Congenital pulmonary airway malformation

Case contributed by Jeremy Jones


Respiratory distress but no features of infection or sepsis.

Patient Data

Age: 10 months
Gender: Male

2 1/2 months old


The mediastinum is shifted to the left due to hyperexpansion of the right lung.  The cardiothymic contour is normal.

10 months old


The right lung, which is relatively oligaemic, has increased in volume with subsequent mediastinal shift.

There is a large area of abnormal hyper-expanded oligaemic lung parenchyma in the anterior portion of the right lung.  The mediastinum is displaced by the mass-effect it produces.

The abnormal lung is not restricted by normal boundaries and crosses the oblique fissure.

Case Discussion

Congenital pulmonary airway malformations are complex pulmonary anomalies that can have a broad range of appearances.

They may result in grossly cystic abnormalities, or, as in this case, result in a more generalized emphysematous appearance.  They may cross the normal boundaries within the lung and exist on either side of the major and minor fissures.

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