Episode 1: Budd-Chiari Syndrome
Script Author and Editor: Dr. Suneet Sood
Narrators: Thong Yi Kun, Alan Koay
So what do you have for us today?
This is the story of a 30 year old man who presented with an upper gastrointestinal bleeding.
Last year, we received a patient who presented with hematemesis, hepatosplenomegaly, and ascites.
The surgeons carried out an endoscopy, and found esophageal varices, which they banded, and he stabilized.
I notice, your patient had hepatosplenomegaly. I can understand the splenomegaly in portal hypertension, but why the hepatomegaly?
That’s a good question. Portal hypertension may be prehepatic, intrahepatic, or posthepatic. The liver is quite normal in prehepatic portal hypertension, and ascites is not a feature. The liver is shrunken in intrahepatic portal hypertension, which usually results from cirrhosis. Ascites is common. The liver is enlarged in posthepatic portal hypertension, and ascites is common. In fact, subsequent MR angiograms showed hepatic vein thrombosis.
That’s interesting. What caused this hepatic vein thrombosis?
Initially we didn’t have proof, but made a guess. We had to take a very detailed history. This provided a clue to the cause of hepatic vein thrombosis.
What was the clue?
The patient gave a history of severe gastroenteritis and dehydration a year earlier, and needed admission and intravenous fluids. In susceptible persons, dehydration may provoke venous thrombosis at various sites, including the hepatic veins, mesenteric veins, cavernous sinus, and other sites.
You said “In susceptible persons, dehydration may provoke venous thrombosis”. What do you mean by susceptible persons?
I mean persons who are at risk of thrombosis, which means persons who have an underlying thrombophilia. The common causes are deficiencies of Protein C, Protein S, or antithrombin III, and the Leiden mutation of factor 5.
So did this patient have any of these?
Yes. Later we evaluated this patient for thrombophilia. Results showed that he had a significant deficiency of Protein C.
If he had a protein C deficiency, can’t he get this problem again?
Yes he can. We put him on a lifelong warfarin therapy.
Thanks. So you are saying two things. One, hepatomegaly with varices indicates posthepatic portal hypertension, not cirrhosis. And two, that dehydration can precipitate venous thrombosis in a patient with thrombophilia.
Partly true. If a patient with portal hypertension has a palpable liver, also think of hepatocellular cancer. Remember that hepatocellular cancer is commoner in cirrhosis, especially cirrhosis associated with Hepatitis B or C.
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