Abstract
The patient was a 20-year-old woman who had undergone medical treatment for essential thrombocythemia for 5 years. She had complained of abdominal distension and jaundice of uncertain etiology when she was thoroughly examined in February 2006. As late as July 2006, the needle biopsy specimen showed congestive liver damage; the abdominal enhanced CT scan, complete hepatic venous obstruction; and the pulsed Doppler ultrasonogram, reverse flow in the right branch of the portal vein with recanalization of the paraumbilical vein. Accordingly, our diagnosis was hepatic venous thrombosis-type Budd-Chiari syndrome (BCS). Follow-up ultrasonography revealed multiple nodules up to 2 cm in diameter in the enlarged liver and showed a heterogeneous solid echoic pattern with a halo. Contrast-enhanced ultrasonography with Sonazoid® was carried out. The imaged nodules demonstrated a spoke-wheel pattern after blood had flowed directly to the center of the liver in the early phase. In the late phase, the stained nodules were more echogenic than the background liver tissue. Liver transplantation was carried out at the request of the patient and her family in March 2007 because of progression of liver failure. Pathologically, the liver explant showed congestive cirrhosis resulting from hepatic venous obstruction. Most of the nodules contained central satellite scar and radial dilated vessels, consistent with the histologic indication of focal nodular hyperplasia (FNH). Ultrasonography using a contrast agent appears to be the method of choice for evaluating dynamic state of liver circulation in BCS and proved invaluable in diagnosing the nodules associated with it in this case.
