Michael Helmrath · Development of a multi-institutional registry for children with operative congenital lung malformations
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Article1 min read·Published Feb 2019Older

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

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Article · Feb 2019 · 1 min read

In brief

In brief

Multi-institutional registry study of 506 children undergoing congenital lung malformation resection across 11 hospitals reveals significant practice variation in prenatal evaluation, surgical timing, and thoracoscopic approach. Most patients underwent elective lobectomy between 1-12 months of age, with CPAM and bronchopulmonary sequestration being the most common pathologies.

Written by the GCMD Library team from the article.

Michael Helmrath

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 manage

Statements in this article

  1. Among 344 prenatally diagnosed congenital lung malformations, the congenital pulmonary airway malformation volume ratio was measured in 49.1% of cases.

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  2. Fetal MRI was performed in 34.3% of prenatally diagnosed congenital lung malformations.

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  3. 26.7% of children with congenital lung malformations had respiratory symptoms at birth.

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  4. 11.6% of children with congenital lung malformations underwent neonatal resection.

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  5. 64.1% of children with congenital lung malformations had surgery at 1-12 months of age.

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  6. 24.3% of children with congenital lung malformations had operations after 12 months of age.

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  7. The median age at resection for congenital lung malformations was 6.7 months with an interquartile range of 3.6-11.4 months.

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  8. Among 230 elective lobectomies performed in asymptomatic patients with congenital lung malformations, thoracoscopy was successfully utilized in 44.3%.

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  9. There was substantial variation across centers in the use of thoracoscopy for elective lobectomy in asymptomatic patients with congenital lung malformations.

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  10. Congenital pulmonary airway malformation was the most common lesion type, accounting for 47.3% of operative congenital lung malformations.

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  11. Intralobar bronchopulmonary sequestration accounted for 21.4% of operative congenital lung malformations.

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  12. There is marked disease heterogeneity in operative congenital lung malformations.

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  13. There is substantial practice variation in preoperative evaluation of congenital lung malformations.

    ResearchConclusion
  14. There is substantial practice variation in operative management of congenital lung malformations.

    ResearchConclusion

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