Pulmonary Agenesis

Etiology:
— Congenital complete absence of the ipsilateral mainstem bronchus and pulmonary artery and lung tissue
— Contralateral lung may develop twice as many as normal alveoli
Imaging CXR: Complete opacification of hemithorax with ipsilateral mediastinal shift and compensatory hyperinflation of contralateral lung
Imaging CT:
— Confirms absence of ipsilateral mainstem bronchus and pulmonary artery
— More distal pulmonary arteries may be fed by collateral vessels such as bronchial arteries
DDX:
Complications:
Treatment:
Clinical: Present with respiratory distress

Radiology Cases of Pulmonary Agenesis

CXR and bronchogram and pulmonary angiogram of pulmonary agenesis — CXR AP and lateral (above) shows complete opacification of the left hemithorax and tracheal and mediastinal shift to the left and hyperexpansion of the right lung. CXR AP taken during a bronchogram (below left) shows absence of the left mainstem bronchus. AP image from a pulmonary angiogram (below right) shows a normal appearing right main pulmonary artery and absence of the left pulmonary artery.

CXR and bronchogram of pulmonary agenesis — CXR AP and lateral (above) shows the right hemithorax, which is completely opacified, to be smaller in size than the left hemithorax and there is hyperexpansion of the left lung and mediastinal shift to the right. AP image from a vintage bronchogram (below) shows contrast filling the airways of the left upper lobe, lingula and left lower lobe. There is no contrast in the right hemithorax.

CXR and CT of pulmonary agenesis — CXR AP (above) shows complete opacification of the left hemithorax with mediastinal shift to the left. Axial CT without contrast of the chest (below) show hyperexpansion of the right lung with herniation across the midline, mediastinal shift to the left, and non-visualization of the left mainstem bronchus.

CXR and CT of pulmonary agenesis — CXR AP (left) shows the right hemithorax to be smaller than the left hemithorax and there is opacity in the right upper and middle lobes and an aerated right lower lobe. There are multiple hemivertebrae and a butterfly vertebra in the thoracic spine causing a scoliosis convex left. Axial CT with contrast of the chest (above right) shows the right pulmonary artery to be absent along with the right upper lobe. Axial CT (below right) shows absence of the right middle lobe of the lung but the right lower lobe of the lung is present and aerated via a small bronchus off of the trachea (not shown) and perfused by collateral vessels arising from the 7 o’clock position off of the aorta.

Gross Pathology Cases of Pulmonary Agenesis

Gross pathological image of right pulmonary agenesis — Gross pathological image shows a normal appearing left mainstem bronchus and left upper lobe and left lower lobe. There is complete absence of the right mainstem bronchus and right lung.