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Original article
One-year outcomes of infants born with congenital diaphragmatic hernia: a national population cohort study
  1. Anna-May Long1,2,
  2. Kathryn J Bunch2,
  3. Marian Knight2,
  4. Jennifer J Kurinczuk2,
  5. Paul Damian Losty3
  6. on behalf of BAPS-CASS
    1. 1 Department of Paediatric Surgery, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK
    2. 2 National Perinatal Epidemiology Unit, University of Oxford, Oxford, UK
    3. 3 Institute of Child Health, University of Liverpool, Liverpool, UK
    1. Correspondence to Professor Paul Damian Losty, Institute of Child Health, University of Liverppol, Liverpool L69 3BX, UK; paul.losty{at}liverpool.ac.uk

    Abstract

    Objective To report outcomes to 1 year, in infants born with congenital diaphragmatic hernia (CDH), explore factors associated with infant mortality and examine the relationship between surgical techniques and postoperative morbidity.

    Design Prospective national population cohort study.

    Setting Paediatric surgical centres in the UK and Ireland.

    Method Data were collected to 1 year for infants with CDH live-born between 1 April 2009 to 30 September 2010. Factors associated with infant mortality are explored using logistic regression. Postoperative morbidity following patch versus primary closure, minimally invasive versus open surgery and biological versus synthetic patch material is described. Data are presented as n (%) and median (IQR).

    Results Overall known survival to 1 year was 75%, 95% CI 68% to 81% (138/184) and postoperative survival 93%, 95% CI 88% to 97% (138/148). Female sex, antenatal diagnosis, use of vasodilators or inotropes, being small for gestational age, patch repair and use of surfactant were all associated with infant death. Infants undergoing patch repair had a high incidence of postoperative chylothorax (11/54 vs 2/96 in infants undergoing primary closure) and a long length of hospital stay (41 days, IQR 24–68 vs 16 days, IQR 10–25 in primary closure group). Infants managed with synthetic patch material had a high incidence of chylothorax (11/34 vs 0/19 with biological patch).

    Conclusion The majority of infant deaths in babies born with CDH occur before surgical correction. Female sex, being born small for gestational age, surfactant use, patch repair and receipt of cardiovascular support were associated with a higher risk of death. The optimum surgical approach, timing of operation and choice of patch material to achieve lowest morbidity warrants further evaluation.

    • congenital diaphragmatic hernia
    • mortality
    • outcomes research
    • prognosis
    • congenital abnorm

    https://doi.org/10.1136/archdischild-2018-316396

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      Footnotes

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

      • Funding This study was funded by Action Medical Research (SP4407, GN1739).

      • Competing interests None declared.

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

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

      • Collaborators The British Association of Paediatric Surgeons Congenital Anomaly Surveillance System (BAPS-CASS) Collaborators: Marven S, Draper E, Johnson P, Brocklehurst P, Howe D, Rankin J, Aslam A, Jawaid W, Wilkinson D, Robb A, Lander A, Burki T, Coates L, Cusick E, Fishman J, Madden N, Adikibi B, Mackinlay G, Sabharwal A, Larcombe C, Curry J, Besarovic S, Ashour K, Johnson P, Carnaghan H, Davenport M, Sugarman I, Nour S, Tsang T, Paul A, Davies B, Mclaughlin D, Puri P, Abel R, Driver C, Mahomed A, Ervine E, Mccallion W, Phelps S, Craigie R, Morabito A, De La Hunt M, Hosie G, Lloyd K, Taylor R, Drewett M, Okoye B, Richards C, King J, Nellihala LP, de La Hunt M, Huddart S.

      • Patient consent for publication Not required.

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