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Budd-Chiari syndrome — ESEGH MCQ

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HardHepatologyBudd-Chiari syndromeESEGH

A 34-year-old woman presents 6 weeks postpartum with progressive abdominal pain and ascites. Examination shows tender hepatomegaly. Her bilirubin is 48 micromol/L and INR 1.5. Doppler ultrasonography shows a patent portal vein with hepatopetal flow and a patent inferior vena cava. The right and middle hepatic veins cannot be identified at their caval confluence; flow in the left hepatic vein is reversed, and multiple intrahepatic venovenous collaterals are present. Echocardiography is normal. JAK2 V617F mutation testing is pending. What is the most likely diagnosis?

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Correct answer: D — Budd-Chiari syndrome

The correct answer is **B, Budd-Chiari syndrome**. The decisive findings are non-visualisation of major hepatic veins, reversed flow in the remaining hepatic vein and intrahepatic venovenous collaterals, which demonstrate hepatic venous outflow obstruction. The postpartum state is an additional prothrombotic risk, while pending JAK2 V617F testing appropriately assesses for an occult myeloproliferative neoplasm. Extrahepatic portal vein thrombosis is excluded by a patent portal vein with hepatopetal flow. Congestive hepatopathy would usually be accompanied by cardiac disease and dilated, pulsatile hepatic veins rather than obstructed veins and venovenous collaterals. Sinusoidal obstruction syndrome generally follows haematopoietic transplantation or relevant drug exposure and affects the hepatic sinusoids rather than producing this major hepatic-vein pattern. Acute fatty liver of pregnancy does not cause hepatic venous outflow obstruction.

Reference: European Association for the Study of the Liver. EASL Clinical Practice Guidelines on vascular diseases of the liver, Budd-Chiari syndrome section. Journal of Hepatology. 2026;84(2):399-456. https://pubmed.ncbi.nlm.nih.gov/41224629/