Development of a multi-institutional registry for children with operative congenital lung malformations
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In brief
In brief
Multi-institutional registry of 506 children undergoing congenital lung malformation resection reveals significant practice variation in prenatal evaluation, surgical timing, and thoracoscopic approach across 11 centers. Most operations occurred at 1-12 months of age, with CPAM and bronchopulmonary sequestration being the most common pathologies.
Written by the GCMD Library team from the article.
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.