Standardized reporting for congenital diaphragmatic hernia--an international consensus

J Pediatr Surg. 2013 Dec;48(12):2408-15. doi: 10.1016/j.jpedsurg.2013.08.014.


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.

Keywords: Apgar score; Congenital diaphragmatic hernia (CDH); Risk stratification; Staging system.

Publication types

  • Consensus Development Conference
  • Multicenter Study
  • Observational Study
  • Practice Guideline

MeSH terms

  • Abnormalities, Multiple / diagnosis
  • Decision Support Techniques*
  • Female
  • Hernia, Diaphragmatic / diagnosis
  • Hernia, Diaphragmatic / mortality
  • Hernia, Diaphragmatic / surgery
  • Hernias, Diaphragmatic, Congenital*
  • Herniorrhaphy
  • Humans
  • Infant, Newborn
  • Logistic Models
  • Male
  • Prospective Studies
  • ROC Curve
  • Registries / standards*
  • Risk Assessment
  • Severity of Illness Index*
  • Survival Rate
  • Treatment Outcome