Case of Hemorrhagic Moyamoya Disease Associated with Von Willebrand Disease.

Suzuki, Yume; Shiba, Masato; Wada, Hideo; Yasuda, Ryuta; Toma, Naoki; Suzuki, Hidenori · World Neurosurg · 2019

case_report · Level V

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Abstract

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.

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