Prophylactic and therapeutic strategies for intraoperative bleeding in women with von Willebrand disease and heavy menstrual bleeding: A systematic review.

BACKGROUND: Optimal peri-operative management for women with Von Willebrand disease (VWD) and heavy menstrual bleeding (HMB) remains undetermined. AIM AND METHODS: To evaluate (pre)operative management in relation to (post)operative bleeding after endometrial ablation (EA) and hysterectomy in VWD women with HMB by performing a database search between 1994 and 2023. RESULTS: Eleven cohort studies and 1 case-report were included, of overall 'low' quality, describing 691 operative procedures. Prophylaxis (Desmopressin, clotting factor concentrates or tranexamic acid) to prevent bleeding was described in 100% (30/30) of EA procedures and in 4% (24/661) of hysterectomies. Bleeding complications despite prophylaxis were described in 13% (3/24) of hysterectomies vs 0% (0/30) in EA. CONCLUSION: VWD women often seem to experience bleeding complications during hysterectomy and all women with VWD received preprocedural hemostatic agents during EA, indicating potential under- and overdosing of current prophylactic strategies. Prospective studies are needed to determine the optimal (pre)operative strategy for gynecological surgical procedures in women with VWD.

Ten-year retrospective evaluation of therapeutic choices and related satisfaction in patients with auricular deformities.

To adequately counsel future patients with auricular abnormalities, this study aimed to analyze the therapeutic choices patients make after consultation concerning their auricle and/or hearing. All patients who visited the auricle consultation between January 2010 and January 2020 were included. This comprises patients with all types of auricular anomalies of both congenital and acquired etiology. Follow-up was performed by telephone to obtain informed consent and to take a short nonvalidated questionnaire regarding their therapeutic choices and satisfaction with these choices. Patient characteristics and information regarding therapeutic choices after consultation were subsequently obtained from the patients' medical files. The main outcome parameters were the patients' therapeutic choices regarding the auricle and hearing. A total of 134 patients (61.2%) were included with a mean follow-up of 3.85 years. For congenital and acquired etiologies, 72.8% and 18.2% declined reconstruction, 14.0% and 18.2% chose autologous, 5.3% and 0% chose alloplastic, 6.1% and 45.4% chose prosthetic, and 1.8% and 18.2% chose another type of reconstruction, respectively. A total of 54.9% declined hearing rehabilitation, while 38.2% chose percutaneous bone conduction device (BCD), 2.0% transcutaneous BCD, and 4.9% BCD on a softband. The overall satisfaction score was 8.7/10 concerning the auricle and 8.3/10 regarding hearing. In conclusion, most patients with a congenital etiology chose to wait or decided against auricular reconstruction, while patients with acquired etiologies mostly opted for reconstruction by a prosthesis. Patients or their caregivers were very satisfied with their choice, regardless of whether this implies reconstruction or no reconstruction, which emphasizes the importance of shared decision-making and thorough counseling.

Women prefer proactive support from providers for treatment of heavy menstrual bleeding: A qualitative study in adult women with moderate or severe Von Willebrand disease.

OBJECTIVE: To explore key factors for successful support in women with moderate or severe Von Willebrand disease (VWD) who are faced with heavy menstrual bleeding (HMB) and surgery. DESIGN: A qualitative study design with focus-group interviews and thematic analysis of the discussions. SETTINGS AND POPULATION: Eleven VWD women aged 41-68 years (median age 58 years) who had had a hysterectomy or bipolar radiofrequency ablation (BRA) because of HMB participated in this study. Three of the 11 participants had VWD diagnosed before surgery. Two focus groups were conducted in the summer of 2012. Patients were identified through participation in a nationwide study on Von Willebrand disease in the Netherlands (WiN study). Inclusion criteria were at least 18 years of age, fluent in Dutch, diagnosed with VWD (based on Von Willebrand factor (VWF) antigen and/or activity levels < 30 IU/dL) and previous surgical therapy for HMB. FINDINGS: The following key factors were identified during focus-group interviews: receiving information, proactive support from providers and considering bleeding disorders as a cause of HMB. Other topics were as follows: experiences with VWD and/or surgery, how relieved patients were when menses stopped, patients hoped that in future, providers would work better together so that women receive the best care. CONCLUSIONS: In this focus-group study among women with VWD who underwent surgery because of HMB, support by professionals could be improved by considering a bleeding disorder in women with HMB, providing information about different types of surgery and shared decision-making regarding type of interventions.

High prevalence of reduced thrombin generation and/or decreased platelet response in women with unexplained heavy menstrual bleeding.

INTRODUCTION: Heavy menstrual bleeding (HMB) is a condition that affects 20%-30% of women of reproductive age. HMB has a multifactorial pathophysiology, which is incompletely understood. HMB symptoms are very common in patients with established haemostasis defects, likewise, women with heavy menstrual bleeding have a higher prevalence of impaired Von Willebrand factor (VWF) levels and function, thrombocytopenia, impaired platelet function and impaired coagulation. The aim of this study was to quantify the prevalence of impaired platelet function, impaired coagulation and reduced VWF activity in patients with HMB. METHODS: We have used thrombin generation (TG), a flow cytometry-based platelet function test and a flow cytometry-based VWF function test to study haemostasis in 58 women (median age: 48.4 years, range 40-60 years) with HMB. In addition, we determined VWF antigen levels and VWF ristocetin co-factor activity in platelet-poor plasma. Reference ranges of platelet function were measured in whole blood of 123 healthy volunteers, while reference ranges of TG were determined in platelet-poor plasma (PPP) of 126 healthy volunteers. RESULTS: Fourteen (24%) patients with HMB had impaired platelet function and 17 (29.3%) patients had impaired coagulation. Five patients (8.6%) had both impaired platelet function and impaired coagulation. Only 2 (3.4%) patients had an impaired VWF function or levels; one of them was in combination with impaired coagulation. CONCLUSION: Our approach in women with HMB using a high precision platelet function test in combination with thrombin generation showed impaired coagulation or impaired platelet function in more than 40% of the patients.