P102: Abnormal umbilical, portal and hepatic venos system-its correlation to associated malformation, congestive heart failure, liver dysfunction and fetal outcome

Abstract

To present our experience in the prenatal diagnosis and surveillance of abnormalities of the fetal central veins. An observational study of 12 fetuses with an abnormal umbilical, potal and umbilical venous system being diagnosed at two centers in Bonn and Trondheim. the abnormality was related to associated malformations, development of congestive heart failure or fetal hydrops, abnormal echogenity of the liver parenchyma and outcome of pregnancy. In group 1, five fetuses had an umbilical vein which went directly into the right atrium, by-passing the liver. No ductus venosus, no portal veins and abnormal hepatic veins were detected. Echogenic foci in the liver were present in absence of portal veins. One fetus with Turner Syndrome dies in utero. The other fetuses had no associated malformations, but congestive heart failure secondary to volume overload and fetal hydrops in two cases. The outcome was uneventful after a short period of disturbed adaption. In group 2 of five fetuses, the umbilical vein went below the liver into the inferior Vena cava or via the iliac vein into the distal IVC. A ductus venosus was present in all but one fetus. Portal veins were absent in all and hepatic veins were abnormal developed in four fetuses. Associated malformations were seen in two cases. The other had a transient cardiomegaly and a favourable outcome. In group 3 two siblings had a normal umbilical vein, but an abnormal located ductus venosus. There was hepatomegaly and polyhydramnios in one and hepatomegaly and increasing ascites in the other. Autopsy showed a congenital cholangiopathy after neonatal death and abruption of pregnancy. Malformation of the umbilical, portal and hepatic venous system are rare, but can be associated with malformations or disturbed cardiac or hepatic function requiring intra-uterine or post partal therapy.