Article1 min read·Published Mar 2019Older

Current operative management of congenital lobar emphysema in children: A report from the Midwest Pediatric Surgery Consortium

Part of Congenital Lung Malformations 6 items

pubmed.ncbi.nlm.nih.gov

Michael Helmrath

Purpose: The purpose of this study was to evaluate the clinical presentation and operative outcomes of patients with congenital lobar emphysema (CLE) within a large multicenter research consortium. Methods: After central reliance IRB-approval, a retrospective cohort study was performed on all operatively managed lung malformations at eleven participating children's hospitals (2009-2015). Results: Fifty-three (10.5%) children with pathology-confirmed CLE were identified among 506 lung malformations. A lung mass was detected prenatally in 13 (24.5%) compared to 331 (73.1%) in non-CLE cases (p < 0.0001). Thirty-two (60.4%) CLE patients presented with respiratory symptoms at birth compared to 102 (22.7%) in non-CLE (p < 0.0001). The most common locations for CLE were the left upper (n = 24, 45.3%), right middle (n = 16, 30.2%), and right upper (n = 10, 18.9%) lobes. Eighteen (34.0%) had resection as neonates, 30 (56.6%) had surgery at 1-12 months of age, and five (9.4%) had resections after 12 months. Six (11.3%) underwent thoracoscopic excision. Median hospital length of stay was 5.0 days (interquartile range, 4.0-13.0). Conclusions: Among lung malformations, CLE is associated with several unique features, including a low prenatal detection rate, a predilection for the upper/middle lobes, and infrequent utilization of thoracoscopy. Although respiratory distress at birth is common, CLE often presents clinically in a delayed and more insidious fashion. Level Of Evidence: Level III

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Article · Mar 2019 · 1 min read

In brief

This multicenter study of 53 children with congenital lobar emphysema reveals distinct clinical patterns: low prenatal detection (24.5%), frequent neonatal respiratory symptoms (60.4%), and predominant upper/middle lobe involvement. Most patients underwent open lobectomy in the first year of life, with thoracoscopic approaches used infrequently.

Written by the GCMD Library team from the article.

Michael Helmrath

Statements in this article

Congenital lobar emphysema accounts for 10.5% of operatively managed congenital lung malformations in children.
ResearchResults
Prenatal detection of congenital lobar emphysema occurs in 24.5% of cases, significantly lower than the 73.1% detection rate for other lung malformations.
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Sixty percent of children with congenital lobar emphysema present with respiratory symptoms at birth, compared to 22.7% of children with other lung malformations.
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The left upper lobe is the most common location for congenital lobar emphysema, accounting for 45.3% of cases.
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The right middle lobe is affected in 30.2% of congenital lobar emphysema cases.
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The right upper lobe is involved in 18.9% of congenital lobar emphysema cases.
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Thirty-four percent of children with congenital lobar emphysema undergo resection in the neonatal period.
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The majority of congenital lobar emphysema resections (56.6%) are performed between 1 and 12 months of age.
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Only 9.4% of congenital lobar emphysema resections are performed after 12 months of age.
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Thoracoscopic excision is used in 11.3% of congenital lobar emphysema cases.
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Median hospital length of stay after congenital lobar emphysema resection is 5.0 days with an interquartile range of 4.0 to 13.0 days.
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Congenital lobar emphysema has a predilection for the upper and middle lobes of the lung.
ResearchConclusions
Congenital lobar emphysema often presents clinically in a delayed and more insidious fashion despite respiratory distress at birth being common.
ResearchConclusions