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Early population-based outcomes of infants born with congenital diaphragmatic hernia
  1. Anna-May Long1,2,
  2. Kathryn J Bunch1,
  3. Marian Knight1,
  4. Jennifer J Kurinczuk1,
  5. Paul D Losty2,3
  6. On behalf of BAPS-CASS
    1. 1National Perinatal Epidemiology Unit, University of Oxford, Oxford, UK
    2. 2Academic Department of Paediatric Surgery, Alder Hey Children’s Hospital, Liverpool, UK
    3. 3Institute of Child Health, University of Liverpool, Liverpool, UK
    1. Correspondence to Professor Paul D Losty, Department of Women and Children’s Health, Institute of Translational Medicine, University of Liverpool, Alder Hey Children’s Hospital NHS Foundation Trust, Liverpool L14 5AB, UK; paul.losty{at}liverpool.ac.uk

    Abstract

    Purpose This study aims to describe short-term outcomes of live-born infants with congenital diaphragmatic hernia (CDH) and to identify prognostic factors associated with early mortality.

    Design A prospective population cohort study was undertaken between April 2009 and September 2010, collecting data on live-born infants with CDH from all 28 paediatric surgical centres in the UK and Ireland using an established surgical surveillance system. Management and outcomes are described. Prognostic factors associated with death before surgery are explored.

    Results Two hundred and nineteen live-born infants with CDH were reported within the data collection period. There were 1.5 times more boys than girls (n=133, 61%). Thirty-five infants (16%) died without an operation. This adverse outcome was associated with female sex (adjusted OR (aOR) 3.96, 95% CI 1.66 to 9.47), prenatal diagnosis (aOR 4.99, 95% CI 1.31 to 18.98), and the need for physiological support in the form of inotropes (aOR 9.96, 95% CI 1.19 to 83.25) or pulmonary vasodilators (aOR 4.09, 95% CI 1.53 to 10.93). Significant variation in practice existed among centres, and some therapies potentially detrimental to infant outcomes were used, including pulmonary surfactant in 45 antenatally diagnosed infants (34%). Utilisation of extracorporeal membrane oxygenation was very low compared with published international studies (n=9/219, 4%). Postoperative 30-day survival was 98% for 182 infants with CDH who were adequately physiologically stabilised and underwent surgery.

    Conclusion This is the first British Isles population-based study reporting outcome metrics for infants born with CDH. 16% of babies did not survive to undergo surgery. Factors associated with poor outcome included female sex and prenatal diagnosis. Early postoperative survival in those who underwent surgical repair was excellent.

    • neonatology
    • outcomes research
    • paediatric surgery
    • congenital diaphragmatic hernia
    • baps-cass

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    Footnotes

    • Contributors PDL, MK and JJK designed and coordinated the study, along with the BAPS-CASS collaborators. A-ML and KJB analysed the data, and A-ML wrote the manuscript, which was reviewed and edited by all other authors.

    • Funding This project was funded by Action Medical Research.

    • Competing interests None declared.

    • Patient consent Detail has been removed from this case description/these case descriptions to ensure anonymity. The editors and reviewers have seen the detailed information available and are satisfied that the information backs up the case the authors are making.

    • Ethics approval The study received ethics committee approval from the London Research Ethics Committee: Ref 09/H0718/10.

    • Provenance and peer review Not commissioned; externally peer reviewed.

    • Collaborators Sean Marven, Elizabeth Draper, Paul Johnson, Peter Brocklehurst, David Howe, Judith Rankin, Adil Aslam, Wajid Jawaid, David Wilkinson, Andrew Robb, Anthony Lander, Tariq Burki, Laura Coates, Eleri Cusick, Julia Fishman, Nicholas Madden, Boma Adikibi, Gordon MacKinlay, Atul Sabharwal, Celia Larcombe, Joe Curry, Sanja Besarovic, Khaled Ashour, Paul Johnson, Helen Carnaghan, Mark Davenport, Ian Sugarman, Shawqui Nour, Thomas Tsang, Anu Paul, Brian Davies, Danielle McLaughlin, Prem Puri, Robin Abel, Christopher Driver, Aneis Mahomed, Evelyn Ervine, William McCallion, Simon Phelps, Ross Craigie, Antonino Morabito, Michael De La Hunt, Gareth Hosie, Karen Lloyd, Rhoda Taylor, Melanie Drewett, Bruce Okoye, Catherine Richards, Juliette King and Simon Huddart.

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