Abstract
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. 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). 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). 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 iii.>
