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Abdominal pain in a 22 year old woman

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Q1: What is the syndrome presented by this patient?

Budd-Chiari syndrome (BCS).

Q2: What is the underlying disease?

Latent myeloproliferative disorder (polycythaemia vera).


Because of clinical evidence of portal hypertension (splenomegaly, oesophageal varices), we considered the possibility of BCS. Ludwiget al suggested the following definition for BCS: “hepatic venous outflow obstruction and its manifestations, regardless of cause, the obstruction being either within the liver or the inferior vena cava between the liver and the right atrium”.1

A colour Doppler sonogram was obtained and confirmed the diagnosis: the major hepatic veins were not seen and an enlarged subhepatic accessory vein was …

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