A Clinical Prediction Rule for the Severity of Congenital Diaphragmatic Hernias in Newborns

ME Brindle, EF Cook, Dick Tibboel, PA Lally, KP Lally

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103 Citations (Scopus)

Abstract

BACKGROUND: Congenital diaphragmatic hernia (CDH) is a condition with a highly variable outcome. Some infants have a relatively mild disease process, whereas others have significant pulmonary hypoplasia and hypertension. Identifying high-risk infants postnatally may allow for targeted therapy. METHODS: Data were obtained on 2202 infants from the Congenital Diaphragmatic Hernia Study Group database from January 2007 to October 2011. Using binary baseline predictors generated from birth weight, 5-minute Apgar score, congenital heart anomalies, and chromosome anomalies, as well as echocardiographic evidence of pulmonary hypertension, a clinical prediction rule was developed on a randomly selected subset of the data by using a backward selection algorithm. An integer-based clinical prediction rule was created. The performance of the model was validated by using the remaining data in terms of calibration and discrimination. RESULTS: The final model included the following predictors: very low birth weight, absent or low 5-minute Apgar score, presence of chromosomal or major cardiac anomaly, and suprasystemic pulmonary hypertension. This model discriminated between a population at high risk of death (similar to 50%) intermediate risk (similar to 20%), or low risk (<10%). The model performed well, with a C statistic of 0.806 in the derivation set and 0.769 in the validation set and good calibration (Hosmer-Lemeshow test, P = .2). CONCLUSIONS: A simple, generalizable scoring system was developed for CDH that can be calculated rapidly at the bedside. Using this model, intermediate-and high-risk infants could be selected for transfer to high-volume centers while infants at highest risk could be considered for advanced medical therapies.
Original languageUndefined/Unknown
Pages (from-to)E413-E419
JournalPediatrics
Volume134
Issue number2
DOIs
Publication statusPublished - 2014

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