Extrahepatic Portal Venous Obstruction due to a Giant Hepatic Hemangioma Associated with Kasabach-Merritt Syndrome

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We describe a patient with extrahepatic portal venous obstruction due to a giant hepatic hemangioma associated with Kasabach-Merritt syndrome. A 67-year-old woman presented with upper abdominal distension and appetite loss. The medical history was not relevant to the current disorder. Initial laboratory tests revealed the following: serum platelet count, 9.9 ×10⁴/μL; serum fibrinogen degradation products, 12 μg/mL; prothrombin time, 1.26; and serum fibrinogen, 111 mg/dL. Computed tomography demonstrated homogenous low-density areas, 15 cm in diameter, in the left lobe of the liver. Common hepatic arteriography revealed a hypervascular tumor with pooling of contrast medium in the delayed phase. The portal venous phase of supramesenteric arteriography revealed obstruction and cavernous transformation of the portal vein. We diagnosed extrahepatic portal venous obstruction due to a giant hepatic hemangioma associated with Kasabach-Merritt syndrome. Laparotomy was performed, and the liver was found to be markedly enlarged. After mobilization of the left lobe, left hepatectomy was performed with intermittent clamping. After resection, Doppler ultrasonography revealed recovery of the portal venous flow. The cavernous transformation shrank. Pathologic examination of the surgical specimen confirmed the presence of a giant benign hepatic cavernous hemangioma. The patient was discharged 16 days after operation. Laboratory data and complications improved after 2 months.