An Unusual Case of Budd-Chiari Syndrome—A Case Report

Abstract

The authors report a rare case of congenital Budd-Chiari syndrome in a twenty-eight-year-old male mongoloid. The patient was submitted to azygous- portal disconnection, because of the syndrome of portal hypertension suppos edly due to cirrhosis of the liver. He died of hemorrhage of the liver on the third postoperative day. Autopsy revealed a congenital fibrotic obstruction of all su prahepatic veins, with a wide, round ligament containing a functional umbilical vein, which had been routinely ligated during surgery. An extensive review of the literature showed no similar report. The authors speculate that the inadver tent interruption of the round ligament, which until then had served as a path way for venous draining of the liver, followed by ligation of the anastomoses between the portal and azygous systems, was the factor that triggered the lethal outcome. Thus, this appears to be the first case of congenital Budd-Chiari syn drome predominantly maintained at the expense of the round ligament of the liver, with a patent vascular branch.