Open AccessMounier-Kuhn Syndrome
Author(s) -
Mărioara Şimon,
Petruţ Vremăroiu,
Fabian Andrei
Publication year - 2014
Publication title -
journal of bronchology and interventional pulmonology
Language(s) - Uncategorized
Resource type - Journals
SCImago Journal Rank - 0.648
H-Index - 33
eISSN - 1944-6586
pISSN - 1948-8270
DOI - 10.1097/lbr.0000000000000068
Subject(s) - medicine , bronchoscopy , tracheobronchomalacia , dilation (metric space) , atrophy , radiology , pathology , mathematics , combinatorics
Mounier-Kuhn syndrome or congenital tracheobronchomegaly is a rare clinical and radiologic condition. It is characterized by a tracheal and bronchial dilation. Fewer than 100 cases have been reported in the medical literature since the original description in 1932. The first utilization of bronchoscopy for diagnosis of this condition was recorded by Lemoine only in 1949. The cause of this condition is not clearly understood; however, histopathologic findings of atrophy of smooth muscles and elastic tissue in the trachea and main bronchi have been observed. Tracheobronchomegaly can be associated with tracheal and bronchial diverticuli.