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.

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Keywords Apgar score, Congenital diaphragmatic hernia (CDH), Risk stratification, Staging system
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Journal Journal of Pediatric Surgery
Lally, K.P, Lasky, R.E, Lally, P.A, Bagolan, P, Davis, C.F, Frenckner, B.P, … Wilson, J.M. (2013). Standardized reporting for congenital diaphragmatic hernia - An international consensus. In Journal of Pediatric Surgery (Vol. 48, pp. 2408–2415). doi:10.1016/j.jpedsurg.2013.08.014