DP41: PIONEERING ADVANCES IN VON WILLEBRAND DISEASE MANAGEMENT: OPTIMIZING PERIOPERATIVE HEMOSTASIS IN A COMMUNITY HOSPITAL SETTING
Justin Scuorzo, DO; Giselle Helo, MD; Benjamin Houseman, MD, PhD
Memorial Healthcare System
Background: Von Willebrand disease (VWD), the most common inherited bleeding disorder, affects approximately 1% of the population and presents significant perioperative hemostatic challenges.1 It is classified into Type 1 (quantitative deficiency), Type 2 (qualitative dysfunction), and Type 3 (severe deficiency). Type 2A VWD, characterized by defective platelet adhesion due to aberrant high-molecular-weight (HMW) multimers, poses a heightened perioperative bleeding risk.
Optimal perioperative management of these patients requires meticulous planning tailored to individual phenotype. Unlike Type 1 vWD, Type 2 vWD is often unresponsive to desmopressin (DDAVP) and requires vWF replacement therapies such as plasma-derived vWF (Humate) and recombinant human vWF (rVWF; Vonvendi).1 The latter has a higher proportion of HMW multimers and prolonged half-life, making it desirable for perioperative care.1 Adjunctive antifibrinolytics, such as tranexamic acid (TXA), further enhance hemostatic stability.
Over the past decade, significant advancements in hemorrhagic disorder management have markedly improved perioperative outcomes. The development of recombinant clotting factors, targeted antifibrinolytic therapies, and individualized hemostatic protocols has enhanced control in inherited bleeding disorders such as hemophilia and VWD.2 Gene therapy offers a promising avenue for sustained endogenous production of deficient clotting factors, which, if adapted for VWD, could transform future management.2 We discuss the perioperative management of a patient with Type 2A vWD undergoing gynecologic surgery.
Case Presentation: A 33-year-old female with Type 2A VWD, diagnosed at 19, was scheduled for elective hysteroscopy, dilation and curettage, and endometrial ablation. Her history included menorrhagia, easy bruising, recurrent epistaxis, and post-cesarean hemorrhage.
Hematologic recommendations included 1300 units of rVWF (Vonvendi) the day before surgery, preoperative TXA (1000 mg), and postoperative TXA (1300 mg every six hours for 48 hours). DDAVP was avoided due to prior inadequate response. To minimize airway trauma, general anesthesia with a laryngeal mask airway (LMA) was selected.
During surgery, an unexpected uterine perforation necessitated laparoscopic repair. To prevent additional airway trauma, fiberoptic-guided endotracheal intubation via the LMA was performed. Intraoperative blood loss was minimal, and postoperative hemostatic support ensured an uneventful recovery and discharge home the same day.
Conclusion: This case highlights multidisciplinary management of vWD in a community hospital setting. Careful perioperative planning and adaptive airway management were critical to optimizing outcomes. The integration of therapeutic modalities, such as emerging hemophilia treatments, holds promise for further improving perioperative hemostatic control in VWD. This case exemplifies how state-of-the-art strategies, combined with a collaborative multidisciplinary approach, are enhancing surgical safety and outcomes for patients with bleeding disorders.
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References
DOI:10.1182/bloodadvances.2020002046
DOI:10.1056/NEJMra2034154
DOI: 10.2147/JBM.S9890