Bilateral congenital lobar emphysema: a rare entity and a therapeutic challenge

Background Bilateral congenital lobar emphysema is an extremely rare condition, and detailed workup of these patients is very important. Operative intervention is usually based on radiological, biochemical, and clinical details. Bronchoscopy gives an added advantage in cases with diagnostic dilemmas...

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Bibliographic Details
Published inAnnals of Pediatric Surgery Vol. 18; no. 1; pp. 91 - 8
Main Authors Garge, Saurabh, Mahalik, Santosh, Jain, Pradeep
Format Journal Article
LanguageEnglish
Published Berlin/Heidelberg Springer Berlin Heidelberg 01.12.2022
Springer
Springer Nature B.V
SpringerOpen
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Summary:Background Bilateral congenital lobar emphysema is an extremely rare condition, and detailed workup of these patients is very important. Operative intervention is usually based on radiological, biochemical, and clinical details. Bronchoscopy gives an added advantage in cases with diagnostic dilemmas and makes the treatment more evidence based. We here present three cases and review the literature for this rare entity. Material and methods We retrospectively collected data about all the cases of bilateral CLE operated by the authors at various centres over the period of 3 years from January 2016 to December 2018. Results We managed three cases of bilateral CLE, and all three had unique details based on which treatment was planned. We operated three cases of this very rare entity and compared our findings with 23 cases from the literature reviewed. Conclusion We propose that bronchoscopy should be an essential component in the management of all cases of CLE. It helps in defining pathology, the severity of the disease, and the decision on which side to be operated first and avoids unnecessary simultaneous lobectomies. We recommend case selection on basis of radiological, biochemical, clinical, and bronchoscopy criteria. Based on this, an approach of sequential lobectomies is less risky and should be advocated in most of the patients.
ISSN:2090-5394
1687-4137
2090-5394
DOI:10.1186/s43159-022-00224-w