Version May 2024
INTRODUCTION — Tracheomalacia (TM) refers to weakness in the airway wall, resulting in excessive airway narrowing during expiration.
The treatment and prognosis of TM in adults are reviewed here. Clinical presentation and diagnosis of TM and TM in children are discussed separately. (See "Tracheomalacia in adults: Clinical features and diagnostic evaluation" and "Congenital anomalies of the intrathoracic airways and tracheoesophageal fistula".)
DEFINITIONS AND TERMINOLOGY
●Expiratory central airway collapse (ECAC) encompasses two distinct pathophysiologic entities: excessive (or expiratory) dynamic airway collapse of the posterior membrane (EDAC) and tracheobronchomalacia (TBM). EDAC is also known as membranous malacia and can coexist with TM, bronchomalacia (BM), and TBM (ie, cartilaginous malacia).
●TM refers to diffuse or segmental weakness of the tracheal wall resulting in abnormal airway narrowing. It can be further subcategorized as cartilaginous TM (limited to the anterior or lateral wall) or circumferential TM (involves anterior, posterior, and lateral walls).
●BM exists when the cartilage weakness involves one or both mainstem bronchi and/or bronchus intermedius.
●TBM exists when the cartilage weakness involves the trachea and one or both mainstem bronchi.
We refer to TM, BM, TBM, and EDAC collectively as TM in this review since all these conditions have a similar clinical presentation, diagnostic evaluation, and therapeutic approach [1], and the terms are frequently used interchangeably in practice. We distinguish between the disorders only when necessary.
ASYMPTOMATIC PATIENTS — Our approach to the management of TM is shown in the algorithm (algorithm 1). First, it is determined whether a patient with TM is symptomatic from their malacia. Asymptomatic patients generally do not require therapy, whereas therapy may be warranted for some symptomatic patients.
Observation — For patients with TM who are asymptomatic, even if TM is severe, we suggest observation rather than an intervention.
We typically follow patients clinically (eg, every 6 to 12 months). At every visit, we inquire about newly developed symptoms, such as cough, dyspnea, or recurrent infections. We may also periodically perform pulmonary function tests (PFTs) with flow volume loop (FVL) assessment (eg, every year) and dynamic airway computed tomography (DACT; eg, every one to two years in those older than 60 years), particularly in those with severe TM. (See "Tracheomalacia in adults: Clinical features and diagnostic evaluation", section on 'Clinical manifestations'.)
If patients develop symptoms attributable to TM, we repeat PFT with FVL assessment and DACT (if not recently performed) and then proceed to dynamic flexible bronchoscopy. (See "Tracheomalacia in adults: Clinical features and diagnostic evaluation", section on 'Diagnostic approach'.)
The rationale for observation is that in most adult patients, TM is progressive, although some patients may progress slowly or not at all [2-5]. In a series of 17 patients with TM who underwent repeat bronchoscopy, worse airway narrowing was detected in 76 percent of patients [3]. A larger series followed 94 patients with TM, tracheobronchomalacia (TBM), or bronchomalacia (BM) for an average of 5.2 years [5]. Among the 36 patients who underwent repeat bronchoscopy, most patients had worse disease, some had stable disease, and none improved. Six of the nine patients with TM progressed to TBM while all five patients with BM progressed to TBM [5].
SYMPTOMATIC PATIENTS — Our approach to the management of symptomatic TM is shown in the algorithm (algorithm 1). For all patients with TM who are symptomatic, we optimize therapy for any underlying contributing disorder (eg, lung disease, gastroesophageal reflux (table 1)) and administer adjunctive therapies if indicated (see 'Initial medical therapy in all patients' below). We generally wait four to eight weeks before reassessing the patient's symptoms. (See 'Assess the response' below.)
Initial medical therapy in all patients
Optimize therapy for underlying disease — For patients with TM who have a coexisting contributing disorder, we optimize therapy for the underlying disorder if feasible (table 1).
The rationale for this approach is that patients with coexisting lung disorders, including asthma and chronic obstructive pulmonary disease, can improve if their medical regimen is optimized, by decreasing the degree of bronchospasm and large pressure swings in the thorax that worsen the collapse of the central airways. Similarly, treatment of inflammation due to gastroesophageal reflux and relapsing polychondritis may improve symptoms and slow the progression of TM. (See "Stable COPD: Initial pharmacologic management" and "An overview of asthma management" and "Medical management of gastroesophageal reflux disease in adults" and "Treatment of relapsing polychondritis".)
The ideal duration for medical optimization is unknown, but we generally trial medical therapy for approximately four to eight weeks and then re-evaluate symptoms [6]. (See 'Assess the response' below.)
Adjunctive therapies — Several adjunctive therapies may be used in conjunction with treatment of the underlying disorder. Although clinicians use these therapies, there is little evidence for their benefit [7]. Adjunctive therapies include the following:
●Breathing techniques – All patients with moderate or severe TM should be educated in the pursed lip breathing (PLB) technique. PLB is a breathing technique that consists of exhaling through tightly pressed lips and inhaling through the nose with the mouth closed. The purpose of this technique is to create pressure inside the airways, thereby extending expiration time, producing a pneumatic split during exhalation, and decreasing the work of breathing.
●Airway clearance techniques – Retained secretions are an important cause of recurrent infections, airway obstruction, and airway/lung injury. Thus, in those with sputum retention, we typically use airway oscillatory devices (eg, flutter valve (figure 1)) or external percussion vests two or three times per day to improve expectoration. Some experts also use mucolytics to improve expectoration, although no data support this approach.
●Pulmonary rehabilitation – For patients with debilitating symptoms of dyspnea or patients with deconditioning, we refer to pulmonary rehabilitation. (See "Pulmonary rehabilitation".)
Assess the response — Following initial medical therapy, we assess the response clinically at four to eight weeks.
Mild or moderate TM — For those who have mild or moderate TM and have persistent or improved symptoms despite optimizing medical therapy, we continue medical therapy and follow patients in a similar fashion to that described above (see 'Observation' above). The rationale for this approach is that the risk of central airway stabilization surgery likely outweighs any benefit in this population.
Patients with mild/moderate TM and persistent symptoms are rarely subjected to a tracheal stent trial. (See 'Stent trial' below.)
Severe TM
Symptoms controlled — For patients with TM who improve following initial medical therapy (see 'Initial medical therapy in all patients' above), we continue to follow clinically for recurrent or progressive symptoms that may warrant additional treatment at a future date. (See 'Observation' above.)
Should symptoms recur despite good control of the underlying disease, we evaluate the patients for a stent trial. (See 'Stent trial' below.)
Persistent symptoms — For most patients with TM who have persistent symptoms despite initial medical therapy (see 'Initial medical therapy in all patients' above), we assess suitability for a stent trial. While some experts skip a stent trial, in our experience, approximately 75 percent of patients with severe TM who undergo a stent trial experience subjective and/or objective improvement and 65 percent will ultimately undergo definitive surgery. In addition, a stent trial also provides objective evidence to help distinguish symptoms due to TM from symptoms due to the underlying disorder, thereby supporting the value of a stent trial before proceeding with central airway stabilization surgery. (See 'Central airway stabilization surgery' below.)
STENT TRIAL
Determining suitability in patients with severe TM — For most patients with severe TM whose primary symptom is dyspnea and in whom initial medical therapy has failed, we suggest performing a stent trial rather than proceeding directly to central airway stabilization surgery. The purpose of a tracheal stent trial is to assess whether symptoms are likely to improve with surgery, since surgery is extensive, has a high morbidity, and requires significant expertise, which is not universally available. (See 'Patients suitable for a stent' below.)
Patients who are not generally suitable include the following:
●Mild/moderate TM
●Primary symptom other than dyspnea
●Isolated bronchial disease distal to the mainstem airways and bronchus intermedius
●Significant tracheomegaly (see 'Mounier-Kuhn syndrome' below and "Tracheomalacia in adults: Clinical features and diagnostic evaluation", section on 'Congenital (tracheomegaly)')
Management of these patients is discussed below. (See 'Patient not suited to a stent trial' below.)
Patients suitable for a stent — For those considered suitable for a stent trial, we perform a baseline functional assessment for pre- and post-stent trial comparison.
Baseline functional assessment — Prior to a stent trial, we obtain a baseline functional assessment. This involves the following:
●Pulmonary function tests (PFTs) with flow volume loop (FVL) assessment
●Six-minute walk test (6MWT)
●Quality of life (QOL) assessment (eg, Cough Quality of Life Questionnaire [CQLQ])
●Dyspnea score (modified medical research council [mMRC] (calculator 1) (table 2))
The purpose of this assessment is to establish a baseline from which the response to a stent trial can be "objectively" measured, bearing in mind that all of these measurements include some subjective component.
Stent trial — Following baseline assessment, we insert an airway stent(s) for one to two weeks.
●Rationale – The purpose of a stent trial is to identify patients who are most likely to benefit from surgical central airway stabilization (eg, tracheobronchoplasty [TBP]), which is extensive surgery that has a high morbidity and requires significant expertise. (See 'Central airway stabilization surgery' below.)
There are no predictors that determine who benefits from a stent trial.
Long-term stents are not an ideal long-term solution given the rate of complications in this patient population. (See 'Long-term stent' below.)
●Stent selection – Several stents are available (eg, Y-silicone stent or uncovered self-expanding metallic stents [USEMAS] (table 3 and picture 1)).
USEMAS are our preferred stent for short stent trials since they have several advantages, including easy placement by flexible bronchoscopy, dynamic expansion, and preservation of mucociliary function. However, long-term use of USEMAS is not recommended for benign airway obstruction as they have a black box warning against their use in the treatment of benign airway disease.
For patients with a large tracheal airway diameter of >20 mm (eg, tracheomegaly), we use a silicone stent since other stents cannot fit properly to adequately assess response. (See "Tracheomalacia in adults: Clinical features and diagnostic evaluation", section on 'Congenital (tracheomegaly)'.)
Stent selection, insertion, and removal are discussed separately. (See "Airway stents".)
●Assessing the response – We assess the response using clinical history and examination and "objective" measures similar to those performed for the baseline assessment (PFTs with FVL, 6MWT, QOL assessment, and dyspnea scores). (See 'Baseline functional assessment' above.)
We use subjective improvement in two out of three of the following parameters to indicate a response to stenting:
•Two out of three of the following respiratory symptoms: dyspnea, cough, and ability to clear secretions.
•One out of two of the following QOL questionnaires: CQLQ and mMRC (calculator 1) (table 2).
•One out of two of the following: spirometry and 6MWT.
We remove the stent(s) following assessment.
●Efficacy – Limited data support stent trials in this population. Several case series and observational studies have reported that short-term stenting often provides immediate improvement of both airflow and symptoms [8-14], although these findings have not been universal [15]. As an example, a retrospective study of 33 patients with severe symptomatic tracheobronchomalacia (TBM) showed that the short-term use of USEMAS improved respiratory symptoms, QOL, and exercise capacity with few complications [11].
Improvement with stent — For symptomatic patients with severe TM who subjectively and objectively improve following stent insertion and who are surgical candidates, we suggest central airway stabilization surgery rather than alternative treatments (eg, positive pressure ventilation, long term stenting, tracheostomy). This generally requires referral to a center with significant experience in airway surgery since expertise is not universal. We consider surgery the only option for definitive therapy leading to long term relief of symptoms and prevention of complications. In rare cases, we use noninvasive positive pressure ventilation to facilitate pulmonary rehabilitation and improve candidacy for surgery. (See "Pulmonary rehabilitation".)
Central airway stabilization surgery — Several surgical treatments for TM have been described, primarily in case series [16-20]. In most cases, open or robotic TBP is performed, but tracheal resection with reconstruction is an alternative. Choosing among these is at the discretion of the surgeon, their expertise, and TM extent.
●Open tracheobronchoplasty (TBP) – TBP encompasses surgical splinting of the posterior wall of the trachea, mainstem bronchi, and bronchus intermedius with polypropylene mesh [18]. When an open approach is used, a right posterolateral thoracotomy is used to access both the thoracic trachea and bilateral bronchi. Polypropylene mesh is sutured to the posterior membranous wall and anchored to the cartilaginous edges of the trachea and bronchi. The sutures are placed in a partial-thickness fashion, resulting in a posterior splint that is entirely external to the airway lumen. This splint reconstitutes a C-shape to the tracheal cartilage and prevents the posterior membrane from intruding into the lumen (picture 2 and image 1). The surgery can be adapted to address different types of TM, such as crescentic or saber-shaped TM or membranous disease. (See "Tracheomalacia in adults: Clinical features and diagnostic evaluation", section on 'Classification'.)
TBP is extensive, involving trachea and mainstem bronchi to the level of the bronchus intermedius, even if disease is anatomically limited. The rationale for this approach is that if treatment is limited to the affected airway only, the disease may progress in the future to untreated segments, resulting in recurrent symptoms.
Observational data report improvement among carefully selected patients who undergo TBP [17-20]. Most studies include patients with TM due to several etiologies including chronic obstructive pulmonary disease, asthma, Mounier-Kuhn syndrome, and interstitial lung disease. Studies report improvement in quality of life, dyspnea, mean exercise capacity, lung function, and functional status compared with baseline.
TBP is associated with considerable postoperative morbidity (up to 50 percent) and a 30-day mortality of 1 percent [20]. Commonly cited adverse effects apart from bleeding and infection include the postoperative need for mechanical ventilation (20 percent; including tracheostomy), pneumonia/bronchitis (11 percent), subcutaneous emphysema (11 percent), and less commonly, mesh erosion (<1 percent) [17].
●Robotic TBP (R-TBP) – TBP can also be performed using a robotic approach. A retrospective study including 42 patients that underwent R-TBP reported that at a median follow-up of 40 months, there were no signs or symptoms of mesh infection or erosion. At a median follow-up of 29 months, patients that underwent R-TBP had a significant improvement in pulmonary function and dyspnea-related quality of life [21,22].
●Tracheal resection and reconstruction – Conventional tracheal resection and reconstruction are utilized in cases of short-segment focal malacia, most commonly in the cervical trachea (eg, 4 cm). Tracheal resection and reconstruction have low morbidity and mortality in experienced hands and provide curative relief without the need for multiple endoscopic interventions. However, like any resection of the trachea, it can have the unintended consequence of renarrowing the trachea (ie, tracheal stenosis). Tracheal resection techniques and their complications are described separately. (See "Clinical presentation, diagnostic evaluation, and management of malignant central airway obstruction in adults" and "Management of non-life-threatening, nonmalignant subglottic and tracheal stenosis in adults", section on 'Surgery'.)
Alternatives — For patients who objectively improve following stent insertion but who decline surgery or are poor surgical candidates, options are limited. We generally apply intermittent noninvasive positive airway pressure (PAP) before considering long-term stenting (ie, silicone stents). In patients with TM who are not surgical candidates, have an existing stoma, and do not require PAP for ventilatory failure, a Montgomery T-tube may be an option. Tracheostomy is reserved for patients with TM who have respiratory failure requiring positive pressure ventilation.
Intermittent noninvasive positive airway pressure — Noninvasive PAP, typically continuous PAP (CPAP), can maintain an open airway and facilitate secretion drainage [23-25]. In patients who also have hypercapnic respiratory failure due to TM, we use bilevel PAP (BPAP). We do not typically use automatically adjusting PAP (APAP) unless it is clear that the set minimum pressure provides adequate support for TM. Notably, the indication for PAP is maintenance of airway patency rather than ventilation.
In practice CPAP or BPAP is often initiated in the hospital during an acute illness but may be initiated as an outpatient. We prefer bronchoscopic titration to find the optimal pressure that adequately treats TM. Bronchoscopic titration is performed during dynamic flexible bronchoscopy while a PAP facial mask is applied; a flexible bronchoscope is introduced into the airway and PAP pressures are increased until airway collapse is <70 percent or a pressure of 16 cm H2O is reached. If bronchoscopic titration is not feasible, we start CPAP at 8 cm H2O and titrate up according to symptoms.
We prefer that the patient initially receive CPAP 24 hours per day. We then gradually transition to intermittent CPAP as tolerated (often at night during sleep, with pursed lip breathing during the day). Newer light-weight devices are commercially available that can deliver noninvasive ventilation during ambulation, although they are poorly studied in this population.
Practical aspects of noninvasive ventilation are discussed separately. (See "Noninvasive ventilation in adults with chronic respiratory failure from neuromuscular and chest wall diseases: Practical aspects of initiation".)
Data to support PAP in patients with TM are limited. Observational data suggest that it does not appear to have a long-term impact on dyspnea or cough [23,24] but may improve sputum production, atelectasis, and need for medical care [23-25].
Long-term stent — There are several types of long-term airway stents available for treating patients with TM (picture 1 and table 3). We prefer a custom-designed silicone Y-shaped stent for the chronic management of TM. The custom-design helps to reduce pressure points and decrease the risk of granulation tissue. Although their insertion requires rigid bronchoscopy and general anesthesia, they are easily repositioned and removed and migration occurs less frequently when compared with tubular silicone stents. Due to a high incidence of stent-related complications in this population, we follow these patients closely both clinically and bronchoscopically.
USEMAS are a poor choice for the long-term treatment of TM. This is because they cannot be easily removed and, compared with silicone stents, long-term USEMAS are more frequently associated with major complications, including stenosis and stent fracture (table 4 and picture 3), which can obstruct and perforate the airway, respectively. As a consequence, the US Food and Drug Administration has issued a black box warning regarding their long-term use for benign airway disease [26,27]. Their short-term use for stent trials is described above. (See 'Stent trial' above.)
Data that support the value of silicone stenting as long-term therapy for patients with TM are limited. Several case series report improvements in symptoms and functional class, but complications are frequent and seen in more than 80 percent [12,14]. In our experience, long-term stents are poorly tolerated and less than 5 percent of those who chose this option continue with it as a life-long therapy. (See "Airway stents", section on 'Stent-related'.)
Stent insertion, complications, follow-up, and removal are discussed separately. (See "Airway stents".)
Montgomery T-tube or TY-tube — A Montgomery T-tube is a type of stent. It has a narrow tube that connects at a right angle to a larger tube, forming the shape of the letter T (figure 2). The narrow tube passes from the tracheal lumen through a cervical stoma, similar to a standard tracheostomy tube. The larger tube functions like a straight tubular tracheal stent, extending both superiorly and inferiorly to the stoma. The inferior aspect of the larger tube can extend into the thoracic trachea (picture 4). The airflow during breathing is through the lumen of the larger tube and the larynx if the narrow tube is capped externally. In contrast, the airflow is through both the narrow tube and the larynx if the narrow tube is uncapped.
Occasionally, a silicone tracheal Y-shaped tube (TY-tube) is used for long-term stenting in patients with diffuse TBM who are not surgical candidates, have an existing stoma, and do not require PAP. A TY-tube is particularly suited to patients with malacia in the cervical or upper thoracic trachea.
Tracheostomy — Tracheostomy is reserved for patients with TM who have coexisting respiratory failure requiring positive pressure ventilation. Even if the TM is diffuse, tracheostomy may be beneficial as a route to deliver PAP. Tracheostomy is a treatment of last resort since it can worsen TM by destroying the tracheal cartilage and weakening the tracheal wall. It can also induce stenosis which may co-exist with TM (figure 2 and picture 4). (See "Tracheostomy: Postoperative care, maintenance, and complications in adults", section on 'Tracheal and stoma stenosis'.)
Tracheostomy tubes can be used as a therapy in patients with short-segment TM since they can bypass the abnormal tracheal segment and splint the abnormal airway open. Longer segments of TM may require longer tracheostomy tubes.
No improvement with stent — Patients who do not improve with a stent trial should have the stent(s) removed, and other causes for the symptoms should be investigated. A poor or lack of response to a stent trial suggests that surgery is unlikely to be beneficial, and that while they may have TM, it is not the predominant driver of their symptoms.
Patient not suited to a stent trial — Treatment for patients who are not generally suitable for a stent trial includes the following:
●Mild/moderate TM – Patients with mild/moderate TM and persistent symptoms are rarely subjected to a tracheal stent trial; in these patients, we persist with supportive medical therapy. (See 'Initial medical therapy in all patients' above.)
●Primary symptom other than dyspnea – When the primary symptom of TM is not dyspnea, we do not perform a stent trial. Examples include patients whose primary symptom is severe paroxysmal cough or recurrent infection (more than three per year). In such cases, a stent trial will probably not yield improvement and the decision regarding surgery must be made based on symptoms and abnormal anatomy alone.
●Isolated bronchial disease distal to the mainstem airways and bronchus intermedius – When patients have bronchomalacia that is isolated to the distal airways (ie, beyond the mainstem bronchi and bronchus intermedius (figure 3)), we do not perform a stent trial based upon the rationale that an airway stent (or surgery) cannot address distal bronchial disease. Patients with both tracheal and bronchial disease, however, can be considered for a tracheal or bronchial stent trial to assess the role of tracheobronchial disease in TBM.
●Significant tracheomegaly – In patients with severe tracheomegaly (eg, Mounier-Kuhn syndrome), a large enough stent may not be available such that some experts do not perform a stent trial and proceed directly to central airway stabilization surgery. (See 'Central airway stabilization surgery' above.)
INVESTIGATIONAL THERAPIES — Case series have shown promising results when using thermoablative techniques (ie, holmium laser) to treat the posterior membrane wall of patients with excessive dynamic airway collapse (EDAC) morphology [28,29]. Despite promising initial results, additional evidence is needed to recommend the use of bronchoscopic thermoablative techniques in this patient population.
While preclinical data in sheep suggest that argon plasma coagulation (APC) may be a feasible modality for thermoablation compared with electrocautery, radiofrequency ablation, and potassium titanyl phosphate laser [30,31], human trials are needed to demonstrate the feasibility and effectiveness of APC treatment of the posterior membrane wall in patients with EDAC.
Tracheal replacement refers to the replacement of the defective trachea with a new trachea made from foreign material, donor tissue, nonviable tissue, autogenous tissue, or engineered tissue [32,33]. It has been considered for decades, but a reliable tracheal replacement device has not been developed [34].
PROGNOSIS — For patients with mild symptoms due to TM, the prognosis is generally favorable and the disease may progress at a rate that minimally impacts life expectancy.
Similarly, for those who undergo successful surgical repair, the outcome is generally good. (See 'Central airway stabilization surgery' above.)
For those with severe TM who are not surgical candidates, the prognosis is more guarded since therapy is more limited in this population. (See 'Alternatives' above.)
MOUNIER-KUHN SYNDROME — For patients with Mounier-Kuhn syndrome (ie, tracheomegaly), treatment is similar to that described in this topic. However, because the airway size is typically larger than most commercial stents, most experts do not perform a stent trial and proceed directly to surgery. (See 'Central airway stabilization surgery' above.)
SUMMARY AND RECOMMENDATIONS
●Asymptomatic patients – For patients with tracheomalacia (TM) who are asymptomatic, we suggest observation rather than an intervention (Grade 2C) (algorithm 1). Since TM is often progressive, we typically follow patients clinically (eg, every 6 to 12 months). At every visit, we inquire about newly developed symptoms, such as cough, dyspnea, or recurrent infections. We also perform periodic pulmonary function testing with flow volume loop assessment (eg, every year) and dynamic airway CT (eg, every one to two years in patients >60 years). The rationale for observation is that in most adult patients, TM is progressive. (See 'Asymptomatic patients' above.)
●Symptomatic patients – For patients with TM who are symptomatic, we optimize therapy for any underlying contributing disorder (eg, lung disease, gastroesophageal reflux (table 1)) and administer adjunctive therapies if indicated (eg, airway clearance, pulmonary rehabilitation, purse-lip breathing) (algorithm 1). We reassess the patient four to eight weeks later. (See 'Symptomatic patients' above and 'Initial medical therapy in all patients' above.)
•Patients who improve – For most patients with mild, moderate, or severe TM who improve with initial therapies, we suggest continued observation and clinical management rather than a stent trial (Grade 2C). These patients are unlikely to benefit from an intervention. (See 'Assess the response' above and 'Mild or moderate TM' above and 'Symptoms controlled' above.)
•Patients who do not improve – For most patients with persistent symptoms despite initial therapies, we assess patient suitability for a stent trial. (See 'Persistent symptoms' above and 'Stent trial' above and 'Determining suitability in patients with severe TM' above.)
●Patients suitable for stent trial – For most patients with severe TM whose primary symptom is dyspnea and in whom initial medical therapy has failed, we suggest performing a stent trial rather than proceeding directly to central airway stabilization surgery (Grade 2C) (algorithm 1). The rationale for this approach is that a stent trial (table 3 and picture 1) assesses the likelihood of responding to definitive surgical repair, which is highly specialized surgery that carries a high morbidity. (See 'Determining suitability in patients with severe TM' above and 'Patients suitable for a stent' above.)
•Patients who improve with stenting – For patients who improve with a stent trial for one to two weeks, our approach is the following (see 'Baseline functional assessment' above and 'Stent trial' above):
-Central airway stabilization – For most patients who improve following stent insertion, we suggest central airway stabilization surgery rather than other alternative therapy (eg, positive airway ventilation, long-term stenting) (Grade 2C). Tracheobronchoplasty is the definitive operative technique used for most patients (picture 2 and image 1), although tracheal resection with reconstruction may be an option in patients with focal, short-segment TM. (See 'Central airway stabilization surgery' above.)
Surgery is the only option for definitive cure, and it may provide long term relief of symptoms and prevent complications, although data are limited and the morbidity is high. Referral to a center with significant surgical experience is necessary since expertise is not universal.
-Alternatives – For patients who improve following stent insertion, but are not surgical candidates or decline surgery, we generally apply intermittent noninvasive positive airway pressure (PAP) before considering long-term stenting (ie, silicone stents). In patients with TM who are not surgical candidates, have an existing stoma, and do not require PAP for ventilatory failure, Montgomery T-tube (figure 2 and picture 4) may be an option. Tracheostomy is reserved for patients with TM who have respiratory failure requiring positive pressure ventilation. (See 'Alternatives' above.)
•Patients who do not improve with stenting – For patients who do not improve after a stent trial, we perform additional diagnostic workup to help identify an alternative cause for the patient's symptoms. (See 'No improvement with stent' above.)
●Patients not suitable for stent trial – In patients who are not suited to a stent trial, our approach is the following (see 'Patient not suited to a stent trial' above):
•For patients with mild to moderate TM or patients with isolated bronchial disease distal to the central airways (figure 3), we do not perform a stent trial since airway surgery will not be helpful or feasible. We continue medical therapy.
•For patients with severe TM, whose primary symptom is not dyspnea, we do not perform a stent trial since it is unlikely to be helpful in determining the potential response to surgery. Treatment is individualized depending upon the reason, but options include persisting with medical therapy or proceeding with surgery without stent trial data.
•In patients with severe tracheomegaly (eg, Mounier-Kuhn syndrome), a large enough stent may not be available such that some experts do not perform a stent trial and proceed directly to central airway stabilization surgery.
●Prognosis – For patients with mild symptoms, the prognosis is generally favorable and the disease may progress at a rate that minimally impacts life expectancy. Similarly, for those who undergo successful surgical repair, the outcome is generally good. For those with severe TM who are not surgical candidates, the prognosis is more guarded since therapy is more limited in this population.
ACKNOWLEDGMENT — The UpToDate editorial staff acknowledges Armin Ernst, MD, FCCP, Kelly Carden, MD, and Sidhu Gangadharan, MD, who contributed to earlier versions of this topic review.
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