What prophylaxis is given prior to surgery in patients with von Willebrand disease (vWD)?

Updated: Dec 30, 2019
  • Author: Eleanor S Pollak, MD; Chief Editor: Srikanth Nagalla, MBBS, MS, FACP  more...
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Answer

A hematologist experienced in the management of bleeding disorders should be consulted prior to all surgical/dental procedures. For prophylaxis in major surgery, rVWF (with or without FVIII) or vWF-containing factor VIII (FVIII) concentrates are the treatment of choice.  For emergency surgery, both vWF and FVIII are required to ensure hemostasis; however, for elective procedures, early infusion of rVWF  will stabilize endogenous FVIII.  Hemostatic levels are maintained until bleeding risk abates: usually 3 to 5 days for minor procedures and 7 to 14 days for major surgery.  [25]

Patients with vWD who undergo major surgery,  particularly those with more severe forms of vWD, require frequent monitoring of plasma levels and access to hemostatic. Repeated VWF supplementation (particularly with plasma-derived FVIII-containing products) may lead to accumulation of FVIII. [25]

In extremely rare cases, vWD patients who receive FVIII concentrates as prophylaxis for surgery may experience venous thromboembolic complications. A literature review by Franchini et al found only 11 reported cases, most of which involved orthopedic procedures. Nevertheless, these authors advise that the need for thromboprophylaxis with vWF/FVIII concentrates in patients with vWD who undergo major surgery should be individually assessed, after a careful risk/benefit analysis. [26]


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