Department of Neurosurgery, Mie University Graduate School of Medicine, Tsu, Mie, Japan.
Department of Neurosurgery, Mie University Graduate School of Medicine, Tsu, Mie, Japan.
World Neurosurg. 2019 Oct;130:335-338. doi: 10.1016/j.wneu.2019.07.106. Epub 2019 Jul 18.
Association of moyamoya disease (MMD) with von Willebrand disease (vWD) is extremely rare.
We first report a 27-year-old female case of MMD concurrent with vWD type 1, which presented as hemorrhagic stroke. The patient underwent a revascularization surgery with perioperative replacement therapy of von Willebrand factor and coagulation factor VIII. No hemorrhagic complications occurred.
The patient overcame postoperative transient neurological events and fully recovered. We discuss appropriate perioperative supplementation of coagulation factors for a revascularization surgery for MMD with vWD.
烟雾病（MMD）与血管性血友病（vWD）相关联的情况极为罕见。
我们首次报告了一例 27 岁女性 MMD 合并 vWD 1 型病例，表现为出血性卒中。患者接受了血运重建手术，并在围手术期补充了血管性血友病因子和凝血因子 VIII。未发生出血性并发症。
患者克服了术后短暂的神经功能障碍，完全康复。我们讨论了 vWD 合并 MMD 血运重建手术时合适的围手术期凝血因子补充方案。
