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Early population-based outcomes of infants born with congenital diaphragmatic hernia

Anna-May Long1,2, Kathryn J Bunch1, Marian Knight1

  • 1National Perinatal Epidemiology Unit, University of Oxford, Oxford, UK.

Archives of Disease in Childhood. Fetal and Neonatal Edition
|January 7, 2018
PubMed

Insights

Congenital diaphragmatic hernia (CDH) affects 16% of infants before surgery, with female sex and prenatal diagnosis being poor prognostic factors. Postoperative survival for surgically treated infants with CDH is excellent.

Area of Science:

  • Pediatric Surgery
  • Neonatology
  • Public Health

Background:

  • Congenital diaphragmatic hernia (CDH) is a serious birth defect.
  • Understanding short-term outcomes and prognostic factors is crucial for improving infant survival.
  • Population-based data are essential for assessing care variations and outcomes.

Purpose of the Study:

  • To describe short-term outcomes for live-born infants with congenital diaphragmatic hernia (CDH).
  • To identify prognostic factors associated with early mortality in infants with CDH.
  • To report on management practices and outcomes across UK and Ireland paediatric surgical centres.

Main Methods:

  • Prospective population cohort study from April 2009 to September 2010.
  • Data collected from all 28 UK and Ireland paediatric surgical centres.
  • Analysis of prognostic factors for death before surgery.

Main Results:

  • 219 infants with CDH were identified; 16% died before surgery.
  • Factors associated with pre-operative mortality included female sex, prenatal diagnosis, and need for inotropes or pulmonary vasodilators.
  • Significant practice variations and use of potentially detrimental therapies were observed.
  • Postoperative 30-day survival was 98% for stabilized infants undergoing surgery.

Conclusions:

  • This is the first British Isles population-based study on CDH outcomes.
  • Female sex and prenatal diagnosis are associated with poor outcomes in infants with CDH.
  • Excellent early postoperative survival is achievable for infants undergoing surgical repair.
Abstract

Keywords:
baps-casscongenital diaphragmatic hernianeonatologyoutcomes researchpaediatric surgery

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