The Mounier‐Kuhn syndrome (MKS), the first case of which was described in 1937 1, is a rare, most likely congenital syndrome, whose main characteristic is an enlarged trachea and main bronchi. This book explores the non-interventional aspects of interventional pulmonology, focusing on diseases of the central airways. 6. * Frequent bronchiectasis MKS disproportionately affects men and results in chronic respiratory tract infections. Mounier-Kuhn syndrome (MKS) is a rare disorder characterized by dilation of the trachea and first to fourth-order bronchi secondary to atrophy of the muscular and elastic tissue. 2). Thorax computed tomographic scan showing the marked dilatation of the trachea, right and left main (A) bronchus, and bilateral bronchiectasis (B). Two axial CT images of the thorax demonstrate marked dilatation of the trachea (T) and right (R) and left (L) main bronchi in this patient with Mounier-Kuhn syndrome. In type 3, diverticular and saccular structures extend to the distal bronchi. * Frequent pneumonia Found inside – Page 453salient clinical features 395 winging of scapulae 397f Marvan's syndrome 255 Massive (gross) ... lower 195 Motor neuropathy 229 Mounier-Kuhn syndrome 50 MS, ... In MKS, there is a clinical and prognostic polymorphism; symptoms vary from minor to major with severe infectious exacerbations and respiratory failure leading to premature death. Clin Radiol. The patient had no family history of chronic obstructive pulmonary diseases. The list of signs and symptoms mentioned in various sources for Mounier-Kuhn syndrome includes the 12 symptoms listed below: * Cough * Inability to expectorate * Congenital tracheal malformation * Repeated infections * Enlarged trachea * Enlarged bronchi * Frequent bronchitis * Frequent bronchiolitis * Frequent pneumonia * Frequent emphysema * Frequent … Pilavaki M, Anastasiadou K, Vlachojanni E, et al. In type 1, there is a slight symmetric dilation in the trachea and main bronchi. The hallmark of it is a dilatation of the main airways which frequently, but not always, causes marked, mainly respiratory, symptoms, and patients usually present with varying degrees of recurrent infections, breathlessness, haemoptysis, dyspnoea. 13. Other diagnostic criteria include a mainstem bronchial diameter of 20-24 mm (right) and 15-23 mm (left) (3). Dr. James M. 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The Mounier-Kuhn syndrome has a clinical presentation similar to that of chronic bronchitis and bronchiectasis. This text also addresses imaging and how it plays a pivotal role in the diagnosis and study of exacerbations.Written by today's top experts, Chronic Obstructive Pulmonary Disease Exacerbat Symptoma empowers users to uncover even ultra-rare diseases. This website uses cookies. Recurrent infections revealed MKS in 88% of cases. It is characterized by distinct dilation of the trachea and bronchi and by recurrent lower respiratory tract infections (LRTIs). This microorganism is one of the most commonly grown microorganisms in patients with the Mounier-Kuhn syndrome. Dyspnea, cough, difficulty in clearing secretions, recurrent bronchitis, pneumonia, and respiratory failure have been reported in patients with the Mounier-Kuhn syndrome.7 These are nonspecific symptoms and are similar to those in patients with chronic [journals.lww.com] 800-638-3030 (within USA), 301-223-2300 (international) The syndrome was first described by P. Mounier-Kuhn in 1932 (1). 1973;24:354â358. About 300 cases have been reported to date. 4). [radiopaedia.org], We also hypothesize that TBM and generalized elastolysis may have etiological commonalities, suggesting a need for further study. All registration fields are required. CheckOrphan offers users an interactive and dynamic platform for all these diseases. 1 Found inside – Page 96The Mounier-Kuhn syndrome is underreported because it may go undiagnosed—clinical signs and symptoms are similar to those of chronic bronchitis, COPD, ... The diagnosis is made through the synthesis of clinical and radiological data. [email protected]. 1995;49:556â558. Murgu SD, Henri GC. The clinical, radiologic, and endoscopic features of congenital tracheobronchomegaly were first described in 1932 by Mounier and Kuhn; the syndrome is characterized by expiratory central airway collapse and airflow limitation because of the excessive narrowing of the central airways during expiration.2 The etiology is uncertain. Received for publication July 13, 2010; accepted July 22, 2010. Indian J Radiol Imaging. Please try after some time. Mounier-Kuhn syndrome or congenital tracheobronchomegaly is characterised by gross dilation of the trachea and bronchi which is a rare pathology due to atrophy or absence of smooth muscle cells and elastin fibres of the tracheobronchial tree.1 It may be associated with other congenital connective tissue disorders such as Ehlers-Danlos syndrome, cutis laxa and Marfan syndrome. A standard form was used to record patient characteristics. [jacpjournal.org], He had a history of chronic bronchitis and recurrent respiratory infections. The Disease Database lists the following medical conditions that Mounier-Kuhn syndrome may cause: Pneumologie. In addition, a saw-tooth appearance was observed on the flow-volume loop (Fig. Dyspnea, cough, difficulty in clearing secretions, recurrent bronchitis, pneumonia, and respiratory failure have been reported in patients with the Mounier-Kuhn syndrome. Please try again soon. 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Mounier-Kuhn syndrome is a rare clinical and radiologic entity characterized by pathologic dilatation of the trachea and bronchi.1 It is a form of expiratory central airway collapse characterized by the softening of the airway wall cartilaginous structures and is usually associated with bronchiectasis and lower respiratory tract infection. He had been diagnosed with and treated for chronic obstructive pulmonary disease. Found insideThe book is intended for radiologists, however, it is also of interest to clinicians in oncology, cardiology, and pulmonology. This open access book focuses on diagnostic and interventional imaging of the chest, breast, heart, and vessels. [backclinicinc.com]. Tracheobronchomegaly (Mounier-Kuhn syndrome): CT diagnosis. Patients of MKS can have varied presentation. 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