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Mounier-Kuhn Syndrome: Evolution of Features Over 15 Years FREE TO VIEW

Joseph Skalski, MD; Jay Ryu, MD
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Mayo Clinic, Rochester, MN

Chest. 2014;146(4_MeetingAbstracts):335A. doi:10.1378/chest.1988173
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SESSION TITLE: Miscellaneous Case Report Posters II

SESSION TYPE: Affiliate Case Report Poster

PRESENTED ON: Tuesday, October 28, 2014 at 01:30 PM - 02:30 PM

INTRODUCTION: The clinical course of a patient with Mounier-Kuhn syndrome over fifteen years is presented including evolution of clinical, physiologic and CT imaging features.

CASE PRESENTATION: A 28 year-old man presented to our clinic for evaluation of chronic cough and recurrent pneumonias since childhood. His symptoms had previously been attributed to asthma. Evaluation including chest CT revealed tracheobronchomegaly with tracheal diverticula and bronchiectasis. He was diagnosed to have Mounier-Kuhn syndrome and was initiated on a program of bronchial hygiene including as-needed antibiotics. In the subsequent years, he noted no respiratory limitations but continued to experience a chronic cough with intermittent episodes of pneumonia. Serial PFTs showed development of moderate airflow obstruction. Serial chest CT demonstrated gradual progression of large and small airway disease as well as mucous plugging. At the age of 42 years, a bronchial stent was inserted into the left main stem bronchus for collapsibility with secretion retention. In recent years his respiratory secretions have grown multidrug-resistant Pseudomonas aeruginosa, Achromobacter species, Aspergillus fumigatus and Mycobacterium avium complex.

DISCUSSION: Mounier-Kuhn syndrome is a congenital disorder characterized by tracheobronchomegaly with inefficient cough and poor mucous clearance. Symptoms include chronic productive cough with recurrent respiratory tract infections often resulting in bronchiectiasis.1 As illustrated in this case, the diagnosis is often delayed for decades. Recurrent respiratory infections in Mounier-Kuhn syndrome often start in childhood and are commonly attributed to asthma or another condition.1 The exact prevalence of Mounier-Kuhn syndrome is unknown, but is likely underdiagnosed. The natural history of Mounier-Kuhn syndrome is largely unknown. There are no large case series, and most case reports describe patients followed for less than a year.1 We present a patient with follow-up over a course of fifteen years demonstrating evolution of clinical, physiologic and imaging findings.

CONCLUSIONS: Congenital pulmonary disorders such as Mounier-Kuhn syndrome should be considered in patients presenting with respiratory symptoms dating back to childhood. The natural history of Mounier-Kuhn appears to be variable but can be progressive in some patients.

Reference #1: Krustins E, Kravale Z, Buls A. Mounier-Kuhn syndrome or congenital tracheobronchomegaly: A literature review Respiratory Medicine 2013;107:1822-1828

DISCLOSURE: The following authors have nothing to disclose: Joseph Skalski, Jay Ryu

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