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Birth prevalence of exomphalos, associated anomalies and outcomes in England & Wales: 1997–2007
  1. CA Smith1,
  2. M Bythell2,
  3. C Rounding3,
  4. ES Draper4
  1. 1Jessop Wing Neonatal Unit, Sheffield, UK
  2. 2Regional Maternity Survey Office, Newcastle, UK
  3. 3National Perinatal Epidemiology Unit, University of Oxford, Oxford, UK
  4. 4University of Leicester, Leicester, UK

Abstract

Background The majority of cases of exomphalos are diagnosed during the antenatal period. Up-to-date information about associated anomalies and outcomes are required to facilitate counselling and management.

Aim To examine the trend in the birth prevalence of exomphalos in England & Wales (E&W) from 1997 to 2007. To describe associated anomalies, outcomes and survival to 1 year.

Method Data for all cases with exomphalos notified to eight Congenital Anomaly Registers across E&W, between 1997 and 2007, was analysed by year of birth.

Results 1191 cases of exomphalos were reported over the 11-year period. During this time the birth prevalence of exomphalos showed a significant increasing trend from 2.9 (95% CI 2.5 to 3.3) to 4.3 (3.9 to 4.7) per 10 000 births (X2 test for trend, p<0.0001).

564 (47.4%) cases were electively terminated. 417 (35%) of all infants, were live born and 307 (25.8%) survived to 1 year of age. Of the 341 infants with isolated exomphalos, 197 (57.8%) were live born and 149 (43.7%) were reported to be alive at 1 year of age.

Chromosomal anomalies were reported in 354 (29.7%) cases, two thirds of these cases were trisomy 18. The other most commonly reported associated anomalies were musculo-skeletal (31.9%), cardiac (20.7%) and neurological (13.4%).

Conclusion There was a significant increase in the birth prevalence of exomphalos in E&W from 1997 to 2007. Approximately half of all exomphalos cases were terminated and a further quarter survived to 1 year of age. However, survival in isolated exomphalos cases was significantly higher than in cases with associated anomalies (43.7% vs 18.6%).

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