Hepatic Venous Outflow Obstruction

 Portal vein thrombosis, systemic inflammatory conditions, some neoplasms such as renal cell carcinoma or lymphoma, artifactual sinusoidal dilatation



• Other causes of parenchymal necrosis/hemorrhage
image Acetaminophen toxicity, ischemic liver injury


image
Sinusoidal Dilatation
In Budd-Chiari syndrome, increased sinusoidal pressure causes sinusoidal dilatation, congestion image, and hepatocellular atrophy image .


image
RBC in Space of Disse
Extravasation of RBC image in the space of Disse (the potential space between the hepatocytes and sinusoidal basement membrane) is caused by increased sinusoidal pressure.

image
Hemorrhagic Necrosis
Hepatocellular necrosis image around the central vein can be seen in HVOO, especially in cases presenting acutely. Inflammation is typically mild or absent, unlike centrizonal necrosis seen in autoimmune hepatitis or adverse drug reaction.

image
Ductular Reaction
Ductular reaction image can be seen in portal tracts as well as centrizonal regions. It is generally mild in portal areas but can be prominent and accompanied by portal inflammation &/or focal bile duct damage. These findings can closely mimic biliary disease.



TERMINOLOGY


Abbreviations




• Hepatic venous outflow obstruction (HVOO)


ETIOLOGY/PATHOGENESIS


Venous Obstruction




• Can occur at different levels of hepatic venous outflow
image Sinusoids or small hepatic veins: Sinusoidal obstruction syndrome (formerly venoocclusive disease)

image Large hepatic veins or inferior vena cava [Budd-Chiari syndrome (BCS)]

image Right heart or pericardial disease

– Right heart failure (either isolated or result of left heart failure)

– Tricuspid valve disease

– Cardiac amyloidosis

– 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
image Nonsurgical decompression by percutaneous transluminal angioplasty with stent: Suitable for webs or limited stenosis

image Image-guided transjugular intrahepatic portosystemic shunt

image 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
image 5-year survival after portosystemic shunt is 75-90%

image 5-year survival after liver transplantation is 60%

• Cardiac disease
image 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

image Normal hepatic vein flow is not seen

image Collaterals attempt to decompress obstruction leading to spider web appearance

image Reverse flow can be seen in portal vein

• For cardiac causes, findings depend on underlying disease

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Apr 20, 2017 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Hepatic Venous Outflow Obstruction

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