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Mounier Kuhn Syndrome or tracheobronchomegaly is a rare disease, characterized by dilatation of the trachea and the main bronchi. Our study concerns a case of 67-years old male patient, smoker, with a chronic cough. Chest scan was requested; it shows an enlargement of the tracheal clarity and the two main bronchi. Meanwhile, the Functional respiratory exploration was normal. The treatment includes mucolytics and pulmonary physiotherapy. Mounier-Kuhn syndrome is rare and the clinical signs are not specific, the positive diagnosis is purely radiological. The treatment is about to free the airways to prevent infection.

Non-neoplastic lesions of central airways are uncommon entities with different etiologies, with either focal or diffuse involvement of the tracheobronchial tree. Clinical symptoms of non-neoplastic tracheobronchial diseases are non-specific, and diagnosis is difficult, especially in the early stages. Three-dimensional computed tomography (3D-CT) is an evaluable tool as it allows to assess and characterize tracheobronchial wall lesions and meanwhile it enables the evaluation of airways surrounding structures. Multiplanar reconstructions (MPR), minimum intensity projections (MinIP), and 3D Volume Rendering (VR) (in particular, virtual bronchoscopy) also provide information on the site and of the length of airway alterations. This review will be discussed about (1) primary airway disorders, such as relapsing polychondritis, tracheobronchophathia osteochondroplastica, and tracheobronchomegaly, (2) airway diseases, related to granulomatosis with polyangiitis, Chron's disease, Behcet's disease, sarcoidosis, amyloidosis, infections, intubation and transplantation, (3) tracheobronchial malacia, and (4) acute tracheobronchial injury. 3D-CT findings, especially with MPR and 3D VR reconstructions, allows us to evaluate tracheobronchial disease morphologically in detail.

Tracheobronchomegaly is a rare disease with congenital abnormal change in respiratory tract; its image features are also very special. In this case, we described a 57-year-old male with cough, expectoration, chest pain, and dyspnea. In our institution, the result of chest CT scan is highly extraordinary, which showed obvious dilation of the trachea and main bronchi, emphysema, and a number of pulmonary bullae, and there was a big bulla with air-fluid level on the lower lobe of the right lung. Fortunately, after wedge resection for the big bulla on the lower lobe of right lung under video-assisted thoracoscopic surgery, this patient’s symptoms were significantly relieved. The clinical manifestations of tracheobronchomegaly lack specificity; this disease has freakish image features. At present, there are no effective treatments for tracheobronchomegaly, which just was an accidental discovery in this patient; we just mainly take surgical measures to treat the big bulla for relieving symptoms.

Tracheobronchomegaly is a rare developmental defect in children, which is characterized by the expansion of the trachea and large bronchi, the diagnosis and treatment of which are made more often in adult practice, when the clinical diagnostic picture acquires the obvious features of Mounier-Kuhn syndrome. In this connection, the need arises for the early detection of this pathology in children and its differentiation from other diseases and malformations of the tracheobronchial tree, which mask a recurrent inflammatory process in the lung and upper respiratory tract, and primarily with those, such as cystic fibrosis and primary ciliary dyskinesia.The paper describes a case of secondary tracheobronchomegaly in a child with obvious clinical manifestations of Mounier-Kuhn syndrome in the presence of bronchiectasis, pulmonary fibrosis, and signs of systemic connective tissue disease. It discusses the possibilities of chest computed tomography in the diagnosis of this defect in children, as well as its causes. The sizes of the trachea and main bronchi in this patient are compared with those in the groups of children of the same age and sex who have cystic fibrosis or diffuse pulmonary fibrosis to identify a more significant role of hereditary connective tissue diseases in the development of secondary tracheobronchomegaly. The authors propose to separate the concepts of tracheobronchomegaly and Mounier-Kuhn syndrome in children and to recognize the threshold for tracheal expansion in these patients having +3SD or more.

Tracheal disorders can create clinical and imaging challenges. They are often misdiagnosed as other pulmonary disorders. Chest radiography has low sensitivity and specificity for proper identification of tracheal disorders. Multidetector-row CT (MDCT) has the inherent advantage of being able to provide high-quality multiplanar reformations, 3D volume-rendered images, virtual bronchoscopy, and minimum intensity projections. These can serve as a roadmap for bronchoscopy or surgical planning. The most important imaging sign of tracheal disease is thickening of the tracheal wall, and the most relevant sign to be evaluated for differential diagnosis is the distribution and appearance of wall thickening. Tracheal abnormalities may be classified based on the tracheal dimensions into increased diameter or decreased diameter to reduce the differential diagnosis. Increased diameter tracheal diseases may be subclassified into diffuse or focal diseases based on the area of involvement, thereby providing a more specific diagnosis.

Mounier-Kuhn syndrome is a rare clinical and radiologic condition. It is characterized by tracheal and bronchial dilation. Diagnosis is made by computed tomography and bronchoscopy. An 81-year-old man presenting with an acute chest pain was referred to the pulmonology department. His chest computed tomographic scan showed a tracheobronchomegaly with an increase in the diameter of both the trachea and right and left main bronchi, associated with pneumomediastinum and fibrosis. Fiberoptic bronchoscopy revealed enlarged trachea and both main bronchi. These findings are consistent with a diagnosis of Mounier-Kuhn syndrome. Besides considering this long-neglected “orphan disease” when diagnosing spontaneous pneumomediastinum, clinicians should also be aware of an underlying Mounier-Kuhn syndrome in patients with recurrent respiratory infections, in order to avoid complications associated with the disease.

Mounier-Kuhn syndrome (MKS) is a rare disorder characterized by recurrent lower respiratory tract infections and bronchiectasis due to dilation of the trachea and bronchi. Diagnosis is made based on clinical suspicion along with radiographic evidence of tracheobronchomegaly. Mucolytic agents and chest physiotherapy have been shown to offer symptomatic improvement, and definitive surgical treatment is reserved for those with persistent symptoms. Herein, we report a case of MKS in a 72-year-old woman with bronchiectasis and recurrent multidrug-resistant lower respiratory tract infections.

Mounier-Kuhn syndrome or tracheobronchomegaly is a rare disease characterized by marked dilation of the trachea and proximal bronchi with recurrent lower tract respiratory infections. Computed tomography and bronchoscopy are the key tools to accomplish the diagnosis. This is a condition with a clinical polymorphism, symptoms vary from minor with preserved respiratory function, to very severe with life threatening exacerbations leading to respiratory failure and premature death. The treatment is mainly symptomatic, stenting or surgery are reserved to extreme cases.Herein, we report two cases of the same condition with different clinical signs and diverse outcome.

Mounier-Kuhn syndrome (MKS) is a rare congenital disease with an autosomal recessive inheritance pattern, characterized by an enlargement of the trachea and bronchi. MKS is secondary to a thinning of the muscular mucosa and atrophy of the longitudinal muscle and elastic fibers of the tracheobronchial tree. As a consequence, tracheal diverticulosis and dilatations in the posterior membranous wall appear, along with bronchiectasis that tend to be cystic in appearance. Overall, there is an impairment of mucocilliary clearance, with an ineffective cough, which predisposes the patient to recurrent lower respiratory tract infections. Clinical manifestations vary from asymptomatic to respiratory failure and death, most patients being diagnosed between the third and fourth decades of life. It is an often undiagnosed disease, with a diagnostic algorithm that includes the use of radiological techniques, alone or in combination with bronchoscopy. Specific diagnostic criteria have been developed, based on patients’ tracheal and main bronchi diameter on chest X-ray and thoracic computed tomography scan. We present the case of a 45-year-old African American man who presented with a history of multiples episodes of pneumonia that required management in the intensive care unit, on whom MKS was diagnosed.

A 51-year-old man known to be affected by Mounier-Kuhn syndrome (MKS). presented to Accident & Emergecy (A&E) with fever, dyspnea and deterioration of his chronic coughs. Increased diameters of his trachea (39 mm), right (30 mm) and left (26 mm) main bronchi were revealed by chest computerized tomography (CT) scan. CT scan showed also ground-glass opacities (GGO) and bronchiectasis in the mid and lower zones of both lungs. COIVD-19 infection was eventually confirmed by RT-PCR. A severe form of COVID-19 could occur even in the early stages of the disease in presence of underlying co-morbidities including MKS, which increases the susceptibility to more recurrent and severe respiratory infections.

Tracheobronchomegaly or Mounier-Kuhn syndrome is a rare disorder characterized by the widening of the trachea and the main bronchi. It is a form of tracheomalacia called ‘cartilaginous malacic’ and is characterized by deformation of the tracheal cartilages and intrusion of the redundant membranous wall into the lumen of the airway. We present a patient with Mounier-Kuhn syndrome managed like patients with tracheomalacia of other aetiologies—a tracheobronchoplasty with a reconstructed D-shaped trachea and stabilization of the posterior membranous wall by attaching a polypropylene mesh to the posterior membranous wall of the trachea and the main bronchi after a trial period with a tracheobronchial Y-shaped silicone stent.

A 49-year-old male ex-smoker was referred for recurrent chest infections requiring one course of antibiotics every winter, occurring over the last 20 years. Each episode is characterised by a productive cough with purulent sputum along with difficulty breathing, chest tightness and fatigue, but without haemoptysis. On some occasions, these symptoms were preceded by fever and rhinorrhoea. Each episode lasted ∼10 days and responded well to antibiotics. He had never been hospitalised for these infections, nor received a chest radiograph or sputum microbiology. He had been told that he developed episodes of pneumonia in his first year of life. Apart from these yearly infections, the patient was asymptomatic during the rest of the year., Mounier-Kuhn Syndrome (MKS) is a rare disease characterised by recurrent chest infections, and dilation of the trachea and main bronchi, most likely to due to atrophy of elastic fibres https://bit.ly/3azhDjr

Patient: Male, 45-year-old Final Diagnosis: Mounier-Kuhn syndrome Symptoms: Heart failure • pneumonia Medication:— Clinical Procedure: — Specialty: Pulmonology Objective: Rare disease Background: Mounier-Kuhn syndrome is a rare clinical condition characterized by marked tracheal and bronchial dilatation. It is commonly associated with multiple respiratory infections due to the inability to mobilize secretions, chronic cough, and decline in lung capacity. Although it is a rare disease, the number of cases reported in the literature has increased, and physicians should be aware of its existence to be able to recognize and diagnose it. Suspicion should arise regarding young adults who develop recurrent airway infections when immunosuppression has been ruled out. Case Report: We present the case of a young adult, previously healthy and immunocompetent, who was diagnosed with Mounier-Kuhn syndrome after 5 years of multiple respiratory infections and multiple respiratory failures, who received intensive care management with multiple complications and difficulties in achieving adequate ventilation. Due to the risk of respiratory failure and major complications, its management was challenging. We were able to treat this patient and prevent complications with a multidisciplinary approach with a strong emphasis on constant and intense pulmonary therapy. Conclusions: Mounier-Kuhn syndrome is rare but does occur, and physicians need to have a high index of suspicion to diagnose it. Its management must be done by a multidisciplinary team, and respiratory therapy should be constantly available at bedside.

This chapter describes such abnormalities of the trachea and bronchi as tracheobronchomegaly, relapsing polychondritis, tracheomalacia, saber-tooth trachea, post-intubation stenosis, bronchiectasis (cylindrical, varicose, cystic), mucous impaction, broncholithiasis, bronchopleural fistula, foreign bodies, and trauma.

Chronic obstructive pulmonary disease (COPD) patients that have evidence of bronchiectasis demonstrate greater symptom severity, more frequent bronchial infections and exacerbations, lower lung function and increased mortality.1 While the association has been recognized, it is not clear if there is any causal relationship between having COPD and developing bronchiectasis. Certainly, the fact that COPD is associated with a high risk for lower respiratory infection2 and/or dysbiosis3 may be part of the explanation but not all patients with COPD develop bronchiectasis.Bronchiectasis has been defined as a dilation of the airways that persists beyond the acute responses to various insults such as infections, aspiration and toxic inhalations. The most accepted criteria as defined by Naidich include direct signs of a bronchial-arterial ratio >1, lack of bronchial tapering, visualization of peripheral bronchi within 1 cm of the costal pleura or in contact with the mediastinal pleura and indirect signs such as peribronchial thickening, mucus plugging, mosaic pattern, central lobular nodules, tree in bud nodules, focal areas of air trapping and atelectasis/consolidation).4 Bronchiectasis is known to develop and progressively worsen over time secondary to a number of conditions including cystic fibrosis, alpha-1 antitrypsin deficiency, primary ciliary dyskinesia, allergic bronchopulmonary mycosis, immunoglobulin deficiencies and post obstructive pneumonia secondary to tumors, foreign bodies and lymphadenopathy. Other less common causes include rheumatoid arthritis and Sjogren's disease, connective tissue disorders such as tracheobronchomegaly (Mounier Kuhn), Marfan's disease, cartilage deficiency (Williams Campbell syndrome), inflammatory bowel disease, HIV, Job's syndrome, and malignancies such as, chronic lymphocytic lymphoma and graft-versus-host disease, as well as yellow nail syndrome and Young's syndrome.5 It has been proposed that bronchiectasis develops as a result of these various disorders leading to structural and functional changes in the airways that increase susceptibility to chronic bronchial infections secondary to potentially pathogenic microorganisms. The persistent presence of these organisms causes chronic inflammation, airway remodeling and further damage to local defense mechanisms further enabling these potentially pathogenic microorganisms to persist in the airways despite the repeated administration of antibiotics thus creating a vicious cycle as proposed by Cole et al.6 With this backdrop it is not surprising that there has been recognition of a high prevalence of bronchiectasis in the COPD population with rates ranging from 4% to 72%.7 The broad prevalence range reported is likely a result of several factors including differences in patient characteristics, evaluations during times of exacerbation versus clinical stability, and whether the prevalence of bronchiectasis was part of a primary or secondary analysis of data. Suffice it to say that the true prevalence of bronchiectasis/COPD overlap remains unknown. Regardless, similar associations have also been noted in asthma8,9 and it has also been recognized that up to 75% of bronchiectasis patients demonstrate signs of upper airway disease and report severe daily symptoms of nasal congestion, facial pain and/or loss of smell.10 Considering the various combinations and permutations of these overlaps, several COPD phenotypes could be generated but it is debatable as to whether defining such overlap syndromes will lead to particularly unique treatment paradigms. Rather, it may be best to simply look at the various components as treatable traits and optimize treatment for each component as outlined by Polverino and colleagues in an excellent review recently published.1 Polverino and colleagues also point to many questions that need to be addressed regarding the COPD/bronchiectasis overlap. We need a set of updated consensus criteria for defining radiological and clinical bronchiectasis in patients with COPD to better establish prevalence and the potential prognostic value of identifying bronchiectasis in COPD patients. New methods to diagnose bronchiectasis independent of vessel diameter are needed, as the classic definition, airway diameter/blood vessel diameter ratio greater than 1, can be misleading as cardiovascular comorbidities can cause increases in blood vessel diameter. There are also no specific biomarkers linking COPD to bronchiectasis such as possible genetic and epigenetic phenomena that may influence susceptibility to infections and response to treatment that renders only a subgroup of COPD patients to develop bronchiectasis. Other questions remain with regard to alterations of the microbiome, so called dysbiosis,3 that may be related to microaspiration11 and/or the potential impacts of inhaled corticosteroid use and frequent antibiotic use.12-15 Inhaled corticosteroids are often recommended for COPD patients with frequent exacerbations whereas recent guidelines in the Cochrane review have concluded that there is insufficient evidence to recommend the use of inhaled steroids in adults with stable bronchiectasis except in specific conditions where the possible benefits in exacerbation reduction outweigh the risks.16 Other areas for study include interventions that may help prevent progression of disease and exacerbations, including the role of other anti-inflammatory molecules such as phosphodiesterase 4 (PDE4) inhibitors or prophylactic antibiotic treatment either systemically or by inhalation.1 These are important questions to address in the overlap population because treatment recommendations are often conflicting in guideline recommendations for COPD patients versus pure bronchiectasis patients. Conversely, macrolide antibiotics have appeared to be effective in patients with bronchiectasis and frequent exacerbations however there continue to be some concerns about the possible use of macrolides and their ability to induce significant cardiovascular adverse effects,17 particularly in COPD patients who are already at risk of higher cardiovascular comorbidity. Furthermore, there is the concern about the development of multi-antibiotic resistance particularly given the reported prevalence of nontuberculous mycobacterial (NTM) infections in COPD patients.18 In this Journal club we review some recent papers that have addressed some of these issues. Suffice it to say that additional prospective trial research is much required. Note: Abstracts are presented in their original, published format and have not been edited to match JCOPDF style.

Introduction The segmentation and skeletonisation of images via computed tomography (CT) airway lumen volumetry provide a new perspective regarding the incorporation of this technique in medical practice. Our aim was to quantify morphological changes in the large airways of patients with acromegaly through CT and, secondarily, to correlate these findings with hormone levels and pulmonary function testing (PFT) parameters. Methods This was a cross-sectional study in which 28 non-smoker patients with acromegaly and 15 control subjects underwent CT analysis of airway lumen volumetry with subsequent image segmentation and skeletonisation. Moreover, all participants were subjected to PFT. Results Compared with the controls, patients with acromegaly presented higher diameters in the trachea, right main bronchus and left main bronchus. The patients with acromegaly also showed a higher tracheal sinuosity index (the deviation of a line from the shortest path, calculated by dividing total length by shortest possible path) than the controls [1.06 (1.02–1.09) vs. 1.03 (1.02–1.04), P = 0.04], and tracheal stenosis was observed in 25% of these individuals. The tracheal area was correlated with the levels of growth hormone (rs = 0.45, P = 0.02) and insulin-like growth factor type I (rs = 0.38, P = 0.04). The ratio between the forced expiratory flow and forced inspiratory flow at 50% of the forced vital capacity was correlated with the tracheal area (rs = 0.36, P = 0.02) and Δ tracheal diameters (rs = 0.58, P

Objectives To describe the imaging features of the central airway pathology, correlating the findings with those in pathology and virtual endoscopy. To propose a schematic and practical approach to reach diagnoses, placing strong emphasis on multidetector computed tomography (MDCT) findings. Methods We reviewed our thoracic pathology database and the central airway pathology-related literature. Best cases were selected to illustrate the main features of each disease. MDCT was performed in all cases. Multiplanar and volume-rendering reconstructions were obtained when necessary. Virtual endoscopy was obtained from the CT with dedicated software. Results Pathological conditions affecting the central airways are a heterogeneous group of diseases. Focal alterations include benign neoplasms, malignant neoplasms, and non-neoplastic conditions. Diffuse abnormalities are divided into those that produce dilation and those that produce stenosis and tracheobronchomalacia. Direct bronchoscopy (DB) visualises the mucosal layer and is an important diagnostic and therapeutic weapon. However, assessing the deep layers or the adjacent tissue is not possible. MDCT and post-processing techniques such as virtual bronchoscopy (VB) provide an excellent evaluation of the airway wall. Conclusion This review presents the complete spectrum of the central airway pathology with its clinical, pathological and radiological features. Teaching points • Dividing diseases into diffuse and focal lesions helps narrow the differential diagnosis. • Focal lesions with nodularity are more likely to correspond to tumours. • Focal lesions with stenosis are more likely to correspond to inflammatory disease. • Posterior wall involvement is the main feature in diffuse lesions with stenosis.

Background and Aim: Mounier-Kuhn syndrome (MKS) is a congenital disorder characterized by tracheobronchomegaly resulting from the absence of elastic fibers in the trachea and main bronchi or atrophy and thinning of the smooth muscle layer. In this syndrome, dead space associated with tracheobronchomegaly increases and discharge of secretions decreases because of ineffective coughing. The most common complications are recurrent lower respiratory tract infections and bronchiectasis. We examined the clinical characteristics, radiological features, and related complications of patients with MKS. Methods: The cases were obtained between September 2007 and November 2015. Computed tomography scans of the chest were used to diagnosis tracheobronchomegaly. Results: All cases (a total of 11) were males with a mean age of 63 ± 13 (range, 38–80) years. The mean diameter of the trachea was 31.53 ± 2.99 mm; the mean transverse diameter was 31.69 ± 3.10 mm and the mean sagittal diameter was 31.36 ± 3.01 mm. Complaints at the time of presentation included chronic cough, purulent sputum, dyspnea, and hemoptysis. There were recurrent pulmonary infections in seven cases, bronchiectasis in six, and tracheal diverticulum in four at the time of diagnosis. Conclusions: In this paper, 11 cases with various rarely seen complications are presented and evaluated in the light of current literature. We recommend that if chronic cough, recurrent pulmonary infections and bronchiectasis seen in a patient, MKS should be kept in mind. This article is protected by copyright. All rights reserved.

Tracheobronchomegaly (TBM) is a rare disease of unknown etiology, characterized by dilatation of the trachea and main bronchi. Despite its rarity, TBM has been reported to accompany several conditions, particularly Ehlers-Danlos syndrome. Herein, we report on a 15-year-old patient who was diagnosed with TBM on the basis of radiologic and endoscopic findings and with Ehlers-Danlos syndrome type IVB presenting with marfanoid features, severe kyphoscoliosis, increased skin elasticity, and ongoing hypotonia since the neonatal period.

A 41-year-old previously healthy man presented to otolaryngology outpatient clinic with recurrent episodes of throat discomfort for last 6 months. There was no associated history of voice change, dysphagia, shortness of breath or cough. He denied any history of chronic productive cough, fever

During bronchoscopy, there are normal longitudinal and lateral movements of the bronchi, which are barely perceptible, but the bronchi are more visible once these movements have ceased. Bronchial examination during acute asthma is almost impossible, but the discovery of a very smooth-flowing mucous, rich in eosinophils, suggests a pulmonary condition compatible with asthma. Dynamic collapse and tracheobronchomegaly have been so far of little interest for physiologists, but they are clearly a part of severe respiratory insufficiency.

Background: Mounier Kuhn Syndrome (MKS) is a rare congenital anomaly characterized by abnormal dilatation of the trachea and main bronchi. The aim of this study is to discuss tracheal volume measurement in MKS, and the pathologies accompanying MKS, especially pulmonary artery enlargement. Materials and Methods: 38 patients, 18 of whom were diagnosed with MKS and 20 as control group, were included in the study. Trachea volume and pulmonary artery diameter were measured through thorax-computed tomography (CT) images of the patients. Accompanying pathologies were recorded. Results: In the measurements done through the CT scans, the trachea volume was found to be 25.45 cm3 in the control group and 44.17 cm3 in the patient group. The most frequent accompanying pathologies were tracheal diverticulum, bronchiectasis and pulmonary artery enlargement. Conclusion: In patients with MKS, there is a significant difference in volume calculation as in trachea diameter. Though bronchiectasis and tracheal diverticulum are known as pathologies most frequently accompanying MKS, to the knowledge of the researchers, pulmonary artery enlargement due to the increase in pulmonary truncus diameter was first emphasized in this article.

A 21- year-old Indian man presented with a history of shortness of breath and cough with expectoration for 2 years. The expectoration was copious in amount and was associated with occasional streaky haemoptysis. For 2 years, he had recurrent wheezing with good symptomatic improvement with bronchodilators. Spirometry performed 2 years previously demonstrated moderate obstructive ventilatory defect without bronchodilator reversibility. He had received multiple courses of oral steroids in the past year for treatment of exacerbations. He required hospitalisation thrice in the past year for infective exacerbations, requiring broad-spectrum antibiotics. His perinatal history was unremarkable and he had a history of pneumonia at 3 months of age. There was no history of recurrent infections in early childhood. The presenting episode commenced with a 2 weeks history of right-side chest pain, fever and increased shortness …

We describe a 75 year old gentleman, admitted to intensive care unit, who develops a significant endotracheal cuff leak. After ruling out cuff puncture we discover the patient has tracheobronchomegaly on a CT thorax. The leak is highly positional but was managed successfully with careful ETT manoeuvring. We discuss the causes of ETT cuff leak and then explain the underlying mechanisms of tracheobronchomegaly.

Amyloid is a heterogeneous family of extracellular proteinaceous deposits characterized by apple‐green birefringence on polarized light microscopy. There are rare case reports of these extracellular deposits accumulating in the upper and central airways. Progressive infiltration may impair glottic and airway function with some cases requiring intervention to improve flow. Bronchoscopy and lung function testing provide dynamic information to monitor for disease progression; however, the recent development of 320 multislice computed tomography (320 CT) enables dynamic, four‐dimensional (4‐D) evaluation of laryngeal and tracheal structure and function and presents as a noninvasive, low‐radiation dose surveillance tool. We reviewed a 43‐year‐old man with primary amyloidosis of the larynx and central airways who presented with an 18‐year history of progressive dysphonia without breathlessness and preserved lung function. 4‐D CT demonstrated marked thickening of supraglottic folds and trachea with marked tracheal dilatation. Despite gross structural abnormalities, dynamic function assessed throughout inspiration and expiration was normal, demonstrating neither rigidity nor dynamic collapse. This combination of structural and functional assessment of the proximal airway by 4‐D CT is a novel application to surveillance for laryngeal and tracheal amyloid.

OUNIER-KUHN SYNDROME (MKS), or tracheobronchomegaly, is a rare disorder of unknown etiology that is characterized by marked dilation of the trachea and major bronchi, due to severe atrophy and thinning, as well as absence of elastic fibers and smooth muscular fibers of the large airways. 1 This syndrome commonly presents in men in the third or fourth decade of life. It is marked by weakness of the membranous and cartilaginous parts of the trachea and main bronchi leading to an insufficient cough, mucous retention, and tracheobronchial wall collapse during respiration. Symptoms can range from well-preserved lung function to refractory respiratory failure. There are fewer than 100 case reports in the literature with only 2 reports of successful lung transplantation for patients with this syndrome. 1–3 Herein, the authors present a case of a patient diagnosed with MKS presenting for bilateral lung transplantation and describe the one-lung ventilation (OLV) strategies that were attempted and utilized.

Resume Introduction le syndrome de Mounier-Kuhn ou tracheobronchomegalie congenitale est une pathologie peu frequente, dont la prise en charge therapeutique est complexe. Nous rapportons ici le cas d’un patient traite par endoscopie interventionnelle. Observation Il s’agit d’un homme de 74 ans dont le diagnostic de tracheobronchomegalie a ete porte en 2003 dans un contexte de degradation de l’etat respiratoire et la survenue de syncopes posturales. Il a tout d’abord beneficie d’une prothese tracheobronchique en Y, en silicone, prolongee par des protheses metalliques au niveau tracheal et bronchique. Le montage est reste stable jusqu’en 2011. Il apparait alors des episodes d’asphyxie liee a la luxation posterieure de la prothese tracheobronchique, par rupture des mailles de la prothese metallique tracheale. Une nouvelle prothese tracheobronchique en Y est alors mise en place, faite « sur mesure », a partir d’une modelisation 3D des voies aeriennes, avec evolution clinique et fonctionnelle tout a fait satisfaisante. Discussion Ce cas clinique est un exemple de prise en charge d’un syndrome de Mounier-Kuhn, par bronchoscopie interventionnelle, avec un recul de 10 ans, et avec pour originalite l’adaptation du materiel prothetique, de maniere individualisee a l’anatomie des voies aeriennes du patient.

Tracheobronchomegaly (also called Mounier–Kuhn syndrome) is a rare disease characterized by flaccid and markedly dilated trachea and main bronchi on inspiration with narrowing or collapse on expiration or cough. It is associated with recurrent lower respiratory tract infection. A 75-year-old man with unexpected giant tracheomegaly had a significant peritubal air leak which impeded an operation. Lumbar epidural anesthesia was performed for a subsequent operation without any sequela. Careful evaluation with chest radiography is basic to exclude a large airway. Chest computed tomography and fiber-optic bronchoscopy provided the diagnosis of a large airway. If a large airway is suspected, these examinations help to evaluate and manage the airway.

A variety of pathologic processes can involve the central airways. Abnormalities may either diffusely or focally involve the tracheal or mainstem bronchial walls. Diseases that diffusely involve the tracheal wall can be subclassified as sparing the membranous trachea or circumferentially involving the tracheal wall. Focal diseases of the trachea and mainstem bronchi include benign and malignant causes. Additionally, congenital and acquired morphologic abnormalities of the trachea will be reviewed.

We present the case of a man with Mounier-Kuhn syndrome (MKS), or tracheobronchomegaly, who was referred to the National Institutes of Health Clinical Research Center because of a potential diagnosis of lymphangioleiomyomatosis (LAM), a rare condition in men. The patient was evaluated using ongoing protocols and provided written informed consent. The case demonstrates the presence of chronic inflammation surrounding the dilated airways and histologic changes of the lung parenchyma with emphysematouslike disruption in areas adjacent to the dilated airways. This finding suggests that damage to the lung parenchyma is an ongoing phenomenon in MKS. Moreover, our analysis of CT images indicates similar abnormalities in areas remote from the dilated airways. Finally, because of increased anatomic dead space, calculation of lung diffusion capacity by the single-breath method yielded abnormally low values that required making a correction for the large anatomic dead space, which can be measured by the single-breath nitrogen washout test.

CONTEXT: Mounier-Kuhn syndrome is a rare congenital condition with distinct dilatation and diverticulation of the tracheal wall. The symptoms may vary and the treatment usually consists of support. CASE REPORT: The patient was a 60-year-old male with recurrent hospital admission. He was admitted in this case due to dyspnea, cough and sputum production. An arterial blood sample revealed decompensated respiratory acidosis with moderate hypoxemia. A chest computed tomography (CT) scan showed dilatation of the trachea and bronchi, tracheal diverticula and bronchiectasis. Flexible bronchoscopy was performed, which revealed enlarged airways with expiratory collapse. Furthermore, orifices of tracheal diverticulosis were also detected. Non-invasive positive pressure ventilation (NPPV) was added, along with long-term oxygen therapy. At control visits, the patient’s clinical and laboratory findings were found to have improved. CONCLUSION: Flexible bronchoscopy can be advocated for establishing the diagnosis and non-invasive mechanical ventilation can be used with a high success rate, for clinical wellbeing in Mounier-Kuhn syndrome. RESUMO CONTEXTO: A síndrome de Mounier-Kuhn é uma condição congênita rara com dilatação e diverticulação distintas da parede traqueal. Os sintomas podem ser variáveis e o tratamento geralmente é de suporte. RELATO DE CASO: Paciente do sexo masculino, de 60 anos, com internação hospitalar recorrente, foi internado neste caso devido a dispneia, tosse e produção de expectoração. A amostra de sangue arterial revelou acidose respiratória descompensada, com hipoxemia moderada. A tomografia computadorizada de tórax mostrou dilatação da traqueia e brônquios, divertículos traqueais e bronquiectasias. Realizou-se broncoscopia flexível, que revelou aumento das vias aéreas com colapso expiratório. Além disso, também foram detectados orifícios de diverticulose traqueal. Foi adicionada ventilação com pressão positiva não invasiva (NPPV) juntamente com a oxigenoterapia a longo prazo. Foram verificadas melhoras dos resultados clínicos e laboratoriais do doente nas visitas de controle. CONCLUSÃO: A broncoscopia flexível pode ser defendida para estabelecer o diagnóstico, e a ventilação mecânica não invasiva pode ser utilizada com alta taxa de sucesso, para bem-estar clínico, na síndrome de Mounier-Kuhn.

Mounier-Kuhn Syndrome (MKS) is a rare disorder derived from the muscular and elastic tissue defects of the trachea and the main bronchial walls, characterized by tracheobronchomegaly. Patients may present with complaints of cough, phlegm, dyspnoea and haemoptysis. Haemoptysis may be minor and mixed with phlegm or it may be massive. Establishment of airway patency is a priority in the management of massive haemoptysis. Cold saline solution, diluted adrenaline or tranexamic acid may be administered via the endobronchial route to stop haemorrhage while establishing the airway patency. Ankaferd Blood Stopper (ABS) has a haemostatic property and can be locally administered to the airway. In this report, we aim to highlight the effects of ABS administered via an endobronchial route for emergency palliation of a patient with MKS presenting with massive haemoptysis.

To illustrate and describe computed tomographic large airway pathology and correlate with fiberoptic bronchoscopy findings. Commonly encountered diseases of the large airway include tracheobronchial amyloidosis, tracheobronchopathia osteochondroplastica, tracheobronchomegaly, laryngotracheobronchial papillomatosis, relapsing polychondritis, Wegener granulomatosis, sarcoidosis, and tracheal stenosis. Computed tomography manifestations and bronchoscopic findings of these selected large airway diseases are discussed.

Background and Aims The Mounier–Kuhn syndrome (MKS) is a rare disease characterized by a pathological dilation of the trachea and the bronchial system. The etiology of the disorder remains elusive, but genetic alterations and degradation of elastic fibers are thought to be involved in the pathogenesis. No causative treatment is available although transplantation is an option for end-stage disease. Here, we describe a patient suffering from MKS who received a double lung transplant at our department. Methods Since a familial clustering of MKS is discussed in the literature, we performed a chromosomal analysis and an array-comparative genomic hybridization (CGH) to search for genetic abnormalities. At the time of transplantation, we collected samples from the bronchi and performed hematoxylin and eosin (HE), Elastic von-Gieson (EVG) and immunohistochemical stains of the explanted MKS bronchus, a control bronchus and of the inflammatory infiltrates. Specimens of main bronchi from the donor lung harvested for transplant served as control. Bronchial smears were taken from both main bronchi of the recipient for microbiological cultures. Results No genetic alterations could be found in chromosomal analysis and in array-CGH. Histological analysis revealed a strong reduction of elastic fibers in the submucosal connective tissue and a diffuse inflammatory infiltrate, mainly comprised of CD4+ cells. In addition, immunohistochemistry showed increased matrix metalloproteinases (MMPs) protein expression of MMP-1, 2, 3 and 9. Conclusions Based on our findings, we hypothesize that MKS is a chronic inflammatory disease characterized by an MMP-mediated degradation of submucosal elastic fibers.

Summary We report bronchoscopic changes observed in children with recurrent lower airways infections (RLAI) and findings in control children undergoing bronchoscopy for causes other than RLAI. Patients and methods Retrospective case-control cohorts study. The clinical records of children who had fiberoptic bronchoscopy (FB) for a history of RLAI without any known underlying disorder between 2007 and 2013 and of control children who required FB for other causes were reviewed. Clinical features, bronchospic findings and bronchoalveolar lavage (BAL) results were assessed. Results Cases were 62 (32 female) children aged 5 years (1–12) and controls 29 children aged 4.5 years (0.5–14). Airway malacia was observed in 32 (52%) vs 4 (13%) ( p = 0.001), profuse respiratory secretions in 34(55%) vs 6 (20%) ( p = 0.007). Endobronchial obstruction: 4 (6.4%) and tracheobronchomegaly were observed only in cases. In cases with profuse respiratory secretions there was a higher prevalence of airways malacia: 64.7% vs 35.7% ( p = 0.04) and of positive BAL cultures: 45.5% vs 13.3% ( p = 0.04). Isolated organisms in cases were non-typable Haemophilus influenzae and Streptococcus pneumoniae most frequently. Pneumocystiis jirovecii , Staphylococcus aureus , and Streptococcus mitis were isolated in controls. Conclusions Half of the children with RLAI had tracheo and/or bronchomalacia, their frequency being in keeping with previous reports and far higher than that observed in controls. It was associated with profuse respiratory secretions and with a higher frequency of positive BAL cultures mostly for non typable H. influenzae and S. pneumoniae which were not isolated in controls.

Mounier-Kuhn syndrome (MKS) or tracheobronchomegaly includes clinical and radiographic findings of tracheobronchial dilatation and recurrent respiratory infections. MKS is a very rare pathology, especially in the paediatric age group which makes it a diagnostic challenge. A 4-year-old girl suffered from dyspnea, recurrent respiratory infections and joint pain. Chest radiography detected peribronchial reinforcement and CT-scan revealed extended tracheal dilatation and bronchiectasis. In addition to MKS our patient was diagnosed with juvenile idiopathic arthritis (JIA) and scleroderma. MKS can be caused by congenital disorder or acquired aetiology. Several connective tissue diseases have been associated with MKS but no cases of JIA or scleroderma are described previously. Our case illustrates that patients who suffer from recurrent respiratory infections with unsatisfactory evolution and unspecific chest X-ray alteration, MKS always has to be considered in the differential diagnosis particularly in patients who suffer from connective tissue diseases.