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Virtual Pediatric Hospital: Paediapaedia: Pulmonary Agenesis Paediapaedia: Chest and Airway Diseases

Pulmonary Agenesis

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed


Clinical Presentation:
Unilateral agenesis is usually asymptomatic. The child usually develops respiratory distress and presents in the first year of life when the remaining lung is compromised, usually by a lower respiratory tract infection. Sixty percent have other congenital anomalies including cardiac lesions, bronchogenic cysts, diaphragmatic hernias, and skeletal anomalies (commonly vertebral or rib anomalies). Agenesis of the right lung is associated with a higher frequency of anomalies than agenesis of the left lung.

Etiology/Pathophysiology:
Due to complete failure of development of one or both of the lung buds.

Pathology:
Not applicable

Imaging Findings:
Asymmetry in chest volume and configuration with overexpansion of the normal lung often causing some mediastinal shift to the contralateral side. The definitive diagnosis is made via pulmonary angiography which shows the pulmonary artery to the affected side to be absent.

DDX:

References:

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