30 Chapter 2 Regardless adequate prophylaxis, we found an increased incidence of intraoperative bleeding events in VWD women who underwent hysterectomy as compared to EA (13% vs 0%) or compared to the incidence of adverse bleeding events in women undergoing a hysterectomy without VWD (4%) as reported by a Cochrane review comparing EA to any type of hysterectomy performed in healthy women with HMB. (26) Also, VWD women received more often blood transfusions during or after hysterectomy compared to the general population. This is likely due to the fact that in a significant part of the women who underwent a hysterectomy, the diagnosis VWD was made after surgery. (3, 21) Blood transfusions have the power to save lives, but this limited resource can also harm patients by transfusion-associated reactions and generate excessive costs. EA offers an alternative to hysterectomy as a surgical treatment for HMB. (26) However, the higher rate of adverse bleeding events in hysterectomy versus EA emphasizes impact of diagnosing possible underlying bleeding disorders in women with HMB before surgery. (26-29) Therefore, this review contributes to importance of attention for underlying bleeding disorders in daily gynecological practice. (30) At least an assessment of the patient’s bleeding history, evaluation for mild bleeding disorder using a validated bleeding assessment tool or screening on bleeding disorders for risk assessment should be part of consultation for women with HMB in optimal gynecologic care process. (31) EA is a reliable technique and safe procedure in VWD women who experience HMB. (15-20, 22) In contrast to healthy women, all women with VWD received preprocedural hemostatic agents during EA. Our findings support current national and international guidelines recommending primary prophylaxis before gynecological surgery in patients with VWD for the prevention of bleeding complications during and after surgery. (32) Taking into consideration the evolved EA techniques and the absence of bleeding complications during EA presented here, the question arises whether it is necessary to give prophylaxis to all, some or maybe none of the women with VWD during the EA procedure. So, optimizing prophylactic strategy is needed to avoid the risk of over-dosing, leading to unnecessary treatment and costs versus risking under-dosing, which may lead to adverse bleeding events. The included studies did not and could not be due to the lack of power, examine the relationship between bleeding complications and the severity of the type of VWD, possible prophylactic and therapeutic perioperative strategies. Prophylaxis was described in 4% of the hysterectomy (20, 22, 23) vs 100% of the EA procedures. (15-20, 22) The diversity of the included study population (VWD type 1, type 2A, type 2N, type 3, acquired), the variousness per type of prophylactic treatment strategy and unknown gynecological confounders, make subpopulation analysis and future recommendations unreliable. Also, the definition of surgery-related bleeding complications differed between the included studies. Although most studies used intra- and postoperative bleeding as a lower limit, (3, 20-22, 24) haematoma, (23) transfusion, (21, 23) or death (21) were also used. Limitations that need to be addressed concern the restriction to English, German and Dutch literature and the heterogeneity and observational retrospective nature of studies. Because of small numbers of included VWD women and the study design, the overall quality of evidence
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