscular dystrophy patients requiring spinal surgery

    Crit Care Med

    (1982)
  • B Milne et al.

    Anesthetic considerations in patients with muscular dystrophy undergoing spinal fusion and Harrington rod insertion

    Can Anaesth Soc J

    (1982)
  • Cited by (164)

    • Emergency planning as part of healthcare transition preparation for patients with Duchenne muscular dystrophy

      2021, Journal of Pediatric Nursing
      Citation Excerpt :

      Patients with DMD have inherent risks with anesthesia, including malignant hyperthermia and rhabdomyolysis when receiving some anesthetic agents, particularly inhaled agents such as halothane, isoflourane and servoflurane. Succinylcholine is known to cause hyperkalemia and acute rhabdomyolysis (Birnkrant et al., 2007). General anesthesia, such as intravenous propofol, is typically well tolerated.

    • NIV in Ventilatory Failure Due to Neuromuscular and Chest Wall Disorders

      2021, Encyclopedia of Respiratory Medicine, Second Edition
    View all citing articles on Scopus

    Dr. Cwik is a full-time employee of the Muscular Dystrophy Association. Dr. Schroth is a recipient of grant monies from and membership on the advisory committee of Families of Spinal Muscular Atrophy. Dr. Sussman is a recipient of grant monies from the Shriner's Hospital Research Fund.

    View full text