Tracheobronchomalacia, Tracheobronchial Compression, and Tracheobronchial Malformations: Diagnostic and Treatment Strategies.

Tracheobronchomalacia (TBM) is an excessive dynamic narrowing of the airway that is greatest with increased mediastinal pressure such as coughing, Valsalva, and forced expiration. Airway compression and/or cartilage malformation is a fixed or static narrowing of the airway typically caused by great vessel malposition and/or abnormalities and may also contribute to airway narrowing. Although imprecise and misleading, the term TBM is often used to represent both problems, static and dynamic airway narrowing, which only serves to confuse and may mislead the treatment team into ineffective therapies. The consequences of airway narrowing caused by dynamic TBM and/or static compression includes a range of clinical signs and symptoms, depending on the location, extent, and severity of the airway collapse. All patients with mild to severe TBM benefit from medical management to optimize airway clearance of mucus. The milder cases of TBM may become asymptomatic with this therapy, allowing time for the child to grow and the airway to enlarge without the consequences of recurrent infections. In cases of more severe TBM with clinical sequelae, more aggressive management may be warranted. Multiple options for surgical intervention are available. This article discusses the details of clinical presentation, evaluation, diagnosis, and a variety of treatments.

Treatment of complex airway stenoses using patient-specific 3D-engineered stents: a proof-of-concept study.

Anatomically complex airway stenosis (ACAS) represents a challenging situation in which commercially available stents often result in migration or granulation tissue reaction due to poor congruence. This proof-of-concept clinical trial investigated the feasibility and safety of computer-assisted designed (CAD) and manufactured personalised three-dimensional (3D) stents in patients with ACAS from various origins. After CAD of a virtual stent from a CT scan, a mould is manufactured using a 3D computer numerical control machine, from which a medical-grade silicone stent is made. Complication rate, dyspnoea, quality of life and respiratory function were followed after implantation. The congruence of the stent was assessed peroperatively and at 1 week postimplantation (CT scan). The stent could be implanted in all 10 patients. The 3-month complication rate was 40%, including one benign mucus plugging, one stent removal due to intense cough and two stent migrations. 9 of 10 stents showed great congruence within the airways, and 8 of 10 induced significant improvement in dyspnoea, quality of life and respiratory function. These promising outcomes in highly complex situations support further investigation on the subject, including technological improvements.​ TRIAL REGISTRATION NUMBER: NCT02889029.

Etiology and Clinical Characteristics of Community-Acquired Pneumonia with Airway Malacia in Children.

Objective: The objective of this article is to study the etiology of community-acquired pneumonia in children with airway malacia. Methods: We retrospectively reviewed the medical records of 428 pneumonia patients. All patients underwent bronchoscopy, and bronchoalveolar lavage samples were processed for microbiological assessment. Results: In a total of 428 cases reviewed, 60 were found to have airway malacia. Pathogens were identified in 44 of the 60 specimens (73.3%), with 32 being single-pathogen infections. The most common pathogen was respiratory syncytial virus (RSV; 20%). Mixed-pathogen infections were observed in 12 patients. Airway malacia patients were younger than those without malacia (10.5 vs. 50 months, respectively; p < 0.001). Compared with those without airway malacia, wheezing, cyanosis and admission to the pediatric intensive care unit were more common in children with airway malacia and their hospital stay was longer. Conclusion: RSV was the most common pathogen in those with airway malacia. Airway malacia was found to aggravate infectious pneumonia.

Laser tracheobronchoplasty: a novel technique for the treatment of symptomatic tracheobronchomalacia.

The management of tracheobronchomalacia is a very challenging problem with few treatment options. This study aims to evaluate the outcomes of a novel surgical treatment for membranous tracheobronchomalacia. A consecutive series of patients with tracheobronchomalacia were treated with two to three holmium laser scarring surgeries of the hyperdynamic tracheal and bronchial walls for the purpose of stiffening them through fibrosis. Patients filled out a Dyspnea Index questionnaire before and after treatment. Ten patients were treated for their tracheobronchomalacia with a mean age of 54 years. Symptoms included severe dyspnea, dry cough, recurrent pulmonary infections, and respiratory failure. Fifty percent of patients presented with wheezing refractory to traditional treatment. Tracheobronchomalacia was associated with gastroesophageal reflux disease (n = 8), obstructive sleep apnea (n = 5), and tracheal stenosis (n = 3). Only 50 % of patients presented with morbid obesity. All cases showed significant improvement of their respiratory symptoms with a mean postoperative difference of 22.3 out of a maximum impairment score of 40 (P < 0.01) on the Dyspnea Index. The mean number of procedures was 2.3 per patient with the average laser energy delivered per procedure of 1600 J. Laser tracheobronchoplasty is a safe, easy to adopt, and effective technique for the treatment of membranous tracheobronchomalacia. It presents a simple alternative to the commonly used procedures like endoluminal stenting and open tracheobronchoplasty.

Tracheobronchomalacia as a Rare Cause of Chronic Dyspnea in Adults.

OBJECTIVE: To emphasize the importance of a careful clinical evaluation to prevent unnecessary interventions and treatments. CLINICAL PRESENTATION AND INTERVENTION: A 76-year-old female patient had been diagnosed with asthma during previous admissions to different hospitals. She had also undergone fiberoptic bronchoscopy (FOB) on 2 occasions for evaluation of right middle lobe atelectasis observed on computed tomography. A repeated FOB revealed tracheobronchomalacia and nodular bronchial amyloidosis. A silicone Y stent was inserted, but the dyspnea increased. Excessive granulation tissue developed, and the patient died despite ventilatory support. CONCLUSION: The stenting technique used did not prevent the development of respiratory failure and death in this patient. Hence, a surgical procedure could be considered as an alternative to stenting in such cases.

Respiratory Conditions Associated with Tracheobronchomegaly (Mounier-Kuhn Syndrome): A Study of Seventeen Cases.

BACKGROUND: Mounier-Kuhn syndrome (MKS) is a rare disorder characterized by enlargement of the trachea and main bronchi and associated with recurrent respiratory tract infections. OBJECTIVE: This multicenter, retrospective study was carried out to describe respiratory conditions associated with tracheobronchomegaly. METHODS: Nine institutions involved in the 'Groupe d'Endoscopie de Langue Française' (GELF) participated in this study. A standard form was used to record patient characteristics, treatments and follow-up from medical charts. RESULTS: Seventeen patients, 53% male, aged 58 ± 18 years at diagnosis were included. Recurrent infections revealed MKS in 88% of cases. Main comorbid conditions were diffuse bronchiectasis in 88% of patients and tracheobronchomalacia in 67% of cases. The exacerbation rate was 1.5 exacerbations/patient/year. The main non-respiratory morbid condition was gastroesophageal reflux disease in 29% of cases. Interventional bronchoscopy was performed in seven patients (41%), consisting of laser (n = 2) and tracheal stenting (n = 5). Complications related to stents were observed in 80% of cases with a mean stent duration of 8 months. Four deaths, including three due to respiratory causes, occurred during follow-up. CONCLUSIONS: This is the largest series of MKS reported in the literature, showing that bronchiectasis and tracheobronchomalacia are the main associated morbid conditions that constitute a challenge for treatment.

[Anesthetic management of an adult patient with tracheobronchomalacia].

General anesthesia was successfully performed in an 86-year-old woman with severe tracheobronchomalacia Tracheobronchomalacia in adult is a very rare disease, characterized by weakness of the trachea and bronchi, causing luminal narrowing during expiration. The patient had laparoscopic cholecystectomy. We used propofol, remifentanil, and rocuronium for induction of anesthesia. For maintenance of anesthesia, oxygen, air, propofol, fentanyl, and remifentanil were used. After the end of operation, we used sugammadex to reverse neuromuscular blockade by rocuronium. The patient showed spontaneous breathing and good awareness, and we extubated. The patient did well, and was discharged from the hospital 3 days after the operation. In this case, no special care such as postoperative CPAP was necessary, but normally, general anesthesia for a patient with tracheobronchomalacia requires attention. We gave general anesthesia satisfactorily in a patient with tracheobronchomalacia.

Surgical treatment for severe pediatric tracheobronchomalacia: the 20-year experience of a single center.

OBJECTIVE: In children with tracheobronchomalacia, surgical management should be reserved for the most severe cases and be specific to the type and location of tracheobronchomalacia. The goal of this study is to describe the presentation and outcomes of children with severe tracheobronchomalacia undergoing surgery. METHODS: Retrospective case series of 20 children operated for severe tracheobronchomalacia at a tertiary hospital from 2003 to 2023. Data were collected on symptoms age at diagnosis, associated comorbidities, previous surgery, age at surgery, operative approach, time of follow-up, and outcome. Surgical success was defined as symptom improvement. RESULTS: The most frequent symptoms of severe tracheobronchomalacia were stridor (50 %), cyanosis (50 %), and recurrent respiratory infections (45 %). All patients had one or more underlying conditions, most commonly esophageal atresia (40 %) and prematurity (35 %). Bronchoscopy were performed in all patients. Based on etiology, patients underwent the following procedures: anterior aortopexy (n = 15/75 %), posterior tracheopexy (n = 4/20 %), and/or posterior descending aortopexy (n = 4/20 %). Three patients underwent anterior aortopexy and posterior tracheopexy procedures. After a median follow-up of 12 months, 16 patients (80 %) had improvement in respiratory symptoms. Decannulation was achieved in three (37.5 %) out of eight patients with previous tracheotomy. The presence of dying spells at diagnosis was associated with surgical failure. CONCLUSIONS: Isolated or combined surgical procedures improved respiratory symptoms in 80 % of children with severe tracheobronchomalacia. The choice of procedure should be individualized and guided by etiology: anterior aortopexy for anterior compression, posterior tracheopexy for membranous intrusion, and posterior descending aortopexy for left bronchus obstruction.

Primary Posterior Tracheopexy at Time of Esophageal Atresia Repair Significantly Reduces Respiratory Morbidity.

PURPOSE: Esophageal atresia with tracheoesophageal fistula (EA/TEF) is often associated with tracheobronchomalacia (TBM), which contributes to respiratory morbidity. Posterior tracheopexy (PT) is an established technique to treat TBM that develops after EA/TEF repair. This study evaluates the impact of primary PT at the time of initial EA/TEF repair. METHODS: Review of all newborn primary EA/TEF repairs (2016-2021) at two institutions. Long-gap EA and reoperative cases were excluded. Based on surgeon preference and preoperative bronchoscopy, neonates underwent primary PT (EA + PT Group) or not (EA Group). Perioperative, respiratory and nutritional outcomes within the first year of life were evaluated. RESULTS: Among 63 neonates, 21 (33%) underwent PT during EA/TEF repair. Groups were similar in terms of demographics, approach, and complications. Neonates in the EA + PT Group were significantly less likely to have respiratory infections requiring hospitalization within the first year of life (0% vs 26%, p = 0.01) or blue spells (0% vs 19%, p = 0.04). Also, they demonstrated improved weight-for-age z scores at 12 months of age (0.24 vs -1.02, p < 0.001). Of the infants who did not undergo primary PT, 10 (24%) developed severe TBM symptoms and underwent tracheopexy during the first year of life, whereas no infant in the EA + PT Group needed additional airway surgery (p = 0.01). CONCLUSION: Incorporation of posterior tracheopexy during newborn EA/TEF repair is associated with significantly reduced respiratory morbidity within the first year of life. LEVEL OF EVIDENCE: Level III.

Outcomes of surgical treatment of tracheobronchomalacia in children.

BACKGROUND: Tracheobronchomalacia (TBM) is characterized by excessive dynamic airway collapse. Severe TBM can be associated with substantial morbidity. Children with secondary TBM associated with esophageal atresia/tracheoesophageal fistula (EA/TEF) and vascular-related airway compression (VRAC) demonstrate clinical improvement following airway pexy surgery. It is unclear if children with severe primary TBM, without secondary etiologies (EA/TEF, vascular ring, intrinsic pulmonary pathology, or complex cardiac disease) demonstrate clinical improvement following airway pexy surgery. MATERIALS AND METHODS: The study cohort consisted of 73 children with severe primary TBM who underwent airway pexy surgery between 2013 and 2020 at Boston Children's Hospital. Pre- and postoperative symptoms as well as bronchoscopic findings were compared with Fisher exact test for categorical data and Student's t-test for continuous data. RESULTS: Statistically significant improvements in clinical symptoms were observed, including cough, noisy breathing, prolonged respiratory infections, pneumonias, exercise intolerance, cyanotic spells, brief resolved unexplained events (BRUE), and noninvasive positive pressure ventilation (NIPPV) dependence. No significant differences were seen regarding oxygen dependence, ventilator dependence, or respiratory distress requiring NIPPV. Comparison of pre- and postoperative dynamic bronchoscopy findings revealed statistically significant improvement in the percent of airway collapse in all anatomic locations except at the level of the upper trachea (usually not malacic). Despite some initial improvements, 21 (29%) patients remained symptomatic and underwent additional airway pexies with improvement in symptoms. CONCLUSION: Airway pexy surgery resulted in significant improvement in clinical symptoms and bronchoscopic findings for children with severe primary TBM; however, future prospective and long-term studies are needed to confirm this benefit.

Tracheobronchomalacia: an unusual cause of debilitating dyspnoea and its surgical management.

Tracheobronchomalacia (TBM) is a progressive weakening of the airways, leading to collapse and dyspnoea. TBM can be misdiagnosed when multiple chronic conditions accompany it. Tracheobronchoplasty (TBP) is indicated for severe symptomatic TBM, diagnosed by bronchoscopy and CT thorax. We report the case of a patient who underwent tracheal resection and reconstruction for continuing dyspnoea post argon therapy, TBP and a failure to tolerate extracorporeal membrane oxygenation-assisted Y-stent insertion. Relevant background history includes asthma, sleep apnoea, reflux, cardiomyopathy and a high body mass index. Bronchoscopy postreconstruction showed patent airways. Airway reconstruction was a viable management option for this patient's TBM. TBP is a treatment option for TBM. In this case, tracheal resection was required to sustain benefit. In addition, surveillance bronchoscopies will be carried out every year.

Tracheobronchoplasty: Indications and Best Approaches.

Tracheobronchomalacia (TBM) is an increasingly recognized abnormality of the central airways in patients with respiratory symptoms. Severe TBM in symptomatic patients warrants screening dynamic CT of the chest and/or awake dynamic bronchoscopy. The goal of surgical repair is to restore the C-shaped configuration of the airway lumen and splint or secure the lax posterior membrane to the mesh to ameliorate symptoms. Robotic tracheobronchoplasty is safe and associated with improvements in pulmonary function and subjective improvement in quality of life.

How risky is it to remove an airway stent?

BACKGROUND: Airway stent removal has traditionally been associated with a high complication rate. Most studies on stent removal are over a decade old, prior to newer anti-cancer therapies, and by including non-contemporary and uncovered metal stents, may not reflect the current practices. We review our experience at Mount Sinai Hospital to report outcomes of stent removal with more contemporary practices. METHODS: A retrospective review was carried out of all the airway stent removals performed between 2018 to 2022, in adult patients with benign or malignant airway diseases. Stents inserted and removal for stent trials for tracheobronchomalacia were excluded from the final analysis. RESULTS: Forty-three airway stents removals in 25 patients were included. Twenty-five (58%) stents were removed in 10 patients with benign diseases, and 18 (42%) stents were removed in the remaining 15 patients with malignant diseases. Patients with benign disease were more likely to have their stent removed (OR 3.88). 63% of the stents removed were silicone. The most common reasons for stent removal were migration (n = 14, 31.1%) and treatment response (n = 13, 28.9%). Rigid bronchoscopy was used in 86% of cases. Ninety-eight percent of removals were accomplished in a single procedure. The median time to removal of stents was 32.5 days. Three complications were noted: hemorrhage (n = 1, 2.3%) and stridor (n = 2, 4.6%); one not directly related to the stent removal. CONCLUSIONS: Covered metal or silicone airway stents, in the era of contemporary stents, better cancer-directed therapies and surveillance bronchoscopies, can be removed safely with the use of rigid bronchoscopy.

Single-center review on safety of biodegradable airway stenting in pediatric population.

BACKGROUND: Tracheobronchomalacia (TBM) and airway stenosis are recognized etiologies of airway obstruction among children. Their management is often challenging, requiring multiple interventions and prolonged respiratory support with associated long-term morbidity. Metallic or silicone stents have been used with mixed success and high complication rates. More recently biodegradable Ella stents (BES) provided an attractive interventional option. OBJECTIVES: We report our experience in the treatment of TBM and vascular airway compression using BES. We deliberately downsized them to minimize intraluminal granulation tissue formation. MATERIALS AND METHODS: Retrospective study over an 8-year period between November 2012 and December 2020 of pediatric patients with severe airway obstruction requiring airway stenting for extubation failure, malacic death spells, recurrent chest infections, or lung collapse. RESULTS: Thirty-three patients (5 tracheal and 28 bronchial diseases) required 55 BES during the study period. The smallest patient weighed 1.8 kg. Median age of patient at first stent implantation was 13.1 months (IQR 4.9-58.3). The majority of the bronchial stents were in the left main bronchus (93%), of which 57% for vascular compression. Repeat stents were used in 19 patients (57.7%), with a range of two to four times. We did not experience erosion, infection, or obstructive granuloma needing removal by forceps or lasering. Three stent grid occluded with secretions needing bronchoscopic lavage. Stent migration occurred in three patients. CONCLUSIONS: BES holds promise as a treatment option with low rate of adverse effects for a specific subset of pediatric patients with airway malacia or vascular compression. Further studies are warranted.

Characteristics analysis of 157 cases of central airway stenosis due to tracheobronchial tuberculosis: A descriptive study.

Background: Tracheobronchial stenosis, particularly central airway stenosis, which frequently results in severe complications such as lung damage, occurs in patients with tracheobronchial tuberculosis (TBTB). Objectives: To analyze the clinical characteristics of patients with central airway stenosis due to tuberculosis (CASTB). Methods: Retrospective analysis was performed on the clinical features, radiological features, bronchoscopic features and treatment of 157 patients who were diagnosed with CASTB in two tertiary hospitals in Chongqing, China, from May 2020 to May 2022. Results: CASTB mostly occurs in young patients and females. Patients with CASTB exhibited different symptoms repeatedly during the disease, especially varying degrees of dyspnea, prompting many patients to undergo bronchoscopic intervention and even surgery. Patients with cicatricial strictures constituted the highest proportion of the TBTB subtype with CASTB and 35.7% of the patients with CASTB were found to have tracheobronchomalacia (TBM) under bronchoscopy. CASTB and TBM mainly involved the left main bronchus. Patients with lower levels of education had higher rates of TBM. Patients with TBM manifested shortness of breath more frequently than patients without TBM. Patients with TBTB who had undergone bronchoscopic interventions have a higher rate of TBM. Conclusions: Despite mostly adequate anti-tuberculosis chemotherapy, patients with TBTB can present with CASTB involving severe scarring stenosis, bronchial occlusion, tracheobronchomalacia and even destroyed lung.

Dynamic computed tomography for evaluation of tracheobronchomalacia in premature infants with bronchopulmonary dysplasia.

INTRODUCTION: Dynamic computed tomography (dCT) gives real-time physiological information and objective descriptions of airway narrowing in tracheobronchomalacia (TBM). There is a paucity of literature in the evaluation of TBM by dCT in premature infants with bronchopulmonary dysplasia (BPD). The aim of this study is to describe the findings of dCT and resultant changes in management in premature infants with TBM. METHODS: A retrospective study of 70 infants was performed. Infants included were <32 weeks gestation without major anomalies. TBM was defined as ≥50% expiratory reduction in cross-sectional area with severity defined as mild (50%-75%), moderate (≥75%-90%), or severe (≥90%). RESULTS: Dynamic CT diagnosed malacia in 53% of infants. Tracheomalacia was identified in 49% of infants with severity as 76% mild, 18% moderate, and 6% severe. Bronchomalacia was identified in 43% of infants with varying severity (53% mild, 40% moderate, 7% severe). Resultant management changes included PEEP titration (44%), initiation of bethanechol (23%), planned tracheostomy (20%), extubation trial (13%), and inhaled ipratropium bromide (7%). CONCLUSION: Dynamic CT is a useful noninvasive diagnostic tool for airway evaluation of premature infants. Presence and severity of TBM can provide actionable information to guide more precise clinical decision making.

Reassessing the role of tracheobronchomalacia in persistent wheezing.

BACKGROUND: Tracheobronchomalacia (TBM) is often manifested as wheezing. Reassessing the role of TBM in persistent wheezing in children is essential. METHODS: We selected children who were diagnosed with TBM by bronchoscopy and who underwent bronchoscopic reexamination for persistent wheezing or chronic cough between January 2009 and July 2019. The clinical and bronchoscopy data were collected and retrospectively reviewed. For statistical analysis, we used the Kaplan-Meier method, Kruskal-Wallis test, and Fisher exact test. RESULTS: A total of 79 patients (57 males and 22 females) were included. The median age of the first TBM diagnosis was 7 (interquartile [IQR] 4-11) months. The median age of the first wheezing episode was 4 (IQR 3-7) months. During the time interval between the two bronchoscopies, malacia lesions resolved in 50 patients (63.3%), improvement was seen in 14 patients (17.7%), no change was observed in 11 patients (13.9%), and the condition was aggravated in 4 patients (5.1%). The malacia lesions in 37 patients resolved before 2 years of age. Among the 50 resolved patients, 22 patients (44.0%) reported wheezing three times or more between bronchoscopy evaluations, and 13 of these 22 patients (59.1%) with atopy or family history of allergic diseases were ultimately diagnosed with bronchial asthma. CONCLUSIONS: In children with persistent wheezing, the role of TBM should be reassessed, especially in those with atopy or family history of allergic diseases, and bronchial asthma should be considered early.

Cautionary tales in the use of magnets for the treatment of long gap esophageal atresia.

BACKGROUND: The use of magnets for the treatment of long gap esophageal atresia or "magnamosis" is associated with increased incidence of anastomotic strictures; however, little has been reported on other complications that may provide insight into refining selection criteria for appropriate use. METHODS: A single institution, retrospective review identified three cases referred for treatment after attempted magnamosis with significant complications. Their presentation, imaging, management, and outcomes were reviewed. RESULTS: All three patients had prior cervical or thoracic surgery to close a tracheoesophageal fistula prior to magnamosis, creating scar tissue that can prevent magnet induced esophageal movement, leading to either magnets not attracting enough or erosion into surrounding structures. Two patients had a reported four centimeter esophageal gap prior to attempted magnamosis, both failing to achieve esophageal anastomosis, suggesting that these gaps were either measured on tension with variability in gap measurement technique, or that the esophageal segments were fixed in position from scar tissue and unable to elongate. One patient had severe tracheobronchomalacia requiring tracheostomy, with improvement in his airway after eventual tracheobronchopexies, highlighting that magnamosis does not address comorbidities often associated with this patient population. CONCLUSIONS: We propose the following inclusion criteria and considerations for magnamosis: an esophageal gap truly less than four centimeters off tension with standardized measurement across centers, cautious use with a history of prior thoracic or cervical esophageal surgery, no associated tracheobronchomalacia or great vessel anomaly that would benefit from concurrent repair, and ideally to be used in centers equipped to manage potential complications. LEVEL OF EVIDENCE: Level IV treatment study.

Posterior tracheopexy for preterm infants with severe bronchopulmonary dysplasia and severe tracheobronchomalacia: A case series.

We report a series of four patients with severe bronchopulmonary dysplasia (BPD) who underwent posterior tracheopexy for severe tracheomalacia (TM). While posterior tracheopexy is an established surgical treatment for TM associated with tracheoesophageal fistula, it has not been previously described in TM associated with BPD. There were no significant intraoperative or postoperative complications from the surgeries. Three of the four patients required tracheostomy and mechanical ventilation, which may reflect the degree of lung disease and other multisystem comorbidities in these patients. More investigation is needed to determine whether posterior tracheopexy is an effective surgical option for TM related to BPD.