Document Detail

Standardized reporting for congenital diaphragmatic hernia - An international consensus.
MedLine Citation:
PMID:  24314179     Owner:  NLM     Status:  In-Data-Review    
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.
Kevin P Lally; Robert E Lasky; Pamela A Lally; Pietro Bagolan; Carl F Davis; Bjorn P Frenckner; Ronald M Hirschl; Max R Langham; Terry L Buchmiller; Noriaki Usui; Dick Tibboel; Jay M Wilson;
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Publication Detail:
Type:  Journal Article    
Journal Detail:
Title:  Journal of pediatric surgery     Volume:  48     ISSN:  1531-5037     ISO Abbreviation:  J. Pediatr. Surg.     Publication Date:  2013 Dec 
Date Detail:
Created Date:  2013-12-09     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  0052631     Medline TA:  J Pediatr Surg     Country:  United States    
Other Details:
Languages:  eng     Pagination:  2408-15     Citation Subset:  IM    
Copyright Information:
© 2013.
UT Health Medical School and Children's Memorial Hermann Hospital, Houston, TX, USA. Electronic address:
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