Surgery in patients with von Willebrand disease

Abstract

Von Willebrand disease (vWD) is the most common inherited bleeding disorder. The biggest challenge in the management of vWD is the difficulty in performing surgical procedures because of bleeding. Treatment guidelines recommend the use of pure von Willebrand factor or von Willebrand factor/factor VIII (vWF/FVIII) concentrate in patients with type 2 or type 3 vWD undergoing surgery, in patients with type 1 vWD undergoing surgery who are unresponsive, and in patients for whom desmopressin acetate is contraindicated. However, there is no consensus on the dosage and optimum levels of these factors to date. The aim of this study was to evaluate the perioperative management of patients with vWD during surgical procedures. Data pertaining to surgical procedures performed at our center from 2003 to 2014 were analyzed retrospectively. All operations, except one appendectomy, were elective, and a plan for the management of hemostasis was prepared for each patient. During this period, 37 surgical procedures (major, minor, and dental) were performed in 23 patients with vWD. Complications occurred in four out of 37 procedures (10.8%). No deaths or life-threatening bleeding occurred during any of the operations and no thromboembolic events were observed. The results from this retrospective study indicate that surgery can be safely performed by providing adequate and timely hemostasis during and after the procedure in patients with vWD. Perioperative and postoperative bleeding complications are rare when patients are closely and carefully monitored.

Perioperative bleeding and thrombotic risks in patients with Von Willebrand disease

“…Adjunctive therapies, such as intravenous immunoglobulins and tranexamic acid, have also been reported. [7,8] Although current measures to mitigate surgical bleeding in WVD appear to be safe and well tolerated in small series, effects of pharmacology on perioperative thrombotic risks remain uncertain. [7–9]…”

<p>Potential Undiagnosed VWD Or Other Mucocutaneous Bleeding Disorder Cases Estimated From Private Medical Insurance Claims</p>

“…[7][8][9][10][11][12][13] Patients with VWD are also exposed to a higher risk of future bleeding complications from pregnancy, childbirth, surgical and dental procedures, and minor injuries, which can be managed with appropriate intervention following diagnosis. [14][15][16] We showed recently, through longitudinal analysis of medical insurance claims data from a commercially insured US population, that VWD patients' need for bleed care (number of medical claims for VWD or bleeding events and prescription claims) was substantially reduced after (vs before) VWD diagnosis. 17 However, misrecognition of VWD was common, with a quarter of patients visiting the same health care specialty for episodic bleed care at least twice before diagnosis.…”

“…Diagnosis is important for appropriate management of VWD, and thereby for minimizing the burden of bleeding symptoms and complications on patients and the health care system. 3,9,[14][15][16] Our previous analysis showed that diagnosis of VWD was associated with improved patient outcomes in terms of a lower frequency of bleed claims after (vs before) diagnosis, but that many patients were misrecognized prior to diagnosis. 17 Our present findings, which suggest (based on computer modeling) that there may be a significant number of patients with symptomatic but undiagnosed VWD or other mucocutaneous bleeding disorders within the commercially insured population, underscore the need for continued health care practitioner education to increase VWD detection and diagnosis and are in line with recent calls for improved VWD community education.…”

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

“…Although clinical symptoms are generally milder than in haemophilia, dosing of perioperative treatment in VWD is more challenging due to variation in VWD types and mutations, interpatient variability of residual endothelial VWF production, VWF secretion and clearance, as well as heterogeneity in types of factor concentrates with different ratios of VWF:Act/FVIII and VWF:Act/VWF antigen (VWF:Ag) . Previous studies have, however, reported that surgical procedures can be performed safely in patients with VWD and that treatment with VWF‐containing concentrates is efficacious …”

“…6,7 Previous studies have, however, reported that surgical procedures can be performed safely in patients with VWD and that treatment with VWF-containing concentrates is efficacious. [8][9][10][11][12][13][14][15][16][17] In many countries, specific target levels are defined in national guidelines to safeguard haemostasis during surgery. These target values are based on expert opinion and limited observational research ( Figure 1).…”