The most common cause of Budd chiari syndrome is
|A||Hepatic vein valve|
a. Budd-Chiari syndrome is an uncommon condition induced by thrombotic or nonthrombotic obstruction to hepatic venous outflow.
b. The syndrome most often occurs in patients with underlying thrombotic diathesis, including myeloproliferative disorders, such as polycythemia vera and paroxysmal nocturnal hemoglobinuria, pregnancy, tumors, chronic inflammatory diseases, clotting disorders, and infections.
c. The classic triad of abdominal pain, ascites, and hepatomegaly is observed in the vast majority of patients but is nonspecific. A high index of suspicion is needed to make the diagnosis.
d. Four main clinical variants have been described: acute liver disease, subacute liver disease, fulminant liver disease, and liver failure.
e. The most common presentation is subacute liver disease complicated by portal hypertension and varying degrees of liver decompensation types:-
i. Acute and subacute form: These forms are characterized by rapid development of abdominal pain, ascites, hepatomegaly, jaundice, and renal failure.
ii. Chronic form: This form of presentation is the most common. Patients present with progressive ascites. Jaundice is absent, and approximately 50% of patients also have renal impairment.
iii. Fulminant form: This form of presentation is uncommon. Fulminant or subfulminant hepatic failure is present along with ascites, tender hepatomegaly, jaundice, and renal failure.
iv. Decompression of the hepatic vasculature should be offered if portal hypertension is the cause of the symptoms.
v. Either surgery or a transjugular intrahepatic portosystemic shunt procedure can be performed.
vi. Liver transplantation should be offered if decompensated liver cirrhosis is present