A Newborn Baby with Difficulty Breathing since Birth

X ray of a newborn baby with difficulty breathing since birth is shown below:

Radiological Findings:  X-ray chest  shows opaque left hemithorax and the entire mediastinum is pulled to the left, the right lung has herniated beyond the midline. The endotracheal tube and Ryles tube are in situ. 

Diagnosis suggested is complete agenesis of left lung. This can be confirmed on CT chest.

Comments: 

The findings seen on chest film are absence of lung, opaque hemithorax, mediastinal shift with hernia of contralateral lung to the affected side with normal and symmetrical bony thorax.

CT chest can be helpful to differentiate the three categories of agenesis

(a) complete agenesis, 

(b) lung aplasia and 

(c) lung hypoplasia.

Lung agenesis is rare, developmental anomaly of the lung. It can be:

1. Complete agenesis, manifested by total agenesis of lung tissue, bronchus and pulmonary vessels.

2. Lung aplasia manifested by a rudimentary bronchus but absent lung tissue and pulmonary vessels.

3. Lung hypoplasia manifested by rudimentary bronchus with hypoplastic lung tissue and pulmonary vessels.

Clinical Discussion:
There may be complete absence of a whole lung and its bronchus. In lung aplasia there is no lung tissue but a blind main bronchus is present. Both anomalies show opaque hemithorax with displacement of the mediastinal structures, diaphragm and contralateral lung.

Agenesis of both lungs is incompatible with life.

Unilateral pulmonary agenesis is associated with a high incidence of congenital anomalies in other organs particularly the cardiovascular system, alimentary tract with anal and esophageal atresia,
tracheo-esophageal fistula and renal agenesis. It can be easily ruled out when performing an ultrasonography.