LearningRadiology - Budd-Chiari Syndrome

Budd-Chiari Syndrome

Hepatic Venous Occlusive Disease

Obstruction to hepatic venous outflow leads to increased sinusoidal pressure producing reversed or delayed flow in portal veins
Many causes
- Idiopathic most common
- Tumor
  - Hepatocellular carcinoma
  - Carcinoma of pancreas
  - Carcinoma of kidneys
  - Metastatic disease
- Blood dyscrasia
  - Leukemia
  - Sickle cell disease
  - Polycythemia vera
- Birth control pills
- Pregnancy
- Pyrrolizidine alkaloids (senecio) found in Jamaican tea
- Membranous diaphragm in suprahepatic IVC
Acutely
- Hepatomegaly and ascites
- Severe symptoms including shock
- Abdominal pain
- Jaundice
- CT
  - Hepatomegaly and ascites
  - Non-visualization of occluded hepatic veins
    - Also seen on MRI
  - Patchy enhanced appearance with dynamic imaging
  - Inversion of portal blood flow results in inside-out enhancement of liver
    - Caudate lobe is hyperdense early, decreased later
    - Periphery is hypodense early, increased later
    - Then enhancement equilibrates
      - Due to reversed portal venous flow
  - Enlarged right inferior hepatic vein
  - Enlarged portal vein (>12mm in adults)
Chronically
- Portal hypertension and variceal bleeding
- Enlargement of caudate lobe
- Collateral circulation through azygous and hemiazygous
  - Visualization of paraumbilical vein
- Non-visualization of hepatic veins
- Inversion of portal blood flow results in inside-out enhancement of liver
  - Periphery is hypodense early
  - Then enhancement equilibrates
  - Due to reversed portal venous flow

Early and delayed phases of liver enhancement
in Budd-Chiari Syndrome

Nuclear medicine shows hot caudate lobe with diminished activity in peripheral zones of liver
Angiography shows large lakes of sinusoidal contrast accumulations
Absence of main hepatic veins

WH/03