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.

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.

A novel ex vivo tracheobronchomalacia model for airway stent testing and in vivo model refinement.

OBJECTIVES: We sought to develop an ex vivo trachea model capable of producing mild, moderate, and severe tracheobronchomalacia for optimizing airway stent design. We also aimed to determine the amount of cartilage resection required for achieving different tracheobronchomalacia grades that can be used in animal models. METHODS: We developed an ex vivo trachea test system that enabled video-based measurement of internal cross-sectional area as intratracheal pressure was cyclically varied for peak negative pressures of 20 to 80 cm H2O. Fresh ovine tracheas were induced with tracheobronchomalacia by single mid-anterior incision (n = 4), mid-anterior circumferential cartilage resection of 25% (n = 4), and 50% per cartilage ring (n = 4) along an approximately 3-cm length. Intact tracheas (n = 4) were used as control. All experimental tracheas were mounted and experimentally evaluated. In addition, helical stents of 2 different pitches (6 mm and 12 mm) and wire diameters (0.52 mm and 0.6 mm) were tested in tracheas with 25% (n = 3) and 50% (n = 3) circumferentially resected cartilage rings. The percentage collapse in tracheal cross-sectional area was calculated from the recorded video contours for each experiment. RESULTS: Ex vivo tracheas compromised by single incision and 25% and 50% circumferential cartilage resection produce tracheal collapse corresponding to clinical grades of mild, moderate, and severe tracheobronchomalacia, respectively. A single anterior cartilage incision produces saber-sheath type tracheobronchomalacia, whereas 25% and 50% circumferential cartilage resection produce circumferential tracheobronchomalacia. Stent testing enabled the selection of stent design parameters such that airway collapse associated with moderate and severe tracheobronchomalacia could be reduced to conform to, but not exceed, that of intact tracheas (12-mm pitch, 0.6-mm wire diameter). CONCLUSIONS: The ex vivo trachea model is a robust platform that enables systematic study and treatment of different grades and morphologies of airway collapse and tracheobronchomalacia. It is a novel tool for optimization of stent design before advancing to in vivo animal models.

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.

Tracheopexy and aortopexy for circumflex aortic arch on a small infant.

Circumflex aortic arch is a rare vascular ring anomaly, which is often associated with tracheobronchomalacia. Aortic uncrossing is the definitive repair of this condition, which is a major high-risk operation; therefore, it is not suitable for small patients such as newborn infants or individuals who have severe comorbidities. We present a case of the circumflex aortic arch with tracheobronchomalacia in a 2.7-kg infant who underwent successful innovative combined surgery of the airway and the aorta.

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.

Paediatric tracheobronchomalacia: Incidence, patient characteristics, and predictors of surgical intervention.

OBJECTIVES: Tracheobronchomalacia (TBM), a condition where an abnormality of the tracheal walls causes collapse during the respiratory cycle, is a common cause of airway obstruction in childhood. TBM can present with a large spectrum of disease severity and underlying pathologies that may be managed medically and surgically, and it is not always clear which patients would most benefit from surgical intervention. We aim to describe the incidence, patient characteristics, and predictors of surgical intervention in a large cohort of paediatric patients. METHODS: We performed a retrospective review of all children diagnosed with TBM to a paediatric Otolaryngology unit in the west of Scotland between 2010 and 2020. Odds ratios for clinical predictors of surgery were calculated using logistic regression with uni- and multivariate analysis. RESULTS: 249 patients were identified of which 219 proceeded to data collection. Primary malacia was noted in 161 (73.5%) and secondary in 58 (26.5%). Causes of secondary malacia included compression by the innominate artery (11%) and vascular rings (7.8%). Surgical interventions were performed in 28 patients (12.8%) including division of vascular ring, aortopexy, and surgical tracheostomy. Multivariate analysis showed secondary TBM, acute life-threatening events, and difficulty weaning from mechanical ventilation were independent risk factors for surgical intervention. CONCLUSIONS: TBM can present with a myriad of airway symptoms and is frequently associated with other airway and mediastinal pathologies necessitating multiple interventions. Children aged <1 year present with a more severe form of the disease and the presence of particular independent risk factors may indicate a need for surgical intervention.

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.

Bronchomalacia in Right Aortic Arch Treated With Descending Aortic Translocation and Airway Splint.

Management of recurrent symptomatic tracheobronchomalacia and tracheobronchial compression after prior aortopexy and tracheobronchopexy is a surgical challenge. In patients with right aortic arch, the course of the aortic arch over the right mainstem bronchus can result in superior and posterior compression of the airway. This report presents 2 cases of recurrent bronchomalacia and bronchial compression treated with descending aortic translocation. The addition of bronchial splinting to aortic translocation effectively relieved airway symptoms.

Current concepts in tracheobronchomalacia: diagnosis and treatment.

Airway collapse from dynamic tracheobronchomalacia (TBM), static compression from vascular compression, and/or tracheobronchial deformation are challenging conditions. Patients are best assessed and managed by a multidisciplinary team in centers specializing in complex pediatric airway disorders. Suspicion is made through clinical history and physical examination, diagnosis of location and severity by dynamic 3-phase bronchoscopy, and surgical treatment planning by MDCT and other studies as necessary to completely understand the problems. The treatment plan should be patient-based with a thorough approach to the underlying pathology, clinical concerns, and combined abnormalities. Patients should undergo maximum medical therapy prior to committing to other interventions. For those children considered candidates for surgical intervention, all other associated conditions, including vascular anomalies, chest wall deformities, mediastinal lesions, or other airway pathologies, should also be considered. Our preference is to correct the airway lesions at the same operation as other comorbidities, if possible, to prevent multiple reoperations with their attendant increased risks. We also strongly advocate for the use of recurrent laryngeal nerve monitoring in all cases of cervical or thoracic surgery to minimize the risks to vocal cord function and laryngeal sensation. Studies that evaluate the effect of these interventions on the patient and caregiver's quality of life are needed to fully grasp the impact of TBM on this challenging patient population.

Clinical survey and predictors for the development of tracheobronchomalacia in preterm infants.

BACKGROUND: Tracheobronchomalacia (TBM) contributes to the increased morbidity and mortality observed in preterm infants. Effective strategies for the prevention of TBM are necessary to achieve better outcomes. We sought to identify risk factors associated with the development of TBM in preterm infants. Optimal cut-off values for each risk factor were also determined. METHODS: A total of 80 infants who were born at 36 week's gestation or earlier and underwent flexible bronchoscopy were included in our study sample. A comparison of demographic and clinical risk factors between those with TBM (n = 35, 44%) and those without TBM (n = 45, 56%) was conducted using multivariate logistic regression analysis. Receiver operating characteristic curve analysis was performed to determine the appropriate cut-off values for predicting the development of TBM. RESULTS: In the multivariate analysis, only peak inspiratory pressure (PIP) and the number of intubation days remained significantly different between infants with and without TBM. Preterm infants with TBM received higher PIP (odds ratio: [OR], 1.067; 95% confidence interval [CI], 1.010-1.128; p = .020) and were intubated for longer (odds ratio [OR], 1.019; 95% CI, 1.003-1.035; p = .016) than those without TBM. Infants who received PIP > 19.5 cmH2 O or were intubated for >79.5 days were associated with a significantly higher risk of presence of TBM. CONCLUSION: High PIP and prolonged intubation were major risk factors for the development of TBM in premature infants. Those who require PIP > 19.5 cmH2 O or intubation >79.5 days warrant bronchoscopy examination for early diagnosis and management of TBM.

[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.

A successful surgical tracheobronchoplasty in a case of expiratory collapse of central airways associated with tracheobronchomalacia in a severely deformed single lung patient.

A 67-year-old male with a severe body deformity and a total collapse of the left lung due to infantile paralysis was admitted to a regional hospital for a spinal fracture. He suffered from cardiopulmonary arrest during the hospitalization. Although extubation was tried several times after resuscitation, he went into cardiopulmonary arrest repeatedly. The expiratory collapse of the central airways due to tracheobronchomalacia was suspected, requiring tracheostomy with persistent positive pressure ventilation. He was transferred to our hospital after several unsuccessful endobronchial interventions. Severe tracheobronchomalacia was diagnosed with dynamic bronchoscopy, and surgical tracheobronchoplasty using a polypropylene mesh was performed. A modified surgical approach was utilized to stabilize the intraoperative respiratory status in this particular patient with a severely deformed body and a single lung. Consequently, the tracheobronchoplasty was completed without intraoperative complications. The postoperative course was also uneventful, and the patient was ventilator-free on postoperative day 7.

Expiratory central airway collapse - an overlooked entity?: Two case reports.

INTRODUCTION: Expiratory central airway collapse is defined by excessive inward bulging of either tracheobronchial posterior membrane or cartilage. The former is called excessive dynamic airway collapse (EDAC), and the latter, depending on the site of collapse, tracheomalacia, bronchomalacia or tracheobronchomalacia. Due to their non-specific symptoms and lack of awareness amongst clinicians they tend to be mislabeled as common obstructive lung disorders, or complicate their course undetected. Particular controversies refer to EDAC sometimes considered just as a symptom of obstructive lung disease and not a separate entity. Nonetheless, a growing body of evidence indicates that EDAC might be present in patients without apparent obstructive lung disease or it might be an independent risk factor in chronic obstructive pulmonary disease or asthma patients. PATIENT CONCERNS: Patient #1 was admitted because of idiopathic chronic cough whereas patient #2 was admitted for differential diagnosis of dyspnea of uncertain etiology. In both patients symptoms were unresponsive to bronchodilators and inhaled corticosteroids. FINDINGS AND DIAGNOSIS: In both patients an excess collapse of tracheobronchial posterior membrane was detected during bronchoscopy; in patient #1, of right main bronchus and right upper lobe bronchus and in patient #2 of right upper lobe bronchus and both main bronchi. Excess central airway collapse in patient #2 was also visualized on expiratory chest CT. In patient #1 spirometry did not reveal obturation, whereas in patient #2 only mild, irreversible, obstruction was revealed, disproportionate to patients significant breathlessness. INTERVENTIONS: Both patients were treated with N-acetylcysteine and adjustable positive expiratory pressure valves. OUTCOMES: Due to aforementioned treatment chronic cough in patient #1 subsided almost completely whereas patient's #2 dyspnea improved significantly. CONCLUSIONS: In presented cases EDAC was an unexpected finding, even though, it firmly corresponded with reported symptoms. Treatment modification led to improvement of patients quality of life.

[Evaluation with chest CT scan of CPAP as a treatment for tracheobronchomalacia in the frame of a Mounier-Kuhn syndrome]. / Évaluation par tomodensitométrie thoracique d'un traitement de la trachéobronchomalacie par pression positive continue dans le cadre d'un syndrome de Mounier­Kuhn.

INTRODUCTION: Tracheobronchomegaly disease is often associated with a tracheobronchomalacia which is responsible for recurrent lower respiratory tract infections. Currently there is no evidence to support any specific treatment for the condition. CASE REPORT: We report the case of a 79 years old patient presenting with tracheobronchomegaly in the context of Mounier-Kuhn syndrome complicated by a tracheobronchomalacia responsible for her symptomatology. The diagnosis of tracheobronchomalacia had been confirmed by high-resolution chest computed tomography (CT) with expiratory slices and virtual bronchoscopy. Treatment with continuous positive airway pressure (CPAP) was proposed, and we confirmed its efficacy using high-resolution chest CT, which showed a decrease in tracheobronchial collapse and a reduction in air trapping. CONCLUSIONS: Three-dimensional virtual bronchoscopy is an interesting tool and a noninvasive method to diagnose tracheobronchomegaly for patients who are at a high anesthetic risk. It is also possible to use it demonstrate the effect of CPAP in tracheobronchomalacia.

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.

The role of the pediatrician in caring for children with tracheobronchomalacia.

INTRODUCTION: Children with tracheobronchomalacia (TBM) experience excessive dynamic collapse of the central airway(s). TBM remains an under-diagnosed condition, and there is on ongoing need to raise awareness amongst pediatricians. AREAS COVERED: The literature from PubMed, MEDLINE, EMBASE and Cochrane Controlled Trials Register electronic databases was searched from 1 January 1980 to 14 January 2020. Eligible studies relating to the diagnosis, investigation and management of tracheobronchomalacia in children were included. In this review, we highlight the clinical symptoms of TBM such as the typical barking cough, wheezing, recurrent lower respiratory tract infections or acute life-threatening events. These symptoms worsen when the child is making increased respiratory efforts, such as during crying, coughing and during intercurrent infective illness. This article focuses on the role of the pediatrician in recognizing the condition, the investigative process, and the medical management based on the clinical severity. The principle of management should be holistic, tackling the medical issues of TBM and associated comorbidities, as providing support to families. EXPERT OPINION: There remains a need to devise objective and reproducible bronchoscopic and radiological definitions of severity of TBM. Further studies looking at long-term outcomes of medical therapies used in TBM are required.