Chronic Budd-Chiari syndrome can present with cirrhosis and signs and symptoms similar to those of other chronic liver diseases. We present a case of Budd-Chiari syndrome discovered during attempted transjugular intrahepatic portosystemic shunting in a patient with decompensated cirrhosis believed to be secondary to hepatitis C. Although the patient had hepatocellular carcinoma, the Budd-Chiari...

The case of a 20 year old woman is reported with Budd-Chiari syndrome in whom lupus anticoagulant and anticardiolipin antibodies were shown; treatment with oral anticoagulants induced a considerable improvement. This treatment was interrupted after one year; interruption was followed by redevelopment of ascites. Further treatment with anticoagulants was continued for five years with noticeable ...

Leebeek FW. Myeloproliferative disease in the pathogenesis and survival of Budd-Chiari syndrome. Haematologica 2006;91: 1712-3. 3. Valla D, Dhumeaux D, Babany G, et al. Hepatic vein thrombosis in paroxysmal nocturnal hemoglobinuria. A spectrum from asymptomatic occlusion of hepatic venules to fatal Budd-Chiari syndrome. Gastroenterology 1987;93:569-75. 4. Hillmen P, Lewis SM, Bessler M, Luzzatt...

Budd-Chiari syndrome (BCS) represents a rare medical entity which has an estimated incidence of 0.1 to 10 people per million every year. It is defined by the obstruction flow in inferior vena cava or hepatic veins. Various classifications have been proposed. So, it can be acute chronic and primary secondary. The form more frequent characterized signs portal hypertension. Liver transplantation u...

To the Editor, We read with interest the article by Shukla and Bhatia [1] in a recent issue of the Journal. The authors found that 6 of their patients could not undergo the standard radiological procedures because of technical reasons; reasons for this technical failure were however, not provided. In our experience of adult patients, the TIPSS procedure is usually possible. Of 58 patients who u...

Budd Chiari Syndrome is a rare disease and can be difficult to diagnose. Most of the investigations to diagnose this disease are invasive. Liver scintiscanning is not only non-invasive, it also shows a typical pattern in this condition. Good correlation exists between Liver scanning and other procedures. A case of Budd Chiari Syndrome with typical clinical and scan findings is described (JPMA 3...

We report a four year old boy who presented with liver failure secondary to anti-thrombin III deficiency related Budd Chiari syndrome. He was treated with TIPSS (transjugular intrahepatic porto systemic shunt) which reversed the encephalopathy, normalised the liver function and improved growth, pre-empting the need for a liver transplantation. This is the first reported case of TIPSS in a child...

Primary malignant tumors of the heart, specifically cardiac sarcomas, are rare and mainly diagnosed at autopsy. Acute Budd-Chiari syndrome is a recognized cause of acute liver failure and has been associated with several rare cardiac tumors: atrial myxoma, caval rhabdomyosarcoma, and primary cardiac adenocarcinoma. We present the first case of a fatal, highly differentiated cardiac synovial sar...