Elsevier

Journal of Pediatric Surgery

Volume 48, Issue 12, December 2013, Pages 2408-2415
Journal of Pediatric Surgery

PAPS Papers
Standardized reporting for congenital diaphragmatic hernia – An international consensus

https://doi.org/10.1016/j.jpedsurg.2013.08.014Get rights and content

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.

Section snippets

Methods

The congenital diaphragmatic hernia study group (CDHSG) was founded in 1995 to prospectively collect data on infants with CDH [17]. This is a voluntary registry collecting data on all live born infants with CDH from a participating institution. Data are collected until death or discharge/transfer from the hospital. The data collected include demographic information as well as treatment information and outcomes. Periodic evaluations of data collection forms have resulted in successive revisions

Results

A total of 2,296 patients were entered into the registry during the time period of this study. These patients were cared for in 53 participating centers. Patients treated at 2 centers were excluded as greater than 40% of their patients did not have a coded defect, leaving 51 participating centers from 9 countries. Additionally excluded were 41 who had either bilateral or Morgagni type defects, and 2 patients who were transferred from the participating center before repair. Overall survival for

Discussion

Congenital diaphragmatic hernia remains a challenging problem. Mortality remains high and while survival has improved in the past decade, morbidity in the survivors is substantial [19], [20], [21], [22]. Because the anomaly occurs infrequently, very few centers treat large numbers of patients. In the past 20 years, there has been a rapid expansion in the number of therapies available for these infants. These include fetal intervention, ECMO, iNO, surfactant, HFOV and numerous pulmonary

Conclusion

In conclusion, patients with CDH can be effectively stratified into stages based on readily available clinical information. Given the wide variation in therapies for CDH and the broad range of patients seen in different institutions, we believe it is important that all clinical reports of patients with CDH be reported by stage and include the patients who do not receive surgical therapy. It is clear that using current therapy, mortality is low for infants with stage I and II lesions. Our group

References (30)

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1

The Members of the Writing Group.

2

Members of the Congenital Diaphragmatic Hernia Study Group are listed in the Appendix.

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