Development and validation of a risk stratification index to predict death in gastroschisis

Background: Gastroschisis is a rare congenital anomaly, the improved surgical management of which has contributed to a survival rate greater than 90%. Development of an accurate risk stratification system to help identify the subset of patients at greatest risk for death may lead to further improvements in outcome. Methods: Infants with gastroschisis were identified from 16 years of the National Inpatient Sample database and the Kids' Inpatient Database using the International Classification of Diseases, Ninth Revision, Clinical Modification procedure code 54.71 (repair of gastroschisis) and an age of less than 8 days. Logistic regression analysis determined which coexisting diagnoses were significantly associated with death. Odds ratios from the logistic regression model were simplified and used as weighting factors to create an additive index. The index was validated using the 2003 Kids' Inpatient Database data set. Results: Intestinal atresia, necrotizing enterocolitis, rare cardiac anomalies, and lung hypoplasia were strongly associated with death and used to create a scoring system with a potential range of 0 to 10. Every point increase on the scale of gastroschisis risk stratification index is associated with a 95% relative increase in the likelihood of death. Conclusion: We have developed a novel index, which is superior to previous classification systems in identifying patients with gastroschisis who are at highest risk for death.