Omphalocele: national current birth prevalence and survival
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In brief
In brief
Swedish national registry study (1997-2016) reports omphalocele birth prevalence of 1/10,000 live births with 59% termination rate and 62% associated malformations. Among continued pregnancies, first-year survival is 87%, with ventricular septal defect being the most common associated anomaly.
Written by the GCMD Library team from the article.
Abstract
Purpose
The increase in prenatal diagnosis together with the high rates of associated anomalies in omphalocele has led to increased rates of termination of pregnancies. The aim of this study was to examine the national Swedish birth prevalence and survival rates among these patients.
Methods
This study is based on a nationwide population-based cohort of all children born in Sweden between 1/1/1997 and 31/12/2016. All omphalocele cases were identified though the Swedish National Patient Register and the Swedish Medical Birth Register. Outcome of malformations and deaths were retrieved from the Swedish Birth Defects Register and the Swedish Causes of Death Register.
Results
The study included 207 cases of omphalocele (42% females). The birth prevalence for omphalocele was 1/10,000 live births. About 62% of the cases had associated malformations and/or genetic disorders; most common was ventricular septal defect. The mortality within the first year was 13%. The rate of termination of pregnancy was 59%.
Conclusion
The national birth prevalence for omphalocele in Sweden is 1/10,000 newborn, with high termination rates. Over half of the pregnancies with prenatally diagnosed omphalocele will be terminated. Among those who continue the pregnancy, 1-year survival rates are high.
Type of study
National register study
Level of evidence
III.