Prevalence, pathophysiology, diagnosis, and treatment of Willebrand syndrome in orthopedic-trauma patients

The von Willebrand's Disease affects about 1-3 % of the population and it i s undiagnosed in most people. Originally described by the Finish physician Erik von Willebrand in 1926 this disorder is associated with mucous membran bleeding including epistaxis, hypermenorhoea and excessive bleeding from s urgery and dental procedures. In von Willebrands disease the patients have a quantitative or qualitative abnormality in the von Willebrand Factor (vWF ) which has two major functions: it serves by bridging between platelets an d injury site in the blood Vessel wall, and it circulates as a complex with factor VIII, protecting it from rapid degradation. More than 20 different types and subtypes of vWD have been described but almost all patients can b e classified as having 1 of the 2 most common types of vWD. About 70% of pa tients have type 1 and about 10-20 % present themself with type 2A and 2B v WD type 3 in about 10 %. By taking a clinical history of bleeding it is imp ortant to think about vWD and to make the appropriate diagnosis. if the vWD is suspected diagnostic tests should include an activated partial thrombop lastin time, bleeding time, factor VIII:C, Ristocetin cofactor, and VWF ant igen. Additional testing of ristocetin induced plattlet adhesion (RIPA), th e multimeric structure and collagen binding test and genanalysis allow diag nosing the different types of v. Willebrand Disease. The treatment of choic e in mild forms is the synthetic agent desmopressin. In patients with sever e type 1, type 2B, ZN and type 3 or in people who do not response to desmop ressin, the appropriate treatment is a factor VIII concentrate that is rich of vWF. Until now no recommendations in the orthopaedic literature have be en found regarding diagnosis and treatment of vWS patients. Based on the ex perience with 43 orthopaedic patients presenting vWD Over the last 15 years a contemporary guideline for the successful perioperative management of vW D in orthopaedic surgery is presented. In a close collaboration between the orthopaedic surgeon and the specialized haemostaseologist every invasive p rocedure in patients with vWD can be undertaken without the risk of excessi ve bleeding.