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.
- Congenital lobar emphysema
- Congenital lung malformations
- Congenital pulmonary airway malformation
ASJC Scopus subject areas
- Pediatrics, Perinatology, and Child Health