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Abnormal umbilical‐portal‐hepatic venous system in two siblings: a sign for congenital cholangiodysplasia
Author(s) -
Hofstaetter C.,
Blaas H.G.,
Isaksen C.,
Bolz A.G.,
EikNes S. H.
Publication year - 2005
Publication title -
ultrasound in obstetrics and gynecology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.202
H-Index - 141
eISSN - 1469-0705
pISSN - 0960-7692
DOI - 10.1002/uog.1984
Subject(s) - medicine , ductus venosus , polyhydramnios , autopsy , umbilical vein , portal venous system , fetus , amniocentesis , inferior vena cava , pregnancy , gestation , portal vein , prenatal diagnosis , surgery , obstetrics , cirrhosis , portal hypertension , biochemistry , genetics , chemistry , in vitro , biology
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. Copyright © 2005 ISUOG. Published by John Wiley & Sons, Ltd.