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Abstract：
BACKGROUND: The safety of laparoscopic hepatectomy for inherited coagulation disorders is unclear; however, the safety of open hepatectomy has been reported in several studies. Herein, we report the first case of a laparoscopic hepatectomy for a patient with von Willebrand Disease (VWD).
METHODS: A 76-year-old male with a history of chronic hepatitis C and VWD type 2B was advised surgical resection of a 4 cm hepatocellular carcinoma in segment 7 of the liver. The patient was diagnosed with VWD in his 40 s due to gastrointestinal bleeding caused by gastric erosion. The von Willebrand factor (VWF) ristocetin cofactor activity was 30%, and VWF large multimer deficiency and increased ristocetin-induced platelet agglutination were observed. The preoperative platelet count was reduced to 3.5 × 104/μL; however, preoperative imaging findings had no evidence of liver cirrhosis, such as any collateral formations and splenomegaly. The indocyanine green retention rate at 15 min was 10%, and his Child-Pugh score was 5 (classification A). Perioperatively, VWF/factor VIII was administered in accordance with our institutional protocol. A laparoscopic partial hepatectomy of the right posterior segment was performed. The most bleeding during surgery occurred during the mobilization of the right lobe of the liver due to inflammatory adhesion between the retroperitoneum and the tumor. Bleeding during parenchymal transection was controlable. The duration of hepatic inflow occlusion was 65 min. The surgical duration was 349 min, and the estimated blood loss was 2150 ml. Four units of red blood cells and fresh frozen plasma were transfused at the initiation of parenchymal transection, and 10 units of platelets were transfused at the end of the parenchymal transection. On postoperative day 1, the transection surface drainage fluid became hemorrhagic, and emergency contrast-enhanced computed tomography showed extravasation in the greater omentum. Percutaneous transcatheter arterial embolization of the omental branch of the right gastroepiploic artery was performed. No further postoperative interventions were required. The patient was discharged on postoperative day 14.
CONCLUSIONS: The indications for laparoscopic hepatectomy in patients with VWD should be carefully considered, and an open approach may still be the standard approach for patients with VWD.
METHODS: A 76-year-old male with a history of chronic hepatitis C and VWD type 2B was advised surgical resection of a 4 cm hepatocellular carcinoma in segment 7 of the liver. The patient was diagnosed with VWD in his 40 s due to gastrointestinal bleeding caused by gastric erosion. The von Willebrand factor (VWF) ristocetin cofactor activity was 30%, and VWF large multimer deficiency and increased ristocetin-induced platelet agglutination were observed. The preoperative platelet count was reduced to 3.5 × 104/μL; however, preoperative imaging findings had no evidence of liver cirrhosis, such as any collateral formations and splenomegaly. The indocyanine green retention rate at 15 min was 10%, and his Child-Pugh score was 5 (classification A). Perioperatively, VWF/factor VIII was administered in accordance with our institutional protocol. A laparoscopic partial hepatectomy of the right posterior segment was performed. The most bleeding during surgery occurred during the mobilization of the right lobe of the liver due to inflammatory adhesion between the retroperitoneum and the tumor. Bleeding during parenchymal transection was controlable. The duration of hepatic inflow occlusion was 65 min. The surgical duration was 349 min, and the estimated blood loss was 2150 ml. Four units of red blood cells and fresh frozen plasma were transfused at the initiation of parenchymal transection, and 10 units of platelets were transfused at the end of the parenchymal transection. On postoperative day 1, the transection surface drainage fluid became hemorrhagic, and emergency contrast-enhanced computed tomography showed extravasation in the greater omentum. Percutaneous transcatheter arterial embolization of the omental branch of the right gastroepiploic artery was performed. No further postoperative interventions were required. The patient was discharged on postoperative day 14.
CONCLUSIONS: The indications for laparoscopic hepatectomy in patients with VWD should be carefully considered, and an open approach may still be the standard approach for patients with VWD.
摘要：
背景：腹腔镜肝切除术治疗遗传性凝血障碍的安全性尚不清楚；然而，在几项研究中已经报道了开放式肝切除术的安全性。在这里，我们报道了1例vonWillebrand病(VWD)患者的腹腔镜肝切除术.
方法：一名76岁男性，有慢性丙型肝炎和VWD2B型病史，建议手术切除肝脏第7段4厘米的肝细胞癌。由于胃糜烂引起的胃肠道出血，该患者在40年代被诊断为VWD。血管性假血友病因子(VWF)里托菌素辅因子活性为30%,观察到VWF大多聚体缺乏和瑞斯托霉素诱导的血小板凝集增加。术前血小板计数降至3.5×104/μL;术前影像学检查结果没有肝硬化的证据，如任何侧支地层和脾肿大。吲哚菁绿在15min时的保留率为10%,他的Child-Pugh得分为5分（A级）。围手术期,VWF/因子VIII根据我们的机构方案进行管理。进行了右后段的腹腔镜部分肝切除术。由于腹膜后和肿瘤之间的炎性粘附，手术期间出血最多发生在肝脏右叶动员期间。实质横切过程中的出血是可控的。肝流入闭塞的持续时间为65分钟。手术时间为349分钟，估计失血量为2150毫升。实质切断术开始时输注四个单位的红细胞和新鲜冷冻血浆，在实质横切结束时输注10单位血小板。术后第1天，横切表面引流液出血，急诊对比增强计算机断层扫描显示大网膜外渗。经皮经导管动脉栓塞右胃上动脉的网膜分支。不需要进一步的术后干预。患者在术后第14天出院。
结论：应仔细考虑VWD患者行腹腔镜肝切除术的适应证，开放入路可能仍是VWD患者的标准入路.
方法：一名76岁男性，有慢性丙型肝炎和VWD2B型病史，建议手术切除肝脏第7段4厘米的肝细胞癌。由于胃糜烂引起的胃肠道出血，该患者在40年代被诊断为VWD。血管性假血友病因子(VWF)里托菌素辅因子活性为30%,观察到VWF大多聚体缺乏和瑞斯托霉素诱导的血小板凝集增加。术前血小板计数降至3.5×104/μL;术前影像学检查结果没有肝硬化的证据，如任何侧支地层和脾肿大。吲哚菁绿在15min时的保留率为10%,他的Child-Pugh得分为5分（A级）。围手术期,VWF/因子VIII根据我们的机构方案进行管理。进行了右后段的腹腔镜部分肝切除术。由于腹膜后和肿瘤之间的炎性粘附，手术期间出血最多发生在肝脏右叶动员期间。实质横切过程中的出血是可控的。肝流入闭塞的持续时间为65分钟。手术时间为349分钟，估计失血量为2150毫升。实质切断术开始时输注四个单位的红细胞和新鲜冷冻血浆，在实质横切结束时输注10单位血小板。术后第1天，横切表面引流液出血，急诊对比增强计算机断层扫描显示大网膜外渗。经皮经导管动脉栓塞右胃上动脉的网膜分支。不需要进一步的术后干预。患者在术后第14天出院。
结论：应仔细考虑VWD患者行腹腔镜肝切除术的适应证，开放入路可能仍是VWD患者的标准入路.
