21 Systematic review INTRODUCTION Von Willebrand Disease (VWD) is the most common autosomal inherited bleeding disorder in women and is classified as: VWD type 1 (too low von Willebrand factor (VWF)-antigen level), VWD type 2 (dysfunctional VWF-antigen) or VWD type 3 (absent of VWF-antigen). Heavy menstrual bleeding (HMB) occurs in 64-74% of women with VWD, for which a significant percentage of women (20%) undergo a surgical procedure (1), (2), (3) Endometrial ablation (EA) was introduced to achieve long-term reduction of HMB without disruption of the anatomy. (4-6) Patients experience a high satisfaction on symptom relief, with a low incidence of bleeding complications (2%) and EA is therefore nowadays predominantly used as primary surgical option in women with HMB. (5-8) However, women with HMB and VWD opt more often for a hysterectomy due to the advantage of permanent relief of HMB. (3, 9, 10) As surgical techniques for both hysterectomy and EA continue to evolve, corresponding changes in bleeding complications rate should be noted, entail this review for VWD women. Apart from this, limited information is available regarding the variety of potential prophylactic treatment strategies in VWD women who underwent EA or hysterectomy. Therefore, we aimed to summarize the up-to-date evidence on bleeding events and to address optimal prophylactic and therapeutic perioperative strategies in VWD women for the surgical procedures of HMB to benefit clinical decision-making. METHODS Protocol and registration This review protocol was registered at PROSPERO (CRD42018104535) (https://www.crd.york. ac.uk/prospero/) and conducted according to the PRISMA 2020 guidelines. (11) All articles reporting on women with any type of VWD and HMB who either underwent second generation EA or hysterectomy were eligible for inclusion. Only articles written in English, German or Dutch, containing original patient data and reporting of outcome bleeding complications and/or satisfaction rate were included. Excluded were studies with unclear reporting of type bleeding disorder or unclear reporting bleeding complications and studies conducted with other type than second-generation EA. The review questions were: 1. Which prophylactic and therapeutic strategies for intraoperative bleeding in VWD women undergoing EA or hysterectomy for HMB have been published? 2. What is the relation of these prophylactic and therapeutic strategies towards intraoperative bleeding in VWD women undergoing EA or hysterectomy? 3. How often did HMB reoccur after EA and if so, where further surgical procedures undertaken? Search and study selection The complete search syntax was developed in collaboration with a librarian. We performed an electronic search in January 2023 in PubMed, EMBASE, Cochrane databases and the Register of Current Controlled Trials (Supplement S1. Full search string all databases). We limited the results to literature published from January 1, 1994, to January 1, 2023, to increase the likelihood that the second-generation EA, currently (the most common used method), was used. 2
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