A 32-year-old woman presents with right upper abdominal pain and abdominal distension. Examination reveals hepatomegaly with ascites. Doppler ultrasound shows absence of flow in the hepatic veins with intraluminal echogenic material. CT confirms thrombus in the hepatic veins with caudate lobe hypertrophy. What is the diagnosis?
- A Portal vein thrombosis
- B Right-sided heart failure
- C Budd-Chiari syndrome ✓
- D Sinusoidal obstruction syndrome
Explanation
Budd-Chiari syndrome is obstruction of hepatic venous outflow at any level from small hepatic veins to the inferior vena cava. Doppler ultrasound showing absent hepatic vein flow with intraluminal thrombus is diagnostic. Caudate lobe hypertrophy is characteristic because the caudate lobe drains directly into the IVC, remaining unaffected when main hepatic veins are occluded. Common associations include myeloproliferative disorders, factor V Leiden mutation, and oral contraceptive use.
Reference: Robbins and Cotran Pathologic Basis of Disease, 10th ed.
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Written and medically reviewed by the StethoPrep medical team.