Mounier-Kuhn syndrome: a systematic analysis of 128 cases published within last 25 years

Abstract

Background and aims: Mounier-Kuhn syndrome is a rare disease with abnormal enlargement of major airways, but epidemiological studies are lacking, and currently 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. The aim of this work is to systematically review cases published in the last 25 years and to use descriptive statistics to summarize the patient demographic and clinical information therein in order to acquire details about patient clinical characteristics.

Methods: Cases published in world literature between 1987 and 2013 were sought and reviewed according to PRISMA guidelines. Cases were included only if patient's age was at least 18 years, and a computed tomography scan with tracheal measurements was available.

Results: An 8:1 male predominance was found in 89 identified reports (128 cases). Mean age was 53.9 years, and average tracheal diameter was 36.1 mm. No correlation between increasing age and increasing tracheal diameter was found. Bronchiectasis, tracheal diverticulosis and tracheobronchial dyskinesia were common (49.2%, 33.6% and 28.9%, respectively). Cough, dyspnea and recurrent respiratory infections (71.1%, 51.6% and 50.8%, respectively) were the most common complaints.

Conclusions: The data confirm that syndrome mostly manifests with nonspecific respiratory symptoms and is significantly more common in males. Importantly, there was no connection between age and airway diameter, a fact that could mean that the enlargement does not progress over time, and its severity depends on some other yet undetermined factors.

Keywords: COPD; bronchiectasis; chronic respiratory infections; congenital airway disease; cough; dyspnea.