Document Detail


An unusual case of Budd-Chiari syndrome--a case report.
MedLine Citation:
PMID:  2964800     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
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.
Authors:
R Corrêa de Araujo; R B Bestetti; J S Oliveira
Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Angiology     Volume:  39     ISSN:  0003-3197     ISO Abbreviation:  Angiology     Publication Date:  1988 Feb 
Date Detail:
Created Date:  1988-04-18     Completed Date:  1988-04-18     Revised Date:  2007-11-15    
Medline Journal Info:
Nlm Unique ID:  0203706     Medline TA:  Angiology     Country:  UNITED STATES    
Other Details:
Languages:  eng     Pagination:  193-8     Citation Subset:  IM    
Affiliation:
Department of Pathology, Faculty of Medicine of Triângulo Mineiro, Uberaba, Brazil.
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MeSH Terms
Descriptor/Qualifier:
Adult
Budd-Chiari Syndrome / complications,  congenital*,  pathology
Down Syndrome / complications
Humans
Liver / pathology
Male
Vena Cava, Inferior / pathology

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