Management of von Willebrand disease for orthognathic surgery: Evidenced-based recommendations and scoping review of the literature

Abstract

Background: The general management of von Willebrand Disease has evolved over the past several decades. Advances in blood product replacement manufacturing have led to changes in perioperative management of the bleeding diathesis in its various subtypes. Surgical management follows changes in medical management, but unique perioperative considerations exist depending on the type of surgery. Orthognathic surgery is considered a major surgery that can have life-threatening complications in patients with von Willebrand Disease (vWD) and updates in evidenced-based management are presented. Objectives: The objectives of this report are to review available literature regarding management of patients with von Willebrand Disease and other bleeding disorder undergoing orthognathic surgery and to provide a case report regarding the management of a patient vWD type 2M undergoing orthognathic surgery. Eligibility criteria: Articles included for review were studies addressing patients with bleeding disorders undergoing orthognathic surgery. Sources of evidence: PubMed and Ovid were queried using the terms “orthognathic surgery OR jaw deformity” AND “von Willebrand OR bleeding disorders OR hemophilia.” Additional references relevant to the review were obtained from the reference lists of available articles ultimately deemed appropriate for inclusion in the review. Methods: A recent example case of a patient with type 2M vWD undergoing orthognathic surgery and the patient's perioperative management is presented for consideration. A scoping review of the literature was undertaken for evaluation of available literature using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses extension for Scoping Review (PRISMA-ScR). Results: Insufficient evidence exists for significant analysis of perioperative management strategies previously reported compared to the currently utilized management strategy for the case presented. A review of the available literature with reference to updated literature regarding management of patients with vWD undergoing surgical procedures other than orthognathic surgery yields primarily case reports, two randomized controlled trials, and one systematic review. The results indicate that an evolution in the general management of patients with vWD undergoing surgery can be applied to patients undergoing orthognathic surgery and that the addition of hemostatic agents such as tranexamic acid may be considered with a low level of evidence. Conclusions: For patients undergoing orthognathic surgery, the use of cryoprecipitate in large quantities has been replaced with the use of Humate-P infusion and will likely be replaced with the use of recombinant von Willebrand factor (VWF). The timing of infusion of adjuncts preoperatively for patients with vWD can potentially be extended to the previous day, and the addition of medications to aid in hemostasis such as tranexamic acid may be considered. Prophylactic use of additional VWF adjuncts should be considered in the postoperative setting, and close monitoring is prudent. The use of newer agents may allow for the expansion of craniomaxillofacial surgery procedures that can be safely considered in patients with vWD.