Right Pulmonary Agenesis with Associated Skeletal Malformation

Pulmonary agenesis is a rare congenital anomaly defined by the total absence of pulmonary parenchyma, bronchi and vessels. It is often associated with other malformations. We report the case of a late revelation of right pulmonary agenesis in a 3 year old patient, presenting with recurrent episodes of respiratory infection. Chest radiography showed an opaque right hemithorax. The diagnosis of right lung agenesis was confirmed by chest CT scan with contrast enhancement. In our case pulmonary agenesis was associated with ipsilateral rib synostosis. CT scan is the main imaging modality for establishing a diagnosis of pulmonary agenesis, as well as demonstrating other associated malformations.