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Development of a multi-institutional registry for children with operative congenital lung malformations. J Pediatr Surg 2020 Jul;55(7):1313-1318

Date

03/19/2019

Pubmed ID

30879756

DOI

10.1016/j.jpedsurg.2019.01.058

Scopus ID

2-s2.0-85062980988 (requires institutional sign-in at Scopus site)   27 Citations

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.

Author List

Kunisaki SM, Saito JM, Fallat ME, St Peter SD, Lal DR, Johnson KN, Mon RA, Adams C, Aladegbami B, Bence C, Burns RC, Corkum KS, Deans KJ, Downard CD, Fraser JD, Gadepalli SK, Helmrath MA, Kabre R, Landman MP, Leys CM, Linden AF, Lopez JJ, Mak GZ, Minneci PC, Rademacher BL, Shaaban A, Walker SK, Wright TN, Hirschl RB, Midwest Pediatric Surgery Consortium

Authors

Christina Bence MD Assistant Professor in the Surgery department at Medical College of Wisconsin
Dave Lal MD, MPH Chief, Professor in the Surgery department at Medical College of Wisconsin




MESH terms used to index this publication - Major topics in bold

Humans
Infant
Infant, Newborn
Lung
Prenatal Diagnosis
Registries
Respiratory System Abnormalities
Retrospective Studies