Standardized reporting for congenital diaphragmatic hernia - An international consensus

Kevin P. Lally, Robert E. Lasky, Pamela A. Lally, Pietro Bagolan, Carl F. Davis, Bjorn P. Frenckner, Ronald M. Hirschl, Max R. Langham, Terry L. Buchmiller, Noriaki Usui, Dick Tibboel, Jay M. Wilson

Research output: Contribution to journalArticlepeer-review

Abstract

Background/purpose: Congenital diaphragmatic hernia (CDH) remains a significant cause of neonatal death. A wide spectrum of disease severity and treatment strategies makes comparisons challenging. The objective of this study was to create a standardized reporting system for CDH. Methods: Data were prospectively collected on all live born infants with CDH from 51 centers in 9 countries. Patients who underwent surgical correction had the diaphragmatic defect size graded (A-D) using a standardized system. Other data known to affect outcome were combined to create a usable staging system. The primary outcome was death or hospital discharge. Results: A total of 1,975 infants were evaluated. A total of 326 infants were not repaired, and all died. Of the remaining 1,649, the defect was scored in 1,638 patients. A small defect (A) had a high survival, while a large defect was much worse. Cardiac defects significantly worsened outcome. We grouped patients into 6 categories based on defect size with an isolated A defect as stage I. A major cardiac anomaly (+) placed the patient in the next higher stage. Applying this, patient survival is 99% for stage I, 96% stage II, 78% stage III, 58% stage IV, 39% stage V, and 0% for non-repair. Conclusions: The size of the diaphragmatic defect and a severe cardiac anomaly are strongly associated with outcome. Standardizing reporting is imperative in determining optimal outcomes and effective therapies for CDH and could serve as a benchmark for prospective trials.

Original languageEnglish
Pages (from-to)2408-2415
Number of pages8
JournalJournal of Pediatric Surgery
Volume48
Issue number12
DOIs
Publication statusPublished - Dec 2013

Keywords

  • Apgar score
  • Congenital diaphragmatic hernia (CDH)
  • Risk stratification
  • Staging system

ASJC Scopus subject areas

  • Surgery
  • Pediatrics, Perinatology, and Child Health

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