It presents with hepatomegaly, ascites and classically paroxysmal nocturnal haemoglobinuria (dark urine in the morning). Caused by membrano...
It presents with hepatomegaly, ascites and classically paroxysmal nocturnal haemoglobinuria (dark urine in the morning). Caused by membranous obstruction of the suprahepatic IVC (i.e. venous outflow) by a congenital web or hepatic venous thrombosis (due to e.g. thrombophilia or metastases). About two-thirds of cases are idiopathic.
US
• Hepatic veins may not be seen.
• Thrombus within hepatic veins.
• Turbulent or reversed hepatic venous flow.
• Marked peripheral collaterals.
• Hepatosplenomegaly.
• Caudate hypertrophy only occurs with chronic Budd-Chiari syndrome.
CT
• Enlarged liver with ascites when acute
• Hyperenhancing central liver segments on early-phase, post-contrast, peripheral hypoenhancement (this pattern reverses on delayed images—the ‘flip-flop’ pattern).
• Absent hepatic veins.
• Caudate hypertrophy with chronic Budd-Chiari syndrome (compensatory enlargement, the caudate is spared as it drains directly into the IVC).
• Dysmorphic appearance to liver, mosaic enhancement.
• Gallbladder wall thickening.
• Increased portal vein diameter.
MRI
• Enhancement as per CT, but may also show absence of flow voids on T1 images if thrombus is present.
INTERVENTION
• A ‘spider’s web’ appearance of collaterals is diagnostic.
• Thrombus in hepatic veins.
• Balloon dilatation of the hepatic venous web may be performed for treatment.
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