Congenital pulmonary agenesis

Case contributed by Dr Mostafa El-Feky

Presentation

Cough and dyspnea. Left sided lung opacity by x-ray (not available).

Patient Data

Age: 15 years
Gender: Male
CT

Single right lung is noted extending to the left hemithorax. 

Single right pulmonary artery

Two right pulmonary veins entering the right atrium

One main right pulmonary bronchus

The heart and mediastinum are shifted towards the left side lying at the left hemithorax posteriorly covered by right lung transthoracic herniation.

Normal left-sided aortic arch and its branches.

Case Discussion

Radiological features are in favor of congenital left pulmonary agenesis. If unilateral, the remaining lung contains a double amount of alveoli but normal bronchi. Pulmonary agenesis and aplasia are difficult to discriminate, agenesis differs from aplasia by the presence of a rudimentary bronchus in the latter. They differ from pulmonary hypoplasia which has immature pulmonary tissues. Patients are usually asymptomatic and diagnosis is usually incidental at childhood, however they have increased susceptibility to infection 1.

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Case information

rID: 67517
Published: 13th Apr 2019
Last edited: 14th Apr 2019
Inclusion in quiz mode: Included
Institution: Dar El Ashaa Center

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