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

Standardized reporting for congenital diaphragmatic hernia--an international consensus.

Author information

1
UT Health Medical School and Children's Memorial Hermann Hospital, Houston, TX, USA. Electronic address: kevin.p.lally@uth.tmc.edu.

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.

KEYWORDS:

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

PMID:
24314179
DOI:
10.1016/j.jpedsurg.2013.08.014
[Indexed for MEDLINE]

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