Medical College of Wisconsin
CTSICores SearchResearch InformaticsREDCap

Current operative management of congenital lobar emphysema in children: A report from the Midwest Pediatric Surgery Consortium. J Pediatr Surg 2019 Jun;54(6):1138-1142

Date

03/23/2019

Pubmed ID

30898401

DOI

10.1016/j.jpedsurg.2019.02.043

Scopus ID

2-s2.0-85063000629 (requires institutional sign-in at Scopus site)   30 Citations

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.

Author List

Kunisaki SM, Saito JM, Fallat ME, St Peter SD, Kim AG, Johnson KN, Mon RA, Adams C, Aladegbami B, Bence C, Burns RC, Corkum KS, Deans KJ, Downard CD, Fraser JD, Gadepalli SK, Helmrath MA, Kabre R, Lal DR, Landman MP, Leys CM, Linden AF, Lopez JJ, Mak GZ, Minneci PC, Rademacher BL, Shaaban A, Walker SK, Wright TN, Hirschl RB, Midwest Pediatric Surgery Consortium

Authors

Christina Bence MD Assistant Professor in the Surgery department at Medical College of Wisconsin
Dave Lal MD, MPH Chief, Professor in the Surgery department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Child
Child, Preschool
Dyspnea
Humans
Infant
Midwestern United States
Pulmonary Emphysema
Respiratory System Abnormalities
Retrospective Studies
Thoracoscopy