Development of a multi-institutional registry for children with operative congenital lung malformations

Midwest Pediatric Surgery Consortium

Research output: Contribution to journalArticle

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.

Original languageEnglish (US)
JournalJournal of pediatric surgery
DOIs
StatePublished - Jan 1 2019
Externally publishedYes

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Registries
Lung
Bronchopulmonary Sequestration
Thoracoscopy
Research Ethics Committees
Multicenter Studies
Patient Care
Cohort Studies
Demography
Parturition
Databases
Guidelines
Research

Keywords

  • Bronchopulmonary sequestration
  • Congenital cystic adenomatoid malformation
  • Congenital lung malformations
  • Congenital pulmonary airway malformation
  • Registry

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health

Cite this

Development of a multi-institutional registry for children with operative congenital lung malformations. / Midwest Pediatric Surgery Consortium.

In: Journal of pediatric surgery, 01.01.2019.

Research output: Contribution to journalArticle

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title = "Development of a multi-institutional registry for children with operative congenital lung malformations",
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.",
keywords = "Bronchopulmonary sequestration, Congenital cystic adenomatoid malformation, Congenital lung malformations, Congenital pulmonary airway malformation, Registry",
author = "{Midwest Pediatric Surgery Consortium} and Shaun Kunisaki and Saito, {Jacqueline M.} and Fallat, {Mary E.} and {St. Peter}, {Shawn D.} and Lal, {Dave R.} and Johnson, {Kevin N.} and Mon, {Rodrigo A.} and Cheryl Adams and Bola Aladegbami and Christina Bence and Burns, {R. Cartland} and Corkum, {Kristine S.} and Deans, {Katherine J.} and Downard, {Cynthia D.} and Fraser, {Jason D.} and Gadepalli, {Samir K.} and Helmrath, {Michael A.} and Rashmi Kabre and Landman, {Matthew P.} and Leys, {Charles M.} and Linden, {Allison F.} and Lopez, {Joseph J.} and Mak, {Grace Z.} and Minneci, {Peter C.} and Rademacher, {Brooks L.} and Aimen Shaaban and Walker, {Sarah K.} and Wright, {Tiffany N.} and Hirschl, {Ronald B.}",
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language = "English (US)",
journal = "Journal of Pediatric Surgery",
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T1 - Development of a multi-institutional registry for children with operative congenital lung malformations

AU - Midwest Pediatric Surgery Consortium

AU - Kunisaki, Shaun

AU - Saito, Jacqueline M.

AU - Fallat, Mary E.

AU - St. Peter, Shawn D.

AU - Lal, Dave R.

AU - Johnson, Kevin N.

AU - Mon, Rodrigo A.

AU - Adams, Cheryl

AU - Aladegbami, Bola

AU - Bence, Christina

AU - Burns, R. Cartland

AU - Corkum, Kristine S.

AU - Deans, Katherine J.

AU - Downard, Cynthia D.

AU - Fraser, Jason D.

AU - Gadepalli, Samir K.

AU - Helmrath, Michael A.

AU - Kabre, Rashmi

AU - Landman, Matthew P.

AU - Leys, Charles M.

AU - Linden, Allison F.

AU - Lopez, Joseph J.

AU - Mak, Grace Z.

AU - Minneci, Peter C.

AU - Rademacher, Brooks L.

AU - Shaaban, Aimen

AU - Walker, Sarah K.

AU - Wright, Tiffany N.

AU - Hirschl, Ronald B.

PY - 2019/1/1

Y1 - 2019/1/1

N2 - 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.

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KW - Congenital cystic adenomatoid malformation

KW - Congenital lung malformations

KW - Congenital pulmonary airway malformation

KW - Registry

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