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

J Pediatr Surg. 2020 Jul;55(7):1313-1318. doi: 10.1016/j.jpedsurg.2019.01.058. Epub 2019 Feb 28.


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.

Publication types

  • Multicenter Study

MeSH terms

  • Humans
  • Infant
  • Infant, Newborn
  • Lung* / abnormalities
  • Lung* / surgery
  • Prenatal Diagnosis
  • Registries*
  • Respiratory System Abnormalities* / diagnosis
  • Respiratory System Abnormalities* / epidemiology
  • Respiratory System Abnormalities* / surgery
  • Retrospective Studies