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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.

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.

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.

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.

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.

Tracheobronchomegaly (Mounier-Kuhn syndrome) is a rare condition characterized by an abnormally enlarged trachea and main bronchi. Herein, we present a case of 79-year-old male with idiopathic pulmonary fibrosis and acute hypoxemic respiratory failure due to multilobar pneumonia. Computed tomography of the chest demonstrated a markedly dilated trachea, with the transverse diameter of 31mm and the sagittal diameter of 30mm. The clinical manifestations as well as its diagnosis, classification, and treatment are discussed.

Summary Mounier-Kuhn syndrome (MKS) or tracheobronchomegaly, and tracheopathia osteoplastica (TO) are rare diseases. MKS is a clinical entity characterized by abnormal dilatation of the trachea and main bronchi. TO is a clinical and pathologic condition characterized by multiple submucosal cartilaginous and osseous nodules lining the upper respiratory tract. Some people with MKS manifest recurrent pneumonia or chronic cough with sputum production, hemoptysis or dyspnea. Occasionally, it is found in asymptomatic individuals. CT scan imaging is the key tool for the diagnosis when showing a dilatation of the trachea and main bronchi. TO is usually benign and asymptomatic, frequently diagnosed incidentally during intubation. Flexible fiberoptic bronchoscopy and laryngoscopy are the key diagnostic tools which typically demonstrate irregular spicules of submucosal bone and cartilage projecting into the tracheobronchial lumen and causing various degrees of airway obstruction. When this aspect is found bronchial biopsy is not mandatory. We report the case of a 26-year-old man with features of both MKS and TO who presented a previous history of productive cough, recurrent pneumonia and dyspnea; admitted for pulmonary infection resulting in a respiratory insufficiency. MKS was diagnosed by CT scan and TO by fiberoptic bronchoscopy. To our knowledge, this is the first time these pathologies are reported in the same patient, and our patient is the second one having MKS who presented with respiratory insufficiency.

Resumo: A síndroma de Mounier-Kuhn é uma doença rara, de etiologia desconhecida e que se presume subdiagnosticada. Cursa frequentemente com infecções respiratórias de repetição e bronquiectasias. A clínica inespecífica, semelhante a outras patologias supurativas brônquicas crónicas, dificulta o diagnóstico, muitas vezes efectuado vários anos após o início dos sintomas. Os autores apresentam uma revisão da literatura a propósito de dois casos clínicos que ilustram a heterogeneidade e as dificuldades diagnósticas desta entidade nosológica. Um dos doentes encontrava-se em idade pediátrica na altura do diagnóstico e outro na quarta década de vida. Abstract: The incidence of the syndrome may be greater than is suspected. Patients present with recurrent respiratory infections and bronchiectasis. The symptoms are non-specific and indistinguishable from those of other chronic respiratory diseases. The diagnosis is often made several years after the first clinical complaints. The authors perform a brief review of the literature and report two cases of Mounier--Kuhn syndrome. One of the patients was diagnosed in childhood and the other in the fourth decade of life. Palavras-chave: Síndroma de Mounier-Kuhn, traqueobroncomegalia, infecções respiratórias de repetição, Key-words: Mounier-Kuhn syndrome, tracheobronchomegaly, recurrent respiratory infections

Summary Tracheobronchomegaly (TBM) (Mounier-Kuhn syndrome) is dilatation of the trachea and major bronchi because of atrophy or absence of elastic fibers and smooth muscle cells. We present a case of TBM with normal pulmonary function test (PFT). The patient was a 37-year-old man with increasing productive cough and without fever, wheezes, chest pain, weight loss or any respiratory disease. Chest helical computed tomography (CT) scan showed tracheomegaly with transversal diameters of the trachea of 44 mm. CT scan showed collapse of the trachea. Few large diverticular out-pouching and openings in the trachea was seen in bronchoscopy. PFT results were normal. PFT in large airway disorders may be normal while abnormalities may indicate underlying small airway disorder. An underlying small airway disorders is responsible for abnormal reports in PFT of these patients. We may need to re-evaluate the role of PFT within follow-up of patients with large airway disorder.

HISTORY OF PRESENT ILLNESS A 49-year-old nonsmoking male initially presented to our institution 3 years ago with a history of steroid-dependent asthma since age 14. Before this evaluation he had been on glucocorticoid (GC) doses as high as 120 mg prednisone daily and had rarely been weaned completely from oral GCs. Chronic symptoms included dyspnea on exertion, episodic wheeze that symptomatically responded to bronchodilator, and cough that was productive of clear sputum. There was no history of chest pain or hemoptysis. Viral upper respiratory tract infections frequently led to exacerbations requiring a transient increase in his oral GC dose. He had noted that the duration of these increases was becoming longer over time. Repeated chest imaging in the past did not show evidence of infiltrates or other significant abnormalities. Confounding factors related to his asthma included allergic rhinitis, gastroesophageal reflux disease, and aspirin intolerance. His aspirin allergy resulted in flares of his asthma and rhinitis symptoms, but he had been successful in avoiding all overthe-counter preparations containing aspirin before and during our evaluation. His medication regimen for his asthma included oral GC, a high-dose inhaled steroid, a long-acting -agonist, and a rescue inhaler. Skin testing demonstrated sensitivity to trees, grasses, weeds, and cats. He was treated aggressively for his rhinitis complaints with nasal steroids as well as new generation antihistamines. His reflux disease was also treated with twice-daily proton pump inhibitor. With this therapy his rhinitis as well as reflux symptoms improved, but he did not experience relief with regard to his wheeze and dyspnea on exertion. Given his aspirin allergy it was decided that he should undergo aspirin desensitization. We felt that his asthma symptoms may improve if his upper respiratory symptoms responded favorably to this procedure. He completed the desensitization procedure without difficulty and was maintained on 650 mg twice a day. His medication regimen was also augmented at that time to include a leukotriene receptor antagonist as well as a 5-lipoxygenase inhibitor. Six months after this, he again reported that his lower respiratory symptoms were progressive. His dose of oral prednisone at that point was 20 mg daily. His past medical history was remarkable for eczema that resolved as a child and osteopenia associated with chronic oral steroid use. There was no history of trauma to the chest. He was a lifelong nonsmoker and had no occupational exposures or occupation-related triggers for his symptoms. He has been employed as a software engineer for the past 25 years. Hobbies were confined to watching movies and walking. Family history was positive for atopy and asthma. The patient lived in a new home, which was carpeted. The house had forced air heating and a humidifier. He also had 3 dogs as pets, but they remained outdoors. Review of systems was remarkable for weight gain associated with his steroid use and profound snoring according to his wife.

Chronic obstructive pulmonary disease (COPD) is present in 10.1% of the population and is the fourth leading cause of death in the United States while the prevalence of asthma in adults is 6.7%. 1 Patients presenting to primary care offices with lower respiratory tract symptoms like dyspnea and wheezing are often diagnosed with COPD or asthma and started on treatment. There are uncommon conditions like Mounier-Kuhn syndrome (MKS) that can present with similar symptoms and it is important for the primary care physician to keep them in mind when evaluating patients. It is important to identify MKS, as early recognition can lead to better management and prevention of complications. We describe a case of an older gentleman with recurrent symptoms of shortness of breath and intermittent spells of productive sputum who was misdiagnosed with COPD for years. Our diagnosis was supported by a clinical history and confirmed by radiographic evidence.

Mounier-Kuhn syndrome is a rare condition characterized by marked dilation of the trachea and main bronchi resulting in bronchiectasis and emphysema. We report a case in which a patient underwent successful double lung transplantation for COPD that was found on pathologic examination of the explanted lungs to be Mounier Kuhn syndrome. To our knowledge this is the first case reporting lung transplantation in this syndrome.

This study set out to determine the frequency of tracheomegaly in brochiectasis as judged by high resolution computed tomography (HRCT) while determining whether the published normal tracheal dimensions derived from radiographic data can be used for CT diagnosis. Seventy-five consecutive adults referred for CT assessment of possible bronchiectasis were studied and compared with a control group of 75 adults being staged for lymphoma. The internal tracheal diameters at aortic arch level of the control group correspond with published radiographic data and using these measurements, 7/42 (17%) patients with bronchiectasis were found to have tracheomegaly, while two of the 33 'symptomatic' patients (i.e. those patients not found to have bronchiectasis) had tracheomegaly. Further analysis confirmed that the bronchiectatic group's tracheal dimensions were significantly different from those of the control group while the 'symptomatic' group are an overlap population. We conclude that tracheomegaly is a frequent finding in bronchiectasis.

sidual capacity, with a mild reduction in the FEV1. Diffusing capacity of the lung for carbon monoxide was mildly reduced as well. This was interpreted as a restrictive ventilatory defect, although he carried a clinical diagnosis of COPD. Twenty years prior to presentation, he had pulmonary tuberculosis. The patient describes frequent bouts of severe dyspnea with a productive cough. Two years prior, a chest radiograph was performed that revealed pneumonia in the right lower lobe and dilation of the trachea. CT was ordered and showed marked dilatation of the trachea and the central bronchi, one of which contained an air-fluid level. The patient was treated with antibiotics, improved, and was discharged. The patient now returns to the emergency department with similar symptoms. A chest radiograph was performed (Fig 1, 2), which showed dilation of the central airways but no focus of pneumonia. CT performed a few days later again showed marked dilatation of the trachea and central airways (Fig 3, 4). The patient was treated with antibiotics and improved.

Tracheobronchomegaly is a rare-probably congenital-disorder. Its clinical appearance is non-specific. The diagnosis is made by radiological methods. This paper describes the typical features in early and advanced stages and outlines the diagnostic approach.

Tracheobronchomegaly, also known as Mounier-Kuhn's Syndrome, is characterized by market dilation of the trachea and main bronchi and has been reported in association with several conditions, particularly connective tissue disease. The pathogenesis and clinical signs of light chain deposition disease are similar to those of light chain amyloidosis, in which these chains are deposited as amorphous material lacking the tinctorial features of the amyloid. We present a case involving both entities, an association that has not been previously reported, and we review the main characteristics of both diseases.

We present a case of tracheobronchomegaly seen in association with ankylosing spondylitis. To the authors' knowledge this combination has not been previously described. A review of the chest radiographs of 30 other patients with ankylosing spondylitis revealed no evidence of tracheal dilatation.

A 36-year-old woman with features of both the Mounier-Kuhn syndrome and the Kenny-Caffey syndrome is described. To our knowledge, this is the first reported case of these syndromes occurring together. Three-dimensional computed tomographic reconstruction of the upper airway revealed marked dilatation of the trachea and main-stem bronchi and several large diverticulae of the trachea.

Three children with tracheomegaly were studied by CT scan at The Children's Hospital of Philadelphia. Normal age-related tracheal dimensions were obtained from a study by Griscom (1). Tracheomegaly was defined as tracheal dimensions greater than 2 SD above normal. In two of our patients, tracheomegaly was associated with intubation and ventilatory support, and in the third, it was associated with recurrent pneumonia and chronic bronchitis. In this article, we discuss the etiologies of tracheomegaly in our patients and review the literature on the subject.