Chiari malformation describes a group of structural defects of the cerebellum, characterized by brain tissue protruding into the spinal canal. Chiari malformations are often associated with myelomeningocele, hydrocephalus, syringomyelia, and tethered cord syndrome. Although studies of etiology are few, an increasing number of specific genetic syndromes are found to be associated with Chiari mal...

Transjugular intrahepatic portosystemic shunt (TIPS) is a new treatment modality in patients of intractable ascites with Budd-Chiari syndrome. We report a young lady who presented with intractable ascites and encephalopathy and diagnosed to have Budd-Chiari syndrome. She was treated with TIPS and on follow-up after 12 months she is free of ascites, encephalopathy and able to perform her daily w...

Budd-Chiari syndrome is defined as hepatic venous outflow tract obstruction regardless of the level of the obstruction (anywhere between the small hepatic veins to the junction of the inferior vena cava and right atrium) or the cause of obstruction. It is frequently associated with hepatomegaly, abdominal pain, ascites and hepatic dysfunction, and patients with Budd-Chiari syndrome often demons...

Introduction: There are four types of Chiari malformations described in the literature. Due to the fact that it is a common finding in the general population (true Chiari being present in 0,75% of the population), Chiari 1 malformation was also called Chiari anomaly. Material and methods: In the last 5 years 17 patients with Chiari I malformation have been treated in our clinic. There were 12 w...

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...

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...

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...

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...

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 ...