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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 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.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.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.

No abstract available. Article truncated after 150 words. A 52-year-old non-smoking, Caucasian male patient with a past medical history of reported chronic obstructive pulmonary disease (COPD), recurrent lower respiratory tract infections, prior history of pneumothorax, and dysphagia presented with fevers and shortness of breathing associated with a productive cough for one week. Clinically, he was mildly tachypneic and chest auscultation revealed crackles bilaterally - more prominent at the left base. A chest radiograph (Figure 1) showed bilateral lower lobe pulmonary opacities (left more than right). Computed tomography (CT) of the chest demonstrated airspace disease in the lower lobes in addition to significant tracheobronchomegaly along with paraseptal emphysema and central bronchiectatic changes (Figures 2 and 3). Upper posterior tracheal wall diverticulae were also noted(Figure 3). Serum α1-antitrypsin level and serum immunoglobulins, including IgE levels, were normal. Our patient declined performing diagnostic bronchoscopy. He had a pulmonary function test performed few months prior to his hospital admission which showed combined …

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.

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.

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.

Giant cell arteritis (GCA) is a large-vessel vasculitis with rarely described respiratory initial manifestations. We report such a case presenting with hoarseness, stridor, cough and dyspnea, in which a tracheomegaly with tracheomalacia was found. No signs of relapsing polychondritis were present. The respiratory symptoms rapidly improved after glucocorticoids and Azathioprine. Tracheomegaly or Mounier-Kuhn syndrome is characterized by marked dilatation of trachea and central bronchi. The differential diagnosis and the possible relationship between tracheomegaly and GCA involving metalloproteinase-induced elastolysis are discussed. This is the first case, to our knowledge, of Mounier-Kuhn syndrome in vasculitis. The association of tracheomegaly with GCA may be underestimated, as the diagnosis is not always obvious on conventional radiographs. A tracheal enlargement finding in GCA requires monitoring to ensure early detection and prevention of spontaneous tracheal rupture. Adding a metalloproteinase inhibitor like Doxycycline to GCA therapy would be rational for the prophylaxis of complications.

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.