C1 Protocol OOPUS V1.3 8-10-2013
Omphalocele Outcome Prediction by Ultrasound Screening and omphalocele
Omphalocele is an anterior abdominal wall defect in which abdominal organs protrude into the umbilical cord. A membrane consisting of peritoneum, mesenchymal connective tissue and amnion covers the area of the defect. The umbilical cord inserts into the top of this hernia. The defect may vary greatly in size. Omphalocele has a prevalence of 1 in 6000 births. It may be part of approximately 70 syndromes, but also occurs in an isolated form. In cases with omphalocele, associated structural anomalies and an abnormal karyotype are found in 10-90% and 10-40% of fetuses, respectively. Around 80% of cases of fetal omphalocele is detected antenatally at
The size of the omphalocele is related to the clinical outcome and possibilities for closure. A large defect may have more consequences for a small fetus than for a larger fetus in terms of respiratory insufficiency or type of surgery, and postnatal management may differ accordingly. The omphalocele circumference/abdominal circumference (OC/AC) ratio, a measure for the relative size of the omphalocele, has been shown to be associated with neonatal outcome, and thus could be of value for antenatal counseling and management. However, it has never been quantified if the OC/AC ratio is related to the type of closure and the probability of neonatal pulmonary hypertension.
Observational multicenter prospective cohort study.
Women pregnant with a fetus with omphalocele.
Primary outcome measure
- type of surgical closure (primary vs. secondary)
- persistent neonatal pulmonary hypertension
Secondary outcome measures
- Composite outcome of perinatal mortality and neonatal morbidity.
Dr. E. Pajkrt, Gynaecologist Department of Gynaecology and Obstetrics, AMC Amsterdam
Academic Medical Centre (AMC) Meibergdreef 9 1105 AZ Amsterdam, the Netherlands
Drs. L. Ruiter, PhD student, Academic Medical Centre, Amsterdam
L. Ruiter@amc.uva.nl / +316 49320999
Case report form
E1E2 PIF/IC OOPUS V1.3 08-10-2013