American College of Chest Physicians Consensus Statement on the Respiratory and Related Management of Patients With Duchenne Muscular Dystrophy Undergoing Anesthesia or Sedation
2007; Elsevier BV; Volume: 132; Issue: 6 Linguagem: Inglês
10.1378/chest.07-0458
ISSN1931-3543
AutoresDavid J. Birnkrant, Howard B. Panitch, Joshua O. Benditt, Louis J Boitano, Edward R. Carter, Valerie Cwik, Jonathan D. Finder, Susan T. Iannaccone, Lawrence Jacobson, Gary L. Kohn, Etsuro K. Motoyama, Richard T. Moxley, Mary Schroth, Girish Sharma, Michael D. Sussman,
Tópico(s)Anesthesia and Sedative Agents
ResumoThis 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. 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. 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 topic. This consensus statement is the product of a panel convened under the auspices of the American College of Chest Physicians' (ACCP) Pediatric Chest Medicine and Home Care NetWorks. The panel consisted of specialists in the areas of anesthesiology, critical care medicine, neurology, orthopedic surgery, pediatric and adult pulmonology, and respiratory therapy. The most current and relevant medical literature was identified and reviewed, obtained by querying PubMed, a service of the National Library of Medicine and the National Institutes of Health (www.pubmed.gov), which includes the MEDLINE database. Consensus of recommendations was achieved through a majority vote of the panel members, and there were no disagreements on any of the recommendations. The purposes of this statement are to aid clinicians involved in the care of patients with DMD undergoing procedures requiring sedation or general anesthesia, to be a resource for other stakeholders in this field, including patients and their families, for use as an up-to-date summary of medical literature on this topic, and to identify areas in need of future research. The statement is divided into sections on the assessment and management of patients before, during, and after procedural sedation or general anesthesia. 1Obtain anesthesiology and pulmonology consultations before procedures involving general anesthesia or procedural sedation.2Perform a pulmonary evaluation that includes measurement of FVC, maximum inspiratory pressure (MIP), maximum expiratory pressure (MEP), peak cough flow (PCF), and oxyhemoglobin 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 increased risk of respiratory complications, defined by an FVC < 50% of predicted, and especially for patients at high risk for complications, defined by an FVC < 30% of predicted, consider preoperative training in the use of noninvasive positive pressure ventilation (NPPV). For patients at high risk of ineffective cough, defined in adults by PCF < 270 L/min or MEP < 60 cm H2O, consider preoperative training in manual and mechanically assisted cough, emphasizing use of mechanical insufflation-exsufflation (MI-E) with a bronchial secretion clearance device (CoughAssist; Respironics; Murrysville, PA).3Refer the patient to a cardiologist for clinical evaluation and optimization of cardiac therapies.4Obtain a nutritional assessment, optimize nutritional status, and consider strategies to manage dysphagia.5Discuss the risks and benefits of general anesthesia or procedural sedation with the patient and guardians, and help them to decide on and implement their decisions regarding resuscitation parameters and, if applicable, advance directives. 1Consider use of a total IV anesthesia technique for induction and maintenance of general anesthesia (eg, propofol and short-acting opioids). The use of depolarizing muscle relaxants such as succinylcholine is absolutely contraindicated because of the risk of fatal reactions.2Optimize the medical setting and personnel in attendance when DMD patients undergo general anesthesia or procedural sedation, and have an ICU available for postprocedure care.3Options for providing respiratory support during maintenance of general anesthesia or procedural sedation for patients with DMD include endotracheal intubation, with use of NPPV to facilitate extubation for selected patients; use of the laryngeal mask airway; mechanical ventilation via a mouthpiece with leak-proof seal; and manual or mechanical ventilation (using conventional ventilators or bilevel positive pressure ventilators designed for noninvasive respiratory support) delivered via a full face mask or nasal mask interface.4Application of ventilation in the assisted or controlled modes should be considered for patients with DMD and an FVC < 50% of predicted, and strongly considered for those with an FVC < 30% of predicted, during induction of and recovery from general anesthesia and throughout procedural sedation. Options for respiratory support during induction of and recovery from general anesthesia or procedural sedation include manual ventilation using a flow-inflated manual resuscitation bag (standard "anesthesia bag") with a full face or nasal mask interface, and mechanical support using a conventional or noninvasive positive pressure ventilator via a full face or nasal mask.5Intraoperatively, monitor Spo2 continuously and, whenever possible, blood or end-tidal carbon dioxide levels. 1Consider extubating DMD patients with FVC < 50% of predicted, and especially those with FVC < 30% of predicted, directly to NPPV. Consider delaying extubation until respiratory secretions are well controlled and Spo2 is normal or baseline in room air. Continuous use of NPPV can then be weaned as tolerated. When applicable, try to utilize the patient's home interface after extubation.2Use supplemental oxygen therapy cautiously. Monitor Spo2 continuously after general anesthesia or procedural sedation. Whenever possible, monitor blood or end-tidal carbon dioxide levels. Assess if hypoxemia is due to hypoventilation, atelectasis, or airway secretions, and treat appropriately.3Use manually assisted cough and MI-E postoperatively in DMD patients with impaired cough, defined in adults as PCF < 270 L/min or MEP < 60 cm H2O.4Optimize postoperative pain control in patients with DMD. If sedation and/or hypoventilation occurs, delay endotracheal extubation for 24 to 48 h or use NPPV.5Obtain a cardiology consultation and closely monitor cardiac and fluid status postoperatively.6Initiate bowel regimens to avoid and treat constipation and consider prokinetic GI medications. Consider gastric decompression with a nasogastric tube in patients with GI dysmotility. Start parenteral nutrition or enteral feeding via a small-diameter tube if oral feeding is delayed for > 24 to 48 h postoperatively. The statement identifies specific areas for future research. 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.1Eagle M Baudouin SV Chandler C et al.Survival in Duchenne muscular dystrophy: improvements in life expectancy since 1967 and the impact of home nocturnal ventilation.Neuromusc Disord. 2002; 12: 926-929Abstract Full Text Full Text PDF PubMed Scopus (761) Google Scholar Patients with DMD are especially vulnerable to the adverse physiologic effects of general anesthesia and procedural sedation.2Morris P Duchenne muscular dystrophy: a challenge for the anesthetist.Paediatr Anaesth. 1997; 7: 1-4Crossref PubMed Scopus (55) Google Scholar3Motoyama EK Davis PJ Smith's anesthesia for infants and children. 7th ed. Elsevier/Mosby, Philadelphia, PA2006Google Scholar4Finder JD Birnkrant D Carl J et al.Respiratory care of the patient with Duchenne muscular dystrophy: an ATS consensus statement.Am J Respir Crit Care Med. 2004; 170: 456-465Crossref PubMed Scopus (627) Google Scholar For example, DMD patients have macroglossia2Morris P Duchenne muscular dystrophy: a challenge for the anesthetist.Paediatr Anaesth. 1997; 7: 1-4Crossref PubMed Scopus (55) Google Scholar and weak upper airway dilator muscles. Sedation and general anesthesia cause relaxation of these muscles, which predisposes to upper airway obstruction. Additionally, DMD patients may have limited mobility of the mandible and cervical spine, which impedes maneuvers (such as the "jaw thrust") that restore upper airway patency. General anesthesia results in decreased functional residual capacity, which can cause lower airway closure, atelectasis, and rapid deterioration in gas exchange. Hypercapnia and hypoxemia can worsen chronic cardiopulmonary abnormalities found in some patients with DMD, such as pulmonary hypertension or cardiac conduction defects. This statement arose from two primary considerations. First, patients with DMD and impaired pulmonary function are at high risk for death when they undergo procedures requiring sedation or general anesthesia.2Morris P Duchenne muscular dystrophy: a challenge for the anesthetist.Paediatr Anaesth. 1997; 7: 1-4Crossref PubMed Scopus (55) Google Scholar Second, because survival of patients with DMD has reached an unprecedented level due to contemporary cardiorespiratory management, there is an increased need for these patients to undergo procedures.5Birnkrant DJ New challenges in the management of prolonged survivors of pediatric neuromuscular diseases: a pulmonologist's perspective.Pediatr Pulmonol. 2006; 41: 1113-1117Crossref Scopus (23) Google Scholar The purposes of this statement are to aid clinicians involved in the care of patients with DMD who undergo procedures requiring sedation or general anesthesia; to be a resource for other stakeholders in this field, including patients and their families; for use as an up-to-date summary of medical literature on this topic; and to identify areas in need of future research. While the primary purpose of this statement is to improve the management and outcomes of patients with DMD who undergo procedural sedation or general anesthesia, the committee wishes to emphasize several related points. First, that efforts should be made to optimize the timing of surgeries and procedures such as scoliosis surgery or gastrostomy placement so that patients undergo procedural sedation or general anesthesia as early in the disease course as possible, preferably when cardiopulmonary function is preserved.6Birnkrant DJ Ferguson RD Martin JE 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; 41: 188-193Crossref PubMed Scopus (30) Google Scholar Second, that the increasing use of glucocorticoids to treat patients with DMD may preserve respiratory muscle strength7Daftary A Crisanti M Kalra M et al.Effect of long-term steroids on cough efficiency and respiratory muscle strength in patients with Duchenne muscular dystrophy.Pediatrics. 2007; 119: e320-e324Crossref PubMed Scopus (70) Google Scholar and decrease the need for procedures such as scoliosis surgery8King WM Ruttencutter R Nagaraja HN et al.Orthopedic outcomes of long-term daily corticosteroid treatment in Duchenne muscular dystrophy.Neurology. 2007; 68: 1607-1613Crossref PubMed Scopus (218) Google Scholar and gastrostomy placement, with the potential to decrease the risk of death due to anesthesia and sedation. Finally, whenever possible, alternatives to general anesthesia and procedural sedation should be considered in patients with DMD who require procedures, such as the use of local anesthetics for pain control.9Zickler RW Barbagiovanni JT Swan KG A simplified open gastrostomy under local anesthesia.Am Surg. 2001; 67: 806-808PubMed Google Scholar This consensus statement is the product of a panel convened under the auspices of the ACCP Pediatric Chest Medicine and Home Care NetWorks. The panel consists of specialists in the areas of anesthesiology, critical care medicine, neurology, orthopedic surgery, pediatric and adult pulmonology, and respiratory therapy. The panel worked on this project from January 2006 to January 2007, primarily via telephone conference calls. The authors disclosed any conflicts of interest and were given complete autonomy by the ACCP. The panel was divided into working groups through which the most current and relevant medical literature was identified and reviewed, obtained by querying PubMed, a service of the National Library of Medicine and the National Institutes of Health (www.pubmed.gov), which includes the MEDLINE database. Only articles written in English were considered. There are few randomized, controlled trials involving the subject of this statement, so this document is a consensus statement derived from expert opinion rather than an evidence-based guideline. Consensus of recommendations was achieved through a majority vote of the panel members, and there were no disagreements on any of the recommendations. The statement is divided into sections on the assessment and management of patients before, during, and after procedural sedation or general anesthesia. Each section consists of a review of the subtopic, followed by a list of specific suggestions. The ACCP Health and Science Policy Committee designates that these recommendations should not be used for performance measurement or for competency purposes because they are not evidence based. 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.10Gozal D Pulmonary manifestations of neuromuscular disease with special reference to Duchenne muscular dystrophy and spinal muscular atrophy.Pediatr Pulmonol. 2000; 29: 141-150Crossref PubMed Scopus (191) Google Scholar11Inkley SR Oldenburg FC Vignos Jr, PJ Pulmonary function in Duchenne muscular dystrophy related to stage of disease.Am J Med. 1974; 56: 297-306Abstract Full Text PDF PubMed Scopus (191) Google Scholar 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 measurement of the patient's blood and/or end-tidal carbon dioxide level should be done if Spo2 is < 95% in room air. Additionally, DMD patients should undergo measurement of the following lung function parameters to assess their risk of respiratory complications and need for perioperative and postoperative assisted ventilation or cough. FVC: FVC is the pulmonary function parameter most frequently reported to have predictive value in assessing the risk of respiratory complications for patients with DMD. To determine predicted values, arm span is usually used to estimate height for patients in a wheelchair, or predictive equations based on ulnar length can be used.12Gauld LM Kappers J Carlin JB et al.Prediction of childhood pulmonary function using ulna length.Am J Respir Crit Care Med. 2003; 168: 804-809Crossref PubMed Scopus (64) Google Scholar The FVC is usually measured with the patient in a seated, upright body position. FVC < 30% of predicted has been identified as a predictor of postoperative respiratory complications and the need for postoperative ventilatory assistance among DMD patients undergoing spinal fusion surgery.13Jenkins JG Bohn D Edmonds JF et al.Evaluation of pulmonary function in muscular dystrophy patients requiring spinal surgery.Crit Care Med. 1982; 10: 645-649Crossref PubMed Scopus (74) Google Scholar14Milne B Rosales JK Anesthetic considerations in patients with muscular dystrophy undergoing spinal fusion and Harrington rod insertion.Can Anaesth Soc J. 1982; 29: 250-254Crossref PubMed Scopus (18) Google Scholar15Almenrader N Patel D Spinal fusion surgery in children with non-idiopathic scoliosis: is there a need for routine postoperative ventilation?.Br J Anaesth. 2006; 97: 851-857Crossref PubMed Scopus (55) Google Scholar However, studies16Marsh A Edge G Lehovsky J Spinal fusion in patients with Duchenne's muscular dystrophy and a low forced vital capacity.Eur Spine J. 2003; 12: 507-512Crossref PubMed Scopus (62) Google Scholar17Harper CM Ambler G Edge G The prognostic value of pre-operative predicted forced vital capacity in corrective spinal surgery for Duchenne's muscular dystrophy.Anesthesia. 2004; 59: 1160-1162Crossref PubMed Scopus (58) Google Scholar18Gill I Eagle M Mehta JS et al.Correction of neuromuscular scoliosis in patients with preexisting respiratory failure.Spine. 2006; 31: 2478-2483Crossref PubMed Scopus (57) Google Scholar suggest that the risks associated with spinal fusion surgery among DMD patients with FVC < 30% of predicted can be greatly reduced by facilitating postoperative extubation with NPPV and by using MI-E to assist with cough. Additionally, percutaneous endoscopic gastrostomy placement has been accomplished in DMD patients with vital capacity well < 30% of predicted through the use of NPPV during induction of and recovery from anesthesia.19Birnkrant DJ Petelenz KM Ferguson RD et al.Use of the laryngeal mask airway in patients with severe muscular dystrophy who require anesthesia or sedation.Pediatr Pulmonol. 2006; 41: 1077-1081Crossref PubMed Scopus (9) Google Scholar A previous consensus conference report20Goldberg A Leger P Hill N et al.Clinical indications for noninvasive positive pressure ventilation in chronic respiratory failure due to restrictive lung disease, COPD, and nocturnal hypoventilation: a consensus conference report.Chest. 1999; 116: 521-534Abstract Full Text Full Text PDF PubMed Scopus (755) Google Scholar recommended NPPV for patients with progressive neuromuscular disease and FVC < 50% of predicted and symptoms of hypoventilation. It is the consensus of this panel that DMD patients with FVC < 50% of predicted measured in the seated, upright body position are at increased risk for respiratory complications when they undergo general anesthesia or procedural sedation, and that patients with FVC < 30% of predicted are at high risk for complications. While diaphragm strength can be relatively preserved in DMD,10Gozal D Pulmonary manifestations of neuromuscular disease with special reference to Duchenne muscular dystrophy and spinal muscular atrophy.Pediatr Pulmonol. 2000; 29: 141-150Crossref PubMed Scopus (191) Google Scholar11Inkley SR Oldenburg FC Vignos Jr, PJ Pulmonary function in Duchenne muscular dystrophy related to stage of disease.Am J Med. 1974; 56: 297-306Abstract Full Text PDF PubMed Scopus (191) Google Scholar patients are often confined to the supine body position during and after surgery. Thus, measurement of both upright and supine FVC may be useful because patients with poor supine FVC values will be especially vulnerable to postoperative atelectasis and hypoxemia. MIP, MEP, and PCF: MIP and MEP are used to assess respiratory muscle strength, and they have clinical utility in DMD.4Finder JD Birnkrant D Carl J et al.Respiratory care of the patient with Duchenne muscular dystrophy: an ATS consensus statement.Am J Respir Crit Care Med. 2004; 170: 456-465Crossref PubMed Scopus (627) Google Scholar10Gozal D Pulmonary manifestations of neuromuscular disease with special reference to Duchenne muscular dystrophy and spinal muscular atrophy.Pediatr Pulmonol. 2000; 29: 141-150Crossref PubMed Scopus (191) Google Scholar Another useful preoperative test is the measurement of PCF.21Suarez AA Pessolano FA Monteiro SG et al.Peak flow and peak cough flow in the evaluation of expiratory muscle weakness and bulbar impairment in patients with neuromuscular disease.Am J Phys Med Rehabil. 2002; 81: 506-511Crossref PubMed Scopus (132) Google Scholar Impairment of these parameters reflects an inability to generate the cough force and velocity necessary for effective clearance of respiratory secretions.4Finder JD Birnkrant D Carl J et al.Respiratory care of the patient with Duchenne muscular dystrophy: an ATS consensus statement.Am J Respir Crit Care Med. 2004; 170: 456-465Crossref PubMed Scopus (627) Google Scholar22McCool FD Global physiology and pathophysiology of cough: ACCP evidence-based clinical practice guidelines.Chest. 2006; 129: 48S-53SAbstract Full Text Full Text PDF PubMed Scopus (220) Google Scholar Patients with a tracheostomy tube and assisted PCF < 160 L/min are at increased risk for failure to achieve tracheostomy tube decannulation, and adult DMD patients who cannot generate a PCF ≥ 270 L/min are at increased risk for pneumonia or atelectasis.23Bach JR Saporito LR Criteria for extubation and tracheostomy tube removal for patients with ventilatory failure: a different approach to weaning.Chest. 1996; 110: 1566-1571Abstract Full Text Full Text PDF PubMed Scopus (453) Google Scholar24Tzeng AC Bach JR Prevention of pulmonary morbidity for patients with neuromuscular disease.Chest. 2000; 118: 1390-1396Abstract Full Text Full Text PDF PubMed Scopus (279) Google Scholar25Bach JR Ishikawa Y Kim H Prevention of pulmonary morbidity for patients with Duchenne muscular dystrophy.Chest. 1997; 112: 1024-1028Abstract Full Text Full Text PDF PubMed Scopus (445) Google Scholar Moreover, young adults with DMD and MEP < 60 cm H2O are likely to have ineffective cough.26Szeinberg A Tabachnik E Rashed N et al.Cough capacity in patients with muscular dystrophy.Chest. 1988; 94: 1232-1235Abstract Full Text Full Text PDF PubMed Scopus (114) Google Scholar Therefore, it is the consensus of the panel that DMD patients with PCF < 270 L/min or MEP < 60 cm H2O are at increased risk for respiratory complications when they undergo procedural sedation or general anesthesia due to impaired cough. The data used to determine these threshold values were obtained from teenage and adult patients, and the values of MEP or PCF that predict increased risk of impaired cough in young children are unknown. It is our consensus opinion that patients with DMD and FVC < 50% of predicted, and especially those with FVC < 30% of predicted, should be considered for preoperative training in the use of NPPV due to their increased risk of respiratory complications. Preoperative training in NPPV should increase the probability of successful use of NPPV during recovery from general anesthesia or sedation and at postoperative endotracheal extubation (see sections II and III below).4Finder JD Birnkrant D Carl J et al.Respiratory care of the patient with Duchenne muscular dystrophy: an ATS consensus statement.Am J Respir Crit Care Med. 2004; 170: 456-465Crossref PubMed Scopus (627) Google Scholar6Birnkrant DJ Ferguson RD Martin JE 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; 41: 188-193Crossref PubMed Scopus (30) Google Scholar18Gill I Eagle M Mehta JS et al.Correction of neuromuscular scoliosis in patients with preexisting respiratory failure.Spine. 2006; 31: 2478-2483Crossref PubMed Scopus (57) Google Scholar27Bach JR Sabharwal S High pulmonary risk scoliosis surgery: role of noninvasive ventilation and related techniques.J Spinal Disord Tech. 2005; 18: 527-530Crossref PubMed Scopus (47) Google Scholar28Lumbierres M Prats E Farrero E et al.Noninvasive positive pressure ventilation prevents postoperative pulmonary complications in chronic ventilator users.Respir Med. 2007; 101: 62-68Abstract Full Text Full Text PDF PubMed Scopus (16) Google Scholar Similarly, adult patients with PCF < 270 L/min or MEP < 60 cm H2O are at risk for ineffective cough, and preoperative training in manual and mechanically assisted cough (MI-E with a bronchial secretion clearance device) [CoughAssist; Respironics; Murrysville, PA]) is suggested, using the techniques described in the referenced articles.24Tzeng AC Bach JR Prevention of pulmonary morbidity for patients with neuromuscular disease.Chest. 2000; 118: 1390-1396Abstract Full Text Full Text PDF PubMed Scopus (279) Google Scholar27Bach JR Sabharwal S High pulmonary risk scoliosis surgery: role of noninvasive ventilation and related techniques.J Spinal Disord Tech. 2005; 18: 527-530Crossref PubMed Scopus (47) Google Scholar29Miske LJ Hickey EM Kolb SM et al.Use of the mechanical in-exsufflator in pediatric patients with neuromuscular disease and impaired cough.Chest. 2004; 125: 1406-1412Abstract Full Text Full Text PDF PubMed Scopus (167) Google Scholar DMD is associated with the development of dilated hypertrophic cardiomyopathy and cardiac dysrhythmias. Patients with DMD are at high risk for perioperative cardiac side effects due to hypoxemia, anemia, and other causes of impaired tissue oxygen delivery. Intravascular fluid shifts can result in congestive heart failure and impaired ventricular preload. These issues are reviewed in the recent consensus statement30American Academy of Pediatrics Section on Cardiology and Cardiac Surgery Cardiovascular health supervision for individuals affected by Duchenne or Becker muscular dystrophy.Pediatrics. 2005; 116: 1569-1573Crossref PubMed Scopus (176) Google Scholar of an expert panel convened by the American Academy of Pediatrics that states that patients with DMD should undergo a cardiac evaluation and optimization of cardiac therapies before anesthesia. Preoperative consultation with a cardiologist is advised for all patients with DMD because heart disease can be severe even among patients with only mild pulmonary involvement, and normal preoperative ECG and echocardiogram findings do not exclude the possibility of postoperative cardiac complications.31Schmidt GN Burmeister MA Lilje C et al.Acute heart failure during spinal surgery in a boy with Duchenne muscular dystrophy.Br J Anaesth. 2003; 90: 800-804Crossref PubMed Scopus (54) Google Scholar Good nutritional support is integral to the proper care of patients with DMD, and the adverse effects of malnutrition on respiratory muscle strength can be profound.4Finder JD Birnkrant D Carl J et al.Respiratory care of the patient with Duchenne muscular dystrophy: an ATS consensus statement.Am J Respir Crit Care Med. 2004; 170: 456-465Crossref PubMed Scopus (627) Google Scholar Therefore, preoperative nutritional status should be evaluated and optimized because poor nutrition can increase postoperative morbidity.32Iannaccone ST Owens H Scott T et al.Postoperative malnutrition in Duchenne muscular dystrophy.J Child Neurol. 2003; 18: 17-20Crossref PubMed Scopus (24) Google Scholar33Pruijs JE van Tol MJ van Kesteren RG et al.Neuromuscular scoliosis: clinical evaluation pre- and postoperative.J Pediatr Orthop B. 2000; 9: 217-220Crossref PubMed Scopus (35) Google Scholar Optimization of preoperative nutritional status may involve the use of NPPV because patients with untreated respiratory failure may become malnourished due to increased work of breathing, or they may b
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