The development of extrahepatic portal obstruction after undergoing multiple operations for a congenital dilatation of the bile duct: report of a case.

As a long-term complication after undergoing a cystenterostomy for a congenital dilatation of the bile duct, liver cirrhosis due to stenosis of the anastomosis or reflux cholangitis has been reported in conjunction with subsequent portal hypertension. We treated a 48-year-old Japanese woman who developed both portal hypertension and pancytopenia after undergoing multiple operations for a congenital dilatation of the bile duct. She underwent a Hassab's operation in July 1994, when an occlusion of the extrahepatic portal vein, which resulted in portal hypertension, was first noted; the liver was microscopically normal. The etiology of the extrahepatic portal obstruction in our patient was most likely due to either repeated inflammation or adhesion at the hepatic hilus. Based on these findings, the differential diagnosis of portal hypertension after an operation for a congenital dilatation of the bile duct should therefore include an extrahepatic portal obstruction in addition to liver cirrhosis.