Q51) In Budd Chiari Syndrome best management for patients when all three hepatic veins are blocked with deranged LFT

a) Liver transplant

b) Side to side porto caval shunt

c) MEso atrial Shunt

d) TIPS


51) a

In most patients with BCS, occlusion of the hepatic veins is caused by thrombosis. The thrombus undergoes organization and ultimately is converted to fibrous tissue that permanently occludes the veins.

The attractiveness of TIPS is that it can be done without a major surgical abdominal operation in patients who have liver disease and thus are often quite ill. Its major disadvantage is a substantial incidence of occlusion of the TIPS that may require repeated revision and hospitalization and often involves recurrence of symptoms

SSPCS Side to side porto caval shunt  proved to be the most widely applied and durable. This technique is indicated only in patients with BCS who have a patent IVC and an IVC pressure that is substantially lower than WHVP or portal pressure

Meso atrial shunt is for when IVC is occluded. It is from SMV to right atrium bypassing the IVC and directly takes blood to the heart

Liver Transplant

It is useful in far advanced, decompensated liver disease, when liver dysfunction has progressed beyond a salvageable state by other portal decompression procedures.

. Indications include the following:

1. Cirrhosis with progressive liver failure that has reached the point of permitting a reasonable prediction that the patient will die within 1 year—the most common indication for LT and the same used widely in other liver disease

2. Failure of a portosystemic shunt or TIPS, usually because of thrombosis, with persistence or recurrence of symptoms and signs of BCS.

3. BCS with unshuntable portal hypertension secondary to thrombosis of the PV, splenic vein, and much of the SMV—a rare indication that applies only if patent blood vessels are available to vascularize the liver allograft.

4. Acute fulminant hepatic failure—a rare indication that we have encountered only once in the past 20 years.

The above question does not mention cirrhosis, choice is between sspcs and Liver transplant. 

Hepatic venous outflow obstruction produces widespread destruction of the hepatic parenchyma by pressure necrosis and ischemia, and the liver damage becomes irreversible in a surprisingly short time; some patients developed cirrhosis within 3 or 4 months of the onset of symptom

Ref Blumgart

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