DOCSLIB.ORG

Mounier‐Kuhn Syndrome: a Systematic Analysis of 128 Cases

Total Page：16

File Type：pdf, Size：1020Kb

Download full-text PDF Read full-text
Mounier‐Kuhn Syndrome: a Systematic Analysis of 128 Cases The Clinical Respiratory Journal REVIEWREVIEW ARTICLE Mounier-Kuhn syndrome: a systematic analysis of 128 cases published within last 25 years Eduards Krustins1,2 1 Department of Internal Medicine, Pauls Stradins Clinical University Hospital, Riga, Latvia 2 Department of Internal Medicine, Riga Stradins University, Riga, Latvia Abstract Key words Background and Aims: Mounier-Kuhn syndrome is a rare disease with abnormal bronchiectasis – chronic respiratory infections enlargement of major airways, but epidemiological studies are lacking, and cur- – congenital airway disease – COPD – cough – dyspnea rently the most available data about it come from case reports, making it difficult to collate changes in a particular patient to those in previously published cases. Correspondence The aim of this work is to systematically review cases published in the last 25 Eduards Krustins, MD, Department of Internal years and to use descriptive statistics to summarize the patient demographic and Medicine, Pauls Stradins Clinical University clinical information therein in order to acquire details about patient clinical Hospital, 13 Pilsonu Street, LV1002 Riga, characteristics. Latvia. Methods: Cases published in world literature between 1987 and 2013 were sought Tel: 00 371 292 431 48 Fax: +371 67614168 and reviewed according to PRISMA guidelines. Cases were included only if email: [email protected] patient’s age was at least 18 years, and a computed tomography scan with tracheal measurements was available. Received: 28 January 2014 Results: An 8:1 male predominance was found in 89 identified reports (128 cases). Revision requested: 03 July 2014 Mean age was 53.9 years,and average tracheal diameter was 36.1 mm.No correlation Accepted: 22 July 2014 between increasing age and increasing tracheal diameter was found. Bronchiectasis, tracheal diverticulosis and tracheobronchial dyskinesia were common (49.2%, DOI:10.1111/crj.12192 33.6% and 28.9%, respectively). Cough, dyspnea and recurrent respiratory infec- Authorship and contributorship tions (71.1%, 51.6% and 50.8%, respectively) were the most common complaints. E. Krustins is the sole contributor to this Conclusions: The data confirm that syndrome mostly manifests with nonspecific manuscript. respiratory symptoms and is significantly more common in males. Importantly, there was no connection between age and airway diameter, a fact that could mean Ethics that the enlargement does not progress over time, and its severity depends on some Dr. Krustins as the sole author vouches for other yet undetermined factors. scientific integrity of the study and explicitly confirms that the manuscript meets the highest ethical standards for authorship. Please cite this paper as: Krustins E. Mounier-Kuhn Mounier-Kuhn syndrome: a systematic systematic analysis analysis of 128 128 cases cases published published within within last 25 last years. 25Clin years. RespirClin J 2014; Respir ••: ••–••.J 2016; DOI:10.1111/ 10: 3–10. Conflict of interest DOI:10.1111/crj.12192.crj.12192. The author has stated explicitly that there are no conflicts of interest in connection with this article. Abbreviations: COPD chronic obstructive pulmonary disease CT computed tomography MKS Mounier-Kuhn syndrome Introduction visualizations of MKS in computed tomography (CT) were described (2, 3), and although numerous case The Mounier-Kuhn syndrome (MKS), the first case of reports are published every year, there have been very which was described in 1937 (1), is a rare, most likely few attempts to summarize them (4–6). congenital syndrome, whose main characteristic is an To the best of the author’s knowledge, there is no enlarged trachea and main bronchi. In 1988, the first review available that has systematically tried to identify The Clinical Respiratory Journal (2016)(2014) • ISSN 1752-6981 31 V©C 2014 2014 John John Wiley Wiley & & Sons Sons Ltd Ltd Congenital tracheobronchomegaly – statistical review Krustins 129 publicaons idenfied in PubMed 39 publicaons idenfied in other sources (Google Scholar, journal database webpages, reference mining) 168 publicaons screened for eligibility 16 arcles excluded as not containing any case descripons 152 publicaons with 247 cases screened for eligibility (see Table S1) 63 further arcles excluded (no CT scan, paent less than 18 years old, no airway measurements given, arcles that could not be translated, full text not available) 89 publicaons with 128 cases included in this review Figure 1. PRISM flowchart of case selection. the published cases and that would have performed a tory medicine (as listed by online SCIMAGO journal statistical analysis of them. Although individual case rank (8): American Journal of Respiratory and reports and previously published reviews do provide Critical Care Medicine, European Respiratory Journal, information about disease characteristics, symptoms Chest, Thorax and Proceedings of the American and already implemented treatment strategies, they do Thoracic Society) were reviewed for meeting and con- not provide the overview of an issue when it is needed. ference supplement not published elsewhere. Refer- As of MKS it is currently unclear when the dilation of ences of acquired articles were screened for additional the airways start and if (or how) it progresses over publications. time. Patients usually present themselves with a The full texts of the articles identified by the search marked dilation, but the frequently asked question were retrieved and reviewed for conformity with the ‘whether the dilation will increase further?’ currently following inclusion criteria: patient was older than 18 cannot be answered. years; tracheo- or bronchomegaly was confirmed with Therefore, the aim of this review is to clarify the a CT scan according to the criteria published by clinical characteristics of the MKS demographics and Breatnach et al. (9) [the criteria originally were devel- symptoms by systematically searching and reviewing oped for use with conventional chest X-ray, and later cases published within the last 25 years, according to validated for CT by Roditi and Weir (10)]; and at least the PRISMA guidelines for systematic reviews (7). one abnormal tracheal or bronchial measurement clearly was given in the publication. Materials and methods Conference abstracts were included only if no sub- sequent publication of the particular case could be PubMed, ScienceDirect and GoogleScholar data- found and if the case met all inclusion criteria men- bases were searched for publications of MKS case tioned above. For the flowchart of case selection, see reports by using terms ‘Mounier-Kuhn syndrome’, Fig. 1. Information about the demographics of the ‘tracheomegaly’, ‘bronchomegaly’ and ‘tracheobron- patient, smoking history (pack years were calculated if chomegaly’. Time was restricted to cases published data were available), airway size, patient’s complaints between January 1987 and December 2012. Articles in and lung ventilation function was searched for in the Japanese, Chinese and Korean were excluded as it was case reports that were included. After the data collec- not possible to translate them. Additionally, the web tion was completed, in a small random sample of 13 pages of the five most influential journals of respira- cases (10%), the gathered information was compared 24 The Clinical Respiratory Journal (2014)(2016) • ISSN 1752-6981 ©VC 2014 John Wiley & Sons Ltd Krustins Congenital tracheobronchomegaly – statistical review Figure 2. Age and gender frequencies for patients diagnosed with Mounier- Kuhn syndrome (MKS). once again with the case reports to check for accuracy (with reasons for exclusion, where applicable) may be – no errors were detected. The final data table was found online at the publisher’s website. analysed using SPSS 20.0 (IBM Corporation, Armonk, There were 114 (89,1%) male and 14 female NY, USA) computer program. Acquired data were (10,9%) patients (a ratio of 8.1:1). The distribution of checked for normality and depending on the results age (Shapiro–Wilk test, P = 0.000) and other continu- either parametric (Student’s t-test, Pearson’s coeffi- ous variables were found to be nonnormal; there- cient of correlation) or nonparametric tests (Mann– fore, nonparametric tests were used for analyses. The Whitney U-test, Spearman’s rank correlation) were average age was 53.9 years, and males were slightly applied. Results of calculations were rounded to one younger than females (53.8 vs 54.9 years, respectively), decimal place. Where information was missing the but this difference was not statistically significant available data was summarized. (P = 0.7, Mann–Whitney U-test). Fig. 2 shows number of patients per age group. Further details about the age Results distribution can be seen in Table 1. The average tracheal size was 36 mm (median and The search in PubMed identified 129 publications mode 34 mm, min 25 mm, max 65 mm). There was no since 1987, and 39 additional publications were found correlation between the patient’s age and tracheal with other methods, with both groups mentioning 173 and/or bronchial diameter, but a weak correlation and 74 cases of MKS, respectively (247 cases in a total could be observed between the tracheal and/or bron- of 168 publications). Sixteen publications were chial diameter and the duration of disease (as defined excluded as the full text of the article could not be by the length of complaints, ρ=0.22, P = 0.045). In acquired. An additional 63 publications did not meet 29.7% (n = 38) of the cases only the tracheal dimen- the inclusion
Load more

Recommended publications
  • Diagnostic Issues in Systemic Lupus Erythematosis
    266 Postgrad Med J 2001;77:266–285 Postgrad Med J: first published as 10.1136/pmj.77.906.268 on 1 April 2001. Downloaded from SELF ASSESSMENT QUESTIONS Diagnostic issues in systemic lupus erythematosis N Sofat, C Higgens Answers on p 274. A 24 year old woman was diagnosed with sys- (4) What other tests (apart from 24 hour urine temic lupus erythematosis (SLE) based on a creatinine clearance) are available to measure few months’ history of a photosensitive skin the glomerular filtration rate? rash, predominantly on her face, arthralgia The patient had a 24 hour urinary protein col- involving both hands and wrists, a positive lection, which showed a 24 hour protein measure- antinuclear antibody (ANA) test and a raised ment of 1.8 g. There was no evidence of cellular antinative double stranded DNA antibody casts on urine microscopy. Her blood results were binding level. She was treated with oral as below (normal values are in parentheses): hydroxychloroquine 400 mg daily and short x Sodium 134 mmol/l (135–145) courses of prednisolone during flare-ups. x Potassium 4.5 mmol/l (3.5–5.0) She was reviewed in clinic for her regular x Urea 7.0 mmol/l (2.5–6.7) follow up appointment when she was found to x Creatinine 173 µmol/l (70–115) be hypertensive on repeated measurements of x Haemoglobin 108 g/l (115–160) her blood pressure, an average value being x White cell count 4.5 × 109/l (4.0–11.0) 150/90 mm Hg. She was also urine dipstick x Platelets 130 × 109/l (150–400) positive for blood and protein.
    [Show full text]
  • The Diseases of Airway-Tracheal Diverticulum: a Review of the Literature
    Review Article The diseases of airway-tracheal diverticulum: a review of the literature Asli Tanrivermis Sayit, Muzaffer Elmali, Dilek Saglam, Cetin Celenk Department of Radiology, Faculty of Medicine, Ondokuz Mayis University, Samsun, Turkey Contributions: (I) Conception and design: A Tanrivermis Sayit; (II) Administrative support: M Elmali, C Celenk; (III) Provision of study materials or patients: A Tanrivermis Sayit; (IV) Collection and assembly of data: A Tanrivermis Sayit, D Saglam; (V) Data analysis and interpretation: A Tanrivermis Sayit, M Elmali, C Celenk; (VI) Manuscript writing: All authors; (VII) Final approval of manuscript: All authors. Correspondence to: Asli Tanrivermis Sayit. Department of Radiology, Faculty of Medicine, Ondokuz Mayis University, 55139, Atakum/Samsun, Turkey. Email: [email protected]. Abstract: Tracheal diverticulum (DV) is a type of paratracheal air cyst (PTAC) that is often asymptomatic and usually detected incidentally by imaging methods. Tracheal DV are divided into two subgroups: congenital and acquired. Dysphagia, odynophagia, neck pain, hoarseness, hemoptysis, choking, and recurrent episodes of hiccups and burping can also be seen in symptomatic patients. Thin-section multidetector computed tomography (MDCT) is useful for diagnosis of tracheal diverticulum. The relationship between DV and tracheal lumen can be demonstrated by axial, coronal, and sagittal reformat multiplanar images. Bronchoscopy can also be used in diagnosis for tracheal DV. However, the connection between DV and tracheal lumen can not be shown easily with bronchoscopy. Conservative treatment is the preferred treatment in asymptomatic patients. Surgical or conservative treatment can be performed for symptomatic patients, depending on patient age and physical condition. Keywords: Trachea; diverticulum (DV); thorax; multidetector computed tomography; tracheal diseases; chronic obstructive pulmonary disease (CODP) Submitted Sep 17, 2016.
    [Show full text]
  • Tracheobronchomegaly
    Thorax: first published as 10.1136/thx.23.3.320 on 1 May 1968. Downloaded from Thorax (1968), 23, 320. Tracheobronchomegaly ZAKI AL-MALLAH1 AND 0. P. QUANTOCK From the University of Mosul, Iraq, and Sully Hospital, Wales Two cases of the rare condition of tracheobronchomegaly are reported. They occurred in people of completely different racial origin and residence. They showed the characteristic features of this condition-loud, rasping, prolonged, remarkably ineffective cough, abnormally wide trachea and major bronchi, laxity of the cartilaginous rings and membranous part of these airways demonstrable on straight chest radiographs and bronchoscopy and confirmed at bronchography. Evidence is submitted of congenital aetiology. Tracheobronchomegaly is a rare congenital apparently healthy young man with no finger club- abnormality with marked widening of the trachea bing. There were scattered wheezes and bilateral basal and major bronchi, in most cases associated with crepitations, mainly anteriorly. No abnormality was chronic recurrent respiratory tract infection. This detected in any other system. The tuberculin test (100 T.U.) was negative and a chest radiograph rare syndrome was first described by Mounier- showed small nodular opacities in the right lung: Kuhn in 1932, but was then given different names these subsequently cleared. Bronchoscopy showed until Katz, LeVine, and Herman (1962), in an reddening and oedema of the mucosa which was excellent review, clearly defined this entity and covered with thick, mucopurulent secretions. No suggested the name tracheobronchomegaly. We intrinsic lesion was seen in the tracheobronchial have been unable to find a report of this syndrome passages, but an abnormal mobility of the posterior http://thorax.bmj.com/ in the British literature and are therefore present- tracheal wall was noted.
    [Show full text]
  • Tracheobronchomalacia and Excessive Dynamic Airway Collapse
    Blackwell Publishing AsiaMelbourne, AustraliaRESRespirology1323-77992006 Blackwell Publishing Asia Pty Ltd? 2006114388406Review ArticleTBM and EDACSD Murgu and HG Colt Respirology (2006) 11, 388–406 doi: 10.1111/j.1400-1843.2006.00862.x REVIEW ARTICLE Tracheobronchomalacia and excessive dynamic airway collapse Septimiu D. MURGU AND Henri G. COLT Pulmonary and Critical Care Medicine, Department of Medicine, University of California School of Medicine, Irvine, CA, USA Tracheobronchomalacia and excessive dynamic airway collapse MURGU SD, COLT HG. Respirology 2006; 11: 388–406 Abstract: Tracheobronchomalacia and excessive dynamic airway collapse are two separate forms of dynamic central airway obstruction that may or may not coexist. These entities are increasingly recognized as asthma and COPD imitators. The understanding of these disease processes, however, has been compromised over the years because of uncertainties regarding their definitions, patho- genesis and aetiology. To date, there is no standardized classification, diagnosis or management algorithm. In this article we comprehensively review the aetiology, morphopathology, physiology, diagnosis and treatment of these entities. Key words: airflow dynamics, bronchomalacia, excessive dynamic airway collapse, tracheobron- chomalacia, tracheomalacia. INTRODUCTION first is that most published studies are case series and retrospective descriptions, many of which report a The purpose of this systematic review is to clarify con- single investigator’s experience with diagnosis and founding issues pertaining to the definition, patho- management. In fact, it is puzzling that despite the physiology, histopathology, aetiology, diagnosis, relative frequency with which TBM and EDAC are pre- classification and treatment of acquired and idio- sumably encountered, multi-institutional or prospec- pathic forms of adult tracheobronchomalacia (TBM) tive studies have not been published.
    [Show full text]
  • Improving Airways Patency and Ventilation Through Optimal
    JMIR RESEARCH PROTOCOLS Lima et al Protocol Improving Airways Patency and Ventilation Through Optimal Positive Pressure Identified by Noninvasive Mechanical Ventilation Titration in Mounier-Kuhn Syndrome: Protocol for an Interventional, Open-Label, Single-Arm Clinical Trial Evelise Lima*, MD; Maria Aparecida Miyuki Nakamura*, PhD; Pedro Rodrigues Genta*, PhD, MD; Ascedio José Rodrigues*, MD; Rodrigo Abensur Athanazio*, PhD, MD; Samia Rached*, MD; Eduardo Leite Vieira Costa*, PhD, MD; Rafael Stelmach*, PhD, MD Pulmonary Division-Heart Institute (InCor), Hospital das Clínicas da Faculdade de Medicina de São Paulo, São Paulo, Brazil *all authors contributed equally Corresponding Author: Evelise Lima, MD Pulmonary Division-Heart Institute (InCor) Hospital das Clínicas da Faculdade de Medicina de São Paulo Dr. Eneas de Carvalho Aguiar, 44 São Paulo Brazil Phone: 55 1126615612 Email: [email protected] Abstract Background: Mounier-Kuhn syndrome or congenital tracheobronchomegaly is a rare disease characterized by dilation of the trachea and the main bronchi within the thoracic cavity. The predominant signs and symptoms of the disease include coughing, purulent and abundant expectoration, dyspnea, snoring, wheezing, and recurrent respiratory infection. Symptoms of the disease in some patients are believed to be pathological manifestations arising due to resident tracheobronchomalacia. Although treatment options used for the management of this disease include inhaled bronchodilators, corticosteroids, and hypertonic solution, there is no consensus on the treatment. The use of continuous positive airway pressure (CPAP) has been reported as a potential therapeutic option for tracheobronchomalacia, but no prospective studies have demonstrated its efficacy in this condition. Objective: The purpose of this is to identify the presence of tracheobronchomalacia and an optimal CPAP pressure that reduces the tracheobronchial collapse in patients with Mounier-Kuhn syndrome and to analyze the repercussion in pulmonary ventilation.
    [Show full text]
  • Diagnosis and Management of Bronchiectasis
    REVIEW CPD Diagnosis and management of bronchiectasis Maeve P. Smith MB ChB MD n Cite as: CMAJ 2017 June 19;189:E828-35. doi: 10.1503/cmaj.160830 ronchiectasis is a chronic, debilitating respiratory condition that affects people of all ages. It is most prevalent in KEY POINTS women and those older than 60 years, and prevalence is • Following a diagnosis of bronchiectasis, it is important to Bincreasing.1 Patients have daily excessive sputum and associated investigate for an underlying cause. symptoms, recurrent chest infections and impaired health-related • Goals of management are to suppress airway infection and quality of life.2,3 In North America, management guidelines are lack- inflammation, to improve symptoms and health-related quality ing. This review discusses best evidence to guide the long-term of life. management of non–cystic fibrosis bronchiectasis in adults, focus- • There are now validated scoring tools to help assess disease ing on the two most common single-entity types of bronchiectasis severity, which can help to stratify management. in adults: idiopathic and postinfectious bronchiectasis4,5 (Box 1). • Good evidence supports the use of both exercise training and Table 1 lists all the types of bronchiectasis by cause. long-term macrolide therapy in long-term disease management. What are the clinical features of bronchiectasis? has better sensitivity but may involve greater radiation doses. Typically, thin section (< 1 mm) slices acquired using a high- First described by Laennec in 1819, bronchiectasis refers to abnor- spatial frequency reconstruction algorithm should be used.18,19 mal permanently dilated airways, which are typically described as cylindrical, varicose or cystic in appearance.10,11 The condition is Determining underlying cause characterized by a vicious cycle of persistent bacterial infection and Determination of the underlying cause may alter management in excessive neutrophilic inflammation owing to impairment of airway as many as 37% of adults presenting with bronchiectasis.5 Rele- defence mechanisms.
    [Show full text]
  • Imaging of the Trachea
    Keynote Lecture Series Imaging of the trachea Jo-Anne O. Shepard, Efren J. Flores, Gerald F. Abbott Division of Thoracic Imaging, Department of Radiology, Massachusetts General Hospital, Harvard Medical School, Boston, MA, USA Correspondence to: Jo-Anne O. Shepard, MD. Thoracic Imaging, Department of Radiology, Massachusetts General Hospital, Founders 202, 55 Fruit Street, Boston, MA 02114, USA. Email: [email protected]. Numerous benign and malignant tracheal diseases may affect the trachea primarily and secondarily. While the posterior anterior (PA) and lateral chest radiograph is the conventional study for initial evaluation of the trachea and central airways, findings may not always be apparent on conventional radiographs, and further evaluation with cross sectional imaging is usually necessary. Computed tomography (CT) is the imaging modality of choice for imaging the trachea and bronchi. Familiarity with the imaging appearances of the normal and diseased trachea will enhance diagnostic evaluation. Keywords: Trachea; tracheal stenosis; tracheal tumor; tracheomalacia Submitted Jan 09, 2018. Accepted for publication Feb 27, 2018. doi: 10.21037/acs.2018.03.09 View this article at: http://dx.doi.org/10.21037/acs.2018.03.09 Introduction mediastinal structures away from the trachea. Visualization of tracheal abnormalities can also be improved by high The trachea extends from the cricoid cartilage to the kilovoltage technique (140 kV). Evaluation of the trachea carina and usually measures 10–12 cm in length. The extra and central airways should be carefully performed on all thoracic trachea is (2–4 cm in length) and transitions to patients presenting with symptoms of stridor, wheezing, the intrathoracic trachea (6–9 cm in length) as it passes “adult-onset” asthma, hemoptysis or recurrent pneumonia.
    [Show full text]
  • Tracheal and Bronchial Surgery
    Tracheal and Bronchial Surgery 1A031 Honorary Editors: Douglas E. Wood, Douglas J. Mathisen, Erino Angelo Rendina Editors: Xiaofei Li, Federico Venuta, David C. van der Zee Associate Editors: Dirk Van Raemdonck, Federico Rea, Jinbo Zhao Xiaofei Li, Federico Venuta, David C. van der Zee Xiaofei Li, Federico Venuta, Editors: Tracheal and Bronchial Surgery Honorary Editors: Douglas E. Wood, Douglas J. Mathisen, Erino Angelo Rendina Editors: Xiaofei Li, Federico Venuta, David C. van der Zee Associate Editors: Dirk Van Raemdonck, Federico Rea, Jinbo Zhao AME Publishing Company Room C 16F, Kings Wing Plaza 1, NO. 3 on Kwan Street, Shatin, NT, Hong Kong Information on this title: www.amegroups.com For more information, contact [email protected] Copyright © AME Publishing Company. All rights reserved. This publication is in copyright. Subject to statutory exception and to the provisions of relevant collective licensing agreements, no reproduction of any part may take place without the written permission of AME Publishing Company. First published 2017 Printed in China by AME Publishing Company Editors: Xiaofei Li, Federico Venuta, David C. van der Zee Cover Image Illustrator: Zhijing Xu, Shanghai, China Tracheal and Bronchial Surgery Hardcover ISBN: 978-988-77841-8-0 AME Publishing Company, Hong Kong AME Publishing Company has no responsibility for the persistence or accuracy of URLs for external or third-party internet websites referred to in this publication, and does not guarantee that any content on such websites is, or will remain, accurate or appropriate. The advice and opinions expressed in this book are solely those of the author and do not necessarily represent the views or practices of AME Publishing Company.
    [Show full text]
  • Brazilian Consensus on Non-Cystic Fibrosis Bronchiectasis Biomarkers
    ISSN 1806-3713 Volume 45, Number 4 July | August 2019 PUBLICAÇÃO OFICIAL DA SOCIEDADE BRASILEIRA DE PNEUMOLOGIA E TISIOLOGIA Volume 45, Number 4 Volume July | August 2019 HIGHLIGHT Brazilian consensus Biomarkers in Chest computed on non-cystic fibrosis community acquired tomography and bronchiectasis pneumonia sleep apnea ISSN 1806-3713 Continuous and Bimonthly Publication, J Bras Pneumol. v. 45, n. 4, July/August 2019 EDITOR-IN-CHIEF Bruno Guedes Baldi - Universidade de São Paulo, São Paulo - SP DEPUTY EDITOR Associação Brasileira Rogerio Souza - Universidade de São Paulo, São Paulo - SP de Editores Científicos ASSOCIATE EDITORS Alfredo Nicodemos da Cruz Santana - HRAN da Faculdade de Medicina da ESCS - Brasília - DF | Area: Doenças pulmonares intersticiais Bruno do Valle Pinheiro - Universidade Federal de Juiz de Fora, Juiz de Fora - MG | Area: Terapia intensiva/Ventilação mecânica Danilo Cortozi Berton - Universidade Federal do Rio Grande do Sul, Porto Alegre - RS | Area: Fisiologia respiratória Publicação Indexada em: Denise Rossato Silva - Universidade Federal do Rio Grande do Sul, Porto Alegre - RS | Area: Tuberculose/Outras infecções respiratórias Latindex, LILACS, Scielo Dirceu Solé - Escola Paulista de Medicina, Universidade Federal de São Paulo, São Paulo - SP | Area: Pneumopediatria Brazil, Scopus, Index Edson Marchiori - Universidade Federal Fluminense, Niterói - RJ | Area: Imagem Copernicus, ISI Web of Fabiano Di Marco - University of Milan - Italy | Area: Asma / DPOC Fernanda Carvalho de Queiroz Mello - Universidade Federal do Rio de Janeiro, Rio de Janeiro - RJ | Area: Tuberculose/ Knowledge, MEDLINE e Outras infecções respiratórias PubMed Central (PMC) Frederico Leon Arrabal Fernandes - Universidade de São Paulo, São Paulo - SP | Area: DPOC/Fisiologia respiratória Giovanni Battista Migliori - Director WHO Collaborating Centre for TB and Lung Diseases, Fondazione S.
    [Show full text]
  • Development of Tracheobronchomegaly
    Anadolu Klin / Anatol Clin Olgu/Case Development of Tracheobronchomegaly during Mechanical Ventilation: An Unusual Case Report Mekanik Ventilasyon Sırasında Gelişen Trakeobronkomegali: Ender Bir Olgu Sunumu Faruk Cicekci Abstract Tracheobronchomegaly, first described in 1932 by Mounier–Kuhn, is a well-known clinical and Anesthesiology, Department of Anesthesiology, Konya Numune radiological entity characterized by marked weakness of the trachea. It can be congenital or Hospital, Konya, Turkey acquired. Some well-known inflammatory and infectious conditions have been associated with the acquired form. Endotracheal intubation can traumatize the trachea and usually results in focal stenosis, but may infrequently cause focal tracheobronchomegaly. It is not clearly un- derstood why some cases result in stenosis and others in dilatation. This study reports the case of a patient whose clinical course was complicated by the development of intrathoracic focal tracheobronchomegaly during mechanical ventilation. Key Words: mechanical ventilation; Mounier–Kuhn syndrome; tracheobronchomegaly Özet Trakeobronkomegali, ilk defa 1932’de Mounier–Kuhn tarafından tarif edilmiş belirgin trakea zayıflığı ile karakterize, konjenital veya kazanılmış olabilen klinik ve radyolojik olarak iyi bilinen bir tablodur. Bazı iyi bilinen enflamasyonlu ve enfeksiyöz durumlar kazanılmış formu ile birlik- Geliş Tarihi /Received : 18.09.2015 Kabul Tarihi /Accepted : 06.11.2015 te olabilmektedir. Endotrakeal entübasyon, trakeayı travmatize edebilmekte ve genellikle fokal Sorumlu Yazar/Corresponding Author darlık ile sonuçlanabilmektedir, ancak çok seyrek olarak fokal trakeobronkomegaliye sebep ola- Faruk Cicekci, M.D. bilmektedir. Bazı olgularda darlık veya dilatasyonun niye oluştuğu açıkça anlaşılamamıştır. Biz, Konya Numune Hospital, Department mekanik ventilasyon sırasında klinik seyri komplike hale gelmiş olan bir intratorasik fokal trake- of Anesthesiology, Yazir Mah.Turgut Ozal Cad.
    [Show full text]
  • Appendices A
    Appendices A. Search Strategy Table A-1. Search strategy for Key Question 1 Search terms Result "PEP therapy"[tiab] OR "PEP mask"[tiab] OR "Oscillating PEP"[tiab] OR "Chest Wall Oscillation"[mh] 2981 OR "postural drainage"[tiab] OR "Drainage, Postural"[mh] OR ((respiratory[tiab] OR lung[tiab] OR Lung[mh] OR chest[tiab]) AND ("Physical Therapy Modalities"[Mesh:NoExp] OR "physical therapy"[tiab] OR physiotherapy[tiab])) OR "HFCC"[tiab] OR ("high frequency"[tiab] AND chest[tiab] AND (compression[tiab] OR oscillation[tiab])) OR "intrapulmonary percussive ventilation"[tiab] OR Frequencer[tiab] OR "lung flute"[tiab] OR autogenic drainage[tiab] OR ACBT[tiab] OR "active cycle of breathing"[tiab] OR "cough assist"[tiab] OR "cough assistance"[tiab] OR "assisted cough"[tiab] OR "MI-E"[tiab] OR "mechanical insufflation-exsufflation"[tiab] OR "thoracic squeeze"[tiab] ((Positive-Pressure Respiration[mh] OR "positive expiratory pressure"[tiab] OR Flutter[tiab] OR 3696 Quake[tiab] OR Cornet[tiab] OR "RC-Cornet"[tiab] OR Acapella[tiab]OR Percussion[mh] OR percussion[tiab] OR percussing[tiab] OR Vibration[mh] OR vibration[tiab] OR oscillating[tiab] OR oscillation[tiab] OR Sound[mh] OR "sound waves"[tiab] OR Bronchoscopy[mh] OR Suction[mh] OR suction*[tiab] OR IPV[tiab] OR "Breathing Exercises"[mh] OR "non-drug"[tiab] OR "non- pharmacological"[tiab]) AND (Airway Obstruction/therapy[mh] OR "airway clearance"[tiab] OR "airway clearing"[tiab] OR "airway obstruction"[tiab] OR "airflow obstruction"[tiab] OR "lung clearance"[tiab] OR "lung clearing"[tiab] OR "sputum
    [Show full text]
  • Huge Tracheal Diverticulum in a Patient with Mounier-Kuhn Syndrome
    European Journal of Case Reports in Internal Medicine Huge Tracheal Diverticulum in a Patient with Mounier-Kuhn Syndrome Michele Mondoni1, Paolo Carlucci1, Elena Maria Parazzini1, Paolo Busatto2, Stefano Centanni1 1Respiratory Unit, San Paolo Hospital, Dept. of Scienze della Salute, Università degli Studi di Milano, Milan, Italy 2UO Pneumologia, San Luca Hospital, USL Nordovest Toscana, Lucca, Italy Doi: 10.12890/2016_000419 - European Journal of Case Reports in Internal Medicine - © EFIM 2016 Received: 01/03/2016 Accepted: 16/03/2016 Published: 15/06/2016 How to cite this article: Mondoni M, Carlucci P, Parazzini EM, Busatto P, Centanni S. Huge tracheal diverticulum in a patient with Mounier-Kuhn syndrome EJCRIM 2016;3:doi:10.12890/2016_000419 Conflicts of Interests: The Authors declare that there are no competing interests. This article is licensed under a Commons Attribution Non-Commercial 4.0 License ABSTRACT Tracheal diverticulum is a rare benign entity. Tracheobronchomegaly (TBM), also known as Mounier-Kuhn syndrome, is a rare disorder characterized by marked dilation of the trachea and main bronchi, associated with thinning or atrophy of the elastic tissue. Because of the weakened trachea and increased intraluminal pressure related to chronic cough, some patients may develop mucosal herniation leading to tracheal diverticulosis. We report the case of a patient with TBM with a huge tracheal diverticulum, diagnosed by bronchoscopy and computed tomography with three-dimensional reconstruction. to our knowledge this is the largest tracheal diameter described in a patient affected by this syndrome. LEARNING POINTS • Tracheal diverticulum is a rare condition that should be considered in the presence of bronchopulmonary disorders characterized by chronic cough and repeated bronchial infection, such as Mounier-Kuhn syndrome.
    [Show full text]