title: Standardized reporting for congenital diaphragmatic hernia--an international consensus.
abstract: OBJECTIVE
  Congenital diaphragmatic hernia (CDH) remains a significant cause of neonatal death. A wide spectrum of disease severity and treatment strategies makes comparisons challenging. The objective of this study was to create a standardized reporting system for CDH.
  
  
  METHODS
  Data were prospectively collected on all live born infants with CDH from 51 centers in 9 countries. Patients who underwent surgical correction had the diaphragmatic defect size graded (A-D) using a standardized system. Other data known to affect outcome were combined to create a usable staging system. The primary outcome was death or hospital discharge.
  
  
  RESULTS
  A total of 1,975 infants were evaluated. A total of 326 infants were not repaired, and all died. Of the remaining 1,649, the defect was scored in 1,638 patients. A small defect (A) had a high survival, while a large defect was much worse. Cardiac defects significantly worsened outcome. We grouped patients into 6 categories based on defect size with an isolated A defect as stage I. A major cardiac anomaly (+) placed the patient in the next higher stage. Applying this, patient survival is 99% for stage I, 96% stage II, 78% stage III, 58% stage IV, 39% stage V, and 0% for non-repair.
  
  
  CONCLUSIONS
  The size of the diaphragmatic defect and a severe cardiac anomaly are strongly associated with outcome. Standardizing reporting is imperative in determining optimal outcomes and effective therapies for CDH and could serve as a benchmark for prospective trials.
authors: UT Health Medical School and Children's Memorial Hermann Hospital, Houston, TX, USA. Electronic address: kevin.p.lally@uth.tmc.edu.
authors: Kevin P
authors: KP
authors: Lally
authors: Robert E
authors: RE
authors: Lasky
authors: Pamela A
authors: PA
authors: Lally
authors: Pietro
authors: P
authors: Bagolan
authors: Carl F
authors: CF
authors: Davis
authors: Bjorn P
authors: BP
authors: Frenckner
authors: Ronald M
authors: RM
authors: Hirschl
authors: Max R
authors: MR
authors: Langham
authors: Terry L
authors: TL
authors: Buchmiller
authors: Noriaki
authors: N
authors: Usui
authors: Dick
authors: D
authors: Tibboel
authors: Jay M
authors: JM
authors: Wilson
mesh_terms: D005260:Female; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D000015:Abnormalities, Multiple / Q000175:diagnosis; D003661:Decision Support Techniques; D006548:Hernia, Diaphragmatic / Q000175:diagnosis / Q000401:mortality / Q000601:surgery; D065630:Hernias, Diaphragmatic, Congenital; D059685:Herniorrhaphy; D016015:Logistic Models; D011446:Prospective Studies; D012042:Registries / Q000592:standards; D018570:Risk Assessment; D012372:ROC Curve; D012720:Severity of Illness Index; D015996:Survival Rate; D016896:Treatment Outcome
keywords: Apgar score; Congenital diaphragmatic hernia (CDH); Risk stratification; Staging system