Chiba Hiroto, Sato Naoya, Takahashi Hiroshi, Suzuki Yoshiki, Azuma Takayasu, Tsukida Shigeyuki, Muto Makoto, Kofunato Yasuhide, Ishigame Teruhide, Kimura Takashi, Kenjo Akira, Ikezoe Takayuki, Marubashi Shigeru
Department of Hepato-Biliary-Pancreatic and Transplant Surgery, Fukushima Medical University, Fukushima, Fukushima, Japan.
Department of Hematology, Fukushima Medical University, Fukushima, Fukushima, Japan.
Surg Case Rep. 2025;11(1). doi: 10.70352/scrj.cr.25-0188. Epub 2025 Jul 31.
Von Willebrand disease (VWD) is the second most common inherited coagulation disorder, and appropriate perioperative management is necessary when considering major surgery. There are few reports of patients with VWD who have undergone hepatectomy, especially minimally invasive hepatectomy. To our knowledge, this is the first reported case of a patient with VWD who successfully underwent robotic hepatectomy with von Willebrand factor (VWF) and factor VIII (FVIII) supplementation.
A 75-year-old female was referred to our hospital because of a liver tumor that was diagnosed during follow-up after hepatitis C treatment. She had also been diagnosed with VWD in her 30s. CT and MRI showed a 24-mm mass in segment 8 of the liver, bordered by the middle hepatic vein (MHV). To ensure safe perioperative management, replacement therapy with a VWF- or FVIII-containing concentrate was administered from preoperative day 1 to POD 14. Robotic extended segmentectomy (segment 8) was performed, with resection of the MHV. Liver parenchyma was dissected using the crush and clamp technique under the Pringle maneuver. Estimated intraoperative blood loss was 160 mL, and total operative time was 601 min. The patient needed 2 units of fresh frozen plasma on POD 1; however, no other transfusions, including red blood cells, were required. Although the patient presented with postoperative ascites and was treated with diuretics, she was discharged on POD 20 without any bleeding event. The final pathological finding was intrahepatic cholangiocarcinoma.
We encountered a patient with intrahepatic cholangiocarcinoma and VWD who was successfully treated with anatomical hepatectomy by robotic-assisted laparoscopic surgery under perioperative replacement therapy with a VWF- or FVIII-containing concentrate. With appropriate perioperative management, major hepatectomy can be applied for VWD patients despite their high risk of postoperative hemorrhagic complications.
血管性血友病（VWD）是第二常见的遗传性凝血障碍，在考虑进行大手术时，需要进行适当的围手术期管理。关于血管性血友病患者接受肝切除术的报道很少，尤其是微创肝切除术。据我们所知，这是首例成功接受补充血管性血友病因子（VWF）和凝血因子VIII（FVIII）的机器人肝切除术的血管性血友病患者。
一名75岁女性因丙型肝炎治疗后随访期间诊断出的肝肿瘤转诊至我院。她在30多岁时也被诊断出患有血管性血友病。CT和MRI显示肝脏第8段有一个24毫米的肿块，以肝中静脉（MHV）为界。为确保围手术期管理安全，从术前第1天至术后第14天给予含VWF或FVIII的浓缩物进行替代治疗。进行了机器人扩大肝段切除术（第8段），切除了MHV。在Pringle手法下，采用挤压和钳夹技术解剖肝实质。估计术中出血量为160毫升，总手术时间为601分钟。患者在术后第1天需要2单位新鲜冰冻血浆；然而，不需要包括红细胞在内的其他输血。尽管患者术后出现腹水并接受了利尿剂治疗，但她在术后第20天出院，没有发生任何出血事件。最终病理结果为肝内胆管癌。
我们遇到了一名患有肝内胆管癌和血管性血友病的患者，在围手术期使用含VWF或FVIII的浓缩物进行替代治疗的情况下，通过机器人辅助腹腔镜手术成功进行了解剖性肝切除术。通过适当的围手术期管理，尽管血管性血友病患者术后出血并发症风险高，但大肝切除术仍可应用于此类患者。
