LearningRadiology - Congenital Lobar Emphysema

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

X-ray

• 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

WH/’91