Congenital lobar emphysema

Congenital lobar emphysema (CLE) describes a malformation of the lung. The affected lung lobe is overstretched and causes compression and displacement of adjacent normal lung tissue.

Most patients suffer from shortness of breath, which is often associated with lower respiratory tract infections, which increase the backflow of air. The first symptoms occur in most patients already in the neonatal period or early childhood. A manifestation in adulthood is rather unusual.

In 40% of cases, concomitant malformations occur, in particular heart defects, skeletal system abnormalities and heart defects.

The etiology is still unclear, but it is suspected in familial cases, a hypoplasia of the bronchial cartilage as the cause. The air is trapped after inhalation in a section of the lung, which now inflates greatly and compresses the surrounding lung tissue. Bronchomalacia, mucosal leaks or stenoses can lead to this valve mechanism.

In rare cases stenosis can also be caused by vascular anomalies or bronchogenic cysts. Inheritance is autosomal dominant in familial cases.