Early population-based outcomes of infants born with congenital diaphragmatic hernia. Issue 6 (4th January 2018)

• Record Type: Journal Article
• Authors: Long, Anna-May; Bunch, Kathryn J; Knight, Marian; Kurinczuk, Jennifer J; Losty, Paul D
• Other Names: author non-byline.; Marven Sean author non-byline.; Draper Elizabeth author non-byline.; Johnson Paul author non-byline.; Brocklehurst Peter author non-byline.; Howe David author non-byline.; Rankin Judith author non-byline.; Aslam Adil author non-byline.; Jawaid Wajid author non-byline.; Wilkinson David author non-byline.; Robb Andrew author non-byline.; Lander Anthony author non-byline.; Burki Tariq author non-byline.; Coates Laura author non-byline.; Cusick Eleri author non-byline.; Fishman Julia author non-byline.; Madden Nicholas author non-byline.; Adikibi Boma author non-byline.; Mackinlay Gordon author non-byline.; Sabharwal Atul author non-byline.; Larcombe Celia author non-byline.; Curry Joe author non-byline.; Besarovic Sanja author non-byline.; Ashour Khaled author non-byline.; Johnson Paul author non-byline.; Carnaghan Helen author non-byline.; Davenport Mark author non-byline.; Sugarman Ian author non-byline.; Nour Shawqui author non-byline.; Tsang Thomas author non-byline.; Paul Anu author non-byline.; Davies Brian author non-byline.; Mclaughlin Danielle author non-byline.; Puri Prem author non-byline.; Abel Robin author non-byline.; Driver Christopher author non-byline.; Mahomed Aneis author non-byline.; Ervine Evelyn author non-byline.; Mccallion William author non-byline.; Phelps Simon author non-byline.; Craigie Ross author non-byline.; Morabito Antonino author non-byline.; La hunt Michael De author non-byline.; Hosie Gareth author non-byline.; Lloyd Karen author non-byline.; Taylor Rhoda author non-byline.; Drewett Melanie author non-byline.; Okoye Bruce author non-byline.; Richards Catherine author non-byline.; King Juliette author non-byline.; Huddart Simon author non-byline.;
• Is Part Of: Archives of disease in childhood. Volume 103:Issue 6(2018)
• Page Start: F517
• Page End: F522
• Publication Date: 2018-01-04
• Subjects: neonatology -- outcomes research -- paediatric surgery -- congenital diaphragmatic hernia -- baps-cass; Infants -- Diseases -- Periodicals; Newborn infants -- Diseases -- Periodicals; Fetus -- Diseases -- Periodicals; 618.920105
• DOI: 10.1136/archdischild-2017-313933
• Languages: English
• ISSNs: 1359-2998

Abstract

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.