Mounier-Kuhn syndrome or congenital tracheobronchomegaly: A literature review

Eduards Krustins, Zaiga Kravale, Atis Buls

Research output: Contribution to journalReview articlepeer-review

38 Citations (Scopus)

Abstract

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. TBM - tracheobronchomegaly, MKS - Mounier-Kuhn syndrome, CT - computed tomography, COPD - chronic obstructive pulmonary disease.

Original languageEnglish
Pages (from-to)1822-1828
Number of pages7
JournalRespiratory Medicine
Volume107
Issue number12
DOIs
Publication statusPublished - Dec 2013
Externally publishedYes

Keywords*

  • Bronchiectasis
  • Chronic respiratory infections
  • COPD
  • Mounier-Kuhn syndrome
  • Tracheobronchomegaly

Field of Science*

  • 3.2 Clinical medicine

Publication Type*

  • 1.1. Scientific article indexed in Web of Science and/or Scopus database

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