TY - JOUR
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 M.
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.
N1 - Funding Information:
This project was supported by intramural funding from each institution within the Midwest Pediatric Surgery Consortium ( www.mwpsc.org ). No competing financial interests exist, and all authors have nothing to disclose.
Publisher Copyright:
© 2019 Elsevier Inc.
PY - 2019/6
Y1 - 2019/6
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
UR - http://www.scopus.com/inward/record.url?scp=85063000629&partnerID=8YFLogxK
U2 - 10.1016/j.jpedsurg.2019.02.043
DO - 10.1016/j.jpedsurg.2019.02.043
M3 - Article
C2 - 30898401
AN - SCOPUS:85063000629
VL - 54
SP - 1138
EP - 1142
JO - Journal of Pediatric Surgery
JF - Journal of Pediatric Surgery
SN - 0022-3468
IS - 6
ER -