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Congenital Diaphragmatic Hernia Associated with Esophageal Atresia: Incidence, Outcomes, and Determinants of Mortality.
MedLine Citation:
PMID:  23177372     Owner:  NLM     Status:  Publisher    
Abstract/OtherAbstract:
BACKGROUND: Congenital diaphragmatic hernia (CDH) and esophageal atresia (EA) are fairly common congenital anomalies, but the occurrence of both is exceedingly rare, with only anecdotal cases reported in the English literature. The primary objective of the current study is to assess the incidence and outcomes of CDH/EA using the Congenital Diaphragmatic Hernia Study Group registry. STUDY DESIGN: The Congenital Diaphragmatic Hernia Study Group registry was queried from January 2000 through August 2011. Patients with CDH/EA were identified. The primary end point was survival and the secondary end point was seeking to identify variables that might explain variances in outcomes. RESULTS: During the study period, 4,888 patients were reported to the Congenital Diaphragmatic Hernia Study Group registry. Twenty-three (0.5%) had an associated EA, with an overall survival significantly lower than the registry mean (26.1% vs 70.3%; p < 0.001). These patients had significantly lower birth weights (1.8 vs 2.94 kg), more cardiac abnormalities, and more chromosomal abnormalities (p < 0.001). Only 6 of the 23 CDH/EA patients had a CDH repair. In addition, the cohort had significantly fewer primary CDH repairs (4.3% vs 39.3%; p < 0.001). Logistic regression analysis identified birth weight, cardiac abnormalities, repair, and surfactant administration to be significantly associated with adverse outcomes. Esophageal atresia was the only variable not independently associated with survival. CONCLUSIONS: In patients with both CDH and EA, survival is significantly lower than other patients with CDH, but not uniformly lethal, therefore, intent to treat is a viable approach. Although the association of CDH with EA has a substantial impact on survival, it is the presence of low birth weight, cardiac and chromosomal abnormalities, surfactant use, and larger defects-and not the mere presence of EA-that influences the outcomes of this unique cohort.
Authors:
Offir Ben-Ishay; Victor M Johnson; Jay M Wilson; Terry L Buchmiller
Publication Detail:
Type:  JOURNAL ARTICLE     Date:  2012-11-20
Journal Detail:
Title:  Journal of the American College of Surgeons     Volume:  -     ISSN:  1879-1190     ISO Abbreviation:  J. Am. Coll. Surg.     Publication Date:  2012 Nov 
Date Detail:
Created Date:  2012-11-26     Completed Date:  -     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  9431305     Medline TA:  J Am Coll Surg     Country:  -    
Other Details:
Languages:  ENG     Pagination:  -     Citation Subset:  -    
Copyright Information:
Copyright © 2013 American College of Surgeons. Published by Elsevier Inc. All rights reserved.
Affiliation:
Department of Surgery, Children's Hospital Boston and Harvard Medical School, Boston, MA. Electronic address: O_ben-Ishay@Rambam.Health.gov.il.
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