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.

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.

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.

First series of minimally invasive, robot-assisted tracheobronchoplasty with mesh for severe tracheobronchomalacia.

OBJECTIVE: Tracheobronchomalacia is a progressive, debilitating disease with limited treatment options. Open tracheobronchoplasty (TBP) is an accepted surgical option for management of severe tracheobronchomalacia. This study examined the outcomes of the first reported series of robot-assisted TBP (R-TBP). METHODS: We retrospectively reviewed the records of patients with clinical suspicion for tracheobronchomalacia who had dynamic computed tomography scan and subsequent R-TBP from May 2016 to December 2017. RESULTS: Four hundred thirty-five patients underwent dynamic computed tomography scan for suspicion of tracheobronchomalacia. Of this group, 42 patients underwent R-TBP. In the surgery group, the median age was 66 years (interquartile range, 39-72 years) and there were 30 women (71%). Respiratory comorbidities included asthma (88%) and chronic obstructive pulmonary disease (52%). The median operative time was 249 minutes (interquartile range, 266-277 minutes). Median hospital length of stay was 3 days (interquartile range, 2-4.75 days), and there were 19 postoperative complications (11 minor and 8 major). There were no mortalities at 90 days. Comparison of preoperative and postoperative pulmonary function testing demonstrated improvement in forced expiratory volume at 1 second by 13.5% (P = .01), forced vital capacity by 14.5% (P < .0001), and peak expiratory flow rate by 21.0% (P < .0001). Quality of life questionnaires also showed improvement with 82% reporting overall satisfaction with the procedure. CONCLUSIONS: R-TBP can be performed with low morbidity and mortality. Early follow-up reveals significant improvement in pulmonary function testing and high patient satisfaction when compared with preoperative baseline. Long-term follow-up is needed to demonstrate the durability of R-TBP and substantiate its role in the management of patients with symptomatic, severe tracheobronchomalacia.

Longitudinal Follow-up of Patients With Tracheobronchomalacia After Undergoing Tracheobronchoplasty: Computed Tomography Findings and Clinical Correlation.

PURPOSE: The purpose of this study was to evaluate intermediate and long-term changes in expiratory tracheal collapsibility by computed tomography (CT) in patients with tracheobronchomalacia following surgical treatment with tracheobronchoplasty and to correlate CT findings with clinical findings. MATERIALS AND METHODS: Between 2003 and 2016, 18 patients with tracheobronchomalacia underwent tracheobronchoplasty and were imaged preoperatively and postoperatively at both intermediate and long-term intervals. Imaging included end-inspiratory and dynamic expiratory phase scans. The cross-sectional area of the airway lumen was measured at 2 standard levels (1 cm above the aortic arch and carina). These measurements were used to calculate % collapsibility. Clinical findings recorded included a questionnaire on symptomatology and a 6-minute walk test. RESULTS: Before surgery, expiratory collapsibility of the upper trachea was 72%±25% (mean±SD) and that of the lower trachea was 68%±22%. On intermediate follow-up (mean, 1.5 y), collapsibility significantly decreased to 37%±21% at the upper trachea and 35%±19% at the lower trachea (P<0.001). On long-term follow-up (mean, 6 y), collapsibility increased to 51%±20% at the upper trachea and 47%±17% at the lower trachea and was significantly worse than on intermediate follow-up (P=0.002). However, collapsibility on long-term follow-up remained significantly lower than preoperative collapsibility (P=0.015). Clinical findings showed a similar trend as quantitative CT measurements. CONCLUSION: Expiratory tracheal collapsibility substantially decreases after tracheobronchoplasty on intermediate follow-up. At long-term follow-up, tracheal collapsibility shows a modest increase, but remains significantly lower than the preoperative baseline. Quantitative measurements from dynamic CT have the potential to play an important role as imaging biomarkers for assessing response to tracheobronchoplasty.

The Prevalence of Gastroesophageal Reflux in Patients With Excessive Central Airway Collapse.

BACKGROUND: Gastroesophageal reflux (GER) is increasingly recognized as an exacerbating or causal factor in several respiratory diseases. There is a high prevalence of GER in infants with airway malacia. However, such data are lacking in adults. METHODS: This retrospective study was conducted to determine the relationship between GER and excessive central airway collapse (ECAC). The study included consecutive patients with ECAC referred to the Complex Airway Center at Beth Israel Deaconess Medical Center who underwent esophageal pH testing for GER between July 2014 and June 2018. RESULTS: Sixty-three of 139 patients with ECAC (45.3%) had documented GER as shown by an abnormal esophageal pH test result. The mean DeMeester score was 32.2, with a symptom association probability of 39.7% of GER-positive patients. Twenty-nine of 63 patients (46%) with GER reported improvement in respiratory symptoms following maximal medical therapy or antireflux surgery without requiring further treatment for ECAC. CONCLUSIONS: GER is prevalent among patients with ECAC, and aggressive reflux treatment should be considered in these patients prior to considering invasive airway procedures or surgery.

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.

Tracheobronchial stenting for airway malacia.

Tracheobronchomalacia is a rare but clinically troublesome condition in paediatrics. The softening of the major airways - which can include some or all of the tracheobronchial tree can lead to symptoms ranging from the minor (harsh barking cough, recurrent chest infections) to severe respiratory difficulties including prolonged ventilator support and 'near death attacks'. The causes are broadly divided into intrinsic softening of the airway wall which is considered a primary defect (e.g. syndromes; post tracheo-oesophageal fistula repair; extreme prematurity) or secondary malacia due to external compression from vascular structures or cardiac components. These secondary changes can persist even when the external compression is relieved, for example, following the repair of a pulmonary artery sling or double aortic arch. For children with severe clinical symptoms attributed to malacia, consideration is given to possible surgical remedies such as an aortopexy for short limited areas of malacia, or long term positive pressure support with CPAP either by non invasive or tracheostomy interface. More recently the role of stenting in children is receiving attention, especially with the development of newer techniques such as bioabsorbable stents which buy time for a natural history of improvement in the malacia to occur. This paper reviews the stents available and discusses the pros and cons of stenting in paediatric airway malacia.

[Characteristics of tidal breathing pulmonary function in children with tracheobronchomalacia].

OBJECTIVE: To investigate the characteristics of tidal breathing pulmonary function in children with tracheobronchomalacia (TBM). METHODS: In this study, 30 children who were diagnosed with TBM using electronic bronchoscopy were enrolled in the observation group; 30 healthy children were recruited in the normal control group. For individuals in each group, the assessment of tidal breath pulmonary function was performed at diagnosis and 3, 6, 9, and 12 months after diagnosis. RESULTS: There were no significant differences in tidal volume, inspiratory time, expiratory time, and inspiratory to expiratory ratio between the two groups (P>0.05). Compared with the control group, the observation group had a significantly higher respiratory rate and significantly lower ratio of time to peak tidal expiratory flow to total expiratory time (TPTEF/TE) and ratio of volume to peak tidal expiratory flow to total expiratory volume (VPTEF/VE). There was a time-dependent increase in TPTEF/TE and VPTEF/VE for TBM children from the time of initial diagnosis to 12 months after diagnosis. CONCLUSIONS: Tidal breathing pulmonary function has characteristic changes in children with TBM. Tidal breathing pulmonary function tends to be recovered with increased age in children with TBM.

Computational fluid dynamics evaluation of excessive dynamic airway collapse.

BACKGROUND: Excessive dynamic airway collapse, which is often caused by the collapse of the posterior membrane wall during exhalation, is often misdiagnosed with other diseases; stents can provide support for the collapsing airways. The standard pulmonary function tests do not necessarily show change in functional breathing condition for evaluation of these type of diseases. METHODS: Flow characteristics through a patient's airways with excessive dynamic airway collapse have been numerically investigated. A stent was placed to support the collapsing airway and to improve breathing conditions. Computed tomography images of the patient's pre- and post-stenting were used for generating 3-Dimensional models of the airways, and were imported into a computational fluid dynamics software for simulation of realistic air flow behavior. Unsteady simulations of the inspiratory phase and expiratory phase were performed with patient-specific boundary conditions for pre- and post-intervention cases to investigate the effect of stent placement on flow characteristic and possible improvements. FINDINGS: Results of post-stent condition show reduced pressure, velocity magnitude and wall shear stress during expiration. The variation in wall shear stress, velocity magnitude and pressure drop is negligible during inspiration. INTERPRETATION: Although Spirometry tests do not show significant improvements, computational fluid dynamics results show significant improvements in pre- and post-treatment results, suggesting improvement in breathing condition.

Cine MRI of Tracheal Dynamics in Healthy Volunteers and Patients With Tracheobronchomalacia.

OBJECTIVE: Bronchoscopy and MDCT are routinely used to assess tracheobronchomalacia (TBM). Recently, dynamic MRI (cine MRI) has been proposed as a radiation-free alternative to MDCT. In this study, we tested cine MRI assessment of airway dynamics during various breathing conditions and compared cine MRI and MDCT measurements in healthy volunteers and patients with suspected TBM. CONCLUSION: Cine MRI was found to be a technically feasible alternative to MDCT for assessing central airway dynamics.

Our Experience on Silicone Y-Stent for Severe COPD Complicated With Expiratory Central Airway Collapse.

BACKGROUND: Expiratory central airway collapse (ECAC) is abnormal central airway narrowing during expiration. ECAC involves 2 different pathophysiological entities as tracheobronchomalacia and excessive dynamic airway collapse (EDAC). Although the exact cause is unknown, chronic obstructive pulmonary disease (COPD) is frequently accompanied by ECAC. Although there are various publications on the relationship between COPD and ECAC, there are very few data for stent placement in patients with tracheobronchomalacia accompanied severe COPD. We share our results for stenting in ECAC among patients with severe COPD. METHODS: The data in this case series were collected retrospectively. The ECAC diagnosis was made during flexible bronchoscopy with severe COPD. Silicone Y-stents were placed via rigid bronchoscopy under general anesthesia. RESULTS: A total of 9 patients' (7 men) data were evaluated with an average age of 67±10.73 years. One patient experienced stent migration on the second day of stenting prompting stent removal. Another patient died 1 month after stenting. Consequently, we evaluated the follow-up data of remaining 7 patients. The changes in forced expiratory volume 1 was not significant for these 7 cases (P=0.51). The modified Medical Research Council (mMRC) score improvement was statistically significant (P=0.03). Functional status improvement was observed in 4 of 7 patients. Of the 7 patients, mean additional follow-up bronchoscopic interventions requirement was 2.2 times. CONCLUSIONS: Our study showed significant decrease in mMRC score with stenting for ECAC in severe COPD. For 2 patients, we experienced severe complications during short-term follow-up period after stenting. Additional follow-up bronchoscopic interventions were required.

[CPAP interest in help of mucus clearance in tracheo-bronchomalacia outpatients]. / Intérêt de la PPC en aide au désencombrement chez le patient présentant une trachéo-bronchomalacie.

The patients suffering of trachea bronchial dyskinesia in a context of trachea bronchomalacia present an important decrease of the expiratory flow during the Forced Expiration Techniques, and during the cough, returning difficult mucus clearance. The use of CPAP for these patients as a pneumatic prothesis can bring a considerable help during the chest physiotherapy techniques, avoiding the tracheobronchial collapse. The objectivation of the efficiency of this technique through a clinical case, its ease of application and control, allows us to propose the attempt of this additive help when the indication and the contraindications are clearly defined.

Advances in Surgical Treatment of Congenital Airway Disease.

Tracheobronchomalacia (TBM) is frequently present in infants and children with congenital heart disease (CHD). Infants with CHD and TBM appear to do worse than those without TBM. The principle of operative intervention for TBM is to improve function of the airway and clinical status. When indicated, conventional surgical options include tracheostomy, aortopexy, tracheoplasty, and anterior tracheal suspension. There is no consensus on the optimal treatment of severe tracheobonchomalacia, which can be associated with a mortality rate as high as 80%. Congenital tracheal stenosis is also frequently associated with CHD (vascular rings, atrioventricular canal defects, and septal defects) and may require concomitant repair. Repair of tracheal stenosis is often associated with distal TBM. This article addresses new techniques that can be performed in corrective surgery for both TBM and congenital tracheal stenosis.

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.

Flat trachea syndrome: a rare condition with symptoms similar to obstructive airway disease.

Flat trachea syndrome, commonly known as 'tracheobronchomalacia', is a central airway disease characterised by excessive expiratory collapse of the tracheobronchial posterior membrane due to weakness in the airway walls. Patients present with symptoms such as chronic cough, dyspnoea and recurrent respiratory tract infections, which are often attributed to more common conditions such as asthma and chronic obstructive pulmonary disease (COPD). The term 'Flat Trachea Syndrome' was first proposed by Niranjan and Marzouk in 2010 following a retrospective study of 28 patients with the condition who underwent surgery for it. The authors advocated the term due to the primary abnormality being collapse of the posterior membranous wall of the central airways as opposed to softening of the tracheal cartilage (tracheobronchomalacia), which they proposed is a misnomer. We present a rare case of a patient with flat trachea syndrome on a history of COPD who initially presented with recurrent respiratory tract infections.