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


Section of Pediatric Surgery, Department of Surgery, University of Michigan and Michigan Medicine, C.S. Mott Children's and Von Voigtlander Women's Hospital, Ann Arbor, MI, USA. Electronic address: [Email]


OBJECTIVE : 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.


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

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