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November 23, 2009

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Consensus statement on the respiratory and related management of patients

with Duchenne muscular dystrophy undergoing anesthesia or sedation

Use of anesthesia or sedation in patients with Duchenne muscular dystrophy (DMD) can have severe adverse physiological effects including death. The December 2007 issue of Chest (Chest 2007; 132; 1977-1986) , the official publication of the American College of Chest Physicians (AACP), presented a consensus statement for the use of anesthesia or sedation in patients with DMD. This statement was convened by the American College of Chest Physicians' Pediatric Chest Medicine and home Care NetWork and was supported by the AACP and Muscular Dystrophy Association. The consensus panel consisted of experts in anesthesiology, critical care medicine, neurology, orthopedic surgery, pediatric and adult pulmonology and respiratory therapy. The statement addresses management of patients before, during and after the use of anesthesia or procedural sedation. Following are the recommendations of the panel.

Prior to general anesthesia or procedural sedation the following suggestion are made for evaluation and management:

• Obtain anesthesiology and pulmonology consultations.
• Perform pulmonary evaluation.
• Obtain clinical evaluation and optimization of cardiac therapies from a cardiologist.
• Nutritional assessment.
• Discuss risks and benefits with patient and guardians.

During general anesthesia or procedural sedation:

• Use intravenous sedation and avoid depolarizing muscle relaxants such as succinylcholine
• Optimize the setting and personnel and have an intensive care unit available.
• Provide respiratory support by a variety of means.
• Continuously monitor oxygen saturation.

After general anesthesia or procedural sedation:

• Remove intubation and provide noninvasive positive pressure ventilation immediately after surgery.
• Use supplemental oxygen therapy cautiously.
• Use manually assisted cough to clear secretions.
• Optimize pain control.
• Obtain cardiology consultation and closely monitor cardiac and fluid status.
• Avoid constipation.
• Provide nutrition if oral feeding is delayed for more than 24 hours.

The area of procedural sedation and anesthesia use in DMD patients lacks information obtained from prospective, randomized clinical trials. The above recommendations are the best conclusions the paned could arrive at lacking this source of information. It leaves a need for evidence from specific prospective studies to develop evidence-based guidelines for the care of DMD patients requiring procedural sedation or general anesthesia. Information from these evidence-based studies may enable the identification of patients who are most likely to benefit from or who are most likely to require specific interventions when anesthesia is required.