A Multidisciplinary Approach to the Anesthetic Management of Patients With Duchenne Muscular Dystrophy

ABSTRACT

Duchenne muscular dystrophy (DMD) is an inherited neuromuscular disorder with multisystem involvement that causes progressive muscular weakness and cardiorespiratory dysfunction. The first comprehensive recommendations regarding anesthetic management are now almost 20 years old and, in the interval, DMD care has evolved. Patients with DMD are experiencing prolongation of survival and, with widespread use of assisted ventilation, cardiac function has become the main determinant of their survival. Newer procedures, such as advanced cardiac interventions, are now performed, in a population of older, medically fragile individuals. Regional anesthetic techniques have advanced, allowing more patients to avoid the risks of inhalational anesthesia, and newer techniques for reversing neuromuscular blockade and minimizing cardiorespiratory depression have become available. Additionally, new medications are being used that have anesthetic implications, including newer guideline‐directed heart failure medications. Overall, it has become increasingly important for perioperative management to be addressed in a multidisciplinary way, with coordination between medical, surgical, and anesthetic clinicians. In this narrative review, we provide information on how DMD's multi‐system involvement impacts perioperative assessment and management, and we advocate for a multidisciplinary approach to perioperative care.

1. Introduction

Duchenne muscular dystrophy (DMD) is a progressive X‐linked recessive neuromuscular disorder caused by mutations in the DMD gene that result in absence or insufficiency of functional dystrophin protein [1]. DMD affects approximately one in 5000 live male births. Dystrophin stabilizes muscle cell membranes [2], and its disruption creates a cycle of inflammation and fibrosis that is perpetuated by muscle contractions. The result is progressive weakness of skeletal muscles and of cardiac muscle. Additionally, dystrophin deficiency affects the brain, as tissue‐specific isoforms are found in the central nervous system [3]. Central nervous system manifestations may include cognitive impairment and neuropsychiatric disorders [4]. Symptoms are first reported at about 3 years of age, with diagnosis at approximately 4–5 years of age [5, 6]. Diagnosis is based on clinical presentation, genetic testing for DMD mutations (primarily deletions), and elevation of serum creatine kinase, which is the basis of newborn screening programs.

While it is a neuromuscular disease, the cardiorespiratory complications of DMD are the source of its most impactful morbidities, and they determine survival [7, 8]. With the aid of assisted ventilation, active cardiac management, and chronic glucocorticoid (GC) therapy [8], most patients can live to 30 years of age and beyond [9, 10]. Overall, treatment with assisted ventilation has likely played the largest role in prolonging survival [9, 10].

Almost 50 years ago, Boltshauser et al. wrote the following regarding the anesthetic management of individuals with DMD: “…We think that these patients are at risk and that, in particular, suxamethonium and halothane should be avoided [11].” Their conclusion was correct, but it took years for those recommendations to become widely accepted, and longer still for broader anesthesia guidelines to enter the literature [12]. Those guidelines were developed to address complications of prolonged survival, including nephrolithiasis requiring lithotripsy and severe dysphagia requiring gastrostomy tube placement [13, 14], occurring in “older” patients who were at increased risk of perioperative complications [14, 15].

Many of the issues addressed in prior guidelines remain relevant [16, 17]. However, patients are now living even longer, and there has been a shift from respiratory to cardiac causes of death [18]. Newer procedural options have emerged, including implantation of ventricular assist devices and heart transplantation [19, 20]. Advances in anesthetic techniques and in medical therapy for heart failure have created new anesthetic considerations [21, 22, 23]. Finally, as we enter further into a new era of emerging DMD therapies [24], the number and nature of advanced procedural interventions, and of medications with anesthetic implications, will almost certainly increase. These trends have prompted this review, in which we present updated recommendations for anesthetic management, and we advocate for a multidisciplinary approach to anesthetic care.

2. Search Strategy

Searches of PubMed, Embase, and Google Scholar were conducted for relevant articles published from January 1, 1980, to May 11, 2026. Search terms included, “Duchenne muscular dystrophy” AND “Anesthesia” or “Sedation,” as well as individual keywords and phrases. Additionally, the authors consulted their personal files. Publications were selected by the authors for inclusion based on relevancy to this article.

3. An Anesthesia‐Focused Overview of DMD

3.1. Respiratory Complications

Age can be viewed as the primary determinant of respiratory complications in individuals with DMD. A few years after loss of ambulation, decline in pulmonary function typically begins—by about 13 years of age in patients not treated with GCs, and by about 16 years of age in GC‐treated individuals. Subsequently, pulmonary function continues to decline throughout the lifespan [8, 17, 25]. Progressive respiratory muscle weakness leads to hypoventilation during sleep and impaired clearance of respiratory secretions. Alveolar hypoventilation and hypercapnia ultimately extend into wakefulness [17]. Physiologically, a sustained increase in respiratory load and work of breathing can occur, due to scoliosis, progressive chest wall stiffness, and microatelectasis, which reduce chest wall and lung compliance [26]. The clinical consequences are atelectasis, respiratory infections, and chronic respiratory failure [26].

Hypotonia of the upper airway muscles, macroglossia, adenotonsillar hypertrophy, and GC‐associated obesity, can lead to OSA [17]. OSA can occur in young individuals with normal pulmonary function and prior to loss of ambulation [27].

Figure 1 illustrates the respiratory complications of DMD.

FIGURE 1.

Respiratory issues in Duchenne muscular dystrophy. Legend: V/Q, ventilation/perfusion. [Color figure can be viewed at wileyonlinelibrary.com]

3.2. Respiratory Standards of Care

Current guidelines endorse an anticipatory approach to respiratory management [8, 12, 28]. By 18–21 years of age, individuals with DMD are typically prescribed cough assistance via mechanical insufflation–exsufflation (MI‐E) and nocturnally assisted lung ventilation via noninvasive ventilation (NIV) [28, 29]. The use of nocturnal NIV and cough assistance is recommended when forced vital capacity (FVC) is below 50% of the predicted value. Assisted coughing is also advised for a peak cough flow (PCF) < 270 L/min or a maximal expiratory pressure (MEP) level < 60 cm H₂O. Additional indications for initiating nocturnal NIV include the presence of clinical or laboratory evidence of hypoventilation or sleep‐disordered breathing [17, 29].

Daytime ventilatory failure is common as individuals with DMD age into adulthood and their weakness progresses. Current guidelines recommend initiation of daytime ventilation when awake daytime pCO₂ is > 45 mmHg, oxyhemoglobin saturation (SpO₂) is < 95% in room air, and/or clinical signs of hypoventilation or respiratory muscle fatigue are present despite nocturnal ventilatory support [17, 28, 29]. Treatment with assisted ventilation has been shown to reduce respiratory complications, improve quality of life, and extend survival [8].

Although NIV is the preferred first‐line approach, tracheostomy remains an alternative [17, 30]. Indications for tracheostomy include patient preference, severe dysphagia causing aspiration of secretions, the inability to use NIV successfully, the need for direct tracheal suctioning to manage secretions, and/or three consecutive failed extubation attempts during a critical illness despite optimal use of NIV and MI‐E [17, 29].

3.3. Cardiac Complications

Cardiac complications may begin subclinically as early as 6–10 years of age and typically progress to dilated cardiomyopathy (DCM) with left ventricular dysfunction. Myocardial damage, dilatation, and remodeling lead to DCM. As a result, arrhythmias are common, including sinus tachycardia, atrial fibrillation, and atrial flutter. Potentially life‐threatening ventricular arrhythmias can occur, especially in individuals with advanced disease [31, 32].

Clinical manifestations of heart failure are often unrecognized until advanced stages, especially when patients become non‐ambulatory and cannot exhibit dyspnea with exertion. In non‐ambulatory individuals, cardiac symptoms may include palpitations, dyspnea at rest, orthopnea, or syncope. However, atypical and non‐specific presentations are common, including loss of appetite, weight loss, delayed gastric emptying, and upper abdominal pain [33]. Individuals with advanced respiratory disease and nocturnal hypoxemia can develop secondary pulmonary hypertension with right ventricular dysfunction.

Active cardiac management is associated with improved long‐term outcomes [33]. According to current guidelines, heart failure therapies such as angiotensin‐converting enzyme inhibitors and β‐blockers should be introduced before the onset of dilated cardiomyopathy [34, 35]. More recently, additional medications, including sacubitril/valsartan and sodium–glucose co‐transporter 2 inhibitors (SGLT2i) have been added to the therapeutic options, based on extrapolation from non‐Duchenne adult heart failure trials and guidelines [36]. Pacemaker implantation is indicated for clinically significant bradycardia or atrioventricular block, and implantable cardioverter‐defibrillators may be considered for ventricular arrhythmias or advanced heart failure [37]. In severe or end‐stage disease, left ventricular assist devices may be used for circulatory support [34] and heart transplantation is an area of active interest [20]. By correcting hypoxemia, nocturnal NIV may benefit patients with heart failure by improving cardiac performance [37].

3.4. Other Complications

Scoliosis may cause loss of sitting ability, pelvic tilt, skin breakdown, and impairment of respiratory function. Strategies to prolong ambulation may delay the onset of scoliosis [38], and patients treated chronically with GCs typically exhibit milder spinal deformities and a reduced need for surgical intervention [29].

Chronic GC therapy has become a standard of care for individuals with DMD as it prolongs ambulation, decreases the prevalence of scoliosis, and improves cardiorespiratory function [39]. However, chronic GC therapy can cause adrenal suppression and acute adrenal insufficiency with surgical stress. GC‐treated individuals also develop osteoporosis, leading to low‐trauma vertebral or long‐bone fractures. Long‐bone fractures may be complicated by fat embolism syndrome, a rare but potentially fatal condition that presents with altered mental status, respiratory distress, and tachycardia [8, 29]. Obesity is a common complication of chronic GC therapy, worsened by increased appetite and decreased caloric needs related to physical limitations.

Individuals with DMD often have gastrointestinal complications due to dysmotility [8]. The clinical presentation can be severe, especially in cases of colonic pseudo‐obstruction, small bowel obstruction, and acute gastric dilatation [39, 40, 41].

In advanced stages of DMD, oropharyngeal muscle weakness commonly leads to dysphagia causing an increased risk of malnutrition and aspiration, with recurrent respiratory infections [42, 43, 44, 45]. Gastrostomy feeding is indicated for aspiration or malnutrition that is refractory to non‐surgical intervention [8].

Older, non‐ambulatory patients with DMD are at risk of symptomatic nephrolithiasis, for which they may require lithotripsy procedures [15]. Contributors include prolonged immobilization, GC therapy, and reduced fluid intake [46].

Finally, individuals with DMD often have elevated serum transaminase levels due to muscle breakdown [47]. When bilirubin and γ‐glutamyl transferase levels are normal, isolated elevations of serum transaminases likely have a muscular origin and are unlikely to reflect liver disease [42].

A summary of relevant aspects of multi‐system involvement is found in Table 1.

TABLE 1.

Multisystem involvement of Duchenne muscular dystrophy.

System	Clinical implications and management
Respiratory system	Respiratory complications are always present in adulthood, driven by progressive respiratory muscle weakness that impairs secretion clearance, upper airway muscle tone, and ventilatory pump function. Reduction of inspiratory and expiratory muscle strength leads to a progressive decline in forced vital capacity. The respiratory consequences are secretion retention, OSA, and hypoventilation. The latter initially occurs during sleep and ultimately extends into wakefulness. OSA may occur prior to loss of ambulation, despite normal pulmonary function. NIV combined with MI‐E are used to treat hypoventilation and clear airway secretions, respectively. Tracheostomy may be considered, especially in patients with severe bulbar dysfunction and difficulty clearing secretions despite the use of MI‐E, and in those who are unable to use NIV.
Airway	Difficult airway management is frequently reported.
Cardiac system	Subclinical myocardial changes can occur as early as 6–10 years of age. Dilated cardiomyopathy and arrhythmias are very frequent in adulthood. Conduction defects occasionally occur. Clinical manifestations of heart failure are often unrecognized until very late, owing to musculoskeletal limitations and atypical symptomatology. Angiotensin‐converting enzyme inhibitors and beta‐blockers are typically initiated before the onset of overt dilated cardiomyopathy. Pacemaker implantation is indicated for bradycardia or atrioventricular block, while implantable cardioverter‐defibrillators may be required in cases of ventricular arrhythmias or advanced heart failure.
Oropharyngeal muscles	Swallowing difficulties are present in the late stage of the disease. Oropharyngeal muscle weakness leads to dysphagia and aspiration risk. Signs and symptoms include mealtimes that are longer than 30 min, recurrent chest infections, unintentional weight loss, and choking when eating or drinking. Gastrostomy tube feeding is indicated in patients with dysphagia‐associated malnutrition and/or aspirations that are refractory to conservative swallowing interventions.
Spinal involvement and bone health	Scoliosis commonly develops around loss of ambulation. Posterior spinal fusion is the preferred treatment for severe progressive scoliosis. Corticosteroid therapy is associated with milder spinal deformities and a reduced need for surgery. Owing to weakness, contractures, and poor balance, there is a high risk of frequent falls and associated fractures. Glucocorticoid‑associated osteoporosis also increases the risk of low‑trauma vertebral and long‑bone fractures. With fractures, there is a risk of fat embolism syndrome.
Nutritional	Children with DMD are at risk of obesity related to glucocorticoid therapy. Contributors include increased appetite, fluid retention, reduced physical activity, and reduced caloric needs after loss of ambulation. In the late stage of the disease, there is a high risk of malnutrition as chewing and swallowing impairment cause reduced oral intake and caloric needs may increase due to increased work of breathing. Gastrostomy tube placement and feeding is indicated for refractory malnutrition or aspiration.
Gastrointestinal	Constipation, gastric and/or abdominal distension are common, due to impaired gastrointestinal motility, with the potential for severe complications, including acute gastric dilatation, and intestinal obstruction or pseudo‐obstruction.
Renal/Urinary	DMD is an independent risk factor for symptomatic nephrolithiasis, particularly in older non‑ambulatory patients.
Hepatic laboratory abnormalities	When bilirubin and γ‑GT are normal, elevated serum transaminases usually reflect muscle involvement rather than a hepatic source.

Abbreviations: DMD, Duchenne muscular dystrophy; γ‑GT, gamma‐glutamyl transferase; MI‐E, mechanical in–exsufflation; NIV, noninvasive ventilation; OSA, obstructive sleep apnea

3.5. Anesthesia‐Related Risks

With regard to muscle relaxants, two main categories should be distinguished: depolarizing neuromuscular blocking agents (NMBAs), such as succinylcholine, and non‐depolarizing NMBAs. Atypical reactions to non‐depolarizing NMBAs may cause delayed onset and prolonged recovery from neuromuscular blockade [21]. Heightened sensitivity to both anesthetic agents and non‐depolarizing NMBAs can amplify chronic weakness and hypotonia, increasing the risk of postoperative complications such as acute respiratory failure and upper airway obstruction [48, 49, 50, 51].

Additionally, when exposed to inhaled anesthetic agents or succinylcholine, individuals with DMD can experience anesthesia‐induced rhabdomyolysis (AIR) and hyperkalemic cardiac arrest [16, 51, 52].

Anesthetic agents that depress respiratory function can exacerbate baseline impairment, causing respiratory failure, and prolonged dependence on mechanical ventilation [50, 51, 52, 53].

Individuals with significant cardiomyopathy have limited ability to tolerate surgical stress, augment cardiac output in response to changes in intravascular volume and compensate for anesthesia‐induced myocardial depression, making them vulnerable to acute heart failure and dangerous arrhythmias.

In patients with advanced disease, neuraxial anesthesia may precipitate respiratory and cardiovascular complications by impairing intercostal and abdominal muscle function and by reducing sympathetic outflow originating from T1 to L2. Neuraxial anesthesia extending above T10 may impair cough effectiveness, secretion clearance, and alveolar ventilation through paralysis of intercostal and abdominal muscles. Compared with epidural anesthesia, spinal anesthesia results in a more profound and less titratable neuraxial block, increasing the risk of respiratory compromise in patients with reduced respiratory reserve [54, 55]. Hypotension is a common side effect of spinal anesthesia, resulting from vasodilation due to sympathetic blockade, with pooling of blood in the extremities, decreased cardiac preload, and reduced cardiac output.

Left ventricular assist device (LVAD) support has important implications for anesthetic management. Unlike normal physiology, where reductions in systemic vascular resistance (SVR) can be compensated by an increase in cardiac output, this compensatory mechanism is absent in patients with an LVAD. Consequently, anesthetic induction–related decreases in SVR may lead to a proportional decline in arterial blood pressure, often necessitating the use of vasoconstrictors to maintain hemodynamic stability [56]. The requirement for chronic systemic anticoagulation in LVAD recipients is a major perioperative consideration, influencing both surgical planning and the safety of regional anesthetic techniques [56].

Factors that decrease intra‐ and postoperative cardiorespiratory function are shown in Figure 2.

FIGURE 2.

Perioperative interactions influencing cardiopulmonary physiology. Legend: FRC, forced vital capacity; NMBAs, neuromuscular blocking agents. [Color figure can be viewed at wileyonlinelibrary.com]

4. Preoperative Evaluation, Planning, and Optimization

4.1. A Multidisciplinary Approach

It is essential to conduct a thorough, multi‐system preoperative assessment that categorizes the patient's extent of muscle weakness and cardiorespiratory involvement, identifies their high‐risk features, and optimizes their preoperative therapies, with special attention to cardiac medications and NIV [17, 50, 51]. Because DMD is a multi‐system disease close collaboration is needed among the patient's anesthesiologist, surgeon, or other procedural specialist, and their cardiologist, pulmonologist, and neuromuscular specialist (NMS) [50, 51]. Other specialists may be relevant, such as those providing nutritional, gastrointestinal, endocrine, palliative, and behavioral care.

A key goal of the multidisciplinary assessment is to allow cardiorespiratory specialists sufficient time to assess baseline status and to optimize care. This might include acquisition of diagnostic studies, such as a cardiac MRI, Holter monitoring, or sleep studies. Sufficient time is also needed to assess the impact of therapeutic changes, such as adjustment of cardiac medications, pacemaker implantation, or training in NIV.

Patients with DMD usually have a longstanding and trusting relationship with their NMS, who is typically a pediatric or adult neurologist. The NMS will often lead a multi‐disciplinary DMD clinic, as recommended in consensus guidelines [29] and by patient advocates [57]. With that structure, patients can undergo preoperative evaluation by multiple specialists during a single clinical visit.

When multiple visits are necessary, a suggested timeline for key appointments follows:

to 8 weeks preoperatively: meeting with coordinating NMS,

4–6 weeks preoperatively: cardiac and respiratory consultations and testing, and

2–4 weeks preoperatively: anesthesia consultation and preoperative testing.

The NMS is also uniquely qualified to assure that procedures align with the patient's goals of care and that any advanced care directives, including those involving tracheostomy and resuscitation, have been thoroughly discussed and are available to the perioperative team. They can consult with palliative care providers as needed.

Patients with DMD have a high prevalence of cognitive, behavioral, and psychological challenges, including intellectual disability, autism spectrum and obsessive‐compulsive disorders, and anxiety [58]. These issues can negatively impact their operative experience and their cooperation with perioperative care. Referral by the NMS to a behavioral specialist can be beneficial, to anticipate problems, and initiate or optimize pharmacologic and behavioral therapies [59]. When available, Child Life and music therapists can provide valuable support.

Good communication between the multidisciplinary team and specialists in the ICU is important, allowing formulation of a tentative postoperative care plan, pre‐arrangement of nursing and respiratory therapy staffing, and assurance of the availability of key specialists, such as those with expertise in NIV and cardiac management. By remaining involved postoperatively, the NMS can provide guidance and support, especially if the patient's clinical course is unstable.

4.2. Respiratory Assessment and Optimization

A comprehensive preoperative respiratory evaluation is crucial for identifying patients with significant respiratory muscle weakness, especially those who require preoperative training in NIV and MI‐E [12, 17, 48, 49]. Respiratory consultation should include a clinical assessment, a chest radiograph, and pulmonary function testing, including measurement of FVC, MEP, maximum inspiratory pressure (MIP), and awake SpO₂ [12, 48, 49]. An SpO₂ below 95% in room air is clinically significant and warrants assessment for hypoventilation, including measurement of carbon dioxide (pCO₂) levels via end‐tidal CO₂ measurement, arterial blood gas (ABG) sampling, or capillary blood gas (CBG) sampling at the earlobe [12, 17, 49]. When signs or symptoms suggest OSA or nocturnal hypoventilation, polysomnography is indicated [17].

Preoperative training in the use of NIV is recommended for patients at high risk of respiratory failure. Specifically, training is strongly recommended for individuals with an FVC < 50% predicted and is considered essential for individuals with an FVC < 30% predicted. Additional indications for NIV training include awake hypercapnia (daytime end‐tidal, arterial, venous, or capillary blood pCO₂ > 45 mmHg or baseline SpO2 < 95% on room air), and sleep study results showing either OSA or hypoventilation, as defined by consensus guidelines [29]. Preoperative training in the use of MI‐E is indicated for individuals with impaired cough effectiveness (FVC < 50% predicted, PCF < 270 L/min, or MEP < 60 cm H₂O) [12, 48, 49]. Whenever possible, patients who are chronically treated with NIV and/or MI‐E should use their home equipment perioperatively. This assures that the necessary equipment is available in the procedural venue and that the patient will be using their properly fitted home NIV and MI‐E masks. Preoperatively, it is important for the respiratory specialist to confirm that the device settings have been optimized [17].

A preoperative respiratory assessment and management pathway is shown in Figure 3.

FIGURE 3.

Respiratory management pathway in the pre‐operative period. Legend: FVC, forced vital capacity; MEP, maximum expiratory pressure; MI‐E, mechanical in–exsufflation; NIV, noninvasive ventilation; OSA, obstructive sleep apnea; PCF, peak cough flow. [Color figure can be viewed at wileyonlinelibrary.com]

4.3. Cardiac Assessment and Optimization

Patients with DMD should undergo comprehensive cardiovascular assessment and optimization of cardiac therapy prior to anesthesia or deep sedation [29, 48]. Major surgical procedures, such as scoliosis correction, are recommended during early childhood, before the development of significant myocardial dysfunction, to minimize perioperative cardiovascular risk [21].

A preoperative electrocardiogram and echocardiogram are indicated, and clinical evidence of arrhythmia should prompt evaluation with Holter monitoring [50]. Pacemaker implantation is recommended for patients with advanced atrioventricular conduction abnormalities before undergoing elective procedures requiring anesthesia [50]. An implantable cardioverter‐defibrillator (ICD) may be indicated for the prevention of sudden cardiac death in patients with a left ventricular ejection fraction < 35% [60, 61]. The consulting cardiologist may elect to obtain other studies in selected patients, such as cardiac MRI [62].

Some of the heart failure medications used to treat advanced cardiomyopathy require specific perioperative management. Beta‐blockers should be continued throughout the perioperative period [63]. SGLT2 inhibitors should be discontinued 3–4 days before surgery to reduce the risk of euglycemic ketoacidosis [63]. In patients receiving renin–angiotensin–aldosterone system inhibitors (RAASi), perioperative continuation is generally reasonable given their established role in heart failure management and the limited evidence supporting routine preoperative discontinuation [63]. For individuals treated with RAASi to manage hypertension, withholding these agents 24 h before surgery may be considered to reduce the risk of intraoperative hypotension [63].

4.4. Prevention of Acute Adrenal Insufficiency

Chronic corticosteroid therapy may suppress the hypothalamic–pituitary–adrenal axis, increasing the risk of acute adrenal insufficiency during surgical stress. In response, the PJ Nicholoff Steroid Protocol was developed. The protocol identifies individuals at risk and provides hydrocortisone dosing guidelines for the day of surgery and postoperatively, stratified by the severity of surgical stress. The latest version of the protocol can be found at this reference [64].

Vamorolone is a “dissociative” steroid that was designed to retain anti‐inflammatory activity while minimizing traditional glucocorticoid side effects [65]. However, the drug does cause adrenal suppression [65] and perioperative stress steroid dosing is indicated for vamorolone‐treated individuals [64].

See Table 2 for a summary of the PJ Nicholoff Steroid Protocol.

TABLE 2.

Prevention of acute adrenal insufficiency during surgical stress using the PJ Nicholoff Steroid Protocol.

Indications for supplemental corticosteroid dosing
Long‐term corticosteroid therapy can suppress the HPA axis, putting patients at risk of acute adrenal insufficiency during surgical stress. All standard daily corticosteroid regimens prescribed to treat Duchenne (prednisone, deflazacort, and vamorolone) used for more than 2 weeks can cause suppression of adrenal function. To prevent this, supplemental hydrocortisone should be administered.
Recommended supplemental dose
The supplemental hydrocortisone dose is based on degree of surgical stress ✓ In case of Minor surgical stress (e.g., local anesthesia < 1‐h, inguinal hernia repair, single tooth extraction, and colonoscopy) hydrocortisone dose: 25 mg or 30 mg/m2 IV. Postoperative taper regimen is not needed. Resume maintenance physiologic dose of corticosteroid once illness, pain, or fever subsides. ✓ In case of Moderate surgical stress hydrocortisone dose: 50 mg or 75 mg/m2 IV. Postoperativeregimen for 24 h: 25 mg IV every 8 h or 50 mg/m2/day every 8 h. Postoperative taper regimen for 24 h. Return to baseline dose over 1–2 days. ✓ In case of Major surgical stress hydrocortisone dose: 100 mg or 100 mg/m2/dose IV. Postoperative regimen for 24–48 h: 50 mg IV every 8 h or 75 mg/m2/day every 8 h. Postoperative taper regimen for 24–48 h. Return to baseline dose over 1–3 days.

Abbreviations: HPA, hypothalamic–pituitary–adrenal (axis).

4.5. Airway Assessment and Preoperative Planning

Difficult intubation is common in individuals with DMD due to macroglossia and upper airway hypotonia, limited mobility of the jaw and/or cervical spine, which is common in older patients, and steroid‐related obesity, which is common in younger patients [66, 67, 68, 69]. These factors, along with decreased chest wall compliance, can also cause difficulties with mask ventilation. In one case series of 12 DMD patients undergoing gastrostomy tube placement, eight patients underwent general anesthesia. Four of the patients required intubation with a video laryngoscope, two required fiberoptic intubation, and one required multiple intubation attempts via direct laryngoscopy [66].

When risk factors for difficult airway are identified, an advance plan is indicated that includes backup emergency measures, with attention to the potential need for nasal or oral fibreoptic intubation. Furthermore, given the high prevalence of delayed gastric emptying in patients with DMD, clinicians should maintain a high index of suspicion for an increased risk of pulmonary aspiration during general anesthesia [70, 71]. Accordingly, measures to reduce aspiration risk, including rapid sequence induction, cricoid pressure, and reverse Trendelenburg positioning during tracheal intubation should be considered [72, 73]. Cricoid pressure may increase the difficulty of tracheal intubation and cause airway obstruction, thereby making manual bag‐mask ventilation significantly more challenging. If intubation is unsuccessful, immediate release of cricoid pressure should be considered [72, 73]. Comprehensive guidelines for difficult airway management apply and should be consulted [72].

4.6. Swallowing and Nutritional Assessment and Management

In advanced DMD, swallowing dysfunction and nutritional status should be carefully evaluated. For selected patients, preoperative evaluation by relevant specialists may be indicated, such as a speech‐language pathologist with expertise in dysphagia, a nutritionist and/or a gastroenterologist. Signs of dysphagia, such as prolonged mealtimes and coughing or choking during eating or drinking, indicate an increased risk of aspiration and of airway clearance problems, as discussed in Section 3.4 [48, 50]. Postoperatively, dysphagia can cause poor oral intake. Therefore, nutritional status should be optimized prior to surgery to reduce the risk of delayed wound healing, wound dehiscence and weakness due to malnutrition, which can impair cough and ventilation [48, 50]. Preoperative nutritional intervention may include evaluation for gastrostomy feeding. Initiation of NIV can have nutritional benefits, as dyspnea can cause decreased oral intake, and work of breathing can increase caloric expenditure [12].

4.7. Risk–Benefit Discussions and Advance Care Planning

The severity of chronic disease appears to have a greater impact on surgical risk than the invasiveness of the planned procedure [68]. Preoperatively, the patient and family should be engaged in a discussion of the potential benefits of surgery vs. the procedural and anesthetic risks [50]. Advance directives, including limitations regarding tracheostomy and resuscitation, should be clearly documented in the medical record prior to surgery. Discussions can be initiated by the patient's NMS; however, if concerns remain regarding the concordance of the procedure with the patient's goals of care, it is also appropriate to involve the palliative care team [74].

4.8. Additional Preoperative Considerations

Creatine kinase (CK) levels are typically elevated in patients with DMD, but they do not correlate with disease severity. In later stages of the disease, CK levels may even return to normal. Therefore, measurement of a baseline preoperative CK level can be useful for assessing perioperative rhabdomyolysis.

Premedication with benzodiazepines should be performed with caution, due to the risk of respiratory depression [51].

Surgery should be scheduled as the first case of the day to minimize the risk of hypoglycemia, particularly in patients with reduced muscle mass [51].

Procedures should be done in specialized centers that are familiar with DMD care. Venues such as ambulatory surgical centers are only appropriate for individuals who are in the early stage of DMD, do not have cardiorespiratory involvement, and are undergoing a low‐risk procedure [51].

Establishing intravenous access can be challenging; ultrasound guidance facilitates peripheral cannulation and is the preferred approach for central vascular access, with peripherally inserted central catheters offering a safer alternative when needed [50]. For patients with severe scoliosis, altered pelvic anatomy, and/or restricted hip mobility, special consideration should be given to intraoperative positioning and padding to reduce the risk of pressure injuries.

While routine prophylactic anticoagulation is not indicated, impaired mobility in non‐ambulatory patients increases the risk of postoperative thrombosis.

Components of the preoperative evaluation are shown in Section 1 of Table 3.

TABLE 3.

Anesthesia and perioperative management standards for patients with Duchenne muscular dystrophy.

1. Preoperative management
Hospital setting	Elective surgery should be done in experienced, fully equipped hospitals; urgent procedures in non‐specialized centers require strict adherence to anesthesia and perioperative care standards. Communication and coordination of management with the clinicians who will provide postoperative care, such as those in the intensive care unit (ICU).
Multi‐disciplinary evaluation and timeline	Preoperative multidisciplinary evaluation is essential to optimize patient care and identify potential risk factors. The suggested timeline for key consultations is as follows: 8 weeks before surgery: evaluation by the patient's coordinating neuromuscular specialist. 4–6 weeks before surgery: cardiology and pulmonology consultations. 2 weeks before surgery: anesthesiology consultation.
Respiratory evaluation	Comprehensive respiratory evaluation to identify the need for preoperative respiratory training and perioperative respiratory support. NIV is recommended for patients with a FVC below 50% of the predicted value. Cough assistance is recommended for patients with a PCF below 270 L/min. Obstructive sleep apnea and potential difficulties with airway management should be carefully assessed and addressed preoperatively.
Airway evaluation	Careful preoperative assessment of potential airway difficulties.
Cardiac evaluation	Preoperative echocardiography and other diagnostics, such as cardiac MRI or Holter monitoring, as indicated, followed by optimization of cardiac therapies.
Swallowing and nutrition assessment	Assessment of signs and symptoms of dysphagia, particularly in advanced disease. Evaluation and optimization of nutritional status.
Behavioral assessment	Assess cognitive, behavioral, and psychological challenges that can impact the patient's perioperative experience and cooperation with medical management. Refer to behavioral specialists and involve Child Life and music therapists, when appropriate and available.
Risks and benefits discussion	Discussion of surgical/anesthetic risks and benefits, with a focus on aligning procedures with the individual patient's overall goals of care.
Advance directives	Revision and documentation of advance directives, including tracheostomy and resuscitation preferences and limitations.
2. Intraoperative management
Anesthetic technique	Regional anesthesia is preferred when feasible (caution is required with spinal anesthesia in cases of severe cardiac dysfunction). If general anesthesia is required, use TIVA and avoid inhalational agents and succinylcholine. Ultra‐short‐acting anesthetic agents are preferred. Non‐depolarizing neuromuscular blocking agents can be used after careful consideration of their potential benefits and limitations.
Management of neuromuscular blockade	Among non‐depolarizing neuromuscular blockers, rocuronium and vecuronium are preferred due to complete reversibility with sugammadex. Quantitative neuromuscular monitoring using the TOF technique is best practice. Confirmation of full neuromuscular recovery requires a TOF ratio ≥ 0.9. Postoperative residual paralysis should be prevented through appropriate dosing of sugammadex, with confirmation of full neuromuscular recovery (TOF ratio ≥ 0.9) by quantitative measurement.
Airway management	Always consider the possibility of a difficult airway.
Anesthetic depth monitoring	Best practice anesthesia depth monitoring to ensure adequate anesthesia and minimize complications.
Acute adrenal insufficiency prevention	Give perioperative hydrocortisone according to surgical stress in long‐term corticosteroid patients.
Intraoperative non‐invasive respiratory support	In patients with FVC < 50% predicted, the use of NIV during the induction and recovery phases of general anesthesia is recommended, as well as throughout procedures requiring deep sedation or regional anesthesia. Instead of intubation, selected patients may be supported with the laryngeal mask airway during maintenance of general anesthesia, or with continuous NIV. However, specialized expertise in noninvasive respiratory management is required to apply these techniques successfully.
3. Postoperative management
Postoperative monitoring	Consider postoperative ICU/HDU admission for patients with respiratory/cardiac risk or receiving opioid infusions.
Extubation strategy	Extubate patients with significant respiratory muscle weakness to NIV, using MI‐E to assist secretion clearance.
Postoperative analgesia	In patients with reduced respiratory function, minimize opioid use with multimodal pain management. Use NIV and MI‐E if respiratory compromise occurs due to opioids or residual pain.

Abbreviations: FVC, forced vital capacity; HDU, high dependency unit; ICU, intensive care unit; MI‐E, mechanical insufflation–exsufflation; NIV, noninvasive ventilation; PCF, peak cough flow; TIVA, total intravenous anesthesia; TOF, train‐of‐four.

5. Intraoperative Management Strategies

5.1. Regional and Neuraxial Anesthesia

For patients with DMD and compromised pulmonary function, regional anesthesia is recommended whenever feasible to minimize respiratory risks associated with general anesthesia [21, 50, 51, 53]. A large number of patients with DMD have successfully undergone regional anesthesia for a wide range of surgical procedures, as described in recent reviews and case reports [22, 56, 61, 75, 76, 77, 78]. Neuraxial techniques include spinal and epidural anesthesia. Peripheral nerve blocks include upper extremity blocks (e.g., supraclavicular and axillary blocks), lower extremity blocks (e.g., femoral–obturator and bilateral popliteal–sciatic blocks), and truncal blocks (e.g., pectoral nerve blocks, transversus abdominis plane block, rectus sheath block, intercostal nerve blocks, and erector spinae plane block). The use of ultrasound has facilitated these procedures, particularly peripheral and truncal blocks [22]. Although ultrasound‐ and electrical stimulation‐guided nerve blocks generally improve the safety and effectiveness of regional anesthesia, muscle responses to electrical nerve stimulation may be unreliable in patients with advanced DMD. So et al. reported two DMD patients undergoing peripheral nerve blocks: a 2‐year‐old boy with mild disease showed the expected muscle contraction in response to nerve stimulation, whereas a 14‐year‐old boy with severe disability showed no muscle response [79]. These findings suggest that the effectiveness of electrical stimulation guidance may vary according to the stage and severity of DMD [79]. In pediatric patients and individuals with cognitive or behavioral disorders, regional anesthesia is commonly combined with sedation to improve patient comfort, cooperation, and procedural success [22].

Neuraxial anesthesia requires caution in patients with severe cardiac systolic dysfunction (ejection fraction < 35%) or marked scoliosis, as scoliosis may make the technique technically challenging, and spinal anesthesia can induce significant hypotension due to sympathetic blockade [12, 51, 53]. In such cases, peripheral nerve blocks may be a safer alternative when feasible [22, 50]. Compared with racemic bupivacaine, levobupivacaine, the pure S(−)‐enantiomer of bupivacaine, may have a more favorable cardiovascular safety profile when used for spinal anesthesia. The pharmacological properties of the S(−)‐enantiomer, including its lower affinity for cardiac sodium channels and weaker depressant effects on cardiac conduction, may contribute to this improved cardiovascular safety profile compared with racemic bupivacaine [80]. Furthermore, when comparing hyperbaric racemic bupivacaine with isobaric levobupivacaine, the hemodynamic effects of spinal anesthesia may also be influenced by the baricity of the intrathecal formulation, which affects the spread of the local anesthetic and, consequently, the extent of sympathetic blockade. Additionally, spinal anesthesia extending above the T10 level may impair intercostal and abdominal muscle function, reducing cough effectiveness and alveolar ventilation. In patients with respiratory muscle weakness meeting the criteria for pre‐procedural training in the use of NIV (see Section 4.2), support with NIV can be beneficial even during spinal anesthesia [50].

5.2. General Anesthesia

Exposure to inhalational anesthetics or succinylcholine places patients with DMD at risk of AIR [21, 51, 52, 68]. Historically, AIR in these patients was often misdiagnosed as malignant hyperthermia [16, 51, 52]. Evidence from a large retrospective study [67] and a recent meta‐analysis [21] indicates that total intravenous anesthesia (TIVA) can be safely administered, with no significant risk of AIR. Nevertheless, some authors recommend perioperative monitoring for AIR in all patients with DMD, regardless of the anesthetic technique used. Indeed, AIR may occur intraoperatively or postoperatively, even in the absence of exposure to volatile anesthetic agents [69]. Accordingly, when general anesthesia is required, TIVA should be preferred, avoiding volatile anesthetics and succinylcholine [21, 52, 67]. Although some authors have reported the use of inhalational anesthesia with halogenated agents and have justified their brief use to facilitate intravenous cannulation [16, 69], current evidence supports the use of TIVA for both induction and maintenance of general anesthesia [21, 67, 68, 81]. Moreover, as intravenous access can often be successfully established after premedication with oral midazolam, administration of 50%–70% nitrous oxide via face mask, and application of topical anesthetic cream, there is limited need for volatile anesthetic agents [62]. Consequently, to prevent inadvertent exposure to volatile anesthetics, a complete washout of the anesthesia machine should be performed before induction [16, 82]. This precautionary recommendation is based on the fact that the minimum triggering concentration of the inhalational agent remains unknown [16].

In patients with DMD, intravenous anesthetics and opioids can depress respiratory and cardiac function; therefore, ultra‐short‐acting agents such as propofol and remifentanil are preferred [21, 67]. Doses should be carefully titrated, and the depth of anesthesia closely monitored using processed electroencephalogram signals, to prevent either intraoperative awareness or drug overdose [50]. The choice of agents for anesthetic induction should be guided by the patient's underlying cardiac comorbidities [62]. Although etomidate has a suppressive effect on adrenal function, it remains an appropriate option for induction in patients with severely impaired myocardial function, given its minimal hemodynamic effects even in the setting of reduced cardiac function [56, 62, 83, 84, 85, 86].

Remimazolam, an ultra‐short‐acting benzodiazepine recently approved for procedural sedation and general anesthesia, has attracted considerable interest because of its rapid onset and offset of action [87]. In addition, its metabolism is independent of hepatic and renal function, it has minimal hemodynamic effects, and its sedative action can be reversed with flumazenil [87]. These characteristics make remimazolam a potentially attractive anesthetic agent for patients with DMD. This hypothesis is supported by two case reports. Horikoshi et al. reported the successful use of remimazolam in combination with remifentanil and rocuronium for induction and maintenance of general anesthesia in a pediatric patient with DMD [88]. Elhamrawy et al. described the use of TIVA with remimazolam and dexmedetomidine, combined with regional anesthesia, in a patient with DMD and an implanted left ventricular assist device undergoing orthopedic surgery [56].

While succinylcholine is absolutely contraindicated in patients with DMD, several studies have shown that non‐depolarizing NMBAs can be used safely in this population after careful consideration of their potential benefits and limitations [21, 67, 89]. An anesthetic technique incorporating a non‐depolarizing NMBA can provide the best intubating conditions, minimize laryngeal adverse effects, and facilitate surgical procedures requiring profound muscle relaxation [85, 90]. However, the administration of non‐depolarizing NMBAs in patients with DMD has been associated with a delayed onset and a markedly prolonged duration of action, which may be up to 4 times longer than that observed in healthy individuals, depending on disease severity [21, 51]. Furthermore, higher doses may be required to shorten onset time [91]. When non‐depolarizing NMBAs are used in patients with DMD, careful dose titration and quantitative neuromuscular monitoring are advisable to minimize the risk of prolonged postoperative neuromuscular blockade. Quantitative neuromuscular monitoring using train‐of‐four (TOF) assessment with acceleromyography or electromyography is recommended to guide administration and recovery [52, 92].

Among non‐depolarizing NMBAs, rocuronium and vecuronium are preferred because their effects can be completely and rapidly reversed with sugammadex [50, 51, 93, 94]. Sugammadex provides reversal of rocuronium‐induced neuromuscular blockade at any depth of block and is more effective than neostigmine in reducing residual neuromuscular block during recovery [95]. Sugammadex dosing should be guided by TOF monitoring. Quantitative confirmation of full neuromuscular recovery (TOF ratio ≥ 0.9) is essential to prevent postoperative residual paralysis [91]. These recommendations are not specific to DMD but reflect general best practices. In addition to their unpredictable efficacy, acetylcholinesterase inhibitors such as neostigmine may increase bronchial secretions, and therefore they are generally not recommended for reversal of NMBAs in patients with DMD, or in patients with poor control of secretions at baseline. When neostigmine is used for reversal of neuromuscular blockade, the risk of increased secretions is reduced by co‐administering glycopyrrolate or atropine. In patients with DMD requiring rapid sequence induction, high‐dose rocuronium should be used in place of succinylcholine, as it offers a comparable onset of action and complete reversal with suggamadex [91, 96].

In place of non‐depolarizing NMBAs, tracheal intubation can be achieved using a combination of propofol, fentanyl, and remifentanil [21, 75]. In addition, Iwata et al. reported successful anesthetic management of laparoscopic cholecystectomy in a patient with Becker muscular dystrophy using TIVA combined with bilateral rectus sheath block and right subcostal transversus abdominis plane block, without the use of muscle relaxants [75]. Finally, a laryngeal mask airway (LMA) may be considered as an alternative airway management strategy during general anesthesia [16, 97]. The LMA can be inserted without neuromuscular blockade [90] and may be used in fasted patients at low risk of aspiration or regurgitation, particularly when adequate access to the patient's head and airway is available [98].

5.3. Deep Sedation

Although general anesthesia is required for many procedures, moderately painful interventions can often be performed under deep sedation while maintaining spontaneous ventilation. A variety of anesthetic drug combinations can be used to achieve this level of sedation. However, commonly used agents such as propofol and opioids may cause significant respiratory depression. In patients with DMD, deep sedation is generally achieved using continuous infusions of dexmedetomidine and/or remifentanil, supplemented with intermittent intravenous ketamine [66, 98]. Dexmedetomidine and ketamine offer combined sedative and analgesic effects, while typically preserving airway reflexes and respiratory drive. The sympathomimetic properties of ketamine can offset the bradycardia and hypotension associated with dexmedetomidine, promoting hemodynamic stability during sedation [98]. Remifentanil, an ultra‐short‐acting opioid, may be a useful adjunct but requires careful monitoring due to the risk of respiratory depression, bradycardia, and hypotension [66, 98].

5.4. Intraoperative Respiratory Management

Several techniques have been described for provision of noninvasive respiratory support during general anesthesia or deep sedation in patients with DMD. They are especially relevant for patients with significant respiratory muscle weakness, such as those meeting the criteria listed in Section 4.2 for pre‐procedural training in NIV.

Boivin et al. [66] reported that three patients with DMD who were treated chronically with NIV successfully underwent radiologically guided gastrostomy under deep sedation while respiratory support was provided with continuous NIV [99]. Other noninvasive intraoperative options include respiratory support with a laryngeal mask airway, or with continuous manual ventilation [14].

However, while avoidance of endotracheal intubation can mitigate the risk of extubation failure, there are substantial challenges and risks to providing procedural respiratory support noninvasively. Individuals with advanced DMD have increased sensitivity to sedatives and intravenous anesthetics. After their administration, obstruction due to upper airway hypotonia, acute hypoventilation and/or apnea may occur, creating a sudden, urgent need for reestablishing oxygenation and ventilation. Even during NIV support, obstruction to airflow can occur, and may be difficult to correct when, as is common in advanced DMD, immobility of the jaw and cervical spine interfere with airway opening maneuvers (e.g., the head tilt/chin lift or jaw thrust). Dysphagia and aspirations can cause lower airway secretion retention, making it challenging to deliver effective NIV, and requiring frequent application of MI‐E. Some risk factors are specific to the planned procedure. In the case of percutaneous gastrostomy placement, oral leak of NIV‐delivered gas can occur while the GI endoscope is in the patient's mouth, with loss of tidal volume. Laryngeal mask airways can be difficult to place, and the laryngeal mask may block the space that is needed for maneuvering the GI endoscope [14]. Additionally, the specialized expertise needed to manage noninvasive intraoperative respiratory support techniques may not be available in all procedural venues.

With these challenges in mind, the use of NIV during induction of, and recovery from anesthesia, with endotracheal ventilation during the procedure itself, may have advantages. This hybrid approach secures the airway during the procedure, allows direct tracheal suctioning of secretions, and may align better with institutional expertise.

5.5. Intraoperative Cardiac Management

Continuous cardiovascular monitoring is essential in patients with DCM, particularly those with a left ventricular ejection fraction below 35%, due to the increased risk of intraoperative hypotension and impaired cardiac output. In these patients, invasive arterial pressure monitoring and close assessment of cardiac output and fluid status are recommended during anesthesia induction, maintenance, and recovery, and throughout the postoperative period [12, 48, 50, 62]. Intermittent measurement of lactate during the intraoperative and postoperative periods may serve as an additional indicator of adequate end‐organ perfusion [62]. Close monitoring of cardiac rhythm should be standard practice, with prompt recognition and treatment of any arrhythmias [62]. Hemodynamic support with inotropic agents, such as dobutamine, may be required in patients with impaired myocardial function to maintain adequate cardiac performance [62].

5.6. Intraoperative Hypothermia

Individuals with DMD, especially those in the advanced stages of the disease, are at increased risk of hypothermia due to the reduced heat produced by dystrophic muscle [50, 51]. Anesthesia further lowers body temperature by inducing peripheral vasodilation and impairing thermoregulatory reflexes. To prevent intraoperative hypothermia, multiple active warming measures should be employed, including forced‐air warming systems, heated blankets, warmed intravenous fluids, and maintaining a warm operating room environment. Preoperative warming has been reported to reduce the decline in core temperature that occurs after induction of anesthesia in the general population and is likely even more beneficial for patients with DMD [51]. Maintaining continuous core temperature surveillance during the perioperative period is strongly recommended.

6. Postoperative Care and Monitoring

6.1. Postoperative Monitoring

Postoperatively, patients at risk of respiratory complications, significant cardiac dysfunction, or those receiving opioid infusion should be considered for admission to an ICU or HDU [50, 51]. These settings allow for close cardiovascular and respiratory monitoring and provide access to key therapies, including NIV and MI‐E. They facilitate a quick response by skilled personnel to adverse events or clinical instability.

6.2. Postoperative Pain Management

Effective analgesia is critical to prevent hypoventilation caused by surgical wound pain, particularly following thoracic, upper abdominal, or spinal procedures [12, 50], and in patients with chronically compromised respiratory function. Use of intravenous opioids requires careful monitoring and titration, to ensure adequate analgesia, while reducing the risk of respiratory depression [12, 50, 67]. Effective, opioid‐sparing pain control strategies include pre‐emptive analgesia and a multimodal pharmacologic approach. This can include the use of intravenous paracetamol, either alone or in combination with nonsteroidal anti‐inflammatory drugs such as ketorolac [100]. However, non‐steroidal anti‐inflammatory drugs should be used cautiously, as they may precipitate rhabdomyolysis crises [101]. Other opioid‐sparing strategies employ additional agents such as N‐methyl‐d‐aspartate receptor antagonists (e.g., ketamine and magnesium), corticosteroids, membrane‐stabilizing agents such as lidocaine, and α₂‐adrenergic agonists (e.g., clonidine and dexmedetomidine) [102]. Preliminary evidence suggests a potential role for preoperative administration of gabapentin or pregabalin in perioperative pain management [103]. When pain management with opioids is necessary, hypoventilation can be managed with NIV or by postponement of extubation for 24–48 h [14, 68]. MI‐E is indicated when pain limits effective coughing [68].

In patients undergoing spinal fusion surgery, continuous epidural anesthetic infusion can provide effective analgesia while reducing inhibitory respiratory effects [104]. More recently, regional techniques using local anesthetics have emerged as effective alternatives for providing postoperative analgesia [21, 22, 62, 85]. These include peripheral nerve blocks, continuous perineural infusions, ultrasound‐guided abdominal wall blocks (e.g., transversus abdominis plane [TAP] blocks), chest wall blocks (e.g., pectoral nerve [Pecs] blocks), and local wound infiltration [50, 105, 106]. Neural targets can be identified using ultrasound guidance and/or nerve stimulation to ensure accuracy and safety.

6.3. Postoperative Respiratory Management

Postoperative respiratory management should be tailored to the patient's preoperative respiratory function [12, 49]. Patients with preserved pulmonary function and effective airway clearance are generally not at increased risk for postoperative pulmonary complications. Conversely, individuals with weakened respiratory muscles need careful monitoring and anticipatory respiratory support.

Continuous SpO₂ monitoring is essential after general anesthesia or procedural sedation [12, 48]. To improve airway clearance and maintain respiratory function in intubated patients, MI‐E via the endotracheal tube should be implemented pre‐extubation [107]. Extubation should be delayed until the patient's SpO₂ stabilizes at normal or baseline values on room air [107]. For patients with significant respiratory muscle weakness, such as those meeting the criteria listed in Section 4.2 for pre‐procedural training in NIV, post‐extubation support should not consist of supplemental oxygen alone. Instead, extubation directly to NIV is indicated, with the use of MI‐E to manage secretions. This strategy has been reported to reduce extubation failures and the associated risk of tracheostomy, and to shorten ICU stays [12, 49, 68, 107, 108]. Active participation of the patient's caregivers in postoperative care can play a critical role in optimizing outcomes [66, 68].

Incentive spirometry is not recommended postoperatively in patients with respiratory muscle weakness, as it may be ineffective, and MI‐E provides a better alternative [12].

7. Summary, Conclusions, and Future Directions

In this article we summarize the pathophysiologic and clinical characteristics that increase the risk of adverse events during anesthesia or sedation in individuals with DMD. These risk factors are especially relevant for individuals with advanced disease, who have long required procedures for fracture management, gastrostomy tube placement and nephrolithiasis and, more recently, have become candidates for advanced cardiac procedures, including ventricular assist device (VAD) placement and even heart transplantation.

This review advocates for a multidisciplinary approach to the anesthetic management of individuals with DMD. Table 3 contains a summary of pre‐, intra‐, and post‐operative multidisciplinary management that can be used as a checklist.

It is important to acknowledge the limitations of this review. Because some DMD centers do not operate on a multidisciplinary model, limited access to some specialities may preclude full implementation of our recommendations. Additionally, this article is not a systematic review; instead, its recommendations are based on expert opinion, supported by selected references. Thus, our recommendations are not evidence‐based.

Standardization of care through dissemination of consensus recommendations has been a key strategy for improving DMD care and outcomes [8, 29]; conversely, variability of care negatively impacts clinical care [109]. We hypothesize that a standardized multidisciplinary approach to perioperative management, as discussed in this review, has the potential to improve patient outcomes. To assess that hypothesis, future studies are needed that compare the multidisciplinary approach to current perioperative management.

Author Contributions

Fabrizio Racca: conceptualization, writing – original draft, methodology, visualization, writing – review and editing, project administration, supervision, resources, investigation. Elena Conti: conceptualization, writing – original draft, methodology, visualization, writing – review and editing, investigation. Giulia Racca: conceptualization, writing – original draft, methodology, writing – review and editing, visualization, investigation. Luigi Barberis: conceptualization, methodology, visualization, writing – review and editing, writing – original draft, investigation. David J. Birnkrant: conceptualization, investigation, writing – original draft, methodology, visualization, writing – review and editing, project administration, supervision, resources.

Funding

This work received no specific grant from any funding agency in the public, commercial or not‐for‐profit sectors. The article processing charge for open access publication was covered by Parent Project Muscular Dystrophy (PPMD), Washington, DC, USA. PPMD had no role in the design or content of this review, nor in the decision to submit it for publication.

Conflicts of Interest

Dr. Birnkrant has a financial interest in patents he developed for respiratory devices. These patents are not currently licensed to a commercial entity.

Data Availability Statement

Data sharing is not applicable to this article, as no new data were created or analyzed in this study.