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

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SUPPLEMENT ARTICLE

The 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

Department of Pediatrics, MetroHealth Medical Center, Case Western Reserve University, Cleveland, Ohio

The author has indicated he has no ﬁnancial relationships relevant to this article to disclose.

ABSTRACT

This is a summary of the presentation on the American College of Chest Physicians consensus statement on the respiratory and related management of patients with Duchenne muscular dystrophy undergoing sedation or anesthesia, presented as part of the program on pulmonary management of pediatric patients with neuromuscular disorders at the 30th Annual Carrell-Krusen Neuromuscular Symposium on Febru-ary 20, 2008.Pediatrics2009;123:S242–S244

T

HE CONSENSUS STATEMENTon the respiratory and related management of patients with Duchenne muscular dystrophy (DMD) undergoing anesthesia or sedation was based on work performed by a multidisciplinary panel of experts convened by the American College of Chest Physicians.1_{Permission was obtained from the journal}

Chestto present the contents of the statement at the Carrell-Krusen meeting and to submit this summary document for publication inPediatrics. The statement’s writing panel consisted of specialists in the areas of anesthesiology, critical care medicine, neurology, orthopedic surgery, pediatric and adult pulmonology, and respiratory therapy. The medical literature was reviewed by querying PubMed (www.pubmed. gov), a service of the National Library of Medicine and the National Institutes of Health. Consensus of recommendations was achieved by majority vote, and there were no disagreements regarding the final recommendations.

The need for this statement derives from the fact that persons with DMD are at high risk of major medical complications when they undergo general anesthesia (GA) or procedural sedation (PS).2_{In addition, survival has reached unprecedented}

levels among people with DMD, requiring them to undergo more procedures involv-ing GA and PS.3

DMD causes weakness of the respiratory muscles, resulting in impaired cough and a progressive decrease in total lung capacity and vital capacity, which makes patients especially vulnerable to the adverse physiologic effects of GA on the respiratory system.4 _{Airway patency can be compromised during PS or GA, which makes respiratory}

management complex.

The American College of Chest Physicians designated these recommendations to not be used for performance measurement or for competency purposes, because they are not evidence based.

PREOPERATIVE ASSESSMENT AND MANAGEMENT

For preoperative assessment, the panel chose tests that are widely available (avoiding the need for special equip-ment), objective and reproducible, simple to understand, and easy to measure (avoiding the need for specialized personnel). The suggested preoperative respiratory tests assess gas exchange, lung volume, and cough effectiveness. To assess gas exchange, the panel advised measuring the patient’s oxyhemoglobin saturation in room air by using pulse oximetry (SpO2). SpO2at⬍95% in room air was defined as a clinically significant abnormality, instigating the

need for measurement of the patient’s blood and/or end-tidal carbon dioxide levels. This SpO2threshold level was

chosen on the basis of consensus opinion and the threshold level identified by Bach et al5_{in their protocols for}

prevention of pulmonary morbidity in patients with neuromuscular diseases. To assess lung volume, the panel advised measuring the patient’s preoperative forced vital capacity (FVC) with the patient in the seated, upright body position. FVC is simple to measure and highly reproducible and, thus, was chosen in preference to more specialized measurements of lung volume. FVC was also chosen because low levels have been shown to predict respiratory complications in persons with DMD.6,7_{Therefore, a 2-tier risk-assessment method was devised to incorporate these}

FVC threshold levels. The panel’s consensus opinion was that patients with DMD are at increased risk of complica-www.pediatrics.org/cgi/doi/10.1542/ peds.2008-2952J

doi:10.1542/peds.2008-2952J

*The consensus statement1_{is summarized}

and excerpted here with permission.

Abbreviations

DMD—Duchenne muscular dystrophy GA— general anesthesia

PS—procedural sedation SpO2—pulse oxygen saturation

FVC—forced vital capacity NPPV—noninvasive positive-pressure ventilation

MEP—maximum expiratory pressure PCF—peak cough ﬂow

Accepted for publication Jan 5, 2009 Address correspondence to David J. Birnkrant, MD, MetroHealth Medical Center, Case Western Reserve University, Department of Pediatrics, Department of Pediatrics, 2500 MetroHealth Dr, Cleveland, OH 44109. E-mail: [email protected].

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tions of GA and PS if preoperative vital capacity is⬍50% of that predicted and at high risk of complications if preoperative FVC is ⬍30% of that predicted. These threshold values are used to identify patients with DMD at risk of postoperative respiratory complications; pa-tients who will benefit from preoperative training in noninvasive positive-pressure ventilation (NPPV) to op-timize the success of postoperative extubation facilitated by NPPV; and patients who need NPPV during induction of and recovery from GA throughout PS. This 2-tiered risk-assessment analyzer is one of the innovations of the consensus statement, as was the emphasis on a proactive approach to the use of assisted ventilation during recov-ery from GA and throughout PS in patients with DMD and low baseline pulmonary function. This strategy has the potential to be applied to patients with chronic re-spiratory insufficiency resulting from diagnoses other than DMD when such patients require GA or PS.

In the consensus statement, the authors advocated pre-operative assessment of cough strength and effectiveness by measuring maximum expiratory pressure (MEP) and peak cough flow (PCF).8,9_{For patients with a preoperative}

PCF of⬍270 L/minute or MEP of⬍60 cm H2O, the panel

advocated preoperative training in and postoperative use of manual and mechanically assisted cough (ie, mechanical insufflation/exsufflation with the CoughAssist device [Re-spironics, Murrysville, PA]). The recommended preopera-tive evaluation also includes cardiac consultation,10

nutri-tional and gastrointestinal evaluation,11 _{and discussions}

with patients and their families regarding their attitudes toward prolonged mechanical ventilator dependency, tra-cheostomy, and advance directives.

INTRAOPERATIVE MANAGEMENT

The intraoperative use of depolarizing muscle relaxants or neuromuscular blockers, such as succinylcholine, is absolutely contraindicated in persons with DMD because of the risk of rhabdomyolysis, hyperkalemia, and cardiac arrest.12_{Inhalational anesthetics are associated with the}

risk of malignant hyperthermia-like reactions in persons with DMD, with the potential for cardiac arrest and sudden death.13_{Thus, the clear trend is away from the}

use of inhaled anesthetics for persons with DMD,14_{and a}

total intravenous anesthetic technique was advocated in the consensus statement.

To minimize the risk of cardiorespiratory complica-tions, the authors also advocated the presence of an anesthesiologist when patients with DMD undergo PS, with full monitoring and safety measures adhering to the guidelines of the American Academy of Pediatrics and the American Society of Anesthesiologists.15,16

Intra-operatively, SpO2 should be monitored continuously,

and whenever possible, end-tidal or blood carbon diox-ide levels should be measured. Medical procedures should be performed in the optimal setting and with the presence of a full complement of skilled personnel, in-cluding a respiratory therapist skilled in the manage-ment of NPPV. An ICU should be available for postop-erative monitoring and medical management.

Options for respiratory support during maintenance of GA or PS include endotracheal intubation, with use of

NPPV to facilitate extubation for selected patients; use of the laryngeal mask airway; and use of mechanical or manual NPPV via lip seal, face mask, or nasal interface. The statement’s authors identified patients with FVC of ⬍50% of that predicted as being at risk and patients with FVC of⬍30% of that predicted as being at high risk of needing assisted ventilation during induction of and recovery from GA and throughout PS.

POSTOPERATIVE ASSESSMENT AND MANAGEMENT

The statement advocated that, for patients with DMD who have been intubated for GA or PS, extubation to NPPV should be considered if the patient has a preoper-ative FVC of⬍50% of that predicted and strongly con-sidered for patients with preoperative FVC of⬍30% of that predicted. Patients who use NPPV chronically should also be extubated to NPPV postoperatively. Con-tinuous NPPV can then be weaned off or back to baseline hours of use per day. Patients with subthreshold FVC whose intraoperative respiratory support was accom-plished by using the laryngeal mask airway or with NPPV should also use NPPV postoperatively. Other rec-ommendations included delaying extubation until respi-ratory secretions are in control and SpO2 is normal or

baseline in room air; extubation to the patient’s usual home NPPV interface whenever possible; and cautious use of supplemental oxygen postoperatively, because oxygen therapy can mask hypoxemia caused by atelec-tasis, hypoventilation, or airway secretions without treating the underlying cause.

The authors advised that patients with preoperative MEP of⬍60 cm H2O or PCF at ⬍270 L/minute should

use manual and mechanically assisted cough postoper-atively. Mechanical insufflation/exsufflation can aug-ment cough, promote deep lung inflation, and treat or prevent atelectasis. Mechanical insufflation/exsufflation can be applied via the endotracheal tube before extuba-tion.

It is important to provide patients with adequate pain control. If sedation occurs as a result of the use of nar-cotics, extubation can be delayed, or NPPV can be used continuously to provide respiratory assistance. When possible, it is desirable to avoid GA when patients with low pulmonary function require procedures. For exam-ple, neuraxial techniques (eg, epidural catheters) can be used in selected patients to achieve pain control while minimizing sedation and respiratory depression. Patients with DMD are at risk of postoperative congestive heart failure, dysrhythmias, and inadequate cardiac output because of fluid shifts caused by transfusions and intra-venous fluid administration. Consider postoperative car-diology consultation, as well as attention to fluid balance and intensive cardiopulmonary monitoring.

Gastrointestinal, nutritional, and pulmonary man-agement are closely interrelated. Bowel regimens should be initiated to treat or avoid constipation and associated inhibition of diaphragmatic excursion. Consider the use of prokinetic gastrointestinal medications and gastric de-compression with a nasogastric tube for patients with gut dysmotility to avoid the adverse effects of gastric insufflation on the diaphragm. Parenteral nutrition or

PEDIATRICS Volume 123, Supplement 4, May 2009 S243

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enteral feeding via a small-diameter tube early in the postoperative course is advised to avoid malnutrition and associated respiratory muscle weakness.

The consensus statement identified areas in need for future research, because this entire area is lacking in controlled, randomized studies.

REFERENCES

1. Birnkrant DJ, Panitch HB, Benditt JO, et al. American College of Chest Physicians consensus statement on the respiratory and related management of patients with Duchenne muscular dys-trophy undergoing anesthesia or sedation.Chest.2007;132(6): 1977–1986

2. Morris P. Duchenne muscular dystrophy: a challenge for the anaesthetist.Paediatr Anaesth.1997;7(1):1– 4

3. Birnkrant DJ. New challenges in the management of prolonged survivors of pediatric neuromuscular diseases: a pulmonolo-gist’s perspective.Pediatr Pulmonol.2006;41(12):1113–1117 4. Gozal D. Pulmonary manifestations of neuromuscular disease

with special reference to Duchenne muscular dystrophy and spinal muscular atrophy.Pediatr Pulmonol.2000;29(2):141–150 5. Bach JR, Ishikawa Y, Kim H. Prevention of pulmonary mor-bidity for patients with Duchenne muscular dystrophy.Chest. 1997;112(4):1024 –1028

6. Harper CM, Ambler G, Edge G. The prognostic value of pre-operative predicted forced vital capacity in corrective spinal surgery for Duchenne’s muscular dystrophy.Anaesthesia.2004; 59(12):1160 –1162

7. Goldberg A, Leger P, Hill N, et al. Clinical indications for non-invasive positive pressure ventilation in chronic respiratory failure due to restrictive lung disease, COPD, and nocturnal hypoventilation: a consensus conference report. Chest. 1999; 116(2):521–534

8. Szeinberg A, Tabachnik E, Rashed N, et al. Cough capacity in patients with muscular dystrophy. Chest. 1988;94(6): 1232–1235

9. Tzeng AC, Bach JR. Prevention of pulmonary morbidity for patients with neuromuscular disease. Chest. 2000;118(5): 1390 –1396

10. American Academy of Pediatrics, Section on Cardiology and Cardiac Surgery. Cardiovascular health supervision for individ-uals affected by Duchenne or Becker muscular dystrophy. Pediatrics.2005;116(6):1569 –1573

11. Iannaccone ST, Owens H, Scott J, Teitell B. Postoperative mal-nutrition in Duchenne muscular dystrophy. J Child Neurol. 2003;18(1):17–20

12. Larsen UT, Juhl B, Hein-Sorensen O, de Fine-Olivarius B. Complications during anesthesia in patients with Duchenne’s muscular dystrophy (a retrospective study). Can J Anaesth. 1989;36(4):418 – 422

13. Girshin M, Muherjee J, Clowney R, Singer LP, Wasnick J. The postoperative cardiovascular arrest of a 5-year-old male: an initial presentation of Duchenne’s muscular dystrophy. Paediatr Anaesth.2006;16(2):170 –173

14. Yemen TA, McClain C. Muscular dystrophy, anesthesia and the safety of inhalational agents revisited, again.Paediatr Anaesth. 2006;16(2):105–106

15. American Academy of Pediatrics; American Academy of Pedi-atric Dentistry; Cote´ CJ, Wilson S; Work Group on Sedation. Guidelines for monitoring and management of pediatric pa-tients during and after sedation for diagnostic and therapeutic procedures: an update.Pediatrics.2006;118(6):2587–2602 16. American Society of Anesthesiologists, Task Force on Sedation

and Analgesia by Non-anesthesiologists. Practical guidelines for sedation and analgesia by non-anesthesiologists. Anesthesi-ology.2002;96(4):1004 –1017

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DOI: 10.1542/peds.2008-2952J

2009;123;S242

Pediatrics

David J. Birnkrant

Dystrophy Undergoing Anesthesia or Sedation

Respiratory and Related Management of Patients With Duchenne Muscular

The American College of Chest Physicians Consensus Statement on the

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DOI: 10.1542/peds.2008-2952J

2009;123;S242

Pediatrics

David J. Birnkrant

Dystrophy Undergoing Anesthesia or Sedation

Respiratory and Related Management of Patients With Duchenne Muscular

The American College of Chest Physicians Consensus Statement on the

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