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Congenital Lobar Emphysema
Congenital lobar emphysema (CLE), also known as congenital lobar
hyperinflation (CLH), is caused by overexpansion of the air spaces of a segment
or lobe of histologically normal lung (Fig. 124.9 ). Operative findings can reveal
large blebs protruding from the lung parenchyma (Fig. 124.10 ), but often the
lobe is anatomically normal in appearance, with the exception of massive
overdistention. Compression of adjacent normal lung and mediastinal structures
frequently occurs, which can result in impairment of gas exchange and lifethreatening circulatory collapse. This process is caused by air trapping from either
a developmental deficiency of supporting cartilage in the bronchus of a particular
lobe or a partially obstructing bronchial lesion, either endobronchial or from
external compression (as with a bronchogenic cyst).
FIGURE 124.9 Congenital lobar emphysema of the left upper lobe in a 3-month-old girl who
presented with decreased breath sounds and rales in this area. Note the left-sided secondary
compression atelectasis of the lower lobe.
Clinical Recognition