Criteria

Features suggestive of diagnosis

There are no formal diagnostic criteria for Budd-Chiari syndrome (BCS); however, the following findings are supportive of the diagnosis:

Clinical[1][6][7][9]​​​​

  • The classic triad of BCS is abdominal pain, ascites, and hepatomegaly. Leg oedema and dilated venous collateral on the trunk can indicate inferior vena cava compression or thrombosis.

Imaging[51]

  • Colour Doppler specific signs include alterations in hepatic and/or caval veins in the form of thrombosis, stenosis, fibrotic cord, or insufficient recanalisation of the vessels.

Venography[1][52]​​​​

  • Presence of a spider web pattern on hepatic venography confirms the diagnosis of BCS.

Pathology[46]

  • The liver in BCS can develop severe centrilobular congestion, hepatocyte necrosis, and fibrosis and can sometimes have co-existing cirrhosis.

Severity criteria

To assess the severity of the chronic form (cirrhosis) of BCS, Child-Pugh classification may be used.[53]​ 

Child-Pugh scoring uses 5 clinical measures of liver disease. Each measure is scored as between 1 and 3 points, with 3 indicating the most severe derangement. The clinical measures are:

Encephalopathy

  • None: 1 point

  • Grade 1 to 2: 2 points

  • Grade 3 to 4: 3 points

Ascites

  • None: 1 point

  • Mild/moderate: 2 points

  • Tense: 3 points

Bilirubin (mg/dL)

  • <2: 1 point

  • 2 to 3: 2 points

  • More than 3: 3 points

Albumin (g/dL)

  • >3.5: 1 point

  • 2.8 to 3.5: 2 points

  • <2.8: 3 points

INR

  • <1.7: 1 point

  • 1.7 to 2.3: 2 points

  • >2.3: 3 points

Chronic liver disease is classified into Child-Pugh class A to C using the scores as above:

  • Class A: 5 to 6 points

  • Class B: 7 to 9 points

  • Class C: 10 to 15 points

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