title: Bilateral congenital diaphragmatic hernia: prognostic evaluation of a large international cohort.
abstract: BACKGROUND
  Congenital diaphragmatic hernia (CDH) is a lethal birth defect, which occurs in 1:2000-3000 live births. Bilateral CDH is a rare form (1%), with a high mortality. This study presents the outcomes of the largest cohort of bilateral CDH patients.
  
  
  METHODS
  The records of patients with bilateral CDH from the Congenital Diaphragmatic Hernia Registry born between 1995 and 2015 were retrospectively analyzed to identify parameters associated with mortality.
  
  
  RESULTS
  Eighty patients with a bilateral CDH were identified. Overall mortality was 74% (n=59). Apgar scores at 1 and 5min were statistically lower in the non-survivors compared to the survivors (median 3.0 and 5.0, versus 6.5 and 8.0, respectively, p<0.001). All survivors were repaired (n=21), compared to 22% of the non-survivors (n=17). The type of repair was equally divided in the survivors (52% primary versus 48% patch), while non-survivors were mainly patch repaired (82% versus 12%). Nineteen were treated with extracorporeal membrane oxygenation (ECMO) (24%), only three of them survived. When calculating the risk on mortality for the patients who lived until repair, ECMO had an adjusted odds ratio for mortality of 10.8 (95% CI: 2.0-57.7) and patch repair 5.2 (95% CI: 0.8-34.9).
  
  
  CONCLUSIONS
  The treatment of bilateral CDH patients remains challenging with a high mortality rate. Lower Apgar-scores, ECMO (probably as a surrogate for the severity of disease), and patch repair were negatively associated with outcome.
  
  
  METHODS
  Level IV study.
authors.affiliation: Department of Pediatric Surgery, Radboudumc-Amalia Children's Hospital, Nijmegen, The Netherlands. Electronic address: Sanne.Botden@radboudumc.nl.
authors.forename: Sanne Mbi
authors.initials: SM
authors.lastname: Botden
authors.affiliation: Department of Pediatric Surgery, Radboudumc-Amalia Children's Hospital, Nijmegen, The Netherlands.
authors.forename: Kim
authors.initials: K
authors.lastname: Heiwegen
authors.affiliation: Radboud Institute for Health Sciences, Department for Health Evidence, Radboudumc, Nijmegen, The Netherlands.
authors.forename: Iris Alm
authors.initials: IA
authors.lastname: van Rooij
authors.affiliation: Department of Pediatric Surgery, Radboudumc-Amalia Children's Hospital, Nijmegen, The Netherlands.
authors.forename: Horst
authors.initials: H
authors.lastname: Scharbatke
authors.affiliation: McGovern Medical School at The University of Texas Health Science Center at Houston and Children's Memorial Hermann Hospital.
authors.forename: Pamela A
authors.initials: PA
authors.lastname: Lally
authors.affiliation: Department of Neonatology, Radboudumc-Amalia Children's Hospital, Nijmegen, The Netherlands.
authors.forename: Arno
authors.initials: A
authors.lastname: van Heijst
authors.affiliation: Department of Pediatric Surgery, Radboudumc-Amalia Children's Hospital, Nijmegen, The Netherlands.
authors.forename: Ivo
authors.initials: I
authors.lastname: de Blaauw
mesh_terms: D000015:Abnormalities, Multiple / Q000401:mortality* / Q000601:surgery; D013505:Digestive System Surgical Procedures / Q000706:statistics & numerical data*; D015199:Extracorporeal Membrane Oxygenation / Q000706:statistics & numerical data; D005260:Female; D065630:Hernias, Diaphragmatic, Congenital / Q000401:mortality* / Q000601:surgery; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D011379:Prognosis; D012042:Registries; D012189:Retrospective Studies; D015996:Survival Rate; D017741:Survivors
keywords: Bilateral; Congenital diaphragmatic hernia; Congenital malformation; Extracorporeal membrane oxygenation