Abnormal umbilical-portal-hepatic venous system in two siblings: a sign for congenital cholangiodysplasia.

We report on two siblings who presented prenatally with a ductus venosus (DV) that was abnormally located between the middle hepatic vein (mHV) and the proximal inferior vena cava (IVC), and with hepatomegaly. The first case presented with polyhydramnios at 33 weeks and therapeutic amniocentesis resulted in premature delivery soon after admission. The neonate died 19 days later and autopsy revealed congenital cholangiodysplasia. The second fetus presented for routine screening at 19 weeks of gestation and was found to have similar abnormalities of the venous system, suggesting the same origin of disease. Termination of pregnancy was performed and autopsy revealed congenital cholangiodysplasia. Our observations suggest that an abnormal umbilical-portal-hepatic venous system may be associated with a congenital malformation of the liver.