Budd Chiari syndrome

Case contributed by Dr Mostafa El-Feky

Presentation

Known chronic liver disease on follow up.

Patient Data

Age: 40 years
Gender: Male

Abdomen

Ultrasound

The liver shows average size and coarse echo-pattern showing patchy echogenicity and volume dysmorphism (prominent caudate and small left lobe).

Hepatic veins are patent and markedly attenuated.

No intrahepatic biliary radicle dilatation. 

The spleen is enlarged measuring 15.5 cm in bipolar diameter with normal echo-pattern.  

Abdomen

MRI

The liver shows the following imaging features:

  • enlarged right lobe span, measuring (19.5 cm)
  • heterogeneous parenchymal enhancement and signal intensities, showing mostly sectorial distribution in territories of the hepatic veins.
  • no evidence of diffusion restriction and no mass effect, with no abnormal enhancement in the arterial phase. These are associated perfusion changes.
  • the hepatic veins (MHV and RHV) show near obliterative occlusion, with a patent retro-hepatic segment of the IVC.
  • the left lobe is atrophic and represented only by a small 3.5 cm exophytic tissue replaced by a focal lesion with benign features including spreading puddle of near venous vascular enhancement and no diffusion restriction, mostly a cavernous hemangioma.

The portal vein and PM venous confluence are patent.

Recanalized dilated and tortuous para-umbilical vein is seen. Multiple dilated left gastric/esophagogastric and lienorenal varices are seen.

The spleen is enlarged (15.5 cm).

Mild free upper abdominal ascites is seen.

Also, a note is made of dilated systemic venous collaterals along the internal mammary and lower chest wall.

Both breasts show prominent mammary tissue, representing gynecomastia.

Case Discussion

Overall hepatic features are suggestive of veno-occlusive disease localized to the hepatic veins with essentially patent IVC and corresponding perfusion changes, mostly representing Budd-Chiari syndrome. Portal hypertension, mild splenomegaly, ascitis and porto-systemic varices are also noted.

The patient is planned for liver transplantation.

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