title: Treatment evolution in high-risk congenital diaphragmatic hernia: ten years' experience with diaphragmatic agenesis.
abstract: OBJECTIVE
  The objective of this study was to evaluate the impact of newer therapies on the highest risk patients with congenital diaphragmatic hernia (CDH), those with agenesis of the diaphragm.
  
  
  BACKGROUND
  CDH remains a significant cause of neonatal mortality. Many novel therapeutic interventions have been used in these infants. Those children with large defects or agenesis of the diaphragm have the highest mortality and morbidity.
  
  
  METHODS
  Twenty centers from 5 countries collected data prospectively on all liveborn infants with CDH over a 10-year period. The treatment and outcomes in these patients were examined. Patients were followed until death or hospital discharge.
  
  
  RESULTS
  A total of 1,569 patients with CDH were seen between January 1995 and December 2004 in 20 centers. A total of 218 patients (14%) had diaphragmatic agenesis and underwent repair. The overall survival for all patients was 68%, while survival was 54% in patients with agenesis. When patients with diaphragmatic agenesis from the first 2 years were compared with similar patients from the last 2 years, there was significantly less use of ECMO (75% vs. 52%) and an increased use of inhaled nitric oxide (iNO) (30% vs. 80%). There was a trend toward improved survival in patients with agenesis from 47% in the first 2 years to 59% in the last 2 years. The survivors with diaphragmatic agenesis had prolonged hospital stays compared with patients without agenesis (median, 68 vs. 30 days). For the last 2 years of the study, 36% of the patients with agenesis were discharged on tube feedings and 22% on oxygen therapy.
  
  
  CONCLUSIONS
  There has been a change in the management of infants with CDH with less frequent use of ECMO and a greater use of iNO in high-risk patients with a potential improvement in survival. However, the mortality, hospital length of stay, and morbidity in agenesis patients remain significant.
authors.affiliation: Department of Surgery, University of Texas Medical School Houston and Memorial Hermann Children's Hospital, Houston, TX, USA. kevin.p.lally@uth.tmc.edu
authors.forename: Kevin P
authors.initials: KP
authors.lastname: Lally
authors.forename: Pamela A
authors.initials: PA
authors.lastname: Lally
authors.forename: Krisa P
authors.initials: KP
authors.lastname: Van Meurs
authors.forename: Desmond J
authors.initials: DJ
authors.lastname: Bohn
authors.forename: Carl F
authors.initials: CF
authors.lastname: Davis
authors.forename: Bradley
authors.initials: B
authors.lastname: Rodgers
authors.forename: Jatinder
authors.initials: J
authors.lastname: Bhatia
authors.forename: Golde
authors.initials: G
authors.lastname: Dudell
mesh_terms: D000013:Congenital Abnormalities / Q000628:therapy; D003964:Diaphragm / Q000002:abnormalities*; D005260:Female; D006548:Hernia, Diaphragmatic / Q000628:therapy*; D065630:Hernias, Diaphragmatic, Congenital; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D011446:Prospective Studies; D012307:Risk Factors; D013997:Time Factors