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DOI 10.1378/chest.07-0458 2007;132;1977-1986 Chest

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

Anesthesia or SedationDuchenne Muscular Dystrophy Undergoing and Related Management of Patients WithConsensus Statement on the Respiratory American College of Chest Physicians

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American College of Chest PhysiciansConsensus Statement on the Respiratoryand Related Management of PatientsWith Duchenne Muscular DystrophyUndergoing 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

This statement on the management of patients with Duchenne muscular dystrophy (DMD)undergoing procedural sedation or general anesthesia represents the consensus opinion of amultidisciplinary panel convened under the auspices of the American College of Chest Physi-cians. Expert recommendations on this subject are needed for several reasons. First, patients withDMD 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 anunprecedented duration of survival. As a result, it is more common for them to requireprocedures involving sedation or general anesthesia. The risks related to anesthesia and sedationfor DMD patients include potentially fatal reactions to inhaled anesthetics and certain musclerelaxants, upper airway obstruction, hypoventilation, atelectasis, congestive heart failure, cardiacdysrhythmias, respiratory failure, and difficulty weaning from mechanical ventilation. Thisstatement includes advice regarding the highly interrelated areas of respiratory, cardiac, GI, andanesthetic management of patients with DMD undergoing general anesthesia or proceduralsedation. The statement is intended to aid clinicians involved in the care of patients with DMDand to be a resource for other stakeholders in this field, including patients and their families. Itis an up-to-date summary of medical literature regarding this topic and identifies areas in needof future research. (CHEST 2007; 132:1977–1986)

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

Abbreviations: ACCP � American College of Chest Physicians; DMD � Duchenne muscular dystrophy;MEP � maximum expiratory pressure; MI-E � mechanical insufflation-exsufflation; MIP � maximum inspiratorypressure; NPPV � noninvasive positive pressure ventilation; PCF � peak cough flow; Spo2 � oxyhemoglobin saturationmeasured by pulse oximetry

Executive Summary

D uchenne muscular dystrophy (DMD) is a pro-gressive neuromuscular disease transmitted by

X-linked inheritance with an incidence of approxi-mately 1 in 3,500 live male births. DMD affects themuscles of respiration and is associated with dilatedcardiomyopathy, which often leads to death from

cardiopulmonary causes. Patients with DMD areespecially vulnerable to the adverse physiologic ef-fects of general anesthesia and procedural sedation,prompting the need for expert recommendations onthis topic. This consensus statement is the product ofa panel convened under the auspices of the Ameri-can College of Chest Physicians’ (ACCP) PediatricChest Medicine and Home Care NetWorks. The

CHEST Special FeatureCONSENSUS STATEMENT

www.chestjournal.org CHEST / 132 / 6 / DECEMBER, 2007 1977



panel consisted of specialists in the areas of anesthe-siology, critical care medicine, neurology, orthopedicsurgery, pediatric and adult pulmonology, and respi-ratory therapy. The most current and relevant med-ical literature was identified and reviewed, obtainedby querying PubMed, a service of the NationalLibrary of Medicine and the National Institutes ofHealth (www.pubmed.gov), which includes theMEDLINE database. Consensus of recommenda-tions was achieved through a majority vote of thepanel members, and there were no disagreements onany of the recommendations. The purposes of thisstatement are to aid clinicians involved in the care ofpatients with DMD undergoing procedures requir-ing sedation or general anesthesia, to be a resourcefor other stakeholders in this field, including patientsand their families, for use as an up-to-date summaryof medical literature on this topic, and to identifyareas in need of future research. The statement isdivided into sections on the assessment and manage-ment of patients before, during, and after proceduralsedation or general anesthesia.

Summary of Specific Suggestions for Evaluationand Management of Patients With DMD BeforeGeneral Anesthesia or Procedural Sedation

1. Obtain anesthesiology and pulmonology con-sultations before procedures involving generalanesthesia or procedural sedation.

2. Perform a pulmonary evaluation that includesmeasurement of FVC, maximum inspiratorypressure (MIP), maximum expiratory pressure(MEP), peak cough flow (PCF), and oxyhemo-globin saturation measured by pulse oximetry(Spo2) in room air. Measure the blood and/or

end-tidal carbon dioxide level if Spo2 is � 95%in room air. For DMD patients at increasedrisk of respiratory complications, defined by anFVC � 50% of predicted, and especially forpatients at high risk for complications, definedby an FVC � 30% of predicted, consider pre-operative training in the use of noninvasive posi-tive pressure ventilation (NPPV). For patients athigh risk of ineffective cough, defined in adults byPCF � 270 L/min or MEP � 60 cm H2O, con-sider preoperative training in manual and me-chanically assisted cough, emphasizing use ofmechanical insufflation-exsufflation (MI-E)with a bronchial secretion clearance device(CoughAssist; Respironics; Murrysville, PA).

3. Refer the patient to a cardiologist for clinicalevaluation and optimization of cardiac therapies.

4. Obtain a nutritional assessment, optimize nu-tritional status, and consider strategies to man-age dysphagia.

5. Discuss the risks and benefits of general anes-thesia or procedural sedation with the patientand guardians, and help them to decide onand implement their decisions regarding re-suscitation parameters and, if applicable, ad-vance directives.

Summary of Specific Suggestions for Managementof Patients With DMD During General Anesthesiaor Procedural Sedation

1. Consider use of a total IV anesthesia techniquefor induction and maintenance of general an-esthesia (eg, propofol and short-acting opioids).The use of depolarizing muscle relaxants suchas succinylcholine is absolutely contraindicatedbecause of the risk of fatal reactions.

2. Optimize the medical setting and personnel inattendance when DMD patients undergo gen-eral anesthesia or procedural sedation, andhave an ICU available for postprocedure care.

3. Options for providing respiratory support dur-ing maintenance of general anesthesia or pro-cedural sedation for patients with DMD in-clude endotracheal intubation, with use ofNPPV to facilitate extubation for selected pa-tients; use of the laryngeal mask airway; mechan-ical ventilation via a mouthpiece with leak-proofseal; and manual or mechanical ventilation(using conventional ventilators or bilevel posi-tive pressure ventilators designed for noninva-sive respiratory support) delivered via a fullface mask or nasal mask interface.

4. Application of ventilation in the assisted orcontrolled modes should be considered forpatients with DMD and an FVC � 50% of

*From MetroHealth Medical Center, Case Western ReserveUniversity (Dr. Birnkrant), Cleveland, OH; Children’s Hospitalof Philadelphia (Dr. Panitch), University of Pennsylvania, Phila-delphia, PA; University of Washington (Dr. Benditt and Mr.Boitano), Seattle, WA; Children’s Hospital (Dr. Carter), Univer-sity of Washington, Seattle, WA; Muscular Dystrophy Association(Dr. Cwik), Tucson, AZ; University of Pittsburgh (Drs. Finderand Motoyama), Pittsburgh, PA; University of Texas Southwest-ern Medical School (Dr. Iannaccone), Dallas, TX; Shriner’sHospital (Drs. Jacobson and Sussman), Portland, OR; Universityof Medicine and Dentistry of New Jersey (Dr. Kohn), Newark,NJ; University of Rochester (Dr. Moxley), Rochester, NY; Uni-versity of Wisconsin (Dr. Schroth), Madison, WI; and RushUniversity School of Medicine (Dr. Sharma), Chicago, IL.Dr. Cwik is a full-time employee of the Muscular DystrophyAssociation. Dr. Schroth is a recipient of grant monies from andmembership on the advisory committee of Families of SpinalMuscular Atrophy. Dr. Sussman is a recipient of grant moniesfrom the Shriner’s Hospital Research Fund.Manuscript received April 18, 2007; revision accepted July 16, 2007.Reproduction of this article is prohibited without written permissionfrom the American College of Chest Physicians (www.chestjournal.org/misc/reprints.shtml).Correspondence to: David J. Birnkrant, MD, FCCP, Department ofPediatrics, MetroHealth Medical Center, 2500 MetroHealth Dr,Cleveland, OH 44109-1900; e-mail: [email protected]: 10.1378/chest.07-0458

1978 Special Feature



predicted, and strongly considered for thosewith an FVC � 30% of predicted, during in-duction of and recovery from general anesthe-sia and throughout procedural sedation. Op-tions for respiratory support during inductionof and recovery from general anesthesia orprocedural sedation include manual ventilationusing a flow-inflated manual resuscitation bag(standard “anesthesia bag”) with a full face ornasal mask interface, and mechanical supportusing a conventional or noninvasive positivepressure ventilator via a full face or nasal mask.

5. Intraoperatively, monitor Spo2 continuouslyand, whenever possible, blood or end-tidalcarbon dioxide levels.

Summary of Specific Suggestions for Managementof Patients With DMD After General Anesthesia orProcedural Sedation

1. Consider extubating DMD patients with FVC� 50% of predicted, and especially those withFVC � 30% of predicted, directly to NPPV.Consider delaying extubation until respiratorysecretions are well controlled and Spo2 is nor-mal or baseline in room air. Continuous use ofNPPV can then be weaned as tolerated. Whenapplicable, try to utilize the patient’s homeinterface after extubation.

2. Use supplemental oxygen therapy cautiously.Monitor Spo2 continuously after general an-esthesia or procedural sedation. Wheneverpossible, monitor blood or end-tidal carbondioxide levels. Assess if hypoxemia is due tohypoventilation, atelectasis, or airway secre-tions, and treat appropriately.

3. Use manually assisted cough and MI-E postop-eratively in DMD patients with impairedcough, defined in adults as PCF � 270 L/minor MEP � 60 cm H2O.

4. Optimize postoperative pain control in patientswith DMD. If sedation and/or hypoventilationoccurs, delay endotracheal extubation for 24 to48 h or use NPPV.

5. Obtain a cardiology consultation and closely mon-itor cardiac and fluid status postoperatively.

6. Initiate bowel regimens to avoid and treatconstipation and consider prokinetic GI medi-cations. Consider gastric decompression with anasogastric tube in patients with GI dysmotility.Start parenteral nutrition or enteral feeding viaa small-diameter tube if oral feeding is delayedfor � 24 to 48 h postoperatively.

Areas in Need of Further Study

The statement identifies specific areas for futureresearch.

Background

DMD is a progressive neuromuscular diseasetransmitted by X-linked inheritance with an inci-dence of approximately 1 in 3,500 live male births.DMD affects the muscles of respiration and isassociated with dilated cardiomyopathy, which oftenleads to death from cardiopulmonary causes. Withcurrent medical management, including the use ofnocturnally assisted ventilation, mean survival nowapproximates 25 years.1 Patients with DMD areespecially vulnerable to the adverse physiologic ef-fects of general anesthesia and procedural seda-tion.2–4 For example, DMD patients have macro-glossia2 and weak upper airway dilator muscles.Sedation and general anesthesia cause relaxation ofthese muscles, which predisposes to upper airwayobstruction. Additionally, DMD patients may havelimited mobility of the mandible and cervical spine,which impedes maneuvers (such as the “jaw thrust”)that restore upper airway patency. General anesthe-sia results in decreased functional residual capacity,which can cause lower airway closure, atelectasis,and rapid deterioration in gas exchange. Hypercap-nia and hypoxemia can worsen chronic cardiopulmo-nary abnormalities found in some patients withDMD, such as pulmonary hypertension or cardiacconduction defects.

This statement arose from two primary consider-ations. First, patients with DMD and impaired pul-monary function are at high risk for death when theyundergo procedures requiring sedation or generalanesthesia.2 Second, because survival of patientswith DMD has reached an unprecedented leveldue to contemporary cardiorespiratory management,there is an increased need for these patients toundergo procedures.5 The purposes of this statementare to aid clinicians involved in the care of patientswith DMD who undergo procedures requiring seda-tion or general anesthesia; to be a resource for otherstakeholders in this field, including patients and theirfamilies; for use as an up-to-date summary of med-ical literature on this topic; and to identify areas inneed of future research.

While the primary purpose of this statement is toimprove the management and outcomes of patientswith DMD who undergo procedural sedation orgeneral anesthesia, the committee wishes to empha-size several related points. First, that efforts shouldbe made to optimize the timing of surgeries andprocedures such as scoliosis surgery or gastrostomyplacement so that patients undergo procedural seda-tion or general anesthesia as early in the diseasecourse as possible, preferably when cardiopulmonaryfunction is preserved.6 Second, that the increasinguse of glucocorticoids to treat patients with DMD




may preserve respiratory muscle strength7 and de-crease the need for procedures such as scoliosissurgery8 and gastrostomy placement, with the poten-tial to decrease the risk of death due to anesthesiaand sedation. Finally, whenever possible, alternativesto general anesthesia and procedural sedation shouldbe considered in patients with DMD who requireprocedures, such as the use of local anesthetics forpain control.9

Methodology and Structure

This consensus statement is the product of a panelconvened under the auspices of the ACCP PediatricChest Medicine and Home Care NetWorks. Thepanel consists of specialists in the areas of anesthe-siology, critical care medicine, neurology, orthopedicsurgery, pediatric and adult pulmonology, and respi-ratory therapy. The panel worked on this projectfrom January 2006 to January 2007, primarily viatelephone conference calls. The authors disclosedany conflicts of interest and were given completeautonomy by the ACCP. The panel was divided intoworking groups through which the most current andrelevant medical literature was identified and re-viewed, obtained by querying PubMed, a service ofthe National Library of Medicine and the NationalInstitutes of Health (www.pubmed.gov), which in-cludes the MEDLINE database. Only articles writ-ten in English were considered. There are fewrandomized, controlled trials involving the subject ofthis statement, so this document is a consensusstatement derived from expert opinion rather thanan evidence-based guideline. Consensus of recom-mendations was achieved through a majority vote ofthe panel members, and there were no disagree-ments on any of the recommendations. The state-ment is divided into sections on the assessment andmanagement of patients before, during, and afterprocedural sedation or general anesthesia. Eachsection consists of a review of the subtopic, followedby a list of specific suggestions. The ACCP Healthand Science Policy Committee designates that theserecommendations should not be used for perfor-mance measurement or for competency purposesbecause they are not evidence based.

I. Respiratory Support and RelatedMedical Management Before General

Anesthesia or Procedural Sedation(Preoperative)

A. Pulmonary Assessment

DMD is characterized by weakness of the dia-phragm, intercostal muscles, and the accessory mus-

cles of respiration, resulting in restrictive pulmonaryimpairment and a progressive decrease in total lungcapacity and vital capacity.10,11 These abnormalitieslead to hypoventilation and impaired cough, whichpredisposes to atelectasis and respiratory failure.Before patients with DMD receive general anesthe-sia or procedural sedation, they should undergomeasurement of Spo2 in room air, and measurementof the patient’s blood and/or end-tidal carbon dioxidelevel should be done if Spo2 is � 95% in room air.Additionally, DMD patients should undergo mea-surement of the following lung function parametersto assess their risk of respiratory complications andneed for perioperative and postoperative assistedventilation or cough.

FVC: FVC is the pulmonary function parametermost frequently reported to have predictive value inassessing the risk of respiratory complications forpatients with DMD. To determine predicted values,arm span is usually used to estimate height forpatients in a wheelchair, or predictive equationsbased on ulnar length can be used.12 The FVC isusually measured with the patient in a seated, up-right body position. FVC � 30% of predicted hasbeen identified as a predictor of postoperative respi-ratory complications and the need for postoperativeventilatory assistance among DMD patients under-going spinal fusion surgery.13–15 However, stud-ies16–18 suggest that the risks associated with spinalfusion surgery among DMD patients with FVC� 30% of predicted can be greatly reduced byfacilitating postoperative extubation with NPPV andby using MI-E to assist with cough. Additionally,percutaneous endoscopic gastrostomy placement hasbeen accomplished in DMD patients with vitalcapacity well � 30% of predicted through the use ofNPPV during induction of and recovery from anes-thesia.19 A previous consensus conference report20

recommended NPPV for patients with progressiveneuromuscular disease and FVC � 50% of predictedand symptoms of hypoventilation. It is the consensusof this panel that DMD patients with FVC � 50% ofpredicted measured in the seated, upright bodyposition are at increased risk for respiratory compli-cations when they undergo general anesthesia orprocedural sedation, and that patients with FVC� 30% of predicted are at high risk for complica-tions. While diaphragm strength can be relativelypreserved in DMD,10,11 patients are often confinedto the supine body position during and after surgery.Thus, measurement of both upright and supine FVCmay be useful because patients with poor supineFVC values will be especially vulnerable to postop-erative atelectasis and hypoxemia.




MIP, MEP, and PCF: MIP and MEP are used toassess respiratory muscle strength, and they haveclinical utility in DMD.4,10 Another useful preoper-ative test is the measurement of PCF.21 Impairmentof these parameters reflects an inability to generatethe cough force and velocity necessary for effectiveclearance of respiratory secretions.4,22 Patients with atracheostomy tube and assisted PCF � 160 L/minare at increased risk for failure to achieve tracheos-tomy tube decannulation, and adult DMD patientswho cannot generate a PCF � 270 L/min are atincreased risk for pneumonia or atelectasis.23–25

Moreover, young adults with DMD and MEP � 60 cmH2O are likely to have ineffective cough.26 Therefore,it is the consensus of the panel that DMD patientswith PCF � 270 L/min or MEP � 60 cm H2O are atincreased risk for respiratory complications whenthey undergo procedural sedation or general anes-thesia due to impaired cough. The data used todetermine these threshold values were obtainedfrom teenage and adult patients, and the values ofMEP or PCF that predict increased risk of impairedcough in young children are unknown.

B. Preprocedure Initiation of NoninvasiveRespiratory Aids

It is our consensus opinion that patients withDMD and FVC � 50% of predicted, and especiallythose with FVC � 30% of predicted, should beconsidered for preoperative training in the use ofNPPV due to their increased risk of respiratorycomplications. Preoperative training in NPPV shouldincrease the probability of successful use of NPPVduring recovery from general anesthesia or sedationand at postoperative endotracheal extubation (seesections II and III below).4,6,18,27,28 Similarly, adultpatients with PCF � 270 L/min or MEP � 60 cmH2O are at risk for ineffective cough, and preoper-ative training in manual and mechanically assistedcough (MI-E with a bronchial secretion clearancedevice) [CoughAssist; Respironics; Murrysville, PA])is suggested, using the techniques described in thereferenced articles.24,27,29

C. Cardiac Assessment

DMD is associated with the development of di-lated hypertrophic cardiomyopathy and cardiac dys-rhythmias. Patients with DMD are at high risk forperioperative cardiac side effects due to hypoxemia,anemia, and other causes of impaired tissue oxygendelivery. Intravascular fluid shifts can result in con-gestive heart failure and impaired ventricular pre-load. These issues are reviewed in the recent con-sensus statement30 of an expert panel convened bythe American Academy of Pediatrics that states that

patients with DMD should undergo a cardiac eval-uation and optimization of cardiac therapies beforeanesthesia. Preoperative consultation with a cardiol-ogist is advised for all patients with DMD becauseheart disease can be severe even among patients withonly mild pulmonary involvement, and normal pre-operative ECG and echocardiogram findings do notexclude the possibility of postoperative cardiac com-plications.31

D. Nutrition and GI Issues

Good nutritional support is integral to the propercare of patients with DMD, and the adverse effectsof malnutrition on respiratory muscle strength canbe profound.4 Therefore, preoperative nutritional sta-tus should be evaluated and optimized because poornutrition can increase postoperative morbidity.32,33 Op-timization of preoperative nutritional status mayinvolve the use of NPPV because patients withuntreated respiratory failure may become malnour-ished due to increased work of breathing, or theymay be unable to eat due to dyspnea. The preoper-ative evaluation should include measurements ofserum albumin and prealbumin to identify patientswho are at risk for poor healing. In addition, preop-erative evaluation and therapy for dysphagia shouldbe considered because loss of the ability to eatpostoperatively can lead to malnutrition.32

E. Advance Directives

DMD is a progressive and potentially fatal disease.Thus, advance directives (including resuscitation pa-rameters) and attitudes toward prolonged depen-dency on mechanical ventilation and tracheostomyshould be discussed with DMD patients and theirguardians preoperatively. Furthermore, decisions re-garding these issues should be clearly articulated andeasily accessible in the medical record.

Summary of Specific Suggestions for Evaluationand Management of Patients With DMD BeforeGeneral Anesthesia or Procedural Sedation

1. Obtain anesthesiology and pulmonology con-sultations before procedures involving generalanesthesia or procedural sedation.

2. Perform a pulmonary evaluation that includesmeasurement of FVC, MIP, MEP, PCF, andSpo2 in room air. Measure the patient’s bloodand/or end-tidal carbon dioxide level if Spo2 is� 95% in room air. For DMD patients atincreased risk for respiratory complications,defined as FVC � 50% of predicted, and espe-cially for patients at high risk for complications,defined as FVC � 30% of predicted, consider




preoperative training in the use of NPPV. Forpatients at high risk for ineffective cough,defined in adults as PCF � 270 L/min or MEP� 60 cm H2O, consider preoperative trainingin manual and mechanically assisted cough,emphasizing the use of MI-E with a bronchialsecretion clearance device.

3. Refer the patient to a cardiologist for clinicalevaluation and optimization of cardiac therapies.

4. Obtain a nutritional assessment, optimize nu-tritional status, and consider strategies to man-age dysphagia.

5. Discuss the risks and benefits of general anes-thesia or procedural sedation with the patientand guardians, and help them to decide on andimplement their decisions regarding resuscita-tion parameters and, if applicable, advancedirectives.

II. Respiratory Support and RelatedMedical Management During General

Anesthesia or Procedural Sedation(Intraoperative)

A. Choice of Pharmacologic Agents for GeneralAnesthesia

Patients with DMD are at increased risk forextreme hyperthermic events and rhabdomyolysiswhen they are exposed to certain anesthetics, espe-cially inhaled agents such as halothane, isofluorane,and servoflurane. Such episodes can cause hyperka-lemia and sudden death from cardiac arrest.2,34

These events mimic malignant hyperthermia, butDMD and malignant hyperthermia are geneticallydistinct diseases. Succinylcholine, a depolarizingmuscle relaxant that can disrupt unstable cell mem-branes, has been linked to acute rhabdomyolysis,hyperkalemia, and cardiac arrest in patients withDMD.35 Indeed, there are numerous reports ofyoung patients in whom previously unsuspectedDMD was diagnosed after sudden death due tohyperkalemic cardiac arrest associated with generalanesthesia.36 While succinylcholine is widely recog-nized to be contraindicated in patients with DMD,more recently it has been suggested that inhaledanesthetic agents should also be considered contra-indicated for patients with DMD.34

B. Choice of Personnel and Medical Setting

Procedural sedation should be performed with ananesthesiologist in attendance and with full monitorsand safety measures, according to the guidelines ofthe American Academy of Pediatrics and the Amer-ican Society of Anesthesiologists.37,38 Intraopera-

tively, monitor Spo2 continuously and, wheneverpossible, blood or end-tidal carbon dioxide levels.Medical procedures involving procedural sedation orgeneral anesthesia should be performed in the opti-mal medical setting (eg, postanesthetic care unit oroperating room) and with a full complement ofskilled personnel (eg, an anesthesiologist experi-enced in the management of DMD and a respiratorytherapist skilled in the management of NPPV) inorder to minimize the risk of respiratory complica-tions.6 An ICU should be available for postproceduremanagement.28

C. Respiratory Support Options DuringMaintenance of General Anesthesia or ProceduralSedation

The options for respiratory support during main-tenance of general anesthesia or procedural sedationwill depend on the nature of the procedure and thetype of anesthetic used (eg, IV vs inhaled). Optionsfor respiratory support include endotracheal intuba-tion, using NPPV to facilitate extubation for selectedpatients; use of the laryngeal mask airway; mechan-ical ventilation via a mouthpiece with a leak-proofseal; and manual or mechanical ventilation usingeither conventional ventilators or bilevel positivepressure ventilators designed for noninvasive respi-ratory support, delivered via a full face mask or nasalmask interface.6,17,19,39–45

D. Respiratory Support Options During Inductionof and Recovery From General Anesthesia orProcedural Sedation

While it is standard practice to provide assisted orcontrolled ventilation during induction of generalanesthesia, DMD patients with chronic respiratoryinsufficiency and limited respiratory reserve will alsobenefit from respiratory support during recoveryfrom general anesthesia and throughout proceduralsedation. Options for respiratory support duringinduction of and recovery from general anesthesia orprocedural sedation include manual ventilation usinga flow-inflated manual resuscitation bag (standardanesthesia bag) with a full face or nasal mask inter-face, and mechanical ventilation using a conven-tional or bilevel positive pressure ventilator de-signed for noninvasive respiratory support, alsodelivered via a full face or nasal mask. Patients whohave been intubated for procedures can be extu-bated directly to NPPV as needed (see section III, A,below).3,6,17–19,27,28,44,46 DMD patients with FVC� 50% of predicted should be considered at in-creased risk, and those with FVC � 30% of pre-dicted should be considered at high risk of needing




assisted or controlled ventilation during induction ofand recovery from general anesthesia and through-out procedural sedation.

Summary of Specific Suggestions for Evaluationand Management of Patients With DMD DuringGeneral Anesthesia or Procedural Sedation

1. Consider use of a total IV anesthesia techniquefor induction and maintenance of general an-esthesia (eg, propofol and short-acting opioids).The use of depolarizing muscle relaxants suchas succinylcholine is absolutely contraindicatedbecause of the risk of fatal reactions.

2. Optimize the medical setting and personnel inattendance when DMD patients undergo gen-eral anesthesia or procedural sedation, andhave an ICU available for postprocedure care.Intraoperatively, monitor Spo2 continuouslyand, whenever possible, blood or end-tidalcarbon dioxide levels.

3. There are several options for providing respi-ratory support during maintenance of generalanesthesia or procedural sedation for patientswith DMD. These options are outlined inSection II, C, above.

4. Application of assisted or controlled ventilationshould be considered for patients with DMDand an FVC � 50% predicted, and stronglyconsidered for those with an FVC � 30%predicted, during induction of and recoveryfrom general anesthesia and throughout proce-dural sedation, using the options for respiratorysupport outlined in Section II, D, above.

III. Respiratory Support and RelatedMedical Management After GeneralAnesthesia or Procedural Sedation

(Postoperative)

A. Respiratory Support

Extubation directly to NPPV should be consideredfor DMD patients with baseline FVC � 50% ofpredicted, and should be strongly considered forthose with FVC � 30% of predicted who have beenendotracheally intubated for general anesthesia orprocedural sedation. Extubation directly to NPPVshould also be considered for any patient usingNPPV preoperatively (see Section II, D, above).27,44

Continuous use of NPPV can then be weaned astolerated,18 except in patients who require NPPV 24h/d at baseline. To maximize the chance of success,consider delaying extubation until respiratory secre-tions are in good control and Spo2 is normal orbaseline in room air.46 If NPPV has been used

preoperatively, it is preferable to extubate the pa-tient to NPPV utilizing his usual interface (homemask or mouthpiece) in order to minimize facial skininjury due to poor mask fit, optimize interfacecomfort, and improve the chance of successful extu-bation.6,27 The best medical setting in which toextubate patients depends on the infrastructure andpreferences of individual clinicians and their institu-tions. However, patients who require noninvasiveventilatory support at baseline may tolerate extuba-tion to NPPV best in the ICU rather than in theoperating room or postanesthetic care unit because itavoids the risk of transporting the patient to the ICUin a clinically unstable condition.28 Postoperative useof NPPV should also be considered for DMD pa-tients with baseline FVC � 50% of predicted, andstrongly considered for those with FVC � 30% ofpredicted, if procedural respiratory support was ac-complished using a laryngeal mask airway or withNPPV (see Section II, C, above).

B. Supplemental Oxygen Therapy

Supplemental oxygen therapy should be used withcaution postoperatively in patients with DMD be-cause oxygen therapy can correct hypoxemia withouttreating the underlying cause (eg, hypoventilation oratelectasis) and oxygen therapy may impair centralrespiratory drive.46,47 Spo2 should be monitoredcontinuously during and after general anesthesia orprocedural sedation until cardiopulmonary status isstable. Whenever possible, assess carbon dioxidelevels through blood gas sampling or end-tidal car-bon dioxide monitoring by capnography. Assess ifhypoxemia is due to hypoventilation, atelectasis, orairway secretions, and treat appropriately.

C. Assisted Cough

Any patient with DMD and evidence of impairedcough (defined, in a teenage or adult patient, bypreoperative PCF � 270 L/min or MEP � 60 cmH2O) will benefit from the use of manually assistedcough maneuvers and the MI-E device postopera-tively. The benefits of MI-E include cough augmenta-tion and deep-lung insufflation to treat or preventatelectasis. MI-E can be useful when pain prevents thepatient from coughing spontaneously, such as aftersurgery on the spine, chest, or abdomen.24,25,27,29 MI-Ecan also be used in patients who are still intubated,applied through the endotracheal tube.

D. Pain Control

Adequate postoperative pain control should not becompromised because of concerns about suppres-sion of respiratory drive. When patients are sedated




after administration of opioid analgesics, adequateventilation can be achieved by using NPPV continu-ously or by delaying endotracheal extubation for 24to 48 h. While pain control is essential, the chance ofsuccessful extubation is optimized in an awake,cooperative patient. In patients undergoing spinalfusion surgery, neuraxial techniques have been usedto achieve analgesia through intermittent or contin-uous infusion of opioids and/or local anesthetics viaepidural catheters, with minimal respiratory sideeffects.48

E. Cardiovascular Management

Patients with DMD are at increased risk forintraoperative and postoperative congestive heartfailure and cardiac dysrhythmias, and they have alimited ability to increase cardiac output in responseto stress. After IV fluid boluses or blood transfusions,which are often required during spinal fusion proce-dures and other major surgeries, patients may haveintravascular fluid imbalance. These issues necessi-tate postoperative cardiology consultation, carefulattention to fluid balance, and intensive cardiopul-monary monitoring.30

F. GI and Nutritional Management

DMD is associated with GI smooth-muscle dys-function. Postoperatively, DMD patients may havegastroparesis, intestinal dysmotility, and constipa-tion, all of which can be exacerbated by pain medi-cations. GI dysfunction can impair postoperativebreathing if distention of the abdomen and increasedintraabdominal pressure occur, hampering diaphrag-matic excursion. Gut dysmotility also increases thelikelihood of gastric distention when NPPV is ap-plied. Thus, gastric decompression may be necessarythrough placement of a nasogastric tube. Preopera-tive and postoperative bowel regimens should beemployed to avoid and treat constipation,33 andselected patients may benefit from pharmacologictherapy with GI smooth-muscle prokinetic agents.The inability to take oral nutrition postoperativelycan exacerbate malnutrition and muscle weakness.32

Therefore, any patient with DMD who cannotachieve adequate oral nutrition within 24 to 48 hafter surgery should receive enteral feeding with asmall-diameter nasogastric or nasoduodenal tube, orshould receive parenteral nutrition if ileus is present.

Summary of Specific Suggestions for Managementof Patients With DMD After General Anesthesia orProcedural Sedation

1. Consider extubating DMD patients with FVC� 50% of predicted, and especially those with

FVC � 30% of predicted, directly to NPPV. Tooptimize the chance of success, consider delay-ing extubation until respiratory secretions are ingood control and Spo2 is normal or baseline inroom air. Continuous use of NPPV can then beweaned as tolerated. When applicable, try toutilize the patient’s home interface after ex-tubation.

2. Use supplemental oxygen therapy cautiously.Monitor Spo2 continuously during and after gen-eral anesthesia or procedural sedation. Wheneverpossible, assess blood or end-tidal carbon diox-ide levels. Assess if hypoxemia is due to hy-poventilation, atelectasis, or airway secretions,and treat appropriately.

3. Use manually assisted cough and MI-E postop-eratively in DMD patients with impairedcough, defined in a teenage or adult patient asPCF � 270 L/min or MEP � 60 cm H2O.

4. Optimize postoperative pain control in patientswith DMD. If sedation and/or hypoventilationoccur, delay endotracheal extubation for 24 to48 h or use NPPV.

5. Obtain a cardiology consultation and closely mon-itor cardiac and fluid status postoperatively.

6. Initiate bowel regimens to avoid and treatconstipation and consider prokinetic GI medi-cations. Consider gastric decompression with anasogastric tube in patients with GI dysmotility.Start parenteral nutrition or enteral feeding viaa small-diameter tube if oral feeding is delayedfor � 24 to 48 h postoperatively.

IV. Areas in Need of Further Study

The area explored in this consensus statement ischaracterized by a lack of prospective, randomizedstudies. Examples of specific studies needed todevelop evidence-based guidelines for the care ofDMD patients undergoing general anesthesia orprocedural sedation include the following: prospec-tive studies that identify the baseline pulmonaryfunction parameters that predict an increased risk ofpostoperative respiratory complications for DMDpatients undergoing different types of procedures;randomized, prospective studies designed to deter-mine which patients with DMD are most likely tobenefit from the use of NPPV and/or MI-E toaccomplish successful postoperative extubation;studies that assess the benefit, safety, and efficacy ofnoninvasive methods of respiratory support duringmaintenance of and recovery from general anesthe-sia; studies that assess the benefit, safety, and effi-cacy of the various techniques of assisted cough,including postoperative use of MI-E; studies of the




specific mechanical settings that optimize the effi-cacy of NPPV and MI-E in patients with DMD;studies that clarify the role of alternative mucusmobilization techniques, such as high-frequencychest wall oscillation and intrapulmonary percussiveventilation; and studies that better define optimalpreoperative and perioperative cardiac and nutri-tional management in DMD patients who requireprocedures.

ACKNOWLEDGMENT: We thank the leadership and staff ofthe ACCP and the Muscular Dystrophy Association for theirsupport of this project.

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DOI 10.1378/chest.07-0458 2007;132;1977-1986 Chest

Sussman Richard T. Moxley, Mary K. Schroth, Girish D. Sharma and Michael D.

Iannaccone, Lawrence E. Jacobson, Gary L. Kohn, Etsuro K. Motoyama,Edward R. Carter, Valerie A. Cwik, Jonathan D. Finder, Susan T.

David J. Birnkrant, Howard B. Panitch, Joshua O. Benditt, Louis J. Boitano, Muscular Dystrophy Undergoing Anesthesia or Sedation

Respiratory and Related Management of Patients With Duchenne American College of Chest Physicians Consensus Statement on the

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