State-of-the-art narrative review: Mounier-Kuhn syndrome and tracheobronchomegaly

Abstract

Mounier-Kuhn syndrome (MKS) or tracheobronchomegaly is an uncommon disease of the central airways. It is characterized by pathological dilatation of the trachea and main bronchi and inevitably leads to recurrent respiratory infections, bronchiectasis, hospitalizations, and results in considerable morbidity and mortality. Despite numerous case reports, there is a shortage of evidence on clinical outcomes and limited data on interventions, thus presenting a significant gap in the literature. Fortunately, new strategies and increasing clinical experience have improved the clinical approach, diagnostic workup, classification, and management of MKS. Articles in English, Spanish, and French were searched from databases, including Pubmed, Google Scholar, Medline, and SCOPUS, using the terms "Mounier-Kuhn syndrome," "Tracheomegaly," "Tracheobronchomegaly," and "Bronchomegaly," without date restrictions. A total of 360 articles with the aforementioned syntax were indexed on Pubmed. This state-of-the-art review attempts to fill a void in the current literature by summarizing the current scientific knowledge and highlighting novel interventional strategies in the management of Mounier Kuhn Syndrome.

Keywords: Airway; Bronchi; Bronchiectasis; Mucociliary; Trachea.