Abstract

BackgroundPrevious studies on the mortality rate of omphalocele are limited. The risk of death of non-isolated omphalocele and that of cases of omphalocele that are diagnosed prenatally by ultrasound are unclear. This study aimed to estimate the perinatal mortality of pregnancies with omphalocele. This study also examined the potential risk of death of non-isolated omphalocele and that of cases that are prenatally diagnosed by ultrasound.MethodsData were retrieved from the national birth defects registry in China, for 1996–2006. Multinomial logistic regression was used to calculate the adjusted odds ratios (AORs) and 95% confidence intervals (CIs) between perinatal mortality and selected maternal and fetal characteristics.ResultsAmong 827 cases of omphalocele, 309 (37.4%) cases resulted in termination of pregnancy and stillbirth, and 124 (15.0%) cases resulted in death in the first 7 days after delivery, yielding a perinatal mortality rate of 52.4% (95% CI: 49.0–55.8%). The late fetal death rate (LFDR) of omphalocele that was diagnosed prenatally by ultrasound was 15.91-fold (AOR: 15.91, 95% CI: 10.18–24.87) higher than that of postnatally diagnosed cases. The LFDR of non-isolated omphalocele was 2.64-fold (AOR: 2.64, 95% CI: 1.62–4.29) higher than that of isolated cases. For the early neonatal death rate, neonates with non-isolated omphalocele had a 2.96-fold (AOR: 2.96, 95% CI: 1.82–4.81) higher risk than isolated cases, but the difference between prenatal ultrasound diagnosis and postnatal diagnosis was not significant.ConclusionsSelected fetal characteristics are significantly associated with the perinatal risk of death from omphalocele. Our findings suggest that improving pregnancy and delivery care, as well as management for omphalocele are important.

Highlights

  • Previous studies on the mortality rate of omphalocele are limited

  • The perinatal mortality rate, late fetal death rate (LFDR), and early neonatal death rate (ENNDR) of omphalocele was 52.4%, 37.4%, and 15.0%, respectively

  • We found that the perinatal mortality of pregnancies with omphalocele was 52.4%, late fetal mortality was 37.4%, and early neonatal mortality was 15.0%

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Summary

Introduction

Previous studies on the mortality rate of omphalocele are limited. The risk of death of non-isolated omphalocele and that of cases of omphalocele that are diagnosed prenatally by ultrasound are unclear. This study examined the potential risk of death of non-isolated omphalocele and that of cases that are prenatally diagnosed by ultrasound. More recent studies have estimated the neonatal mortality of omphalocele using prenatal and neonatal databases from certain hospitals These databases were confined to one hospital with a small number of cases, and the reported estimates with a wide range did not truly present the risk of death from omphalocele in the general population [11,12,13,14]. Previous studies did not independently investigate the risk of death from omphalocele with associated malformations using prenatal diagnosis by ultrasound because of potential confounding effects. The magnitude of effect estimates for the risk of death from non-isolated omphalocele and prenatally diagnosed cases by ultrasound has been imprecisely assessed

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