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

Midwest Pediatric Surgery Consortium

Research output: Contribution to journalArticle

Abstract

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.

Original languageEnglish (US)
Pages (from-to)1138-1142
Number of pages5
JournalJournal of pediatric surgery
Volume54
Issue number6
DOIs
StatePublished - Jun 1 2019
Externally publishedYes

Fingerprint

Pediatrics
Lung
Emphysema
Length of Stay
Parturition
Thoracoscopy
Research Ethics Committees
Congenital lobar emphysema
Cohort Studies
Retrospective Studies
Newborn Infant
Pathology
Research

Keywords

  • Congenital lobar emphysema
  • Congenital lung malformations
  • Congenital pulmonary airway malformation

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health

Cite this

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

In: Journal of pediatric surgery, Vol. 54, No. 6, 01.06.2019, p. 1138-1142.

Research output: Contribution to journalArticle

@article{fb85e0d5f3e84a90a3a6ae55a3835b39,
title = "Current operative management of congenital lobar emphysema in children: A report from the Midwest Pediatric Surgery Consortium",
abstract = "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.",
keywords = "Congenital lobar emphysema, Congenital lung malformations, Congenital pulmonary airway malformation",
author = "{Midwest Pediatric Surgery Consortium} and Shaun Kunisaki and Saito, {Jacqueline M.} and Fallat, {Mary E.} and {St. Peter}, {Shawn D.} and Kim, {Aimee G.} and Johnson, {Kevin N.} and Mon, {Rodrigo A.} and Cheryl Adams and Bola Aladegbami and Christina Bence and Burns, {R. Cartland} and Corkum, {Kristine S.} and Deans, {Katherine J.} and Downard, {Cynthia D.} and Fraser, {Jason D.} and Gadepalli, {Samir K.} and Helmrath, {Michael A.} and Rashmi Kabre and Lal, {Dave R.} and Landman, {Matthew P.} and Leys, {Charles M.} and Linden, {Allison F.} and Lopez, {Joseph J.} and Mak, {Grace Z.} and Minneci, {Peter C.} and Rademacher, {Brooks L.} and Aimen Shaaban and Walker, {Sarah K.} and Wright, {Tiffany N.} and Hirschl, {Ronald B.}",
year = "2019",
month = "6",
day = "1",
doi = "10.1016/j.jpedsurg.2019.02.043",
language = "English (US)",
volume = "54",
pages = "1138--1142",
journal = "Journal of Pediatric Surgery",
issn = "0022-3468",
publisher = "W.B. Saunders Ltd",
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T1 - Current operative management of congenital lobar emphysema in children

T2 - A report from the Midwest Pediatric Surgery Consortium

AU - Midwest Pediatric Surgery Consortium

AU - Kunisaki, Shaun

AU - Saito, Jacqueline M.

AU - Fallat, Mary E.

AU - St. Peter, Shawn D.

AU - Kim, Aimee G.

AU - Johnson, Kevin N.

AU - Mon, Rodrigo A.

AU - Adams, Cheryl

AU - Aladegbami, Bola

AU - Bence, Christina

AU - Burns, R. Cartland

AU - Corkum, Kristine S.

AU - Deans, Katherine J.

AU - Downard, Cynthia D.

AU - Fraser, Jason D.

AU - Gadepalli, Samir K.

AU - Helmrath, Michael A.

AU - Kabre, Rashmi

AU - Lal, Dave R.

AU - Landman, Matthew P.

AU - Leys, Charles M.

AU - Linden, Allison F.

AU - Lopez, Joseph J.

AU - Mak, Grace Z.

AU - Minneci, Peter C.

AU - Rademacher, Brooks L.

AU - Shaaban, Aimen

AU - Walker, Sarah K.

AU - Wright, Tiffany N.

AU - Hirschl, Ronald B.

PY - 2019/6/1

Y1 - 2019/6/1

N2 - 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.

AB - 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.

KW - Congenital lobar emphysema

KW - Congenital lung malformations

KW - Congenital pulmonary airway malformation

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