Oshika Hiroyuki, Koyama Yukihide, Tsuzaki Koichi, Ida Kohmei, Andoh Tomio
Department of Anesthesiology, Mizonokuchi Hospital, Teikyo University School of Medicine, Kawasaki, Japan.
Department of Anesthesia, Nippon Koukan Hospital, 1-2-1 Koukan-dori, Kawasaki-ku, Kawasaki-shi, Kawasaki, Kanagawa Prefecture, 210-0852, Japan.
JA Clin Rep. 2019 Aug 27;5(1):54. doi: 10.1186/s40981-019-0276-4.
Von Willebrand disease (VWD) is the most common inherited bleeding disorder in humans. Coagulopathies such as VWD are evidently risk factors for post-surgical bleeding. Perioperative management of patients with VWD remains controversial and is a major clinical concern.
A 5-year-old girl was scheduled for tonsillectomy under general anesthesia. Preoperative laboratory tests revealed prolongation of activated partial thromboplastin time and a mild decrease in von Willebrand factor (VWF) activity. Prophylactic administration of desmopressin or VWF was not performed. During tonsillectomy, oozing from the surgical wound was uncontrollable by conventional hemostasis techniques, but complete hemostasis was ensured by plasma-derived coagulation factor VIII concentrate containing VWF.
Pediatric patients with mild abnormalities in preoperative laboratory tests may have coagulopathies. Prophylactic intervention and/or the preparation of a sufficient amount of coagulation factor VIII concentrate containing VWF may be required in patients suspected of having VWD or with mild VWF deficiency.
血管性血友病（VWD）是人类最常见的遗传性出血性疾病。诸如VWD之类的凝血障碍显然是术后出血的危险因素。VWD患者的围手术期管理仍存在争议，是一个主要的临床关注点。
一名5岁女孩计划在全身麻醉下进行扁桃体切除术。术前实验室检查显示活化部分凝血活酶时间延长，血管性血友病因子（VWF）活性轻度降低。未进行去氨加压素或VWF的预防性给药。在扁桃体切除术中，常规止血技术无法控制手术伤口的渗血，但通过含有VWF的血浆源性凝血因子VIII浓缩物确保了完全止血。
术前实验室检查有轻度异常的儿科患者可能存在凝血障碍。对于疑似患有VWD或VWF轻度缺乏的患者，可能需要进行预防性干预和/或准备足够量的含有VWF的凝血因子VIII浓缩物。
