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Budd-Chiari syndrome

Case contributed by Mostafa Elfeky
Diagnosis almost certain

Presentation

Abdominal distension, pain, and jaundice for weeks.

Patient Data

Age: 50 years
Gender: Female

Triphasic liver CT

ct

The liver shows cirrhotic changes with irregular borders and markedly enlarged caudate lobe with heterogeneous patchy enhancement on the delayed phase.

There are linear non-enhancing structures along the different phases of the study, notably in the right lobe mostly representing thrombosed main hepatic veins. The inferior vena cava is compressed by the enlarged caudate lobe.

The spleen is mildly enlarged with normal features. Mildly dilated splenic vein. Dilated gastro-esophageal collaterals.

Moderate free ascites.

Diffusely thickened edematous wall of the gallbladder.

Right crossed fused renal ectopia.

Case Discussion

Budd-Chiari syndrome is one of the veno-occlusive diseases affecting the liver. The majority of cases are primarily due to thrombosis of the hepatic veins. Other diagnostic features include ascites, hepatomegaly and delayed enhancement of the peripheral zones of the liver. Caudate lobe enlargement occurs more in the subacute and chronic stages.

Crossed fused renal ectopia is more common to occur on the right side. Here the left kidney is located on the right side and fused to the lower pole of the right kidney. The left renal artery is turned to the right to supply the left kidney. Each kidney has its separate pelvicalyceal system and ureter.

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