title: 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.
authors.affiliation: aIstanbul University, Cerrahpasa Medical Faculty and Oncology Institute, Department of Pediatric Hematology/Oncology bIstanbul University, Faculty of Dentistry, Department of Oral and Maxillofacial Surgery cIstanbul University, Istanbul Medical Faculty, Department of Orthopedics and Traumatology dHaydarpaşa State Hospital, Urology Clinic eThe Haemophilia Society of Turkey, Istanbul, Turkey.
authors.forename: Bulent
authors.initials: B
authors.lastname: Zulfikar
authors.forename: Basak
authors.initials: B
authors.lastname: Koc
authors.forename: Gulsum
authors.initials: G
authors.lastname: Ak
authors.forename: Fatih
authors.initials: F
authors.lastname: Dikici
authors.forename: İhsan
authors.initials: İ
authors.lastname: Karaman
authors.forename: Ata Can
authors.initials: AC
authors.lastname: Atalar
authors.forename: Fikret
authors.initials: F
authors.lastname: Bezgal
mesh_terms: D000293:Adolescent; D000328:Adult; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012189:Retrospective Studies; D016896:Treatment Outcome; D055815:Young Adult; D014842:von Willebrand Diseases / Q000150:complications / Q000601:surgery*