von Willebrand disease (vWD) is an inherited bleeding disorder that is characterized by a quantitative or qualitative deficiency of the von Willebrand factor (vWF). Type 3 is the most severe form of vWD with a near-complete absence of vWF and a significantly increased risk of excessive bleeding and hematoma during a surgical procedure. To date, no data on surgical and hemostatic management of a type 3 vWD patient undergoing body-contouring surgery has been published. We report the case of a 47-year-old woman with type 3 vWD requiring medically indicated abdominoplasty after massive weight loss due to bariatric surgery. The case was successfully managed with individualized bodyweight-adapted substitution of recombinant vWF vonicog alfa and tranexamic acid under close monitoring of vWF and factor VIII activity. For further risk stratification, we propose the multidisciplinary treatment of patients with severe vWF undergoing elective plastic surgery in specialized centers providing around-the-clock laboratory testing and access to a blood bank. In addition, strict hemostasis during surgery and early postoperative mobilization with fitted compression garments are recommended to further reduce the risk of bleeding and thromboembolic complications.
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