31st July 2023

Budd-Chiari Syndrome

The eponymous name for hepatic vein occlusion. Typically caused by a thrombus but can also be secondary to infiltration by a tumour.

The classic triad includes right upper quadrant pain, ascites and hepatomegaly. The clinical features are summarised in Table 3.

Table 3: Features of Budd-Chiari syndrome

Budd-Chiari syndrome is exceptionally rare in the absence of a procoagulant condition, and its presence should trigger investigation for the cause of hypercoagulability. A rarer alternative is the direct extension of a tumour mass into the hepatic vein. The causes are shown in Table 2.

Table 4: Causes of Budd-Chiari syndrome

If Budd-Chiari syndrome is suspected, the initial radiological investigation is typically ultrasound with doppler flow studies, which is highly sensitive for hepatic vein occlusion. An alternative is a CT with contrast, for which hypertrophy of the caudate lobe is a characteristic sign (highly specific but only seen in ~50% of cases).

The management is variable and will typically be made by the hepatology team. Lifelong anticoagulation is often required, though this may be contraindicated with concurrent oesophageal varices. The occlusion may need to be bypassed with a surgical shunt or a transjugular intrahepatic portosystemic shunt (TIPSS). If fulminant liver failure is present, the patient may be eligible for a liver transplant.