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Standardized reporting for congenital diaphragmatic hernia – An international consensus.

Authors :
Lally, Kevin P.
Lasky, Robert E.
Lally, Pamela A
Bagolan, Pietro
Davis, Carl F.
Frenckner, Bjorn P.
Hirschl, Ronald M.
Langham, Max R.
Buchmiller, Terry L.
Usui, Noriaki
Tibboel, Dick
Wilson, Jay M.
Source :
Journal of Pediatric Surgery; Dec2013, Vol. 48 Issue 12, p2408-2415, 8p
Publication Year :
2013

Abstract

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. [Copyright &y& Elsevier]

Details

Language :
English
ISSN :
00223468
Volume :
48
Issue :
12
Database :
Supplemental Index
Journal :
Journal of Pediatric Surgery
Publication Type :
Academic Journal
Accession number :
92682418
Full Text :
https://doi.org/10.1016/j.jpedsurg.2013.08.014