Congenital Lobar Emphysema

• Severe overinflation of a lobe, usually causing respiratory distress

• Male predominance

• Only 1/3 are manifest at birth; most are some weeks later

• Associated with congenital heart disease 15% of time (PDA, VSD)

• Predilection for the left upper lobe and less so for RML

Imaging

• Hazy, mass-like density immediately following birth

• Overinflation and air-trapping after several days

• Contralateral mediastinal shift

• Vascular markings are widely separated

 DDx — aspiration of foreign body with air-trapping, space occupying endobronchial lesion, adenomatoid malformation at birth

Clinically

 • About 90% suffer respiratory distress during first few days of life

 • Cyanosis

 • Course is rapid and may be fatal unless emphysematous lobe is resected

Congenital Lobar Emphysema, left upper lobe. There is hyperaeration of the left upper lobe (black arrow) manifest by hyperlucency in this region. The left lower lobe is compressed by the over-expanded upper lobe (white arrow). The expanded lung displaces the mediastinum to the right (red arrow).