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Virtual Pediatric Hospital: Paediapaedia: Congenital Lobar Emphysema (CLE) Paediapaedia: Neonatal Chest Diseases

Congenital Lobar Emphysema (CLE)

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


Clinical Presentation:
Usually have symptoms of respiratory distress in the first week of life. Although most present in the first 6 months of life, they can have a delayed presentation. The symptoms are dependent on the degree of compression of the normal lung.

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. icon gif

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% ) icon gif, right middle lobe (32% )icon gif, and right upper lobe (20% )icon gif. 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. icon gif

DDX:

References:

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