Estimating disease severity of congenital diaphragmatic hernia in the first 5 minutes of life

Background/Purpose: Congenital diaphragmatic hernia (CDH), occurring approximately once in every 2,400 live births, remains a significant cause of perinatal death and morbidity. Risk assessment tools for congenital diaphragmatic hernia derived at single institutions fail to predict outcome at other institutions. Without a generally applicable risk assessment tool it is impossible to determine whether the current variation in outcomes is caused by differences in treatment or to variations in the types of patients treated. The authors report a broadly applicable risk assessment tool for newborns with CDH derived from multiinstitutional data. Methods: Survival data on 322 consecutive liveborn infants with CDH were collected using data from 71 institutions. Demographic and early treatment results were evaluated by univariate analysis. Items useful in an early stratification system were examined using a multivariate logistic regression analysis. The predictive equation developed was applied to the next series of evaluable patients. Results: A total of 1,054 patients with CDH were evaluated from 1995 to 1999 with an overall survival rate of 64%. For the first 322 patients, factors associated with outcome included birth weight, Apgar scores, gestational age, race, immediate distress, presence of a cardiac anomaly, and prenatal diagnosis. Multivariate analysis showed that birth weight and Ei-minute Apgar scores were most useful in a predictive equation. A logistic equation using these 2 variables could separate the next 673 patients into high, intermediate, and low risk of death, and this correlated closely with the actual outcome. Conclusion: Stratifying neonates with CDH into broad risk groups should allow better comparison of outcomes data from different centers, reserving novel and high-risk strategies for patients with a high likelihood of dying. J Pediatr Surg 36:141-146, Copyright (C) 2001 by W.B. Saunders Company.