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Budd-Chiari syndrome


Step 1: View clinicals

A 29 year old man presents with icterus, right upper abdominal pain, and persistent abdominal swelling with intermittent ankle and scrotal swelling for 1 year. There is no history of blood transfusions, intravenous drug abuse or sexual promiscuity. There is no family history of liver disease. He is not on any drugs and was in good health prior to the onset of symptoms. He only drinks socially.

Step 2: Order all relevant investigations

Ultrasound Abdomen

Ascites is present. Marked hepatosplenomegaly with enlargement of the caudate lobe of the liver is noted.

Doppler Hepatobiliary System

The inferior vena cava is dilated and partially occluded by thrombus. Moderate portal hypertension is present, with no evidence of intrahepatic venous obstruction.

Coagulation Profile

The bleeding time, clotting time, PT/INR and APTT are all within normal parameters.

HBsAg + Hepatitis C Abs

Negative for Hepatitis B surface antigen Negative for Hepatitis C antibodies

Step 3: Select appropriate management

Pegylated interferon
Liver Transplantation

Score: ★★☆