The estimated prevalence of von Willebrand’s disease (vWD) worldwide has been widely reported as near 1%. This makes vWD the most common inherited bleeding disorder. Although the condition can present with varying degrees of severity, it is usually mild and its presence often goes undiagnosed until significant hemorrhagic stress is placed on the patient, such as during major orthopedic surgical procedures. Given the heightened prevalence of the disorder in the general population, it is very important for all surgeons to be aware of its presentation, treatment options, and the side effects of these treatments in order to effectively reduce hemorrhagic and other medical complications related to vWD. von Willebrand’s disease is categorized as a disorder of primary hemostasis involving platelet dysfunction and the inability to produce sufficient plasma concentrations of von Willebrand factor (vWF). It is also associated with qualitative deficits in the structure of vWF which prevents normal function during the clotting cascade. Normal levels and structure of vWF act not only to initiate primary hemostasis but also to stabilize factor VIII. In more clinically significant cases of vWD, recombinant factor VIII protein maybe needed in addition to replacement vWF to address all deficiencies in both primary and secondary hemostases .
HSS Journal, an academic peer-reviewed journal, is published twice a year, February and September, and features articles by internal faculty and HSS alumni that present current research and clinical work in the field of musculoskeletal medicine performed at HSS, including research articles, surgical procedures, and case reports.