Abdominoplasty in a Patient With Type 3 von Willebrand Disease: A Case Report.

ABSTRACT: 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.

Into a brave new world: Haemophilia A & von Willebrand Disease Surgery with novel therapies.

INTRODUCTION: Haemophilia & von Willebrand disease are both recognised inherited bleeding disorders. With increased access to highly efficient and safe replacement and novel therapies, management of surgical interventions in this group can be safely managed by experienced multidisciplinary teams. AIM: To review the evidence for managing surgery in the era of novel therapies. METHOD: We explore four cases and establish the role of the clinical nurse specialist within the surgical pathway. RESULTS: All of these cases evidence the continued important role of both the multidisciplinary planning prior to any surgical interventions in people with bleeding disorders and the key role of the Nurse Specialist in ensuring this plan is then implemented. Key focuses of communication with all parties involved in the patient journey, performing education for the patient, family and wider healthcare team about the underlying bleeding disorder and the importance of time critical medicines being given on time is essential. CONCLUSION: These cases demonstrate that individuals with bleeding disorders are at risk of developing other rare conditions alongside their life long condition, in addition to comorbidities associated with ageing. Evidence for rare plus rare is likely to be minimal as demonstrated within the cases, and recognition of how to reach out to international peers in the field is important. Sharing complex case management at national & international meetings and in publication has never been so important.

Utilization of a surgical database to provide care and assess perioperative treatment and outcomes in patients with bleeding disorders.

OBJECTIVES: To describe the Indiana Hemophilia and Thrombosis Center (IHTC) surgical database, its key components, and exploratory analyses of surgeries conducted between 1998 and 2019. METHODS: Surgical data across bleeding disorders collected retrospectively (1998-2006) and prospectively (2006-2019) were analyzed. Perioperative hemostasis, complications, and surgical plan deviations were compared by bleeding disorder diagnosis and data collection period. RESULTS: Within the 21-year period, 3246 procedures were conducted in 1413 patients with a diagnosis of von Willebrand disease (vWD), hemophilia A (HA), hemophilia B (HB), and other bleeding disorders. Majority of the procedures were minor (63.3%), and median number of surgeries per patient was 1 (range: 1-22). Adequate perioperative hemostasis was achieved in 90.9%, complications occurred in 13.6%, and surgical plan deviations occurred in 31.3% of procedures. Inadequate perioperative hemostasis and surgical plan deviations occurred more frequently in procedures involving HB compared with other bleeding disorders. Complications were not significantly different across bleeding disorders (p = .164). The prospective data collection period was associated with higher rates of hemostatic efficacy (92.4% vs. 88.3%; p < .001), complications (14.3% vs. 12.3%; p < .001), and plan deviations (34.2% vs. 25.1%; p < .001). CONCLUSION: The surgical database is an important resource in surgical management in patients with bleeding disorders. Further evaluation will facilitate use for the development of predictive models and principles of care.

Successful Perioperative Management of Orthotopic Cardiac Transplantation in a Pediatric Patient With Concurrent Congenital von Willebrand Disease and Acquired von Willebrand Syndrome Using Recombinant von Willebrand Factor.

Von Willebrand disease (VWD) is the most common bleeding disorder and reportedly affects 1:1,000 of the world's population. There are three subtypes of VWD characterized by a quantitative defect (types 1 and 3 VWD) or a qualitative defect (type 2 VWD). Type 1 VWD results in a partial deficiency of von Willebrand factor (VWF) and affects approximately 75% of individuals with VWD, whereas type 3 VWD results in a severe or complete deficiency of VWF. Individuals with type 2 VWD subtypes (types 2A, 2B, 2M, and 2N VWD) express a dysfunctional VWF protein that has impaired interactions with platelets or factor VIII. The majority of individuals with VWD have mild type 1 VWD and occasionally require bolus infusions of VWF for severe bleeding or major surgery. A subset of patients, especially those with type 2A or 3 VWD, may require more frequent VWF replacement or prophylaxis for refractory bleeding or bleeding prevention, respectively. Acquired von Willebrand syndrome (AVWS) is a rare bleeding disorder that primarily occurs as a result of an underlying disease or other pathologic mechanism. Cases of AVWS associated with heart valve defects, left ventricular assist devices, or congenital cardiac disease result from high shear stress in the circulation that induces VWF unfolding and subsequent proteolysis of high-molecular-weight multimers by ADAMTS-13. In rare instances, plasma-derived factor VIII-containing VWF concentrates have been administered to individuals with AVWS for persistent or challenging bleeding events. In this case report, the hemostatic challenges and the perioperative management of cardiac transplantation surgery using a novel recombinant VWF product in a pediatric patient diagnosed with AVWS concomitant with congenital type 1 VWD are described. Written informed consent was obtained from the patient's mother for this case report. The diagnosis of congenital VWD remains a challenge because of multiple potential modifiers that can alter VWF laboratory results. Concurrent conditions, such as congenital heart disease and the rare secondary condition of AVWS, in addition to congenital VWD, can further affect interpretation of coagulation studies. This can result in delays in diagnosis, increase severity of the bleeding phenotype, and complicate hemostatic management in individuals at risk for bleeding and thrombosis. A multidisciplinary approach, including anesthesiologists, cardiologists, cardiovascular surgeons, hematologists, and pharmacists, is critical to achieving optimal patient outcomes, as highlighted in this case report. As diagnostic capabilities and understanding of VWD broaden, future studies evaluating alternative treatment approaches for individuals with various types of VWD would be of great benefit to the medical community.

Do patients with von Willebrand disease exhibit higher blood loss and revision rates in hip and knee arthroplasty? A case-control study.

BACKGROUND: The aim of the study was to compare the perioperative blood loss, need for transfusion and one-year revision rates in patients undergoing hip and knee arthroplasty who also have a diagnosis of von Willebrand disease (VWD) with a matched control group. METHODS: A retrospective single-centre case-control study was conducted. Fifty-eight patients with VWD and 116 controls (1:2 match) who were operated for primary or revision hip and knee arthroplasty at our hospital were included. Blood loss, haemoglobin (Hb)-drop, need for blood transfusion, intraoperative complications and revision rates within one year were noted in all cases. Outcome measures for subgroups of the primary hip, primary knee, revision hip and revision knee procedures, were also analysed. RESULTS: The mean perioperative Hb-drop was 3.47 (±1.27) g/dL and blood loss was 293 (±97) ml for the VWD group while Hb-drop was 2.85 (±1.21) g/dL and blood loss was 232 (±105) mL for the control group (P < .001). There were no significant increased transfusion rates (P = .264) and revision rates in the VWD group (P = .634). Patients having primary hip surgery had significantly higher Hb-drop (3.68 ± 1.25 g/dL vs 2.62 ± 1.19 g/dL; P = .003), higher blood loss (293 vs 203 mL; P = .002) and increased need for a transfusion (21% vs 2.6%; P = .038) compared to the controls. No outcome measure was found to be significantly different for primary and revision knee surgery. CONCLUSIONS: The results of this study suggest that patients with VWD undergoing primary or revision total hip and knee arthroplasty have higher levels of blood loss than the control cohort. Perioperative protective measures including meticulous surgical techniques should be considered.

Hepatectomy in patients with inherited blood coagulation disorders can be safely performed with adequate coagulation factor replacement.

BACKGROUND: Haemophilia and von Willebrand disease (VWD) are common inherited bleeding disorders. Although patients with haemophilia or VWD have a high risk of hepatitis virus infection and hepatocellular carcinoma (HCC), little is known about the safety of liver resection in these patients. METHODS: From 2006 to 2016, there were seven hepatectomies with haemophilia A and three hepatectomies with VWD for malignant liver tumours at tertiary care hospitals in Japan and Switzerland. To evaluate the safety of hepatectomy in the blood coagulation disorder group (BD group), short-term outcomes in these patients were compared with 20 hepatectomies (non-BD group) for HCC, matched to a 2:1, operative procedure, period and background liver. RESULTS: Ten liver resections were performed in patients with haemophilia or VWD with administration of recombinant FVIII or VWF concentrate. Comparison of the BD vs non-BD group revealed no significant differences in the operative time (327 vs 407 minutes, P = 0.359), estimated blood loss (730 vs 820 mL, P = 0.748), red blood cell transfusion rate (10.0% vs 5.0%, P = 0.605), major complication rate (Clavien-Dindo grade III or IV) (10.0% vs 5.0%, P = 0.605) or mortality rate (0% vs 0%, P > 0.999). Additionally, the length of the postoperative hospital stay was similar between the two groups (13 vs 14 days, P = 0.296). CONCLUSION: Liver resection for treatment of HCC in patients with haemophilia or VWD can be safely performed through an appropriate perioperative administration protocol of coagulation factors.

Surgical procedures in patients with severe haemophilia 1997­2014. / Kirurgiske inngrep hos pasienter med alvorlig blødersykdom 1997­2014.

BACKGROUND: As a result of good medical treatment, the life expectancy of patients with severe haemophilia is now approaching normal. This implies a growing need for treatment of lifestyle- and age-related disease. This article describes surgery in patients with severe haemophilia in the period 1997-2014. MATERIAL AND METHOD: Data were retrieved from the registry linked to the national treatment service for surgery, intervention and advanced diagnostics for haemophilia. The patients were categorised according to type of haemophilia and type of intervention in orthopaedic and non-orthopaedic surgical procedures. RESULTS: A total of 825 surgical procedures were undertaken in 286 patients. The number of procedures increased from 21 in 1997 to 66 in 2014. This increase was associated with non-orthopaedic interventions: altogether 4 such procedures were undertaken in 1997 and 45 in 2014. The number of orthopaedic interventions varied somewhat from year to year, but no clear trend was evident. INTERPRETATION: With increased life expectancy, we are seeing a growing need for treatment of diseases that are not causally related to haemophilia. Doctors with little experience of patients with severe haemophilia will need to deal with lifestyle- and age-related disease in this patient group.

Analysis of current perioperative management with Haemate® P/Humate P® in von Willebrand disease: Identifying the need for personalized treatment.

INTRODUCTION: Patients with Von Willebrand disease (VWD) are regularly treated with VWF-containing concentrates in case of acute bleeding, trauma and dental or surgical procedures. AIM: In this multicentre retrospective study, current perioperative management with a von Willebrand factor (VWF)/Factor VIII (FVIII) concentrate (Haemate® P) in patients with VWD was evaluated. PATIENTS/METHODS: Patients with VWD undergoing minor or major surgery between 2000 and 2015, requiring treatment with a VWF/FVIII concentrate (Haemate® P), were included. Achieved VWF activity (VWF:Act) and FVIII during FVIII-based treatment regimens were compared to predefined target levels in national guidelines. RESULTS: In total, 103 patients with VWD (148 surgeries) were included: 54 type 1 (73 surgeries), 43 type 2 (67 surgeries) and 6 type 3 (8 surgeries). Overall, treatment resulted in high VWF:Act and FVIII levels, defined as ≥0.20 IU/mL above predefined levels. In patients with type 1 VWD, respectively, 65% and 91% of trough VWF:Act and FVIII levels were higher than target levels. In patients with type 2 and type 3 VWD, respectively, 53% and 57% of trough VWF:Act and 72% and 73% of trough FVIII levels were higher than target level. Furthermore, FVIII accumulation over time was observed, while VWF:Act showed a declining trend, leading to significantly higher levels of FVIII than VWF:Act. CONCLUSION: High VWF:Act and accumulation of FVIII were observed after perioperative FVIII-based replacement therapy in patients with VWD, both underlining the necessity of personalization of dosing regimens to optimize perioperative treatment.

Laboratory monitoring of replacement therapy for major surgery in von Willebrand disease.

Von Willebrand disease (VWD) is an inherited haemorrhagic disorder caused by a quantitative or qualitative defect of von Willebrand factor (VWF), a multimeric plasma glycoprotein that plays a key role in platelet adhesion to the subendothelium and acts as a carrier of factor VIII (FVIII) in blood. Patients with VWD experience bleeding symptoms that are mainly localized in mucous membranes and soft tissues, and their severity depends on the degree of the primary reduction in VWF and the secondary deficiency of FVIII in plasma. Because VWD patients are also at increased risk of perioperative bleeding, a prophylactic treatment aimed to correct the dual haemostatic defect (i.e. VWF and FVIII) is warranted. This review summarizes knowledge on the current management of patients undergoing major surgery, focusing on the peri-surgical laboratory monitoring of replacement therapy with VWF/FVIII concentrates. We suggest to monitor plasma levels of FVIII coagulant activity in the postoperative period rather than a surrogate maker of platelet-binding VWF activity as the ristocetin cofactor assay and its recent modifications.

Efficacy and safety of a VWF/FVIII concentrate (wilate® ) in inherited von Willebrand disease patients undergoing surgical procedures.

INTRODUCTION: Surgical procedures in von Willebrand disease (VWD) patients may require prophylactic treatment with exogenous von Willebrand factor (VWF) and coagulation factor VIII (FVIII) to prevent excessive bleeding. Wilate® is a plasma-derived, double virus-inactivated, highly purified, freeze-dried VWF/FVIII concentrate, containing both factors in a physiological activity ratio of 1:1. AIM: To investigate the efficacy and safety of wilate® in maintaining haemostasis in VWD patients undergoing surgical procedures. METHODS: This prospective, open-label multinational clinical study documents 28 individuals who underwent 30 surgical procedures managed with wilate® . Twenty-one patients had VWD Type 3, and 21 surgeries were major. Efficacy was assessed intra- and postoperatively by the surgeon and investigator, respectively, and adjudicated by an Independent Data Monitoring Committee, using an objective scale based on blood loss, transfusion requirements and postoperative bleeding and oozing. Treatment success (primary endpoint) was determined using a composite assessment algorithm and was formally assessed. RESULTS: Surgical prophylaxis with wilate® was successful in 29 of 30 procedures. The overall rate of success was 96.7% (98.75% CI: 0.784, 1.000). All 21 surgeries in patients with VWD Type 3 were managed successfully. There was no accumulation of VWF or FVIII after multiple dosing, and no thromboembolic events or inhibitors to VWF or FVIII were observed. CONCLUSIONS: Wilate® demonstrated effective prevention and treatment of bleeding in inherited VWD patients undergoing surgery, with no clinically significant safety concerns.

Perioperative bleeding and thrombotic risks in patients with Von Willebrand disease.

Von Willebrand disease (VWD) is an inherited bleeding disorder that often manifests clinically with hemorrhage after invasive procedures. We investigated the association between a diagnosis of VWD and bleeding and thrombotic outcomes following major non-cardiac surgery in a large national database from the United States. Patients age ≥45 years requiring major non-cardiac surgery were identified from Healthcare Cost and Utilization Project's National Inpatient Sample data. Von Willebrand disease, perioperative major adverse cardiovascular events (MACE), thrombotic events, and hemorrhage were defined by ICD9 diagnosis codes. From 2004 to 2013, a total of 10,581,621 hospitalizations for major non-cardiac surgery met study inclusion criteria and VWD was identified in 3765 (0.036%). In adjusted analyses, patients with VWD were significantly more likely to develop post-operative hemorrhage than patients without VWD (5.5 vs. 1.9%, p < 0.001; adjusted OR 3.49, 95% CI 3.03-4.03), but had similar odds of perioperative MACE and thrombotic events. Thus, a diagnosis of VWD was associated with increased risks of bleeding with non-cardiac surgery, without a corresponding reduction in perioperative thrombosis in comparison to patients without VWD. Perioperative management of patients with hereditary bleeding disorders and mitigation of thrombotic risks requires further study.

Practical aspects of DDAVP use in patients with von Willebrand Disease undergoing invasive procedures: a European survey.

INTRODUCTION: Desamino D-arginine vasopressin (DDAVP or desmopressin) is a useful and effective haemostatic treatment for patients with von Willebrand Disease (VWD). However, there are still issues regarding in which subtypes of VWD DDAVP is appropriate and little consensus on its use in different surgical settings. We also lack information concerning the appropriate laboratory parameters that should be monitored. AIM: The European Haemophilia Therapy Strategy Board (EHTSB) wished to investigate published information and clinical use of DDAVP in VWD patients. METHODS: We conducted a literature survey on management of VWD during surgical interventions and undertook a survey of specialist haematologist centres across Europe to assess current management of VWD patients. RESULTS: DDAVP is ineffective in type 3 VWD and its use in type 2B remains controversial due to the possibility of thrombocytopenia. It can, however, be used effectively to cover minor surgery and dental procedures in most other VWD patients. For major surgery there is wider use of factor concentrate in preference to DDAVP depending on the subtype of VWD. We give consensus recommendations on the use of DDAVP for surgical interventions in VWD including laboratory parameters that denote an adequate response and contraindications to its use. CONCLUSIONS: DDAVP can be recommended to cover invasive procedure in selected patients with VWD, however, we need more information and systematic recording of adverse events associated with DDAVP use in VWD. A companion paper will be published covering the use of factor concentrates in VWD patients.

Total ankle replacement in patients with von Willebrand disease: mid-term results of 18 procedures.

von Willebrand disease (VWD) is a recognized cause of secondary ankle osteoarthritis (OA). Few studies have examined orthopaedic complications and outcomes in VWD patients treated for end-stage ankle OA with total ankle replacement (TAR). To determine the clinical presentation, intraoperative and postoperative complications and evaluate the mid-term outcome in VWD patients treated with TAR. Eighteen patients with VWD with mean age 47.3 years (range = 34.0-68.7) were treated for end-stage ankle OA with TAR. The mean duration of follow-up was 7.5 years (range = 2.9-13.2). Intraoperative and perioperative complications were recorded. Component stability was assessed with weight-bearing radiographs. Clinical evaluation included range of motion (ROM) tests using a goniometer and under fluoroscopy using a lateral view. Clinical outcomes were analysed by a visual analogue scale, the American Orthopaedic Foot and Ankle Society hindfoot score and Short Form (36) Health Survey (SF-36) health survey. One patient sustained an intraoperative medial malleolar fracture. In two patients delayed wound healing was observed. Two secondary major surgeries were performed. Pain level decreased from 8.2 ± 0.9 (range = 7-10) preoperatively to 1.1 ± 1.2 (range = 0-4) postoperatively. Significant functional improvement including ROM was observed. All categories of SF-36 score showed significant improvement in quality of life. Mid-term results of TAR in patients with VWD are encouraging. The total rate of intraoperative and postoperative complications was 33.3%. However, longer term outcomes are necessary to fully understand the clinical benefit of TAR in patients with VWD.

Wilate use in 47 children with von Willebrand disease: the North London paediatric haemophilia network experience.

Children with von Willebrand disease (VWD) in whom DDAVP is ineffective or contraindicated require treatment with a coagulation factor concentrate containing von Willebrand factor (VWF) and factor VIII (FVIII). The aim of this study was to monitor the safety, efficacy and tolerability of Wilate(®) (a VWF:FVIII concentrate with a 1:1 ratio) used across the North London Paediatric Haemophilia Network since May 2010. In total, 47 children (aged 0.0-17.0 years) with type 1 (n = 28), type 2 (n = 7), type 3 (n = 10) and acquired VWS (n = 2) have been treated for bleeds, surgery and/or prophylaxis using 260 000 IU Wilate(®). Analysis of dose and frequency of treatment show expected responses to treatment with mean doses of 55, 50 and 50 IU kg(-1) for bleeds, surgery and prophylaxis respectively. Most bleeds responded to a single treatment. Surgical procedures were covered with clinician approved dosing schedules with 95% (39/41) reported as having excellent or good efficacy. There was no accumulation of FVIII or VWF and no thromboembolic events. This case series confirms the efficacy, safety and tolerability of Wilate(®) in neonates, children and adolescents when used on-demand, prophylactically and in the surgical setting.

[Total ankle replacement in patients with bleeding disorders]. / Endoprothetischer Ersatz des oberen Sprunggelenks bei Patienten mit Gerinnungsstörungen.

BACKGROUND: Total ankle replacement (TAR) is a well-accepted treatment option in patients with end-stage ankle osteoarthritis. However, published literature on patients with bleeding disorders treated with TAR is limited. Therefore, we carried out this prospective study to analyze mid-term postoperative results in patients with bleeding disorders treated by TAR. METHODS: A total of 34 patients with end-stage ankle osteoarthritis--14 patients with hemophilia type A and 20 patients with von Willebrand disease (VWD)--treated by TAR were included in this prospective study. The mean age of patients was 46.0 ± 9.0 years. Intraoperative and postoperative complications were recorded. The postoperative pain relief and functional results including range of motion (ROM) and American Orthopaedic Foot and Ankle Society (AOFAS) hindfoot score were assessed after a mean follow-up of 6.3 ± 3.4 years. Additionally, the quality of life was analyzed using the SF-36 questionnaire. The alignment of prosthesis components was assessed using weight-bearing conventional radiographs. The results were compared with those obtained in the control group, including 72 and 33 patients with post-traumatic and rheumatoid ankle osteoarthritis respectively. RESULTS: One patient sustained an intraoperative medial malleolar fracture. In total, three revision surgeries were necessary in our patient cohort. There was significant pain relief from 8.2 ± 0.8 to 0.9 ± 1.0, as assessed using a visual analog scale. All categories of the SF-36 score showed significant improvement. The average ROM increased from 20.1° ± 6.9° to 27.5° ± 7.4°. The AOFAS hindfoot score increased from 34.5 ± 10.0 to 82.4 ± 10.2 of a maximum of 100 points. Radiographic assessment showed the neutral alignment of prosthesis components in all patients. The postoperative clinical and radiographic outcomes were comparable in both patients with hemophilia and those with VWD. Patients with bleeding disorders had significantly higher pain relief and significantly lower ROM than the patients in the control group with ankle osteoarthritis of post-traumatic or rheumatoid etiology. CONCLUSION: Our prospective study revealed encouraging mid-term outcomes after TAR in patients with bleeding disorders. However, this surgery should be limited to highly experienced foot and ankle surgeons. Furthermore, this patient cohort requires a multidisciplinary approach to ensure a good outcome.

Predicting operative bleeding in elective pediatric surgeries using the Pediatric Bleeding Questionnaire (PBQ).

To test the Pediatric Bleeding Questionnaire's (PBQ) utility for predicting excessive bleeding during elective surgery, we retrospectively evaluated 60 pediatric patients who had been preoperatively investigated for von Willebrand disease. Of 58 patients with bleeding scores (BS) ≤2 (within normal range), 1 hemorrhaged during surgery. Two had a BS of >2 (positive BS); however, neither bled excessively during surgery. The resulting sensitivity, specificity, positive predictive values, and negative predictive values of the PBQ are 0%, 97%, 0%, and 98%, respectively. On the basis of this small sample, we cannot recommend the PBQ for screening in this setting.

Perioperative hemostasis management in patients with von Willebrand disease: an institutional experience.

OBJECTIVES: Patients with von Willebrand disease (vWD) undergoing surgery are routinely treated with von Willebrand factor (vWF)/factor VIII (FVIII) concentrate to control bleeding risk, but consensus is lacking on optimal dosing. This study aimed to evaluate the efficacy and safety of tailored doses of vWF/FVIII concentrate according to intervention-associated bleeding risk in vWD patients undergoing surgery. METHODS: This was a retrospective analysis of vWD patients who underwent surgical procedures at a haemophilia centre. Patients received vWF/FVIII concentrate with dosage and duration of treatment dependent on intervention type (dental, gynaecological, abdominal or orthopaedic/traumatic) and bleeding risk (moderate/high). RESULTS: Eighty-three surgical procedures (42 patients) were included. Median preoperative loading doses of vWF/FVIII concentrate were 29.9 IU/kg and 35.7 IU/kg for interventions with moderate ( n  = 16) or high ( n  = 67) bleeding risk, respectively. The median perioperative dose was highest in orthopaedic or trauma-related surgery (140 IU/kg) and lowest in dental or gynaecological interventions (76.4 IU/kg and 80.0 IU/kg, respectively). During follow-up, no bleeding or other complications were observed in 95% of patients. CONCLUSIONS: Individually tailored doses of vWF/FVIII concentrate according to intervention-associated bleeding risk were effective in preventing postoperative bleeding, with few complications observed. These doses may be used as guidance in routine clinical care.