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 supposedly 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 suprahepatic 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 inadvertent interruption of the round ligament, which until then had served as a pathway 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 syndrome predominantly maintained at the expense of the round ligament of the liver, with a patent vascular branch.

journal_name

Angiology

journal_title

Angiology

authors

Corrêa de Araujo R,Bestetti RB,Oliveira JS

doi

10.1177/000331978803900211

subject

Has Abstract

pub_date

1988-02-01 00:00:00

pages

193-8

issue

2

eissn

0003-3197

issn

1940-1574

journal_volume

39

pub_type

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