Shaun M Kunisaki1, Jacqueline M Saito2, Mary E Fallat3, Shawn D St Peter4, Aimee G Kim5, Kevin N Johnson5, Rodrigo A Mon5, Cheryl Adams6, Bola Aladegbami2, Christina Bence7, R Cartland Burns8, Kristine S Corkum9, Katherine J Deans10, Cynthia D Downard3, Jason D Fraser4, Samir K Gadepalli5, Michael A Helmrath6, Rashmi Kabre9, Dave R Lal7, Matthew P Landman8, Charles M Leys11, Allison F Linden12, Joseph J Lopez10, Grace Z Mak12, Peter C Minneci10, Brooks L Rademacher11, Aimen Shaaban6, Sarah K Walker7, Tiffany N Wright3, Ronald B Hirschl5. 1. 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: skunisa1@jhmi.edu. 2. Division of Pediatric Surgery, Department of Surgery, Washington University School of Medicine, St. Louis, MO, USA. 3. Division of Pediatric Surgery, Hiram C. Polk, Jr., M.D. Department of Surgery, University of Louisville, Norton Children's Hospital, Louisville, KY, USA. 4. Division of Pediatric Surgery, Department of Surgery, Children's Mercy Hospital, Kansas City, MO, USA. 5. 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. 6. Division of Pediatric Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA. 7. Division of Pediatric Surgery, Department of Surgery, Medical College of Wisconsin, Milwaukee, WI, USA. 8. Division of Pediatric Surgery, Department of Surgery, Indiana University School of Medicine, Indianapolis, IN, USA. 9. Division of Pediatric Surgery, Department of Surgery, Ann & Robert H. Lurie Children's Hospital of Chicago, Northwestern University Feinberg School of Medicine, Chicago, IL, USA. 10. Center for Surgical Outcomes Research, The Research Institute and Department of Surgery, Nationwide Children's Hospital, Ohio State University College of Medicine, Columbus, OH, USA. 11. Division of Pediatric Surgery, Department of Surgery, University of Wisconsin, Madison, WI, USA. 12. Section of Pediatric Surgery, Department of Surgery, Comer Children's Hospital, University of Chicago Medicine and Biological Sciences, Chicago, IL, USA.
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.
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.