Journal of thoracic imaging
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Review Case Reports
Congenital tracheobronchomegaly (Mounier-Kuhn syndrome): a report of 10 cases and review of the literature.
Mounier-Kuhn syndrome is a congenital abnormality of the trachea and main bronchi characterized by atrophy or absence of elastic fibers and thinning of muscle, which allows the trachea and main bronchi to become flaccid and markedly dilated on inspiration with narrowing or collapse on expiration or cough. The abnormal airway dynamics and pooling of secretions in broad outpouchings of redundant musculomembranous tissue between the cartilaginous rings predispose to the development of chronic pulmonary suppuration, bronchiectasis, emphysema, and pulmonary fibrosis. ⋯ In men it is diagnosed when the transverse and sagittal diameters of the trachea exceed 25 mm and 27 mm, respectively, and when the transverse diameters of the right and left main bronchi exceed 21.1 mm and 18.4 mm, respectively. The diagnosis can be confirmed easily by computed tomography.
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Plain film signs of COPD, spirometric evidence of airflow obstruction, and smoking history were correlated in a group of 182 men aged 32 to 85 years (average, 57.5 years) who presented for evaluation of possible pulmonary disability. There were 148 current or past smokers (range, 0.66 to 150 pack-years; average, 31.89 pack-years) and 34 lifetime nonsmokers. A single observer, who had no knowledge of the other parameters, prospectively evaluated posteroanterior chest radiographs for 11 signs of COPD. ⋯ We found a statistically significant association between smoking and airflow obstruction on spirometry (P less than 0.001) and an equally significant association between smoking and radiographic signs of COPD on plain chest films (P less than 0.001). Both airflow obstruction and radiologic signs of COPD were generally absent in lifetime nonsmokers. The plain film signs of COPD were only of moderate value in predicting spirometric evidence of airflow obstruction in smokers; spirometric evidence is not the gold standard for the presence of COPD, however, and the strong association between smoking and these radiologic signs may indicate that in smokers the presence of plain film signs of COPD reflects morphologic abnormality in the lungs indicative of disease.(ABSTRACT TRUNCATED AT 250 WORDS)