The absence of extrahepatic portal bifurcation is a rare anomaly and is considered to be caused by anomalous development of the portal vessels early in fetal life. The portal system develops from two main fetal vessels, the left umbilical vein and right vitelline vein. An anomalous connection of these two vessels early in fetal life results in the absence of bifurcation of the portal vein. We report a case who suffered from biliary atresia complicated with the absence of bifurcation of the portal vein. The patient had an anomalous left hepatic portal vein that ran along the cranial side of the hepatic hilar ductal plate. This is the first report of biliary atresia that was complicated with the absence of extrahepatic portal bifurcation.
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Accepted: December 8, 2012
Received in revised form: November 20, 2012
Received: August 28, 2012
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