Portal vein thrombosis, systemic inflammatory conditions, some neoplasms such as renal cell carcinoma or lymphoma, artifactual sinusoidal dilatation
•
Other causes of parenchymal necrosis/hemorrhage
Acetaminophen toxicity, ischemic liver injury
TERMINOLOGY
Abbreviations
•
Hepatic venous outflow obstruction (HVOO)
ETIOLOGY/PATHOGENESIS
Venous Obstruction
•
Can occur at different levels of hepatic venous outflow
Sinusoids or small hepatic veins: Sinusoidal obstruction syndrome (formerly venoocclusive disease)
Large hepatic veins or inferior vena cava [Budd-Chiari syndrome (BCS)]
Right heart or pericardial disease
–
Right heart failure (either isolated or result of left heart failure)
–
Tricuspid valve disease
–
Constrictive pericarditis
Pathogenesis
•
Liver changes result from hepatic venous congestion, increased hepatic and sinusoidal pressure, and necrosis
•
Secondary sinusoidal thrombosis extending into hepatic and portal veins may contribute to parenchymal damage and fibrosis
CLINICAL ISSUES
Presentation
•
Subacute presentation (< 6 months) is most common with painful hepatomegaly, mild jaundice, and ascites
•
Less commonly, presents as chronic liver disease or cirrhosis
•
Rare cases have fulminant presentation with acute liver failure
Laboratory Tests
•
Mild elevation of transaminases; marked increase in acute cases
•
Alkaline phosphatase elevation is common
Treatment
•
Decompression procedures in BCS
Nonsurgical decompression by percutaneous transluminal angioplasty with stent: Suitable for webs or limited stenosis
Image-guided transjugular intrahepatic portosystemic shunt
Surgical decompression with portosystemic shunt
•
For cardiac etiologies, treat underlying disease
•
Liver transplant necessary in cases with advanced fibrosis
•
Hematological work-up is essential to identify cause of thrombosis
Prognosis
•
BCS
5-year survival after portosystemic shunt is 75-90%
5-year survival after liver transplantation is 60%
•
Cardiac disease
Depends on type and severity of underlying illness
IMAGING
Radiographic Findings
•
For BCS, ultrasound with Doppler flow studies is initial tool of choice
•
Hepatic scintigraphy, CT, and MR can also contribute to diagnosis
•
Hepatic venography was considered gold standard in BCS, but it is now restricted to diagnostically challenging cases
Normal hepatic vein flow is not seen
Collaterals attempt to decompress obstruction leading to spider web appearance
Reverse flow can be seen in portal vein
•
For cardiac causes, findings depend on underlying disease
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