title: Actual outcome in infants with congenital diaphragmatic hernia: the role of a standardized postnatal treatment protocol.
abstract: BACKGROUND
  Congenital diaphragmatic hernia (CDH) is a severe congenital anomaly with a high rate of mortality and morbidity.
  
  
  OBJECTIVE
  Our aim was to determine a possible effect of standardized treatment on outcome in infants with CDH.
  
  
  METHODS
  All prenatally diagnosed patients with unilateral CDH born alive between January 2006 and December 2009 at the Erasmus MC or the University Hospital Mannheim were eligible for inclusion. Patients who underwent a fetal tracheal occlusion were excluded. From November 1, 2007, all CDH patients were treated according to a standardized treatment protocol. Patients were divided into two chronological groups according to their date of birth: without standardized treatment (group 1, Jan 2006-Oct 2007) and with standardized treatment (group 2, Nov 2007-Dec 2009). Outcome measures were mortality by day 28, bronchopulmonary dysplasia (BPD), defined as oxygen dependency at day 28, and need for extracorporeal membrane oxygenation (ECMO) therapy. Uni- and multivariate analyses were performed.
  
  
  RESULTS
  167 patients were included. By day 28, 18% of the infants had died. Of the patients who were alive at day 28, 49% had BPD. An ECMO procedure was performed in 31% of the patients. Overall mortality for all included patients was 22%. In group 1, overall mortality was 33% and in group 2 overall mortality was 12% (p = 0.004). A standardized treatment protocol was independently associated with a reduced risk for mortality by day 28 (OR 0.28, 95% CI 0.11-0.68). Higher observed-to-expected lung-to-head ratios were independently associated with a lower risk for mortality by day 28 (OR 0.97, 95% CI 0.95-0.99), BPD (OR 0.97, 95% CI 0.94-0.98) and need for ECMO (OR 0.98, 95% CI 0.96-0.99). An intrathoracic position of the liver was independently associated with an increased risk for BPD (OR 3.12, 95% CI 1.41-6.90) and need for ECMO therapy (OR 3.25, 95% CI 1.54-6.88).
  
  
  CONCLUSIONS
  Survival rates in patients with CDH increased significantly after the implementation of a standardized treatment protocol.
authors.affiliation: Department of Pediatric Surgery, Erasmus MC-Sophia, Rotterdam, The Netherlands.
authors.forename: L
authors.initials: L
authors.lastname: van den Hout
authors.forename: T
authors.initials: T
authors.lastname: Schaible
authors.forename: T E
authors.initials: TE
authors.lastname: Cohen-Overbeek
authors.forename: W
authors.initials: W
authors.lastname: Hop
authors.forename: J
authors.initials: J
authors.lastname: Siemer
authors.forename: K
authors.initials: K
authors.lastname: van de Ven
authors.forename: L
authors.initials: L
authors.lastname: Wessel
authors.forename: D
authors.initials: D
authors.lastname: Tibboel
authors.forename: I
authors.initials: I
authors.lastname: Reiss
mesh_terms: D001997:Bronchopulmonary Dysplasia / Q000150:complications / Q000401:mortality; D002985:Clinical Protocols; D015199:Extracorporeal Membrane Oxygenation; D006548:Hernia, Diaphragmatic / Q000150:complications / Q000401:mortality / Q000628:therapy; D065630:Hernias, Diaphragmatic, Congenital; D006801:Humans; D007223:Infant; D007231:Infant, Newborn; D012189:Retrospective Studies; D016896:Treatment Outcome