[Treatment of tracheo(broncho)malacia in children]. / Comment je traite la trachéo(broncho)malacie de l'enfant ?

Tracheomalacia (TM) is characterized by tracheal collapse due to an intrinsic anomaly resulting in a lack of rigidity of the cartilaginous rings and/or the posterior membrane during expiration, coughing or crying. It may also be secondary to external compression or acquired during endobronchial diseases. TM is commonly associated with other syndromes or airway abnormalities. Tracheomalacia can be localized or diffused and if the main bronchi are involved, the term of tracheobronchomalacia (TBM) is used. The most common symptoms include expiratory stridor, barking cough and recurrent respiratory tract infections. If tracheal weakness is severe, Acute Life Threating Events (ALTE) or Brief Resolved Unexplained Event (BRUE) can occur. While mild forms usually do not require any treatment, severe TBM may require medical and/or surgical management. Amongst several possible treatments, including tracheostomy, noninvasive ventilation and airway stenting, the pexy surgical approach (posterior, anterior tracheopexy or aortopexy) is currently the favoured option.

La trachéomalacie (TM) est caractérisée par un collapsus trachéal plus ou moins important durant l'expiration, lors des efforts de toux ou des pleurs. Elle peut être due à une anomalie intrinsèque, par manque de rigidité des anneaux cartilagineux et/ou de la membrane postérieure. Elle peut aussi avoir une origine extrinsèque, soit secondaire à une compression externe, soit acquise dans le cadre de pathologies endo-bronchiques. Elle peut enfin être associée à certains syndromes ou malformations des voies respiratoires. La TM peut être localisée ou généralisée, et si les bronches principales sont atteintes, on parlera de trachéobronchomalacie (TBM). Les symptômes les plus courants sont : un stridor expiratoire, une toux aboyante, et des infections respiratoires récurrentes. Dans les cas les plus sévères, des événements menaçant la vie de l'enfant (Acute Life-Threatening Event «ALTE¼ ou Brief Resolved Unexplained Event «BRUE¼) peuvent survenir. Alors que les formes légères ne requièrent généralement pas de traitement, la TBM sévère peut nécessiter une prise en charge médicale et/ou chirurgicale. Parmi les divers choix thérapeutiques, incluant notamment la trachéostomie, la ventilation non invasive et les stents trachéaux, l'approche chirurgicale par pexie (aortopexie, trachéopexie postérieure ou antérieure) est actuellement l'option favorite.

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.

Expiratory Central Airway Collapse in Adults: Corrective Treatment (Part 2).

Corrective treatment of expiratory central airway collapse (ECAC) consists of placement of airway stents or tracheobronchoplasty (TBP). The indication for corrective treatment is severe central airway collapse (>90 %), and severe symptoms that cause decline in quality of life. Patients are selected to undergo a trial of tracheal "Y" stent placement. If symptoms improve (positive trial) they undergo a TBP, provided they are good surgical candidates. Patients who are considered poor surgical candidates because of the severity of comorbidities can be offered permanent stenting to palliate symptoms. The anesthetic management of airway stent placement and TBP is complex. This article reviews the medical management and corrective treatment of ECAC, anesthetic management of airway stent placement, and considerations during TBP.

Expiratory Central Airway Collapse in Adults: Anesthetic Implications (Part 1).

Expiratory central airway collapse (ECAC) is a general term that incorporates tracheobronchomalacia (TBM) and excessive dynamic airway collapse (EDAC). TBM and EDAC are progressive, degenerative disorders of the tracheobronchial tree, causing airway collapse. Induction of general anesthesia can trigger intraoperative airway collapse in patients with these conditions. This crisis presents as the sudden inability to ventilate, which can lead to life-threatening hypoxemia and hypercapnia. This article reviews the definition, pathophysiology, diagnosis, and anesthetic implications of ECAC.

Tracheobronchomalacia and Excessive Dynamic Airway Collapse: Medical and Surgical Treatment.

Excessive central airway collapse (ECAC) is characterized by excessive narrowing of the airway lumen during exhalation leading to dyspnea, cough, mucostasis, recurrent respiratory infections, and poor quality of life. Tracheobronchomalacia and excessive dynamic airway collapse are heterogeneous entities of ECAC and are characterized by a diverse nonspecific symptom profile. Although the pathophysiology of airway mechanics as well as morphology in both entities is different, current evidence so far shows no practical benefit in making such distinction since both have similar symptoms and the diagnostic and therapeutic work-ups are the same. The diagnosis of ECAC should be based on dynamic flexible bronchoscopy and/or dynamic computed tomography scan as well as clinical symptoms that are not fully explained by other lung diseases. Initial treatment of symptomatic ECAC includes treatment of coexisting conditions (such as chronic obstructive pulmonary disease, asthma, gastroesophageal reflux disease, and vocal cord dysfunction) and supportive treatment of dynamic central airway collapse (antibiotics for respiratory infections, aggressive therapy, pulmonary physiotherapy, pulmonary rehabilitation, and continuous positive airway pressure). A short-term stent trial in selected patients with severe symptomatic ECAC is needed to assess whether patients will have improvement in symptoms and thus identify patients who will benefit from surgical central airway stabilization. A multidisciplinary airway team in highly specialized centers with experience in the evaluation and treatment of this patient population is essential for optimal outcomes.

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.

Paediatric Tracheomalacia.

Intrathoracic tracheomalacia is characterized by increased compliance of the central airway within the thorax. This leads to excessive dynamic collapse during exhalation or periods of increased intrathoracic pressure such as crying. Extrathoracic tracheomalacia involves dynamic collapse of the airway between the glottis and sternal notch that occurs during inhalation rather than exhalation. The tone of the posterior membrane of the trachea increases throughout development and childhood, as does the rigidity of the tracheal cartilage. Abnormalities of airway maturation result in congenital tracheomalacia. Acquired tracheomalacia occurs in the normally developed trachea due to trauma, external compression, or airway inflammation. Although tracheomalacia can be suspected by history, physical examination, and supportive radiographic findings, flexible fiberoptic bronchoscopy remains the "gold standard" for diagnosis. Current treatment strategies involve pharmacotherapy with cholinergic agents, positive pressure ventilation, and surgical repair.

Surgical Treatment of Tracheobronchomalacia: A novel approach.

Tracheobronchomalacia, as a whole, is likely misdiagnosed and underestimated as a cause of respiratory compromise in pediatric patients. Currently, there is no standardized approach for the overall evaluation of pediatric tracheobronchomalacia (TBM) and the concept of excessive dynamic airway collapse (EDAC); no grading score for the evaluation of severity; nor a standardized means to successfully approach TBM and EDAC. This paper describes our experience standardizing the approach to these complex patients whose backgrounds include different disease etiologies, as well as a variety of comorbid conditions. Preoperative and postoperative evaluation of patients with severe TBM and EDAC, as well as concurrent development of a prospective grading scale, has allowed us to ascertain correlation between surgery, symptoms, and effectiveness on particular tracheal-bronchial segments. Long-term, continued collection of patient characteristics, surgical technique, complications, and outcomes must be collected given the overall heterogeneity of this particular population.

Short-Term Use of Uncovered Self-Expanding Metallic Airway Stents for Severe Expiratory Central Airway Collapse.

BACKGROUND: Patients with severe symptomatic expiratory central airway collapse (ECAC) undergo a stent trial to determine whether they are candidate for tracheobronchoplasty. Most stent trials were done using silicone stents. However, there was a higher number of silicone stent-related complications. OBJECTIVES: The aim of this study was to evaluate the safety and efficacy of short-term uncovered self-expanding metallic airway stents (USEMAS) in patients with ECAC. METHODS: This was a retrospective review. Baseline measurements were compared to those obtained after 7-14 days. Measurements included: Modified Medical Research Council (mMRC), Cough Quality of Life Questionnaire (CQLQ), spirometry testing, and 6-Minute Walk Test (6MWT). Stent- and procedure-related complications were reported. RESULTS: 33 patients (median age, 52 years) underwent the USEMAS trial. Presenting symptoms were dyspnea in 100%, intractable cough in 90.3%, recurrent infection in 42.2%, and inability to clear secretions in 21.4%. Dyspnea, cough, and secretion clearance improved in 88, 70, and 57%, respectively. Overall, there was a significant improvement in mMRC (p < 0.001), CQLQ (p = 0.015), and 6MWT (p = 0.015). There was 1 airway infection, 1 stent migration, and 1 pneumothorax. The median duration of USEMAS was 7 days. All stents were removed without any complications. At the time of stent removal, no granulation tissue was observed in 30.9%, and mild granulation tissue was observed in 69.1%. CONCLUSION: The short-term USEMAS trial improves respiratory symptoms, quality of life, and exercise capacity with few complications in patients with severe symptomatic ECAC when performed by a multidisciplinary airway team in highly specialized centers with experience in the evaluation and treatment of this patient population.

[Tracheobronchoplasty for Severe Diffuse Tracheomalacia]. / Tracheobronchoplastie bei schwerer diffuser Tracheobronchomalazie.

Patients with diffuse airway instability due to tracheobronchomalacia or excessive dynamic airway collapse are typically highly symptomatic, with marked dyspnoea, recurrent bronchopulmonary infections and excruciating intractable cough. Silicone stents achieve immediate symptom control, but are - due to the typical complications associated with stent treatment - usually not an option for long-term treatment. The aim of surgical intervention is definitive stabilisation of the trachea and of both main bronchi by posterior splinting of the Paries membranaceus with a polypropylene mesh. This operation is an appropriate treatment option for patients with documented severe tracheobronchomalacia or excessive dynamic airway collapse and is ultimately the only therapy that can achieve permanent symptom control. The success of the operation, however, depends on many factors and requires close interdisciplinary collaboration.

Spirometer-controlled cine magnetic resonance imaging used to diagnose tracheobronchomalacia in paediatric patients.

Tracheobronchomalacia (TBM) is defined as an excessive collapse of the intrathoracic trachea. Bronchoscopy is the gold standard for diagnosing TBM; however it has major disadvantages, such as general anaesthesia. Cine computed tomography (CT) is a noninvasive alternative used to diagnose TBM, but its use in children is restricted by ionising radiation. Our aim was to evaluate the feasibility of spirometer-controlled cine magnetic resonance imaging (MRI) as an alternative to cine-CT in a retrospective study. 12 children with a mean age (range) of 12 years (7-17 years), suspected of having TBM, underwent cine-MRI. Static scans were acquired at end-inspiration and expiration covering the thorax using a three-dimensional spoiled gradient echo sequence. Three-dimensional dynamic scans were performed covering only the central airways. TBM was defined as a decrease of the trachea or bronchi diameter >50% at end-expiration in the static and dynamic scans. The success rate of the cine-MRI protocol was 92%. Cine-MRI was compared with bronchoscopy or chest CT in seven subjects. TBM was diagnosed by cine-MRI in seven (58%) out of 12 children and was confirmed by bronchoscopy or CT. In four patients, cine-MRI demonstrated tracheal narrowing that was not present in the static scans. Spirometer controlled cine-MRI is a promising technique to assess TBM in children and has the potential to replace bronchoscopy.

Prevalence and Clinical Characteristics of Pediatric Lower Airway Malacia: Case Series from a Tertiary Center in Turkey.

Introduction: Lower airway malacia (LAM) is characterized by a reduction in the cross-sectional luminal area during quiet respiration. There is no gold standard diagnostic test; however, flexible fiberoptic bronchoscopy (FFB) is most frequently utilized. The exact prevalence and incidence of LAM are unknown. This study aimed to determine the prevalence rates of pediatric patients diagnosed with LAM, offer a detailed understanding of their demographic and clinical characteristics, and investigate distinctions between two specific types of LAM, namely, tracheomalacia (TM) and bronchomalacia (BM). Materials and Methods: Patients younger than 18 years diagnosed with LAM using FFB were included in this retrospective case series. Demographic and clinical characteristics and comorbid disorders were compared between patients with isolated BM and those with isolated TM or tracheobronchomalacia (TM/TBM). Results: Among 390 patients who underwent FFB, 65 (16.6%) were diagnosed with LAM, 16 (24.6%) with TM, and 56 (86.2%) with BM. The median age at diagnosis was 15 months. Among them, 59 (90.8%) had other comorbidities; gastrointestinal (GI) disorders were the most common (38.5%). The most common indications for bronchoscopy were recurrent/prolonged lower respiratory tract infections (LRTI) or wheezing (43.1%), while the most frequently observed respiratory physical examination finding was stridor (35.4%). Patients with TM/TBM had significantly higher frequencies of premature births, stridor, retraction, and GI disorders. Conclusion: Patients with stridor without typical laryngomalacia features or recurrent or prolonged LRTI should undergo prompt evaluation for LAM. The potential coexistence of GI disorders such as gastroesophageal reflux disease and swallowing dysfunction should also be considered.

Cardiovascular-associated tracheobronchial obstruction in children.

Tracheobronchial compression of cardiovascular origin is an uncommon and frequently unrecognised cause of respiratory distress in children. The compression may be due to encircling vessels or dilated neighbouring cardiovascular structures. Bronchoscopy and detailed radiography, especially computed tomography and magnetic resonance imaging, are among the most powerful diagnostic tools. Few previous reports have addressed the relationship between bronchoscopic findings and underlying cardiovascular anomalies. The objective of this study was to correlate bronchoscopic and radiographic findings in children with cardiovascular-associated airway obstruction. A total of 41 patients were recruited for the study. Patients with airway obstruction were stratified on the basis of the aetiology of the cardiovascular structures and haemodynamics into an anatomy-associated group and a haemodynamics-associated group. In the anatomy-associated group, stenosis and malacia were found with comparable frequency on bronchoscopy, and the airway obstructions were mostly found in the trachea (71% of patients). In the haemodynamics-associated group, malacia was the most common bronchoscopic finding (85% of patients), and nearly all locations of airway involvement were in the airway below the carina (90% of patients). The tracheal compression was usually caused by aberrant systemic branching arteries in the anatomy-associated group. In the haemodynamics-associated group, the causal relationships varied. Tracheal compression was often caused by lesions of the main pulmonary artery and aorta, whereas obstruction of the right main bronchus was caused by lesions of the main pulmonary artery and right pulmonary artery. The causes of left main bronchus compression were more diverse. In summary, the bronchoscopic presentations and locations are quite different between these two groups.

External stabilization for severe tracheobronchomalacia using separated ring-reinforced ePTFE grafts is effective and safe on a long-term basis.

PURPOSE: Tracheobronchomalacia is a respiratory disorder that results from abnormal cartilage development. Since 2003, we have performed external stabilization for severe tracheobronchomalacia using separated ring-reinforced expanded polytetrafluoroethylene (ePTFE) grafts. The aim of this study was to analyze the short- and long-term outcomes of our procedure. METHODS: We reviewed the charts of patients who underwent external tracheobronchial stabilization from January 2003 through December 2012. Separated ring-reinforced ePTFE grafts were fixed to the anterior and posterior lesions of malacia independently, with confirmation of a well-opened lumen by bronchoscopy. RESULTS: Five children with severe tracheobronchomalacia underwent six operations. The median follow-up period was 43 months (range 11-109 months). There was no operative mortality associated with the procedure. One patient required removal of a section of the graft owing to the development of bronchial granulation tissue at the site of fixation. All patients showed improved respiratory function, and tracheal growth was confirmed by follow-up computed tomography. CONCLUSIONS: External stabilization with separated ring-reinforced ePTFE grafts for severe tracheobronchomalacia is effective, less invasive than alternatives and preserves the growth potential of the affected airway segment.

[Diagnostic values of bronchoscopy and multi-slice spiral CT for congenital dysplasia of the respiratory system in infants: a comparative study].

OBJECTIVE: To investigate and compare the diagnostic values of bronchoscopy and multi-slice spiral computed tomography (CT) for congenital dysplasia of the respiratory system in infants. METHODS: Analysis was performed on the clinical data, bronchoscopic findings and multi-slice spiral CT findings of 319 infants (≤1 years old) who underwent bronchoscopy and/or multi-slice spiral CT and were diagnosed with congenital dysplasia of the respiratory system. RESULTS: A total of 476 cases of congenital dysplasia of the respiratory system were found in the 319 infants, including primary dysplasia of the respiratory system (392 cases) and compressive dysplasia of the respiratory system (84 cases). Of the 392 cases of primary dysplasia of the respiratory system, 225 (57.4%) were diagnosed by bronchoscopy versus 167 (42.6%) by multi-slice spiral CT. There were significant differences in etiological diagnosis between bronchoscopy and multi-slice spiral CT in infants with congenital dysplasia of the respiratory system (P<0.05). All 76 cases of primary dysplasia of the respiratory system caused by tracheobronchomalacia were diagnosed by bronchoscopy and all 17 cases of primary dysplasia of the respiratory system caused by lung tissue dysplasia were diagnosed by multi-slice spiral CT. Of the 84 cases of compressive dysplasia of the respiratory system, 74 cases were diagnosed by multi-slice spiral CT and only 10 cases were diagnosed by bronchoscopy. CONCLUSIONS: Compared with multi-slice spiral CT, bronchoscopy can detect primary dysplasia of the respiratory system more directly. Bronchoscopy is valuable in the confirmed diagnosis of tracheobronchomalacia. Multi-slice spiral CT has a higher diagnostic value for lung tissue dysplasia than bronchoscopy.

Coordination of Care for Expiratory Central Airway Collapse: A Structured Process for a Multifaceted Disease.

Common respiratory symptoms, including dyspnea, cough, sputum production, and recurrent infections, frequently remain without a clear cause and may be the result of expiratory central airway collapse (ECAC). Establishing the diagnosis and appropriate treatment plan for patients with ECAC is challenging and benefits from a multidisciplinary approach. A coordinator role is crucial in this process to ensure optimal patient-centered outcomes. We describe the coordination of care in the process of diagnosing and treating ECAC. The coordinator leads the organization of the multiple services involved in the care of patients with ECAC, including pulmonary medicine, interventional pulmonology, radiology, and thoracic surgery, as well as hospital inpatient staff. From initial screening to evaluation and management with airway stents and corrective treatment with tracheobronchoplasty, the ECAC coordinator oversees the entire process of care for patients with ECAC.