Budd Chiari Syndrome associated with angiomyolipoma of the liver

Abstract

We wish to report an unusual complication of hepatic angiomyolipoma. The patient was admitted to this Hospital with a two-week history of progressive abdominal pain, distension and peripheral oedema. Laboratory evaluation revealed albumin 30 g/l, bilirubin 48μmol/l, AST 114iu/l, INR 1.55, Cr 137μmol/l and ascitic protein 25 g/l. CT abdomen (Fig. 1) demonstrated a 8.0×9.7×7.3 cm hypoattenuating mass in the central liver, involving segments 4a, 6, 7 and 8. There was associated extensive ascites and caudate lobe hypertrophy. Ultrasound, triphasic CT and MRI showed patent portal veins with hepatopetal flow. The hepatic veins were poorly visualised and CT scan suggested thrombus in the left hepatic vein. On subsequent venography it was not possible to cannulate the hepatic veins and there was extrinsic compression of the inferior vena cava with pressures of 20 mmHg below and 16 mmHg above the lesion. The right atrial pressure was 9 mmHg. Targeted tru-cut™ percutaneous ultrasound guided biopsy of the hepatic mass following correction of coagulopathy was consistent with the diagnosis of hepatic angiomyolipoma.