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 edema and dilated venous collateral on the trunk can indicate inferior vena cava compression or thrombosis.

Imaging[51]

  • Color Doppler specific signs include alterations in hepatic and/or caval veins in the form of thrombosis, stenosis, fibrotic cord, or insufficient recanalization 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 coexisting 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)

  • Less than 2: 1 point

  • 2 to 3: 2 points

  • More than 3: 3 points

Albumin (g/dL)

  • More than 3.5: 1 point

  • 2.8 to 3.5: 2 points

  • Less than 2.8: 3 points

INR

  • Less than 1.7: 1 point

  • 1.7 to 2.3: 2 points

  • More than 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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