Abstract
OBJECTIVE: Von Willebrand disease (VWD) is an inherited bleeding disorder caused by quantitative or qualitative deficiencies in von Willebrand factor (VWF). Reports of intracranial aneurysms complicated by VWD, particularly ruptured aneurysms, are rare. Optimal perioperative management during endovascular treatment in this patient group remains unclear. CASE PRESENTATION: Here, we report the case of a female in her 50s with type 1 VWD who presented with sudden headache and nausea. Imaging revealed a subarachnoid hemorrhage due to a ruptured anterior communicating artery (Acom) aneurysm. Considering the high risk of surgical bleeding, endovascular therapy was administered. DSA demonstrated a 5.8 × 4.4-mm aneurysm, and a Woven EndoBridge (W-EB) SL device (7 × 3 mm; Terumo Corporation, Tokyo, Japan) was successfully deployed. Periprocedural hemostatic management was performed in consultation with hematology specialists, and a factor VIII/VWF concentrate was administered post-procedure to maintain VWF, which was continued for 3 days post-procedure (intravenous bolus infusions of 2000 units). Antiplatelet therapy was not administered. The postoperative course was uneventful, and no intracranial or access site bleeding occurred. Follow-up angiography on postoperative day 7 revealed thrombus formation within the device and no inflow into the aneurysmal bleb. At 4 months postoperatively, the presence of minor residual flow within the W-EB (Bicêtre Occlusion Scale Score [BOSS] grade 1) was confirmed, and its expected complete occlusion was observed during subsequent follow-ups. CONCLUSION: This case demonstrates that W-EB placement can be safely performed in patients with VWD and may reduce hemorrhagic risk by avoiding the need for antiplatelet therapy.