Tani Aya, Yoshida Hiroshi, Mamada Yasuhiro, Taniai Nobuhiko, Kawano Yoichi, Uchida Eiji
Surgery for Organ Function and Biological Regulation, Graduate School of Medicine, Nippon Medical School, Toyko, Japan.
J Nippon Med Sch. 2010 Oct;77(5):269-72. doi: 10.1272/jnms.77.269.
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.
我们描述了一名因巨大肝血管瘤伴卡萨巴赫-梅里特综合征导致肝外门静脉阻塞的患者。一名67岁女性因上腹部胀满和食欲减退就诊。病史与当前疾病无关。初始实验室检查结果如下：血清血小板计数9.9×10⁴/µL；血清纤维蛋白原降解产物12µg/mL；凝血酶原时间1.26；血清纤维蛋白原111mg/dL。计算机断层扫描显示肝脏左叶有直径15cm的均匀低密度区。肝动脉造影显示一个高血管性肿瘤，延迟期有造影剂积聚。肠系膜上动脉造影的门静脉期显示门静脉阻塞及海绵样变性。我们诊断为巨大肝血管瘤伴卡萨巴赫-梅里特综合征导致的肝外门静脉阻塞。行剖腹手术，发现肝脏明显肿大。游离左叶后，间断阻断血流行左肝切除术。切除术后，多普勒超声显示门静脉血流恢复。海绵样变性缩小。手术标本的病理检查证实存在巨大良性肝海绵状血管瘤。患者术后16天出院。2个月后实验室数据及并发症情况改善。
