Congenital LobarEmphysema

Congenital LobarEmphysema

Congenital Lobar EmphysemaGeneral

Progressive overdistension of lobe at birth

Male predominance 3:1

Associated with CHD in 15% (PDA,VSD)

Symptoms: respiratory distress (90%) andcyanosis < 6 mos

Mortality: 10%

Treatment:surgical resection

Congenital Lobar EmphysemaTheories of Etiology

Immaturity of bronchial cartilage

Endobronchial obstruction (fold, web,mucus)

Bronchial compression from vascularstructure

e.g. PDA, aberrant left pulmonary artery

Congenital Lobar EmphysemaLocation

LUL

RML

RUL

Two lobes

Lobe(s) involved

43%

35%

20%

5%

Frequency

Mass-like opacity following birth

Lucency develops over next two weeks

Air-trapping occurs in affected lobe

Compression atelectasis of adjacentlobes

Shift of mediastinum away from lesion

Congenital Lobar EmphysemaX-ray Appearance