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      Recurrent Lower Respiratory Tract Infections Due to Mounier-Kuhn Syndrome

      case-report

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          Abstract

          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.

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          Most cited references 11

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          Mounier-Kuhn syndrome or congenital tracheobronchomegaly: a literature review.

          Mounier-Kuhn syndrome or congenital tracheobronchomegaly is a chronic airway condition which for currently unknown reasons mostly affects males. It is commonly overlooked on conventional chest X-rays, and is considered to be rare, but the prevalence might be higher as commonly assumed. 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. Although at least 200 case reports have been published, there have been only a few attempts to review them, and none in the last 20 years. Due to the lack of clinical trials and wide variability of case-report format, a systematic review was deemed not feasible, therefore PubMed and Medline databases were searched using terms "Mounier-Kuhn syndrome", "tracheobronchomegaly", "tracheomegaly", and "bronchomegaly", without any time restrictions, to summarize currently known facts about the syndrome. To the authors' best knowledge, the result is currently the most comprehensive review of previously published literature about the congenital tracheobronchomegaly, and summarizes what's known about symptoms, prevalence, disease associations, and treatment options for this syndrome.
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            Airway stenting and tracheobronchoplasty improve respiratory symptoms in Mounier-Kuhn syndrome.

            Mounier-Kuhn syndrome (MKS) is a condition characterized by tracheobronchomegaly resulting from the loss or atrophy of musculoelastic fibers within the airway wall. Concomitant tracheobronchomalacia is seen in most patients with MKS, often leading to significant respiratory compromise due to bronchiectasis, increased dead space, and impaired secretion clearance.
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              Tracheobronchoplasty for severe tracheobronchomalacia: a prospective outcome analysis.

              Central airway stabilization with silicone stents can improve respiratory symptoms in patients with severe symptomatic tracheobronchomalacia (TBM) but is associated with a relatively high rate of complications. Surgery with posterior tracheobronchial splinting using a polypropylene mesh has also been used for this condition but to date has not been evaluated prospectively and objectively for patient outcomes. To evaluate the effect of surgical tracheobronchoplasty on symptoms, functional status, quality of life, lung function, and exercise capacity in patients with severe and symptomatic TBM. A prospective observational study in which baseline measurements were compared to those obtained 3 months after surgical tracheobronchoplasty. Of 104 referred patients to our complex airway center for severe TBM, 77 had baseline measurements. Of this group, 57 patients had severe malacia and underwent stent placement for central airway stabilization. Of those, 37 patients reported improvement in respiratory symptoms and 35 were considered for surgical tracheobronchoplasty. Two patients were excluded from surgery for medical reasons. Median age was 61 years (range, 39 to 83 years), 20 patients were men, 11 patients (31%) had COPD, 9 patients (26%) had asthma, and 4 patients (11%) had Mounier-Kuhn syndrome. Thirty-three patients (94%) presented with severe dyspnea, 26 patients (74%) with uncontrollable cough, and 18 patients (51%) reported recurrent pulmonary infections. Two patients (3%) presented with respiratory failure requiring mechanical ventilation. After surgery, quality of life scores improved in 25 of 31 patients (p < 0.0001), dyspnea scores improved in 19 of 26 patients (p = 0.007), functional status scores improved in 20 of 31 patients (p = 0.003), and mean exercise capacity improved in 10 patients (p = 0.012). In experienced hands, surgical central airway stabilization with posterior tracheobronchial splinting using a polypropylene mesh improves respiratory symptoms, health-related quality of life, and functional status in highly selected patients with severe symptomatic TBM.
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                Author and article information

                Journal
                Cureus
                Cureus
                2168-8184
                Cureus
                Cureus (Palo Alto (CA) )
                2168-8184
                4 June 2021
                June 2021
                : 13
                : 6
                Affiliations
                [1 ] Internal Medicine-Pulmonology, University of Illinois College of Medicine Peoria, Peoria, USA
                [2 ] Pulmonary and Critical Care Medicine, University of Illinois College of Medicine Peoria, Peoria, USA
                [3 ] Internal Medicine-Pediatrics, University of Illinois College of Medicine Peoria, Peoria, USA
                Author notes
                Collin J. O'Bryan cobrya3@ 123456uic.edu
                Article
                10.7759/cureus.15437
                8253498
                34249578
                34320c66-9c52-49f3-abfb-7c18f10ec45d
                Copyright © 2021, O'Bryan et al.

                This is an open access article distributed under the terms of the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original author and source are credited.

                Categories
                Radiology
                Infectious Disease
                Pulmonology

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