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.

Dynamic PEEP Study: A Non-invasive Diagnostic Exam to Assess for Effective PEEP in Children with Severe BPD.

OBJECTIVE: Tracheobronchomalacia (TBM) is common in neonates with bronchopulmonary dysplasia (BPD) and is associated with higher morbidity. This study evaluates the value of a CT protocol to assess the degree of TBM and gauge the adequacy of prescribed PEEP. STUDY DESIGN: Four infants with severe BPD on invasive mechanical ventilation underwent a chest CT protocol, including limited reduced-dose expiratory scans with varying PEEP levels. RESULTS: Baseline PEEP was adjusted in all subjects after performing the Dynamic PEEP CT. In two infants, the PEEP was increased due to significant TBM and in the other two without signs of TBM PEEP was decreased. The clinical course improved in all patients after adjusting PEEP. CONCLUSION: A "Dynamic PEEP" study may be reliable and non-invasive imaging modality for the evaluation of adequate ventilator settings in infants with severe BPD who are not optimal candidates for bronchoscopy.

A 70-Year-Old Man With Cough and Recurrent Respiratory Infections.

CASE PRESENTATION: A 70-year-old man was referred for evaluation of recurrent respiratory infections requiring antibiotics and chronic cough over 3 years. Two months prior to presentation, he started to develop blood-tinged sputum but not frank hemoptysis. He otherwise denied any fever, chills, night sweats, or weight loss. He had dyspnea during the respiratory infections but not otherwise. His medical history was significant for chronic rhinitis without sinusitis and a low serum IgM level. He was a never smoker and a farmer but otherwise had no significant or specific exposures or travel history. His family history was significant for alpha-1 antitrypsin deficiency in his mother.

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.

Advanced Therapies for Severe Tracheobronchomalacia: A Review of the Use of 3D-Printed, Patient-Specific, Externally Implanted, Bioresorbable Airway Splints.

Tracheobronchomalacia is a condition of dynamic collapse of the trachea and mainstem bronchi. The clinical significance of tracheobronchomalacia depends on its severity. Mild cases may be medically managed with limited symptomology, while severe cases require advanced therapies, lengthy hospital stays, and carry significant morbidity and mortality. Current therapies for severe tracheobronchomalacia include tracheostomy with prolonged mechanical ventilation, aortopexy, tracheobronchopexy, and intraluminal metallic, silicone, or bioresorbable stents. Three-dimensional (3D)-printed, patient-specific, bioresorbable airway splinting is a novel treatment option that is undergoing investigation in a cohort of critically ill children with severe tracheobronchomalacia. At the time of our last review of our data, 29 splints had been implanted in 15 children with intrathoracic tracheobronchomalacia. The median follow-up was 8.5 months. There were 12 long-term survivors, and all but one lived at home. This article discusses the details of our institution's development and use of 3D-printed, patient-specific, bioresorbable splints for treatment of severe tracheobronchomalacia in the pediatric population.

[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.

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.

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.

Tracheal opening manoeuvre (PEEP-20) in a patient with bronchopulmonary dysplasia and severe tracheobronchomalacia with neurally adjusted ventilatory assist (NAVA).

Bronchopulmonary dysplasia (BPD) is occasionally associated with tracheobronchomalacia, and it is this combination that can lead to serious outcomes. The most severe cases require tracheostomies, ventilatory support and eventually even tracheal stents or surgery. Ventilation in patients with tracheomalacia is complicated without a good patient-ventilator synchrony; the neurally adjusted ventilatory assist (NAVA) mode is potentially beneficial in these cases. This case report presents a patient affected by BPD and severe tracheobronchomalacia who was tracheostomised and ventilated 24 hours a day and who suffered from episodes of airway collapse despite using the NAVA mode. It was necessary to increase the positive end-expiratory pressure to 20 cmH2O (the PEEP-20 manoeuvre) for several minutes during an episode; this allowed the trachea to remain open and allowed us to optimise the patient's ventilation. This strategy has previously been described in a patient with tracheomalacia, reducing the frequency and need for sedation in the following episodes.

Pediatric Airway Stent Designed to Facilitate Mucus Transport and Atraumatic Removal.

OBJECTIVE: The goal was to develop a pediatric airway stent for treating tracheobronchomalacia that could be used as an alternative to positive pressure ventilation. The design goals were for the stent to allow mucus flow and to resist migration inside the airways, while also enabling easy insertion and removal. METHODS: A helical stent design, together with insertion and removal tools, is presented. A mechanics model of stent compression is derived to assist in selecting stent design parameters (pitch and wire diameter) that provide the desired amount of tracheal support, while introducing the minimal amount of foreign material into the airway. Worst-case airway area reduction with stent support is investigated experimentally using a pressurized tracheal phantom matched to porcine tracheal tissue properties. The stent design is then evaluated in a porcine in vivo experiment. RESULTS: Phantom testing validated the mechanics model of stent compression. In vivo testing demonstrated that the stent was well tolerated by the animal. Since the helical design covers only a small portion of the epithelium, mucus transport through the stented region was minimally impeded. Furthermore, the screw-like stent resisted migration, while also providing for atraumatic removal through the use of an unscrewing motion during removal. CONCLUSION: The proposed stent design and tools represent a promising approach to prevent airway collapse in children with tracheobronchomalacia. SIGNIFICANCE: The proposed technology overcomes the limitations of existing airway stents and may provide an alternative to maintaining children on a ventilator.

An Update on Tracheal and Airway Collapse in Dogs.

Tracheal and airway collapse (bronchomalacia) are common causes of chronic cough in middle-aged to older dogs in which weakening of cartilage within the respiratory system leads to narrowing of airways, irritation, inflammation, partial to complete airway obstruction, and other secondary effects. Tracheomalacia occurs in small-breed dogs, whereas bronchomalacia can occur in any size dog. Successful treatment involves correct identification of the problem, recognition of concurrent disease processes, and appropriate medical therapy. Surgical intervention and intraluminal stenting are readily available so it is important to understand indications for such procedures.

Williams-Campbell syndrome complicated with pulmonary hypertension and Type 2 respiratory failure: An adult case report.

Williams-Campbell syndrome, is a rare disorder characterized by a deficiency of cartilage in subsegmental bronchi, leading to distal airway collapse and bronchiectasis, which typically affects the fourth- to sixth-order bronchi. This article reported a 31-year-old female patient who was diagnosed with Williams-Campbell syndrome with pulmonary hypertension and Type 2 respiratory failure due to extensive cystic bronchiectasis. CT of the thorax showed the affected bronchi had characteristic ballooning on inspiration and collapse on expiration.

Biodegradable airway stents: Novel treatment of airway obstruction in children.

BACKGROUND: Tracheobronchomalacia is the most common cause of congenital airway obstruction in infants. An alternative for surgical approach (aortopexy) can be metallic airway stents. Usually, they are not considered as a first choice because of the relatively high risk of complications. Recent years have brought encouraging reports of biodegradable stents applied in children. OBJECTIVES: The aim of this study was to report our experience in the treatment of airway malacia using biodegradable stents. MATERIAL AND METHODS: Six polydioxanone (PDS), self-expanding custom-made stents (ELLA-CS) were implanted in 2 children: 3 in the patient with left main bronchus occlusion due to postpnemonectomy syndrome and 3 stents in the baby with tracheomalacia. RESULTS: Airway collapse was always relieved after stent expansion. Both patients needed repeated stenting because of limited stent lifespan. All the stents were implanted without complications through a rigid bronchoscope. The baby with stented main bronchus died because of irreversible lung lesion. CONCLUSIONS: This small study shows that biodegradable airway stents seem to be an attractive option in the treatment of tracheobronchomalacia in children. We consider this method to be safe, effective, repeatable, and reversible in small children with growing airways. As a time-buying procedure they can be especially useful in the treatment of tracheobronchomalacia.

Technique, pitfalls, quality, radiation dose and findings of dynamic 4-dimensional computed tomography for airway imaging in infants and children.

This retrospective review of 33 children's dynamic 4-dimensional (4-D) computed tomography (CT) images of the airways, performed using volume scanning on a 320-detector array without anaesthesia (free-breathing) and 1.4-s continuous scanning, was undertaken to report technique, pitfalls, quality, radiation doses and findings. Tracheobronchomalacia (airway diameter collapse >28%) was recorded. Age-matched routine chest CT scans and bronchograms acted as benchmarks for comparing effective dose. Pitfalls included failure to administer intravenous contrast, pull back endotracheal tubes and/or remove nasogastric tubes. Twenty-two studies (67%) were diagnostic. Motion artefact was present in 16 (48%). Mean effective dose: dynamic 4-D CT 1.0 mSv; routine CT chest, 1.0 mSv, and bronchograms, 1.4 mSv. Dynamic 4-D CT showed tracheobronchomalacia in 20 patients (61%) and cardiovascular abnormalities in 12 (36%). Fourteen children (70%) with tracheobronchomalacia were managed successfully by optimising conservative management, 5 (25%) underwent surgical interventions and 1 (5%) died from the presenting disorder.

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.