Hepatic Vein
Outflow Obstruction
Epidemiology and
Etiology:
- Etiologies
(thrombus):
- Chronic
liver disease
- Amebic
abscess
- Sarcoidosis
- hypercoagulable disorder
- OCP
- Pregnancy
- Heart
failure (cardiac cirrhosis)
Common sites:
Gross features:
- Thrombi
may have recanalized and not be evident on
imaging (ex. Cryptogenic cirrhosis)
- Massively
engorged liver
- Vena cava
obstruction (if present):
- Dilated
abdominal wall and chest veins
- Leg edema
- Thrombosis
of large portal veins (10-20%)
- Segmental
hyperplasia of caudate lobe (if inferior right hepatic vein is spared) or
other focal area of liver
Histologic
features:
- Perivenular necrosis (zone 3)
- Intraparenchymal hemorrhage
- Fibrosis
- Venocentric pattern if pure
hepatic vein thrombosis
- Intimal fibrosis of large and
medium-sized hepatic veins
- Multiple
layers usually
- Recanalized lumens
- Nodular
regenerative hyperplasia maybe
- Cardiac
sclerosis (“cirrhosis”):
- Mild atrophy
of zone 3 hepatocytes
- Sinusoidal
dilatation
- Venocentric fibrous septation
- Complete
nodules RARELY
Immunophenotype:
Marker:
|
Sensitivity:
|
Specificity:
|
|
|
|
Molecular features:
Other features:
- Budd-Chiari syndrome:
- Hepatomegaly
- RUQ
pain
- Ascites
- Liver
failure
- Complications:
References:
- Wanless IR (2004), in: Surgical
Pathology of the GI Tract, Liver, Biliary
Tract, and Pancreas.