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Development of a multi-institutional registry for children with operative congenital lung malformations
Shaun M Kunisaki1, Jacqueline M Saito2, Mary E Fallat3
1Section of Pediatric Surgery, Department of Surgery, University of Michigan and Michigan Medicine, C.S. Mott Children's and Von Voigtlander Women's Hospital, Ann Arbor, MI, USA.
Insights
This study created a registry of pediatric congenital lung malformation (CLM) resections, revealing significant variations in care. Future research aims to develop evidence-based guidelines for optimal patient management.
Area of Science:
- Pediatric Surgery
- Thoracic Surgery
- Congenital Malformations
Background:
- Congenital lung malformations (CLMs) represent a spectrum of developmental abnormalities in the pediatric population.
- Characterizing contemporary management and outcomes is crucial for improving patient care.
Purpose of the Study:
- To establish a multi-institutional registry for children undergoing congenital lung malformation (CLM) resection.
- To delineate the demographics, management strategies, and clinical outcomes of a current cohort of pediatric CLM patients.
Main Methods:
- A retrospective cohort study utilizing a secure, web-based database.
- Data collected from 11 children's hospitals between 2009 and 2015.
- Inclusion of pathologically confirmed CLMs.
Main Results:
- 506 patients were included, with congenital pulmonary airway malformation (CPAM) and intralobar bronchopulmonary sequestration being the most common diagnoses.
- The median age at resection was 6.7 months, with significant variation in surgical timing and approach (e.g., thoracoscopy utilization).
- Preoperative evaluation methods like congenital pulmonary airway malformation volume ratio and fetal MRI showed inconsistent application.
Conclusions:
- This multicenter study highlights considerable heterogeneity in CLM presentation and significant practice variations in preoperative assessment and surgical management.
- Further registry studies are planned to inform the development of evidence-based guidelines for optimizing pediatric CLM care.
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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