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One-year outcomes of infants born with congenital diaphragmatic hernia: a national population cohort study.

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.

Authors+Show Affiliations

Department of Paediatric Surgery, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK. National Perinatal Epidemiology Unit, University of Oxford, Oxford, UK.National Perinatal Epidemiology Unit, University of Oxford, Oxford, UK.National Perinatal Epidemiology Unit, University of Oxford, Oxford, UK.National Perinatal Epidemiology Unit, University of Oxford, Oxford, UK.Institute of Child Health, University of Liverpool, Liverpool, UK.No affiliation info available

Pub Type(s)

Journal Article

Language

eng

PubMed ID

31154421

Citation

Long, Anna-May, et al. "One-year Outcomes of Infants Born With Congenital Diaphragmatic Hernia: a National Population Cohort Study." Archives of Disease in Childhood. Fetal and Neonatal Edition, 2019.
Long AM, Bunch KJ, Knight M, et al. One-year outcomes of infants born with congenital diaphragmatic hernia: a national population cohort study. Arch Dis Child Fetal Neonatal Ed. 2019.
Long, A. M., Bunch, K. J., Knight, M., Kurinczuk, J. J., & Losty, P. D. (2019). One-year outcomes of infants born with congenital diaphragmatic hernia: a national population cohort study. Archives of Disease in Childhood. Fetal and Neonatal Edition, doi:10.1136/archdischild-2018-316396.
Long AM, et al. One-year Outcomes of Infants Born With Congenital Diaphragmatic Hernia: a National Population Cohort Study. Arch Dis Child Fetal Neonatal Ed. 2019 Jun 1; PubMed PMID: 31154421.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - One-year outcomes of infants born with congenital diaphragmatic hernia: a national population cohort study. AU - Long,Anna-May, AU - Bunch,Kathryn J, AU - Knight,Marian, AU - Kurinczuk,Jennifer J, AU - Losty,Paul Damian, AU - ,, Y1 - 2019/06/01/ PY - 2018/10/15/received PY - 2019/02/28/revised PY - 2019/03/02/accepted PY - 2019/6/3/entrez KW - congenital abnorm KW - congenital diaphragmatic hernia KW - mortality KW - outcomes research KW - prognosis JF - Archives of disease in childhood. Fetal and neonatal edition JO - Arch. Dis. Child. Fetal Neonatal Ed. N2 - 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. SN - 1468-2052 UR - https://www.unboundmedicine.com/medline/citation/31154421/One-year_outcomes_of_infants_born_with_congenital_diaphragmatic_hernia:_a_national_population_cohort_study L2 - http://fn.bmj.com/cgi/pmidlookup?view=long&pmid=31154421 DB - PRIME DP - Unbound Medicine ER -