Editorial

Mounier-Kuhn Syndrome: Overcoming a Lack of Recognition

Authors: Elie G. Abdallah MD, Rendell W. Ashton MD

Abstract

In 1897, Czyhlarz first described the finding of tracheobronchomegaly after noting the abnormality in an autopsy.1 Thirty-five years later, Mounier-Kuhn described the clinical presentation of cough with reduced effectiveness and large sputum production, with the radiographic appearance and bronchoscopic findings associated with enlarged major airways.2 The prevalence of the disease is not known, but is almost certainly underestimated, as a considerable number of cases remain subclinical and would only be recognized if seen incidentally on an imaging study. In addition, many clinicians have little knowledge of, or experience with the disease and may not recognize it even if imaging is performed. Even those physicians who are aware of the condition and identify it have only scant evidence on which to base their clinical management. They are forced to rely on the fewer than 100 case reports published in the literature to treat their patients.

This content is limited to qualifying members.

Existing members, please login first

If you have an existing account please login now to access this article or view purchase options.

Purchase only this article ($25)

Create a free account, then purchase this article to download or access it online for 24 hours.

Purchase an SMJ online subscription ($75)

Create a free account, then purchase a subscription to get complete access to all articles for a full year.

Purchase a membership plan (fees vary)

Premium members can access all articles plus recieve many more benefits. View all membership plans and benefit packages.

References

1. Czyhlarz ER. Uber ein pulsiondivertikel der trachea mit bemerkungen d'uber das verhalten der elastichen fasern an normalen tracheen und bronchien. Centralb Allg Pathol Anat 1897, pp. 721–728.
 
2. Mounier-Kuhn P. Dilatation de la trachee constatations radiographiques et bronchoscopiques, Lyon Med 1932;150:106–109.
 
3. Menon B, Aggarwal B, Iqbal A. Mounier-Kuhn syndrome: report of 8 cases of tracheobronchomegaly with associated complications South Med J 2008;101:83–87.
 
4. Shah SS, Karnak D, Mason D, et al. Pulmonary transplantation in Mounier-Kuhn syndrome: a case report. J Thorac Cardiovasc Surg 2006;131:757–758.
 
5. Drain AJ, Perrin F, Tasker A, et al. Double lung transplantation in a patient with tracheobronchomegaly (Mounier-Kuhn syndrome). J Heart Lung Transplant 2006;25:134–136.