Congenital lobar emphysema
Progressive over distension of lobe at birth
Male predominance 3:1
Associated with CHD in 15% (PDA, VSD)
Symptoms: respiratory distress (90%) and cyanosis < 6months
Mortality 10%
Treatment surgical resection
Theories and etiology:
1. Immaturity of bronchial cartilage
2. Endobronchial obstruction ( fold, web mucous)
3. Bronchial compression from vascular structure
e.g. PDA, aberrant left pulmonary artery
Lobes involved:
LUL 43%
RML 35%
RUL 20%
Both lobes 5%
X ray appearance:
1. Mass like opacity following birth
2. Lucency develops over next tow weeks
3. Air trapping occurs in affected lobe
4. Compression atelectasis of adjacent lobes
5. Shift of mediastinum away from lobes