Congenital Lobar Emphysema (CLE)

Michael P. D'Alessandro, M.D.
Peer Review Status: Internally Peer Reviewed

Etiology/Pathophysiology:
Congenital overdistension of the lobe can be due to an intrinsic airway obstruction or alveolar overgrowth. Usually only one lobe is involved. A definite etiology is seen in only 50% of patients.

Pathology:
Intrinsic cartilage anomaly or compression by an extrinsic vascular structure.

Imaging Findings:
Progressive overdistension of a lobe that compresses adjacent lobes and causes mediastinal shift to the contralateral side is seen. The overdistended lobe appears oligemic. The most frequently affected lobes are left upper lobe (43% ) , right middle lobe (32% ), and right upper lobe (20% ). It is rarely seen in the lower lobes. CLE can begin by having fluid in it, causing it to look like an opaque lung mass, but then the fluid clears via resorption and is replaced by air and it takes on its normal cystic appearance.

DDX:

• Congenital Pulmonary Airway Malformation - Usually has multiple cysts, unlike congenital lobar emphysema which has only one cyst.
• Diaphragmatic Hernia - Loops of bowel in thorax usually easily identified, can be confirmed via upper GI.
• Bronchial obstruction due to foreign body or mucous plug - Seen in older children
• Extrinsic obstruction from vascular structures
• Congenital lung cyst

References:

• Correlapedia
• Thoracopedia

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