Fact-sheet: Pulmonary agenesis
Updated on 06/30/2020 at 9:15 AM
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Definition
Defined by the complete absence of bronchopulmonary or vascular structures on the affected side.
Clinical features
Isolated unilateral pulmonary agenesis is generally clinically silent.
However, in right-sided agenesis, clinical symptoms can be pronounced due to extrinsic compression of the displaced left main bronchus lumen, resulting from transmediastinal herniation, exerted by the descending aorta and the thoracic spine.
Bronchomalacia may occur, potentially leading to recurrent infections or even acute respiratory failure.
CT
Small, retracted opaque hemithorax.
Mediastinal shift toward the opaque hemithorax produces an appearance similar to complete pulmonary atelectasis.
The contralateral lung herniates to occupy the abnormal hemithorax to a variable extent.