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Budd-Chiari syndrome — ABIM Board MCQ

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HardGastroenterology/HepatologyBudd-Chiari syndromeABIM Board

A 39-year-old woman with JAK2-positive polycythemia vera presents with 2 weeks of progressive abdominal distention and right upper-quadrant pain. Examination shows tender hepatomegaly and ascites. Echocardiography shows normal right ventricular function and no pericardial disease. Doppler ultrasonography demonstrates a patent portal vein and inferior vena cava, nonvisualization of the major hepatic veins, caudate-lobe enlargement, and intrahepatic venovenous collaterals. Which of the following pathophysiologic processes most likely accounts for her presentation?

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Correct answer: A — Thrombotic occlusion of the major hepatic veins

The correct answer is A. This patient has primary Budd-Chiari syndrome due to thrombotic obstruction of the major hepatic veins. Polycythemia vera is a major acquired thrombophilic risk factor, and hepatic venous obstruction produces painful hepatomegaly, ascites, hepatic dysfunction, caudate enlargement, and intrahepatic venovenous collaterals. Portal-vein thrombosis causes presinusoidal portal hypertension but does not explain nonvisualized hepatic veins. Sinusoidal obstruction syndrome affects terminal venules and usually follows hematopoietic transplantation or exposure to endothelial toxins. Right-sided heart failure can cause congestive hepatopathy, but normal cardiac findings and obstructed hepatic veins argue against it. Hepatic arterial occlusion causes ischemic injury rather than this portal-hypertensive syndrome.

Reference: Garcia-Pagán JC, Valla DC. Primary Budd-Chiari Syndrome. New England Journal of Medicine. 2023;388:1307-1316. https://www.nejm.org/doi/full/10.1056/NEJMra2207738