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The Budd–Chiari syndrome is a rare disorder resulting from obstructed hepatic venous outflow. It may be due to a congenital obliteration of the hepatic veins. However, acquired cases related to hypercoagulable states or myeloproliferative disorders are more frequent. Transjugular intrahepatic portosystemic shunting (TIPS), side-to-side portocaval decompressive surgery, and liver transplantation are often necessary to improve the survival rate.
We describe an unusual case associated with polycythemia rubra vera (PRV) treated successfully with angioplasty and thrombolysis. Similar cases have rarely been reported in the medical literature.
2. Case Report
A 25-year-old man with PRV was admitted with progressive abdominal pain, hepatosplenomegaly, ascites and lower limbs œdema. Prominent subcutaneous veins were present over the abdomen (Fig. 1) . The venous pattern was suggestive of an inferior vena cava obstruction with blood flow directed from the sapheno-femoral junction to the costal margins. Unlike portal hypertension, the peri-umbilical veins (caput medusae) were not particularly distended. Pressure applied over the liver failed to distend the jugular veins (negative hepatojugular reflux).
Fig. 1Prominent subcutaneous veins over the abdomen with blood flow directed from the sapheno-femoral junction to the costal margins.
Duplex sonography revealed an extensive thrombosis of the three main hepatic veins (Fig. 2(a), uniphasic flow). Thrombosis of the inferior vena cava was also present and was confirmed by venography. There were no signs of pulmonary embolism. Inferior vena cava angioplasty and catheter-directed thrombolysis was performed. The catheter was introduced percutaneously via the basilic vein of the left arm. A bolus of 10 mg tissue-plasminogen activator (t-PA) was given locally, followed by 2 mg/h for 24 h. Complete revascularization was observed with duplex sonography after the treatment (Fig. 2(b), biphasic flow). The PRV was treated with hydroxyurea. No recurrence of thrombosis was observed after one year.
Fig. 2(a) Uniphasic flow in the main hepatic veins on duplex sonography. (b) Biphasic flow in the main hepatic veins after angioplasty and thrombolysis.
The diagnosis of Budd–Chiari syndrome must be suspected in any patient with ascites and evidence of hepatic congestion. It is important to differentiate Budd–Chiari syndrome from portal hypertension due to hepatic cirrhosis. The subcutaneous venous pattern and the absence of a hepatojugular reflux may be suggestive. Screening for hepatic vein occlusion can be done by duplex sonography and confirmed by venography.
Unless hepatic congestion is relieved, the natural history of Budd–Chiari syndrome is generally progressive liver failure and death. Once the diagnosis has been made and the cause identified, treatment must be undertaken without delay. Angioplasty and catheter-directed thrombolysis may be effective and life saving in acute cases avoiding late complications such as liver failure and hepatic transplantation.
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