Síndrome de Mounier Kuhn: uma causa rara de traqueomalácia: revisão da Literatura

Abstract

Introduction: Mounier-Kuhn Syndrome is a rare cause of tracheobronchomegaly, which ischaracterized by the pathological dilation of the trachea and main bronchi. The etiology, pathophysiology, and risk factors remain unknown. Multiple respiratory infections are the most common complications. The diagnosis is established by chest computed tomography, and supportive measures are the mainstay of treatment. However, surgery and lung transplant may be considered in severe cases. Objectives: To produce a literature review of articles published in the last 20 years on the main digital scientific platforms. Materials and Methods: The terms “Mounier-Kuhn Syndrome,” “Tracheobronchomegaly,” and “Congenital tracheobronchomegaly” were used on the platforms PubMed, Bireme, Scielo, Embase and UpToDate. Reviews, systematic reviews, meta-analyses, and randomized clinical trials published in the last 20 years in English, Portuguese, or Spanish with adults older than 18 years were included; those in other languages, without open access, or without a DOI were excluded. Results: The search strategy found 84 articles, which were selected in two steps. The first step excluded 20 articles based on their titles, and the second step excluded 21 after analyzing the abstracts of the remaining publications. A total of 41 articles were elegible for this review. Conclusion: The etiology and pathophysiology remain unknown. Clinical features are nonspecific and may lead to alternative diagnoses. Surgical treatment seems to be promising for symptom relief and improvement in quality of life. However, lung transplantation does not have well-defined indications, and further studies are required to expand the understanding of its outcomes