title: Birth prevalence and survival of exomphalos in england and wales: 2005 to 2011.
abstract: 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 (24+ 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).
  
  
  CONCLUSIONS
  We report a higher birth prevalence than has previously been reported. The proportion of infants surviving with exomphalos remained unchanged over the time period.
authors.affiliation: Centre for Environmental and Preventive Medicine, Queen Mary University of London, London.
authors.forename: Anna
authors.initials: A
authors.lastname: Springett
authors.forename: Elizabeth S
authors.initials: ES
authors.lastname: Draper
authors.forename: Judith
authors.initials: J
authors.lastname: Rankin
authors.forename: Catherine
authors.initials: C
authors.lastname: Rounding
authors.forename: David
authors.initials: D
authors.lastname: Tucker
authors.forename: Sylvia
authors.initials: S
authors.lastname: Stoianova
authors.forename: Diana
authors.initials: D
authors.lastname: Wellesley
authors.forename: Joan K
authors.initials: JK
authors.lastname: Morris
mesh_terms: D000025:Abortion, Eugenic / Q000706:statistics & numerical data; D000022:Abortion, Spontaneous / Q000453:epidemiology* / Q000401:mortality; D000293:Adolescent; D000328:Adult; D002869:Chromosome Aberrations; D002887:Chromosomes, Human, Pair 18 / Q000235:genetics; D004739:England / Q000453:epidemiology; D005260:Female; D006330:Heart Defects, Congenital / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology* / Q000401:mortality; D006554:Hernia, Umbilical / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology* / Q000401:mortality; D006801:Humans; D050498:Live Birth; D008297:Male; D008875:Middle Aged; D009421:Nervous System Malformations / Q000150:complications / Q000175:diagnosis / Q000453:epidemiology* / Q000401:mortality; D011247:Pregnancy; D011296:Prenatal Diagnosis; D015995:Prevalence; D012042:Registries; D050497:Stillbirth; D016019:Survival Analysis; D014314:Trisomy / Q000175:diagnosis* / Q000235:genetics; D000073842:Trisomy 18 Syndrome; D014852:Wales / Q000453:epidemiology
keywords: exomphalos; omphalocele; prenatal diagnosis; prevalence; survival