Sato Koki, Kuroda Shintaro, Kobayashi Tsuyoshi, Shimizu Seiichi, Ohira Masahiro, Tahara Hiroyuki, Ide Kentaro, Fujii Teruhisa, Ohdan Hideki
Department of Gastroenterological and Transplant Surgery, Hiroshima University Hospital, 1-2-3, Kasumi, Minami-ku, Hiroshima, 734-8551, Japan.
Department of Gastroenterological and Transplant Surgery, Hiroshima University Hospital, 1-2-3, Kasumi, Minami-ku, Hiroshima, 734-8551, Japan.
Int J Surg Case Rep. 2018;44:131-134. doi: 10.1016/j.ijscr.2018.02.033. Epub 2018 Feb 24.
Although von Willebrand disease (VWD) is a common inherited bleeding disorder, very few cases of surgery in patients with VWD have been reported.
A 77-year-old man was referred to our hospital for treatment of hepatocellular carcinoma (HCC) based on type C chronic hepatitis. He had also been treated for VWD in the hematology department of another hospital. Partial hepatectomy was performed with the administration of factor VIII/von Willebrand factor concentrate just before and after the operation. The perioperative course was uneventful, and the patient was discharged 12 days after surgery.
VWD causes dysfunction of the platelet and destabilization of the blood clotting factor VIII. The patient was successfully treated with measurement of activated partial thromboplastin time (APTT) as an index for the management of hemostasis.
This report describes a rare case of a successful perioperative management of hepatectomy in a patient with VWD.
尽管血管性血友病（VWD）是一种常见的遗传性出血性疾病，但VWD患者接受手术的病例报道极少。
一名77岁男性因丙型慢性肝炎并发肝细胞癌（HCC）转诊至我院治疗。他曾在另一家医院的血液科接受过VWD治疗。手术前后均给予VIII因子/血管性血友病因子浓缩物，随后进行了部分肝切除术。围手术期过程顺利，患者术后12天出院。
VWD导致血小板功能障碍和凝血因子VIII不稳定。通过监测活化部分凝血活酶时间（APTT）作为止血管理指标，该患者得到了成功治疗。
本报告描述了1例VWD患者肝切除术围手术期成功管理的罕见病例。
