Elsevier

Journal of Hepatology

Volume 32, Issue 3, March 2000, Pages 516-520
Journal of Hepatology

Case Report
Successful treatment of chronic Budd-Chiari syndrome with a transjugular intrahepatic portosystemic shunt

https://doi.org/10.1016/S0168-8278(00)80405-5Get rights and content

Abstract

Budd-Chiari syndrome is characterized by obstruction of the hepatic venous outflow tract. Therapeutic options for chronic Budd-Chiari syndrome are limited. We report the case of a 28-year-old woman who presented with recurrence of chronic Budd-Chiari syndrome with total obstruction of all major hepatic veins. Due to worsening liver function over the course of 1 year, she had to be listed for liver transplantation. Because of therapy-refractory ascites, declining renal function and severe esophageal varices, a transjugular intrahepatic portosystemic shunt (TIPS) was placed, planned as a bridge to transplantation. Following TIPS, a marked recovery of liver function could be observed, accompanied by disappearance of ascites, esophageal varices, and normalization of kidney function. Therefore, the patient could be removed from the waiting list for liver transplantation. This case demonstrates for the first time that the use of TIPS in chronic Budd-Chiari syndrome may result in marked recovery of liverfunction.

Section snippets

Case Report

In April 1994, a 23-year-old woman was referred to our hospital with a 3-week history of abdominal distension, right upper quadrant pain, nausea and vomiting. Her past medical history was remarkable for use of oral contraceptives for 4 years.

On examination, the patient had marked hepatosplenomegaly and ascites. At that time, the complete blood cell count (CBC) including platelets (430 000/mm3) and chemistry were normal. The liver profile showed moderate elevation of transaminases, total

Discussion

The Budd-Chiari syndrome (BCS) results from obstruction of the hepatic venous outflow tract due to occlusion of the hepatic veins and/or the IVC. The pathophysiologic features in acute BCS are caused by the inability of blood to drain from the liver, which leads to hepatic congestion, ischemia, and death of pericentral hepatocytes. Eventually, chronic liver disease develops and is followed by features of portal hypertension and cirrhosis. Due to separate drainage into the IVC by small and

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