Findings: A selective catheterization and injection through a transjugular approach of the hepatic vein confluence. This shows no significant opacification of left or middle hepatic vein with only slight opacification of the right hepatic vein 1-2 cm distal to the tip of the catheter. A small network of collateral veins is opacified representing the typical "spider web" appearance of hepatic vein occlusion. This is consistent with Budd-Chiari syndrome.

Image #2 shows a TIPS with stent placement from the right hepatic vein to the right portal vein. Injection of this stent shows extensive filling defects within the stent. Also, filling defects within the splenic vein near its confluence with the superior mesenteric vein and in the portal vein are present consistent with thrombosis.

Image #3 shows injection of the patient's TIPS after treatment with Urokinase and mechanical thrombectomy. The stent is now patent.

Diagnosis: Budd-Chiari Syndrome treated with TIPS.

Discussion: Budd-Chiari syndrome is a term applied to a group of diseases involving hepatovenous outflow obstruction, which may also affect the intrahepatic inferior vena cava. This syndrome can be classified as primary or secondary, depending on the cause and physiology. In the primary type, membranous obstruction of hepatic veins occurs. The membranous obstruction is of unknown etiology. Theories include a congenital embryologic abnormality or possibly may have been acquired due to infection, injury or phlebitis. Secondary Budd-Chiari syndrome results from obstruction of the major hepatic veins from hematologic disorders such as sickle cell anemia, myeloproliferative disorders, thrombocytosis, polycythemia vera. Also, hypercoagulable state such as pregnancy, protein C and S deficiency, antithrombin III deficiency, and collagen vascular disorders are potential etiologic causes. In this patient, a specific cause was not identified.

Clinical manifestations of Budd-Chiari include abdominal pain, hepatomegaly, ascites, and abnormalities of liver enzymes.

In this patient, a TIPS was performed (transjugular intrahepatic portal systemic shunt). This shunt is most commonly performed in patients with variceal bleeding. Less common indication is Budd-Chiari syndrome. However, a relative contraindication would be portal vein thrombosis. This procedure forms a shunt between the hepatic and portal vein system causing decompression of the portal vein. Gradients between the two circulations of less than 12 mm Hg are often achievable. However, complications include acute or chronic thrombosis of the shunt, bleeding, and hepatic encephalopathy.

In this patient, immediate injection of the shunt showed filling defects in the shunt suggestive of thrombosis as well as filling defect in the portal vein and splenic vein also suggestive of thrombosis. This was treated with Urokinase and thrombectomy and subsequent image showed development of complete patency of the shunt.

References:
Beheshti M, Jones, M. Journal of Vascular Interventional
Radiology, 7:277-281, 1996.

Baum, Pentecost. Abram's Angiography, 1997.

Gore, Levine, Laufer. Textbook of Gastrointestinal Radiology, 1994.

Blum V, Rossie M, et al. Budd-Chiari Syndrome: Technical,
Hemodynamic, and Clinical Results of Treatment with Transjugular
Intrahepatic Portosystemic Shunt. Radiology 1995; 197:805-811.

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