title: Characteristics and outcome and the omphalocele circumference/abdominal circumference ratio in prenatally diagnosed fetal omphalocele.
abstract: OBJECTIVE
  To evaluate the outcome of fetuses with prenatally diagnosed omphalocele and to investigate the predictive value of the omphalocele circumference/abdominal circumference (OC/AC) ratio - a measure for the relative size of the omphalocele.
  
  
  METHODS
  This study includes all fetuses prenatally diagnosed with omphalocele at our centre between 1995 and 2007. Medical records and footage of ultrasound examinations were reviewed. Omphalocele was classified in four groups: isolated, chromosomal, syndromic, and multiple anomalies.
  
  
  RESULTS
  Eighty-eight cases were identified: 21 (24%) were isolated and 67 had additional structural anomalies. Of the 44 fetuses (50%) with chromosomal anomalies, 2 had omphalocele as a solitary finding. Fifty-three pregnancies (60%) were terminated because of the size of the lesion or associated structural or chromosomal anomalies. Twenty-one cases resulted in a live birth, of which 17 were vaginal deliveries (81%, all uncomplicated) including 3 cases of giant omphalocele (≥5 cm). The OC/AC ratio was found predictive for herniation of the liver, respiratory insufficiency and type of surgical reconstruction. Currently, 12/88 fetuses (14%) are alive and well, including 2 infants with multiple anomalies.
  
  
  CONCLUSIONS
  Identification of omphalocele should arouse suspicion of genetic abnormalities, even in cases that appear isolated. The OC/AC ratio may influence counselling regarding the postnatal course.
authors.affiliation: Department of Obstetrics and Gynaecology, Academic Medical Center, Amsterdam, The Netherlands. c.e.kleinrouweler@amc.uva.nl
authors.forename: C E
authors.initials: CE
authors.lastname: Kleinrouweler
authors.forename: C F
authors.initials: CF
authors.lastname: Kuijper
authors.forename: M M
authors.initials: MM
authors.lastname: van Zalen-Sprock
authors.forename: I B
authors.initials: IB
authors.lastname: Mathijssen
authors.forename: C M
authors.initials: CM
authors.lastname: Bilardo
authors.forename: E
authors.initials: E
authors.lastname: Pajkrt
mesh_terms: D000293:Adolescent; D000328:Adult; D036861:Delivery, Obstetric / Q000706:statistics & numerical data; D005260:Female; D005315:Fetal Diseases / Q000000981:diagnostic imaging / Q000453:epidemiology*; D006554:Hernia, Umbilical / Q000000981:diagnostic imaging / Q000453:epidemiology* / Q000601:surgery; D006801:Humans; D007231:Infant, Newborn; D008297:Male; D009426:Netherlands / Q000453:epidemiology; D012189:Retrospective Studies; D016216:Ultrasonography, Prenatal; D055105:Waist Circumference; D055815:Young Adult