Budd-Chiari syndrome

Case contributed by Dr Henry Knipe

Presentation

Upper abdominal pain. JAK negative essential thrombocythemia.

Patient Data

Age: 35 years
Gender: Female

Upper abdomen

CT

Mottled peripheral hypodensity to the right liver, mainly in the periphery of segments V, VI, VII and VIII. The right and middle hepatic veins do not opacify. The left and caudate lobes of the liver have a normal appearance with normal opacification of enlarged left hepatic veins. The portal veins opacify normally. As best as can be determined the peripheral right portal vein branches opacify normally. The hepatic artery is not well seen secondary to timing of the CT. Splenomegaly.

Cholecystectomy. Adrenal glands, kidney and pancreas have a normal appearance. No free fluid or free gas.

Bilateral pleural effusions, larger on the left. Minor bibasal atelectasis.

Conclusion:

Appearances are of right liver hypoperfusion, probably from venous congestion from complete right and middle hepatic vein thrombosis. This represents Budd-Chiari syndrome. 

Case Discussion

Ultrasound confirmed the presence of occlusive thrombus within the right and middle hepatic veins in keeping with Budd-Chiari syndrome

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