Pathophysiology
1) key event is a decrease of hepatic venous flow 2) classically described as formation of clot at the junction of hepatic veins and vena cava Causes – 3) hypercoagulable states (factor V Leiden, lupus, polycythemia vera, myeloproliferative disorders, sickle cell) 4) infectious (amebic liver abscess, hydatid cysts) 5) cancer (hepatocellular carcinoma) 6) pregnancy 7) membranous webs (40% of cases in Far East)
Signs and Symptoms
1) sudden onset of hepatomegaly 2) abdominal pain 3) ascites 4) liver failure 5) onset can be fulminant (associated with pregnancy), acute, or chronic
Histology/Gross Pathology
1) liver parenchymal hemorrhage 2) central zone congestion 3) liver cell necrosis
Associated Conditions
1) at risk for re-thrombosis, even after transplantation 2) requires lifelong anticoagulation
Treatment
1) fulminant presentation with liver failure requires liver transplantation 2) chronic presentation with cirrhosis requires liver transplantation Some remaining function and reserve options include – 3) thrombolytic agents 4) shunt procedures using a “side-to-side” structure (e.g., mesocaval or atrial-caval shunt that has a differential pressure of at least 10 mmHg 5) transjugular intrahepatic shunt (TIPS) procedure
Tips for USMLE
if the patient is a 26 year-old white female who has lupus with acute onset of abdominal pain, abdominal distention, and palpable liver, think Budd-Chiari
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