Shaun M Kunisaki1, Jacqueline M Saito2, Mary E Fallat3, Shawn D St Peter4, Dave R Lal5, Kevin N Johnson6, Rodrigo A Mon6, Cheryl Adams7, Bola Aladegbami2, Christina Bence5, R Cartland Burns8, Kristine S Corkum9, Katherine J Deans10, Cynthia D Downard3, Jason D Fraser4, Samir K Gadepalli6, Michael A Helmrath7, Rashmi Kabre9, Matthew P Landman8, Charles M Leys11, Allison F Linden12, Joseph J Lopez10, Grace Z Mak12, Peter C Minneci10, Brooks L Rademacher11, Aimen Shaaban7, Sarah K Walker5, Tiffany N Wright3, Ronald B Hirschl6. 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. Division of Pediatric Surgery, Department of Surgery, Medical College of Wisconsin, Milwaukee, WI, USA. 6. 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. 7. Division of Pediatric Surgery, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, 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
INTRODUCTION: The purpose of this study was to develop a multi-institutional registry to characterize the demographics, management, and outcomes of a contemporary cohort of children undergoing congenital lung malformation (CLM) resection. METHODS: After central reliance IRB approval, a web-based, secure database was created to capture retrospective cohort data on pathologically-confirmed CLMs performed between 2009 and 2015 within a multi-institutional research collaborative. RESULTS: Eleven children's hospitals contributed 506 patients. Among 344 prenatally diagnosed lesions, the congenital pulmonary airway malformation volume ratio was measured in 49.1%, and fetal MRI was performed in 34.3%. One hundred thirty-four (26.7%) children had respiratory symptoms at birth. Fifty-eight (11.6%) underwent neonatal resection, 322 (64.1%) had surgery at 1-12 months, and 122 (24.3%) had operations after 12 months. The median age at resection was 6.7 months (interquartile range, 3.6-11.4). Among 230 elective lobectomies performed in asymptomatic patients, thoracoscopy was successfully utilized in 102 (44.3%), but there was substantial variation across centers. The most common lesions were congenital pulmonary airway malformation (n = 234, 47.3%) and intralobar bronchopulmonary sequestration (n = 106, 21.4%). CONCLUSION: This multicenter cohort study on operative CLMs highlights marked disease heterogeneity and substantial practice variation in preoperative evaluation and operative management. Future registry studies are planned to help establish evidence-based guidelines to optimize the care of these patients. LEVEL OF EVIDENCE: Level II.
INTRODUCTION: The purpose of this study was to develop a multi-institutional registry to characterize the demographics, management, and outcomes of a contemporary cohort of children undergoing congenital lung malformation (CLM) resection. METHODS: After central reliance IRB approval, a web-based, secure database was created to capture retrospective cohort data on pathologically-confirmed CLMs performed between 2009 and 2015 within a multi-institutional research collaborative. RESULTS: Eleven children's hospitals contributed 506 patients. Among 344 prenatally diagnosed lesions, the congenital pulmonary airway malformation volume ratio was measured in 49.1%, and fetal MRI was performed in 34.3%. One hundred thirty-four (26.7%) children had respiratory symptoms at birth. Fifty-eight (11.6%) underwent neonatal resection, 322 (64.1%) had surgery at 1-12 months, and 122 (24.3%) had operations after 12 months. The median age at resection was 6.7 months (interquartile range, 3.6-11.4). Among 230 elective lobectomies performed in asymptomatic patients, thoracoscopy was successfully utilized in 102 (44.3%), but there was substantial variation across centers. The most common lesions were congenital pulmonary airway malformation (n = 234, 47.3%) and intralobar bronchopulmonary sequestration (n = 106, 21.4%). CONCLUSION: This multicenter cohort study on operative CLMs highlights marked disease heterogeneity and substantial practice variation in preoperative evaluation and operative management. Future registry studies are planned to help establish evidence-based guidelines to optimize the care of these patients. LEVEL OF EVIDENCE: Level II.
Authors: Abigail J Engwall-Gill; Sherwin S Chan; Kevin P Boyd; Jacqueline M Saito; Mary E Fallat; Shawn D St Peter; Stephanie Bolger-Theut; Eric J Crotty; Jared R Green; Rebecca L Hulett Bowling; Sachin S Kumbhar; Mantosh S Rattan; Cody M Young; Joseph K Canner; Katherine J Deans; Samir K Gadepalli; Michael A Helmrath; Ronald B Hirschl; Rashmi Kabre; Dave R Lal; Matthew P Landman; Charles M Leys; Grace Z Mak; Peter C Minneci; Tiffany N Wright; Shaun M Kunisaki Journal: JAMA Netw Open Date: 2022-06-01