Subglottic stenosis and endobronchial disease in granulomatosis with polyangiitis.

OBJECTIVES: To describe tracheobronchial disease in patients with granulomatosis with polyangiitis (GPA) and evaluate the utility of dynamic expiratory CT to detect large-airway disease. METHODS: Demographic and clinical features associated with the presence of subglottic stenosis (SGS) or endobronchial involvement were assessed in a multicentre, observational cohort of patients with GPA. A subset of patients with GPA from a single-centre cohort underwent dynamic chest CT to evaluate the airways. RESULTS: Among 962 patients with GPA, SGS and endobronchial disease were identified in 95 (10%) and 59 (6%) patients, respectively. Patients with SGS were more likely to be female (72% vs 53%, P < 0.01), younger at time of diagnosis (36 vs 49 years, P < 0.01), and have saddle-nose deformities (28% vs 10%, P < 0.01), but were less likely to have renal involvement (39% vs 62%, P < 0.01). Patients with endobronchial disease were more likely to be PR3-ANCA positive (85% vs 66%, P < 0.01), with more ENT involvement (97% vs 77%, P < 0.01) and less renal involvement (42% vs 62%, P < 0.01). Disease activity in patients with large-airway disease was commonly isolated to the subglottis/upper airway (57%) or bronchi (32%). Seven of 23 patients screened by dynamic chest CT had large-airway pathology, including four patients with chronic, unexplained cough, discovered to have tracheobronchomalacia. CONCLUSION: SGS and endobronchial disease occur in 10% and 6% of patients with GPA, respectively, and may occur without disease activity in other organs. Dynamic expiratory chest CT is a potential non-invasive screening test for large-airway involvement in GPA.

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.

Acquired tracheobronchomalacia developed following voice prosthesis implantation.

Acquired tracheobronchomalacia (ATBM) is a condition in which the tracheobronchial wall and cartilage progressively lose their rigidity, resulting in dynamic collapse during exhalation. In this report, we present a case of ATBM that developed following voice prosthesis implantation. To the best of our knowledge, this is the first documented case of such a condition in the medical English literature based on a PubMed search. A 63-year-old man was referred to National Kyushu Cancer Center in Japan with complaints of pharyngeal pain and a laryngeal tumor. The tumor was diagnosed as laryngeal cancer, and the patient underwent laryngectomy. Three months after the surgery, we implanted a voice prosthesis through a tracheoesophageal puncture. Two months after implantation, the patient experienced dyspnea. This condition was subsequently diagnosed as ATBM through computed tomography and bronchofiberscope examinations. After the removal of the voice prosthesis, there has been no progression of ATBM for over five years. While ATBM may not be a common occurrence in the practice of head and neck surgeons, it should be considered as a potential complication when patients report dyspnea following voice prosthesis implantation.

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.

[Relapsing polychondritis]. / La polychondrite chronique atrophiante.

Relapsing polychondritis is a rare systemic disease. Clinical manifestations are variable and the condition is frequently associated with other diseases. Chondritis typically involves ears, nose, costal cartilages and upper airways. Other classical features include scleritis and episcleritis, mucocutaneous lesions, arthralgia and constitutional symptoms. Repeated inflammation of cartilaginous structures may lead to deformities of the ears, the nose and the airways. Tracheobronchomalacia and aortitis of the ascending portion resulting in aortic regurgitation or aneurysm are feared complications. Treatment is mainly based on systemic corticosteroids alone or in association with immunosuppressants. Several biological immunosuppressive agents are reported effective in refractory disease.

Dynamic Tracheobronchial Compression in Steep Trendelenburg Position With Pneumoperitoneum for Robotic-Assisted Cystectomy: A Case Report.

Tracheobronchomalacia is a weakness of the trachea and bronchi due to abnormal cartilage and muscular support leading to airway obstruction. We report a case of an adult former smoker without pulmonary symptoms who underwent robotic-assisted laparoscopic cystectomy in the steep Trendelenburg position. After repeated episodes of hypoxemia, bronchoscopic examination revealed collapse of the distal trachea and bronchi, supporting a diagnosis of tracheobronchomalacia. Tracheomalacia is an underdiagnosed condition in patients with a smoking history and may mimic other obstructive diseases. The anesthesiologist should remain vigilant to the possibility of airway collapse in former smokers, specifically in cases of increased intrathoracic pressure.

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.

Tracheobronchomalacia.

The term tracheobronchomalacia refers to excessively compliant and collapsible central airways leading to symptoms. Although seen as a coexisting condition with various other pulmonary condition, it may cause symptoms by itself. The condition is often misdiagnosed as asthma, bronchitis or just chronic cough due to a lack of specific pathognomonic history and clinical findings. The investigation revolves around different modes of imaging, lung function testing and usually confirmed by flexible bronchoscopy. The treatment widely varies based on the cause, with most cases treated conservatively with non-invasive ventilation. Some may require surgery or stent placement. In this article, we aim to discuss the pathophysiology behind this condition and recognize the common symptoms and causes of tracheobronchomalacia. The article will highlight the diagnostic steps as well as therapeutic interventions based on the specific cause.

Silent tracheobronchial chondritis in a patient with a delayed diagnosis of relapsing polychondritis.

Relapsing polychondritis is a very rare autoimmune disease characterised by a relapsing inflammation of hyaline, elastic and fibrous cartilaginous tissues. The incidence is estimated to be between 3.5 and 4.5 per million people per year. Clinical signs and symptoms can be very subtle, and if left undiagnosed for a prolonged period, airway involvement can cause fibrosis of the tracheobronchial wall, leading to a fixed tracheobronchial stenosis. Eventually, this can progress to life-threatening tracheobronchomalacia due to irreversible damage and loss of tissue integrity. We report an elderly man who presented with recurrent bilateral ear inflammation and intermittent polyarthritis who was diagnosed with relapsing polychondritis with asymptomatic involvement of his large airways.

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.

Critical airway involvement in relapsing polychondritis.

Relapsing polychondritis (RP) is a rare multisystem disease characterised by progressive inflammation and destruction of cartilaginous structures. Airway involvement in RP occurs in approximately 50% of cases. We present a 70-year-old woman with a diagnosis of early adult onset asthma. She required multiple hospital admissions for recurrent episodes of acute severe dyspnoea for 1 year. These were treated as asthma. Months later she developed saddle nose deformity and hoarseness of voice. CT revealed tracheal/bronchial wall thickening and luminal narrowing. Based on these findings, RP was diagnosed. Insertion of a tracheobronchial stent was necessary due to severe tracheobronchomalacia. This intervention improved the patient's dyspnoea immediately. This case is reported to raise awareness of airway involvement in RP and discuss its current management. Early diagnosis of RP is essential to allow prompt treatment and to decrease the risk of life-threatening airway collapse.

3-D printouts of the tracheobronchial tree generated from CT images as an aid to management in a case of tracheobronchial chondromalacia caused by relapsing polychondritis.

This report concerns a 67 year old male patient with known advanced relapsing polychondritis complicated by tracheobronchial chondromalacia who is increasingly symptomatic and therapeutic options such as tracheostomy and stenting procedures are being considered. The DICOM files from the patient's dynamic chest CT in its inspiratory and expiratory phases were used to generate stereolithography (STL) files and hence print out 3-D models of the patient's trachea and central airways. The 4 full-sized models allowed better understanding of the extent and location of any stenosis or malacic change and should aid any planned future stenting procedures. The future possibility of using the models as scaffolding to generate a new cartilaginous upper airway using regenerative medical techniques is also discussed.

Tracheomalacia/Tracheobronchomalacia and hyperdynamic airway collapse.

Tracheobronchomalacia (TBM) and hyperdynamic airway collapse (HDAC) can be debilitating diseases associated with decreased functional capacity and poor quality of life, although there is no standard definition of this complex condition, and there are numerous terms used to describe it. The diverse etiology associated with TBM and HDAC can obscure and delay an accurate diagnosis for years. A thorough medical history is important in understanding possible causes and in guiding diagnostic testing. Medical history may also suggest what treatments may be most beneficial.

[Tracheobronchomalacia in adults: breakthroughs and controversies]. / Trachéobronchomalacie de l'adulte: avancées et controverses.

Tracheobronchomalacia (TBM) in adults is a disease defined by a reduction of more than 50% of the airway lumen during expiration. It encompasses many etiologies that differ in their morphologic aspects, pathophysiological mechanisms and histopathologies. TBM is encountered with increasing frequency, as it is more easily diagnosed with new imaging techniques and diagnostic bronchoscopy, as well as because of its frequent association with Chronic Obstructive Pulmonary Disease (COPD), which represents the most frequent etiology for acquired TBM in adults. A distinction between TBM in association with failure of the cartilaginous part of the airways and TBM affecting only the posterior membranous part is emerging since their physiopathology and treatment differ. The therapeutic management of TBM should be as conservative as possible. Priority should be given to identification and treatment of associated respiratory diseases, such as asthma or COPD. Surgery addressing extrinsic compression (thyroid goiter or tumor, for example) may be necessary. Noninvasive ventilation can be considered in patients with increasing symptoms. Endoscopic options, such as the placement of stents, should only be used as palliative or temporary solutions, because of the high complication rates. Symptomatic improvement after stenting might be helpful in selecting patients in whom a surgical management with tracheobronchoplasty can be useful.

Tracheobronchomalacia in adults.

Severe, diffuse tracheobronchomalacia (TBM) is an underrecognized cause of dyspnea, recurrent respiratory infections, cough, secretion retention, and even respiratory insufficiency. Patients often have comorbidities, such as asthma or chronic obstructive pulmonary disease, and inappropriate treatment for these conditions may precede eventual recognition of TBM by months or years. Most of these patients have an acquired form of TBM in which the etiology in unknown. Diagnosis of TBM is made by airway computed tomography scan and flexible bronchoscopy with forced expiration. The prevailing definition of TBM as a 50% reduction in cross-sectional area is nonspecific, with a high proportion of healthy volunteers meeting this threshold. The clinically significant threshold is complete or near-complete collapse of the airway. Airway stenting may treat TBM, although complications resulting from indwelling prostheses often limit the durability of stents. Surgical stabilization of the airway by posterior splinting (tracheobronchoplasty) effectively and permanently corrects malacic airways. Proper surgical selection is facilitated by a short-term stent trial.

Tracheobronchomalacia treated by inserting a long T-tube into the left main bronchus.

An 88-year-old woman with advanced Parkinson's disease (stage V on the Yahr scale) had difficulty in expectoration and underwent tracheostomy in 1999. In July 2004, granulation tissue was formed in the tracheal lumen at the tip of the tracheostomy tube, and a standard type silicone T-tube was inserted. Thereafter, she was cared for at home, where she was doing well until early March 2009, when a decrease in SaO(2) and difficulty in sputum aspiration were noted. Bronchoscopy showed crescent type tracheobronchomalacia involving the trachea down to the orifice of the left main bronchus. Considering its localization, an intact right main bronchus, a history of tracheostomy tube placement resulting in granulation tissue formation in the lower trachea, and the future need for frequent sputum aspiration, we inserted a long T-tube into the left main bronchus, which is easily replaceable and facilitates sputum aspiration. Right-lung ventilation was maintained through a side aperture made in the long T-tube. After its insertion, her respiratory status stabilized, secretion drainage improved, and she was discharged for treatment at home. Herein, we describe a tracheobronchomalacia patient in whom airway patency was achieved by inserting a long T-tube with a side aperture into the left main bronchus.

[Etiology and airway management in children with tracheobronchomalacia].

OBJECTIVE: To investigate etiology and airway management in children with tracheobronchomalacia. METHOD: Bronchoscopic examinations were performed in 671 children. The cases with tracheomalacia and bronchomalacia were analyzed in etiopathogenesis and summarized their therapy simultaneously. RESULT: Bronchoscopic examination indicated tracheomalacia and bronchomalacia in 148 cases, tracheomalacia in 77 cases and bronchomalacia in 71 cases. Among the cases with tracheomalacia, compression by vascular rings was found in 46 cases, incorporated congenital esophageal atresia with tracheoesophageal fistula was found in 5 cases, tracheomalacia was associated with tracheostoma and mechanical ventilation in 6 cases, with congenital airway malformation in 11 cases and isolated tracheomalacia was found in 4 cases. Among the cases with bronchomalacia, incorporated congenital cardiovascular malformation was found in 64 cases, congenital airway malformation in 6 cases and isolated bronchomalacia in 1 case. Ten children with anomalous innominate artery underwent aortopexy, twelve children with dextro-aorta arch with concomitant aberrant left subclavian artery and double aorta underwent arches vascular ring lysis, six children with pulmonary sling underwent plasty. Severe malacia segments were resected directly in four children during operation. Mechanical ventilation was performed in 38 children. Tracheostoma was performed in 4 children to treat tracheomalacia and bronchomalacia, it could relieve symptom to a certain extent. In 2 children metal stents were inserted into the bronchus for the treatment of bronchomalacia, one was successful and the other needed re-insertion of stent again, these two patients underwent balloon-dilatation in distal part of stent afterwards. CONCLUSION: The congenital cardiovascular malformation was the main reason to develop tracheobronchomalacia in children. The symptom of majority of the cases with cardiovascular malformation would be improved within 6 months after surgical intervention. In severe cases, treatments included mechanical ventilation and tracheostoma. Stenting could be applied in refractory cases, but it had certain limitations.