Development of a multi-institutional registry for children with operative congenital lung malformations.
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.
Journal of pediatric surgery
Lung Diseases/congenital; Registries; Infant, Newborn; Pulmonary Surgical Procedures
Bronchopulmonary sequestration; Congenital cystic adenomatoid malformation; Congenital lung malformations; Congenital pulmonary airway malformation; Registry
Kunisaki, Shaun M.; Saito, Jacqueline M.; Fallat, Mary E.; St Peter, Shawn D.; Lal, Dave R.; Johnson, Kevin N.; Mon, Rodrigo A.; Adams, Cheryl; Aladegbami, Bola; Bence, Christina; Burns, R Cartland; Corkum, Kristine S.; Deans, Katherine J.; Downard, Cynthia D.; Fraser, Jason D.; Gadepalli, Samir K.; Helmrath, Michael A.; Kabre, Rashmi; Landman, Matthew P.; Leys, Charles M.; Linden, Allison F.; Lopez, Joseph J.; Mak, Grace Z.; Minneci, Peter C.; Rademacher, Brooks L.; Shaaban, Aimen; Walker, Sarah K.; Wright, Tiffany N.; Hirschl, Ronald B.; and Midwest Pediatric Surgery Consortium, "Development of a multi-institutional registry for children with operative congenital lung malformations." (2019). Manuscripts, Articles, Book Chapters and Other Papers. 880.