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Birth Prevalence and Survival of Exomphalos in England and Wales: 2005 to 2011

Lookup NU author(s): Professor Judith Rankin


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Background: Exomphalos occurs in 2.2 per 10,000 births with 76% of these babies surviving to discharge. The aim of this study was to determine the birth prevalence and survival of babies with this anomaly in England and Wales. Methods: Six BINOCAR regional congenital anomaly registers in England and Wales (covering 36% of births) between 2005 and 2011 provided cases for this study. Cases included live births, stillbirths (241 weeks' gestation), late miscarriages (20-23 weeks' gestation), and terminations of pregnancy with fetal anomaly. Results: The overall birth prevalence was 3.8 (95% confidence interval [CI]: 3.6-4.0) per 10,000 births; 1.4 (1.2-1.6) for isolated cases, 1.2 (1.1-1.4) for cases with multiple anomalies, and 1.2 (1.1-1.4) for cases with chromosomal anomalies. The live birth prevalence was 0.8 (0.7-0.9), 0.5 (0.4-0.6), and 0.1 (0.0-0.1) per 10,000 live births, respectively. Edwards syndrome, congenital heart defects, and nervous system anomalies were the most common anomalies associated with exomphalos. A prenatal diagnosis was made in 83% of isolated, 95% of multiple, and 99% of chromosomal cases. Fifty-five percent of isolated and multiple cases were live born, whereas 85% of cases with chromosomal anomalies resulted in a termination of pregnancy with fetal anomaly. The 1-year survival of live born babies with an isolated exomphalos was 92% compared with 81% in cases with multiple anomalies and 27% in cases with chromosomal anomalies (p<0.001). Conclusion: We report a higher birth prevalence than has previously been reported. The proportion of infants surviving with exomphalos remained unchanged over the time period. (C) 2014 Wiley Periodicals, Inc.

Publication metadata

Author(s): Springett A, Draper ES, Rankin J, Rounding C, Tucker D, Stoianova S, Wellesley D, Morris JK

Publication type: Article

Publication status: Published

Journal: Birth Defects Research. Part A: Clinical and Molecular Teratology

Year: 2014

Volume: 100

Issue: 9

Pages: 721-725

Print publication date: 01/09/2014

Online publication date: 01/09/2014

Acceptance date: 29/07/2014

ISSN (print): 1542-0752

ISSN (electronic): 1542-0760

Publisher: John Wiley & Sons, Inc.


DOI: 10.1002/bdra.23301


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