MYOMA-ERYTHROCYTOSIS SYNDROME, PRESENTING WITH ACUTE HEPATIC VEIN THROMBOSIS.
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A 31-year-old man with Child's class A micronodular cirrhosis, left lobe hypertrophy, and a transjugular intrahepatic portosystemic shunt (TIPS) which had been placed 6 months earlier, was admitted for recurrent esophageal bleeding and a portosystemic gradient of 42 mmHg. Balloon occlusion portography documented unsuspected ostial thrombosis of the previously patent left hepatic vein. This was considered the cause of the pressure rise. As it was not possible to insert a second TIPS in parallel, the shunt, stented originally with 10-mm Wallstents, was overdilated to 12 mm, and two 12-mm Palmaz stents were placed coaxially, reducing the portosystemic pressure gradient to 13 mmHg.
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Stenosis of the hepatic venous outflow anastomosis is rare after liver transplantation. Hepatic venous outflow obstruction affects 5.1% to 7% of transplanted patients. Clinical findings among children include massive ascites and abdominal pain and laboratory findings demonstrate altered liver function tests and coagulopathy. In this article, we report a case of hepatic venous thrombosis occurring 22 days after living-related liver transplantation. The patient was treated with hepatic venoplasty and stent implantation.
We report a 24-year-old Japanese female hospitalized with jaundice and ascites. She exhibited hepatosplenomegaly, severe liver dysfunction, and slight polycythemia with an increase in serum levels of beta-thromboglobin and platelet factor 4. Bone marrow was hypercellular with an increase in progenitor cells. The aggregation response of platelets to ADP and to collagen was markedly increased. Venography revealed narrowed hepatic veins with "spider web' sign. Liver biopsy revealed hepatic congestion. Budd-Chiari syndrome was diagnosed, and was thought to be due to thrombosis related to myeloproliferative disorder. Liver transplant was successful in relieving symptoms.
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