Department of Emergency and Critical Care Medicine, University of Miyazaki Hospital, Miyazaki, Japan.
Am J Case Rep. 2022 Aug 30;23:e936690. doi: 10.12659/AJCR.936690.
BACKGROUND von Willebrand disease (VWD) is characterized by a bleeding tendency due to abnormalities in von Willebrand factor (VWF). Severe traumatic brain injury (TBI) can induce secondary coagulopathy and hemostatic disorders. We herein present a rare case of multiple trauma, including severe TBI, in a patient with VWD who was successfully treated with repeated factor VIII/VWF transfusion in addition to standard critical care. CASE REPORT A 22-year-old man with type 2A VWD sustained head and lower limb injuries in a traffic accident and was comatose. Computed tomography indicated multiple trauma, including severe TBI (left-sided traumatic epidural hematoma, left-sided traumatic subdural hematoma, traumatic subarachnoid hemorrhage, skull fracture, and skull base fracture). The patient underwent emergency craniotomy for hematoma removal, external decompression, and intracranial pressure monitoring along with massive transfusion and repeated perioperative transfusion of factor VIII/VWF concentrates according to the level of bleeding. He recovered consciousness and eventually survived without neurological deficits. CONCLUSIONS Multiple trauma including TBI in patients with VWD is a critical condition. The active transfusion of factor VIII/VWF is essential for controlling hemorrhage early and in the perioperative period.
血管性血友病（VWD）的特征是由于血管性血友病因子（VWF）异常导致出血倾向。严重创伤性脑损伤（TBI）可引起继发性凝血功能障碍和止血紊乱。本文报告了一例罕见的 VWD 患者合并多处创伤，包括严重 TBI，除了标准重症监护外，还通过反复输注 VIII 因子/VWF 成功治疗。病例报告：一名 22 岁的 2A 型 VWD 患者在交通事故中头部和下肢受伤，处于昏迷状态。计算机断层扫描显示多处创伤，包括严重 TBI（左侧创伤性硬脑膜外血肿、左侧创伤性硬脑膜下血肿、创伤性蛛网膜下腔出血、颅骨骨折和颅底骨折）。患者接受紧急开颅血肿清除术、外减压术和颅内压监测，并根据出血程度进行大量输血和反复围手术期输注 VIII 因子/VWF 浓缩物。他恢复了意识，最终没有神经功能缺损而存活。结论：VWD 患者合并多处创伤，包括 TBI，是一种危急情况。积极输注 VIII 因子/VWF 对于控制早期和围手术期出血至关重要。
