Case of Hemorrhagic Moyamoya Disease Associated with Von Willebrand Disease

World Neurosurg. 2019 Oct:130:335-338. doi: 10.1016/j.wneu.2019.07.106. Epub 2019 Jul 18.

Abstract

Background: Association of moyamoya disease (MMD) with von Willebrand disease (vWD) is extremely rare.

Case description: 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.

Conclusions: 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.

Keywords: Coagulation factor; Hemorrhagic moyamoya disease; Von Willebrand disease.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Factor VIII / metabolism
  • Female
  • Hemorrhage / diagnosis
  • Hemorrhage / etiology
  • Hemorrhage / surgery*
  • Humans
  • Moyamoya Disease / diagnosis
  • Moyamoya Disease / etiology
  • Moyamoya Disease / surgery*
  • von Willebrand Disease, Type 1 / complications
  • von Willebrand Disease, Type 1 / diagnosis
  • von Willebrand Disease, Type 1 / surgery*
  • von Willebrand Factor / metabolism

Substances

  • von Willebrand Factor
  • Factor VIII