Von Willebrand disease: screening, diagnosis, and management

Von Willebrand disease (vWD), a hemorrhagic disorder mimicking a defect in platelet function, is the most commonly inherited coagulopathy, resulting in a deficiency that may prolong bleeding time and increase risk for major bleeding complications during surgery. Von Willebrand factor (vWF) serves a dual role in hemostasis: mediating the initial platelet adhesion to damaged endothelium at the site of vessel injury and stabilizing coagulation factor VIII, an important cofactor in the generation of a fibrin clot. Although quantitative or qualitative defects in vWF protein can manifest as a mild to severe bleeding disorder, many cases of vWD remain subclinical, barring major invasive stimuli, and undetected by either patient or clinician. Nevertheless, the frequency of this coagulation disorder would almost ensure that every plastic surgeon will encounter affected patients, making a thorough understanding of vWD and its management absolutely necessary. Surprisingly, there is little information concerning vWD in the plastic surgery literature. Our goal is to familiarize the plastic surgeon with vWD, including physiology, diagnostic criteria, classification, and molecular basis for multiple vWD variants, and diagnosis and management.