title: Congenital diaphragmatic hernia: defect size correlates with developmental defect.
abstract: OBJECTIVE
  The aim of our study was to elucidate if the defect size reflects the magnitude of the developmental defect in patients with CDH.
  
  
  METHODS
  All patients recorded in the CDH Study Group registry between January 1, 2007, and December 31, 2010, and with defect classification were included in the study. They were divided according to defect size (A-D) and compared for: gestational age (GA), birth weight (BW), number of abnormal organ systems, prevalence of associated anomalies, cardiovascular malformations (CVM), chromosomal anomalies, liver in the chest, and hernia sac.
  
  
  RESULTS
  A total of 1350 of 1778 patients had defect classification: 173 A, 557 B, 438 C, and 182 D. Mortality rate was 0.6%, 5.3%, 22.6%, and 45.6% in group A, B, C, and D, respectively, (p<0.0001; p for trend <0.0001). GA, BW, prevalence of associated anomalies, particularly CVM, number of abnormal organ systems, and prevalence of sac were significantly different between the groups, with a significant reduction of GA, BW, and prevalence of sac. There was an increase in prevalence of associated anomalies, liver in the chest, and number of abnormal systems as the defect size increased.
  
  
  CONCLUSIONS
  Defect size is directly correlated with mortality rate, prevalence of other anomalies (particularly CVM), and number of abnormal systems, and inversely with GA, BW, and prevalence of hernia sac. The defect size may be a marker for the magnitude of developmental abnormality, thereby explaining its relationship with the outcome.
authors.affiliation: Bambino Gesù Children's Research Hospital, Rome, Italy.
authors.forename: Francesco
authors.initials: F
authors.lastname: Morini
authors.forename: Laura
authors.initials: L
authors.lastname: Valfrè
authors.forename: Irma
authors.initials: I
authors.lastname: Capolupo
authors.forename: Kevin P
authors.initials: KP
authors.lastname: Lally
authors.forename: Pamela A
authors.initials: PA
authors.lastname: Lally
authors.forename: Pietro
authors.initials: P
authors.lastname: Bagolan
mesh_terms: D000015:Abnormalities, Multiple / Q000175:diagnosis* / Q000453:epidemiology / Q000401:mortality; D006548:Hernia, Diaphragmatic / Q000145:classification / Q000401:mortality / Q000473:pathology; D065630:Hernias, Diaphragmatic, Congenital; D006801:Humans; D007231:Infant, Newborn; D015995:Prevalence; D011379:Prognosis; D012042:Registries; D012720:Severity of Illness Index
keywords: Congenital diaphragmatic hernia; Defect size; Developmental defect; Embryology; Prognosis