Skip to main content

Main menu

  • Home
  • Current issue
  • Past issues
  • Authors/reviewers
    • Instructions for authors
    • Submit a manuscript
    • Peer reviewer login
  • Journal club
  • Alerts
  • Subscriptions
  • ERS Publications
    • European Respiratory Journal
    • ERJ Open Research
    • European Respiratory Review
    • Breathe
    • ERS Books
    • ERS publications home

User menu

  • Log in
  • Subscribe
  • Contact Us
  • My Cart

Search

  • Advanced search
  • ERS Publications
    • European Respiratory Journal
    • ERJ Open Research
    • European Respiratory Review
    • Breathe
    • ERS Books
    • ERS publications home

Login

European Respiratory Society

Advanced Search

  • Home
  • Current issue
  • Past issues
  • Authors/reviewers
    • Instructions for authors
    • Submit a manuscript
    • Peer reviewer login
  • Journal club
  • Alerts
  • Subscriptions

Emphysema and bronchiectasis in a 48-year-old male

M.L. Gupta, J. Sinha, C. Bhandari, S. Singh
Breathe 2011 8: 169-172; DOI: 10.1183/20734735.004411
M.L. Gupta
1Dept of Chest and TB, SMS Medical College
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • For correspondence: drmlgupta@sify.com
J. Sinha
2Dept of Radiodiagnosis, Getwell Polyclinic Clinic, Jaipur, India
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
C. Bhandari
1Dept of Chest and TB, SMS Medical College
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
S. Singh
1Dept of Chest and TB, SMS Medical College
  • Find this author on Google Scholar
  • Find this author on PubMed
  • Search for this author on this site
  • Article
  • Figures & Data
  • Info & Metrics
  • PDF
Loading

Introduction

A 48-yr-old male was admitted to the hospital with a complaint of chronic cough with mucoid expectoration for the past 27 years. His symptoms frequently worsened in the form of fever, increase in the amount of expectoration and foul smell of expectoration. The patient also complained of difficulty in coughing up sputum. Cough had been associated with exertional dyspnoea for the previous 10 yrs. There was no history of haemoptysis, wheezing, allergic reactions, weight loss or chest pain.

The patient had been on bronchodilators and antibiotics intermittently with partial relief in symptoms during this period. There was no significant family history of similar or other illnesses.

On general physical examination, grade-I clubbing was present. On auscultation, the patient had bilateral coarse crepitations over the chest. When the patient was asked to cough, it was weak/ineffective and there was a loud, rasping sound. Routine blood counts, biochemistry and serum α1-antitrypsin levels (1.58 g·L−1) were within normal limits. Frontal view chest radiograph and high-resolution computed tomography (CT) of the thorax were performed (figs 1 and 2).

Figure 1
  • Download figure
  • Open in new tab
  • Download powerpoint
Figure 1

Chest radiograph.

Figure 2
  • Download figure
  • Open in new tab
  • Download powerpoint
Figure 2

High-resolution CT.

Task 1

Interpret the chest radiograph and CT images.

Answer 1

Chest radiography (fig. 1) suggests extensive emphysema in both lungs and cystic changes suggestive of bronchiectasis in the left lung. The CT scan section (fig. 2), above the aortic arch, shows an enlarged and irregularly shaped trachea.

A high-resolution CT section (fig. 3) just below the carina showed marked centrilobular and panacinar emphysema in both lungs. A cystic form of bronchiectasis was also seen in places. Both main bronchi appear dilated.

Figure 3
  • Download figure
  • Open in new tab
  • Download powerpoint
Figure 3

High-resolution CT just below the carina.

Figure 4
  • Download figure
  • Open in new tab
  • Download powerpoint
Figure 4

Coronal image of thoracic CT.

Figure 5
  • Download figure
  • Open in new tab
  • Download powerpoint
Figure 5

Expiratory CT.

Task 2

  • 1)How would you describe the coronal image of thoracic CT (fig. 4)?

  • 2)What does the expiratory CT image suggest (fig. 5)?

  • 3)Suggest a possible diagnosis for this patient.

Answer 2

  • 1) The coronal reconstruction image of thoracic CT shows an undulating/scalloping appearance of the inner wall of trachea, suggestive of tracheal diverticulosis.

  • 2) The complete collapse of the trachea on expiratory CT indicates tracheal flaccidity and tracheomalacia.

  • 3) The strong possibility of tracheo bronchomegaly (Mounier–Kuhn syndrome) should be considered in this patient. However, it would be neces sary to know the exact diameter of the trachea and/or bronchi at a certain specified level.

A thoracic CT section in the axial plane, 2 cm above the aortic arch, showed a tracheal diameter of 42.6 mm (fig. 6). These findings are consistent with the diagnosis of tracheobronchomegaly.

Figure 6
  • Download figure
  • Open in new tab
  • Download powerpoint
Figure 6

Thoracia CT section in the axial plane.

On fibreoptic bronchoscopy, an enlarged trachea was seen. Other findings during bronchoscopy were prominence of cartilaginous rings and a diverticulum in the left subglottic region (fig. 7). There was a marked diminution of tracheal diameter during expiration. These findings further support the diagnosis of tracheobronchomegaly in this patient.

Figure 7
  • Download figure
  • Open in new tab
  • Download powerpoint
Figure 7

Cartilaginous and a diverticulum (arrow) in the left subglottic region.

Task 3

How would you manage this patient?

Answer 3

The patient should be referred to a longterm physical therapy treatment programme. Infective exacerbations will need frequent courses of antibiotics. The diffuse nature of the pathology precludes any form of surgical treatment. However, the use of tracheobronchial prostheses in advanced cases has been reported [1].

Discussion

The aetiology of this rare syndrome is not clearly understood, although associations with cutis laxa in children [2], Ehlers–Danlos syndrome in adults [3], occurrence in two siblings [4] and absence of tracheobronchial muscle fibres and myenteric plexus [5] suggest that a developmental or congenital connective tissue defect is responsible for this disease. However, presentation of tracheobronchomegaly in the third or fourth decade of life and the absence of a connective tissue disorder in most cases favour an acquired cause. Shin et al. [6] have suggested that chronic irritants, such as cigarette smoke or environmental pollution, might play an important role in the pathogenesis of this disease.

The diagnosis of tracheobronchomegaly can be made on routine chest radiography, bronchography, chest CT and magnetic resonance imaging. For an adult, any diameter of the trachea, right main bronchus and left main bronchus that exceeds 3.0, 2.4 and 2.3 cm, respectively, on a standard chest radiograph is diagnostic of tracheobronchomegaly [7]. On chest CT, tracheal diameter of >3 cm measured 2 cm above the aortic arch, a diameter of right and left main bronchus of >2.0 cm and >1.8 cm, respectively, are considered diagnostic features of this syndrome [8]. Other causes of tracheal widening, e.g. cutis laxa and Ehlers–Danlos syndrome [9], were not considered in the present case.

Tracheal diverticulosis is another important feature of tracheobronchomegaly, and is seen in about one-third of patients. Atrophied elastic and muscular tissues between two cartilages tend to bulge in the form of diverticulosis, resulting in a scalloping appearance of the air column of the tracheal tree.

Among the lung parenchyma changes, a combination of bronchiectasis and emphysema is unique to this syndrome. This happens because of the abnormal flaccidity of the trachea, which predisposes to ineffective cough and retained secretions, resulting in bronchiectasis. Expiratory collapse of the tracheobronchial tree causes emphysema in these patients. Only a few other conditions have concomitant emphysema and bronchiectasis. These include α1-antitrypsin deficiency [10] and, rarely, anorexia nervosa [11]. Both of these could safely be excluded in this case.

The present case not only met all the radiological criteria required for the diagnosis of this syndrome, but also exhibited all possible bronchoscopic findings in such a case. To conclude, Mounier–Kuhn syndrome should be suspected in any patient with chronic productive cough with underlying emphysema and bronchiectasis. Careful attention to central airways, in such settings, is obligatory to discover this uncommon disorder.

The authors would like to thank M. Gupta for his secretarial help.

Footnotes

  • Statement of interest

    None declared.

  • ©ERS 2011

References

  1. ↵
    1. Barakat J,
    2. Belleguic C,
    3. Le Garff G,
    4. et al
    . [Treatment of tracheobronchomegaly with Ultraflex prosthesis. A case report.] Rev Pneumol Clin 2002; 58: 19–22.
    OpenUrl
  2. ↵
    1. Wanderer AA,
    2. Ellis EF,
    3. Goltz RW,
    4. et al
    . Tracheobronchomegaly and cutis laxa in a child: physiologic and immunologic studies. Pediatrics 1969; 44: 709–715.
    OpenUrlAbstract/FREE Full Text
  3. ↵
    1. Aaby GV,
    2. Blake HA
    . Tracheobronchomegaly. Ann Thorac Surg 1966; 2: 64–70.
    OpenUrlCrossRefPubMed
  4. ↵
    1. Johnston RF,
    2. Green RA
    . Tracheobronchomegaly: Report of five cases and demonstration of familial occurrence. Am Rev Respir Dis 1965; 91: 35–50.
    OpenUrlPubMed
  5. ↵
    1. Nadich DP,
    2. Webb WR,
    3. Muller NL,
    4. et al
    . Computed Tomography and Magnetic Resonance of the Thorax, 3rd Edn., Philadelphia: Lippincott-Raven, 1999; 161–290.
  6. ↵
    1. Shin MS,
    2. Jackson RM,
    3. Ho KJ
    . Tracheobronchomegaly (Mounier–Kuhn syndrome): CT diagnosis. Am J Roentgenol 1988; 150: 777–779.
    OpenUrlPubMed
  7. ↵
    1. Himalstein MR,
    2. Gallagher JC
    . Tracheobronchomegaly. Ann Otol Rhinol Laryngol 1973; 82: 223–227.
    OpenUrlAbstract/FREE Full Text
  8. ↵
    1. Blake MA,
    2. Chaoui AS,
    3. Barish MA
    . Thoracic case of the day: Mounier-Kuhn syndrome (tracheobronchomegaly). Am J Roentgenol 1999; 173: 824–825.
    OpenUrl
  9. ↵
    1. Choplin RH,
    2. Wehunt WD,
    3. Theros EG
    . Diffuse lesions of the trachea. Semin Roentgenol 1983; 18: 38–50.
    OpenUrlCrossRefPubMed
  10. ↵
    1. Jones DK,
    2. Godden D,
    3. Cavanagh P
    . α-1 Antitrypsin deficiency presenting as bronchiectasis. Br J Dis Chest 1985; 79: 301–304.
    OpenUrlPubMed
  11. ↵
    1. Cook VJ,
    2. Coxson HO,
    3. Mason AG,
    4. et al
    . Bullae, bronchiectasis and nutritional emphysema in severe anorexia nervosa. Can Respir J 2001; 8: 361–365.
    OpenUrlPubMed
View Abstract
PreviousNext
Back to top
Vol 8 Issue 2 Table of Contents
Breathe: 8 (2)
  • Table of Contents
  • Index by author
Email

Thank you for your interest in spreading the word on European Respiratory Society .

NOTE: We only request your email address so that the person you are recommending the page to knows that you wanted them to see it, and that it is not junk mail. We do not capture any email address.

Enter multiple addresses on separate lines or separate them with commas.
Emphysema and bronchiectasis in a 48-year-old male
(Your Name) has sent you a message from European Respiratory Society
(Your Name) thought you would like to see the European Respiratory Society web site.
CAPTCHA
This question is for testing whether or not you are a human visitor and to prevent automated spam submissions.
Print
Citation Tools
Emphysema and bronchiectasis in a 48-year-old male
M.L. Gupta, J. Sinha, C. Bhandari, S. Singh
Breathe Dec 2011, 8 (2) 169-172; DOI: 10.1183/20734735.004411

Citation Manager Formats

  • BibTeX
  • Bookends
  • EasyBib
  • EndNote (tagged)
  • EndNote 8 (xml)
  • Medlars
  • Mendeley
  • Papers
  • RefWorks Tagged
  • Ref Manager
  • RIS
  • Zotero

Share
Emphysema and bronchiectasis in a 48-year-old male
M.L. Gupta, J. Sinha, C. Bhandari, S. Singh
Breathe Dec 2011, 8 (2) 169-172; DOI: 10.1183/20734735.004411
del.icio.us logo Digg logo Reddit logo Technorati logo Twitter logo CiteULike logo Connotea logo Facebook logo Google logo Mendeley logo
Full Text (PDF)

Jump To

  • Article
    • Introduction
    • Discussion
    • Footnotes
    • References
  • Figures & Data
  • Info & Metrics
  • PDF

Subjects

  • CF and non-CF bronchiectasis
  • Lung imaging
  • Pulmonary pharmacology and therapeutics
  • Respiratory clinical practice
  • Tweet Widget
  • Facebook Like
  • Google Plus One

More in this TOC Section

  • Lung cancer screening by volume CT
  • In pursuit of the primary
  • A rare complication in a case of nonsmall cell lung carcinoma
Show more Expert opinion

Related Articles

Navigate

  • Home
  • Current issue
  • Archive

About Breathe

  • Journal information
  • Editorial board
  • Press
  • Permissions and reprints
  • Advertising

The European Respiratory Society

  • Society home
  • myERS
  • Privacy policy
  • Accessibility

ERS publications

  • European Respiratory Journal
  • ERJ Open Research
  • European Respiratory Review
  • Breathe
  • ERS books online
  • ERS Bookshop

Help

  • Feedback

For authors

  • Intructions for authors
  • Publication ethics and malpractice
  • Submit a manuscript

For readers

  • Alerts
  • Subjects
  • RSS

Subscriptions

  • Accessing the ERS publications

Contact us

European Respiratory Society
442 Glossop Road
Sheffield S10 2PX
United Kingdom
Tel: +44 114 2672860
Email: journals@ersnet.org

ISSN

Print ISSN: 1810-6838
Online ISSN: 2073-4735

Copyright © 2023 by the European Respiratory Society