Congenital pulmonary airway malformation

Case contributed by Dr Jeremy Jones

Presentation

Respiratory distress but no features of infection or sepsis.

Patient Data

Age: 10 months
Gender: Male
X-ray

2 1/2 months old

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

X-ray

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 generalised 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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Case information

rID: 23511
Case created: 20th Jun 2013
Last edited: 18th Feb 2017
Systems: Paediatrics, Chest
Inclusion in quiz mode: Included

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