Chiari's syndrome

Budd Chiari syndrome

A condition defined by obstruction of the hepatic veins and its clinical manifestations, regardless of the cause (except congestive heart failure), where the obstruction is either within the liver on in the inferior vena cava between the liver and the right atrium.
 
Aetiology
Thrombosis in the hepatic veins (majority of cases) due to coagulation secondary to polycythaemia vera, pregnancy, postpartum, oral contraceptive use, intravascular lesions including malignancy, myeloproliferative disorders, paroxysmal nocturnal haemoglobinuria (PNH), infection (e.g., schistosomiasis), intravascular webs.
 
Clinical findings
Abdominal pain, ascites, jaundice, hepatomegaly, and eventually cirrhosis, liver failure and hepatic encephalopathy.

Lab
Abnormal LFTs, increased liver enzymes.

DiffDx
Liver failure, GI bleeding, starvation.
 
Management
Unsatisfactory.

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