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Congenital lobar emphysema: experience with 21 cases
C L Thakral1, D C Maji, M J Sajwani
1Department of Pediatric Surgery, Royal Hospital, P.O. Box 1331, Postal Code 111, Muscat, Sultanate of Oman.
Pediatric Surgery International
|April 24, 2001
Summary
Congenital lobar emphysema (CLE) affects infants of all races, with upper lobe cases being more severe than middle lobe. Mild middle lobe CLE may respond to conservative treatment, but infection often necessitates surgery.
Area of Science:
- Pediatric Surgery
- Pulmonology
- Medical Genetics
Background:
- Congenital lobar emphysema (CLE) is a rare congenital lung malformation.
- Previous literature suggested CLE predominantly affects male Caucasian infants.
Purpose of the Study:
- To review the presentation, diagnosis, treatment, and outcomes of CLE cases.
- To evaluate the severity and management of CLE based on lobe involvement.
Main Methods:
- Retrospective chart review of 21 CLE cases treated between September 1988 and August 1999.
- Analysis of patient demographics, clinical presentation, lobe distribution, symptom severity, and treatment outcomes.
Main Results:
- All 21 patients were non-Caucasian.
- Upper lobe involvement (14 cases) was associated with more severe symptoms than middle lobe involvement (7 cases).
- Mildly symptomatic cases, particularly those involving the middle lobe, often required surgery if infection developed.
Conclusions:
- CLE affects infants of diverse ethnicities, challenging previous assumptions.
- Upper lobe CLE generally presents with more severe symptoms compared to middle lobe CLE.
- Conservative management may be effective for mild middle lobe CLE, but surgical intervention is frequently necessary, especially with infection.