title: Joint surgery in von Willebrand disease: a multicentre cross-sectional study.
abstract: BACKGROUND
  Joint bleeds are reported by 23% of von Willebrand disease (VWD) patients and associated with orthopaedic surgery. Limited data are available on joint surgery in VWD.
  
  
  OBJECTIVE
  To assess the prevalence, indications, management and complications of joint surgery in VWD patients.
  
  
  METHODS
  804 VWD patients with historically lowest von Willebrand factor (VWF) activity ≤30 U dL-1completed a questionnaire on joint bleeds, joint damage and orthopaedic surgery. We retrieved additional medical file data of patients who underwent surgery on large joints (shoulder, elbow, hip, knee or ankle).
  
  
  RESULTS
  116 out of 804 patients (14%) reported large joint surgery. Compared to VWD patients without previous orthopaedic surgery, these 116 patients reported more frequently a history of joint bleeds and joint damage (41% vs. 20%, P < 0.001 and 61% vs. 20%, P < 0.001). Medical file data on 126 large joint surgeries in 79 VWD patients revealed that this surgery was associated with joint damage due to prior joint bleeds in 24% of the procedures. Preoperative clotting factor correction (CFC) to prevent bleeding was administered in most cases (81%). Documentation on postoperative bleeding was found in 23 surgeries (18%).
  
  
  CONCLUSIONS
  Large joint surgery is reported by 14% of VWD patients, related to joint bleeds in 24% and seems associated with bleeding complications frequently despite perioperative CFC.
authors.affiliation: Van Creveldkliniek, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: K P M
authors.initials: KPM
authors.lastname: van Galen
authors.affiliation: Department of Haematology, University Medical Center Groningen, University of Groningen, Groningen, The Netherlands.
authors.forename: K
authors.initials: K
authors.lastname: Meijer
authors.affiliation: Department of Orthopaedic Surgery, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: H C
authors.initials: HC
authors.lastname: Vogely
authors.affiliation: Department of Thrombosis and Haemostasis and Einthoven Laboratory for Experimental Vascular Medicine, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: J
authors.initials: J
authors.lastname: Eikenboom
authors.affiliation: Van Creveldkliniek, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: R E G
authors.initials: REG
authors.lastname: Schutgens
authors.affiliation: Department of Paediatric Haematology, Erasmus University Medical Center-Sophia Children's Hospital, Rotterdam, The Netherlands.
authors.forename: M H
authors.initials: MH
authors.lastname: Cnossen
authors.affiliation: Department of Paediatric Haematology, Academisch Medisch Centrum, Emma Children's Hospital, Amsterdam, The Netherlands.
authors.forename: K
authors.initials: K
authors.lastname: Fijnvandraat
authors.affiliation: Jon J van Rood Center for Clinical Transfusion Medicine, Sanquin Research, Leiden, The Netherlands.|Department of Clinical Epidemiology, Leiden University Medical Center, Leiden, The Netherlands.
authors.forename: J G
authors.initials: JG
authors.lastname: van der Bom
authors.affiliation: Department of Haematology, Radboud University Medical Center, Nijmegen, The Netherlands.
authors.forename: B A P
authors.initials: BAP
authors.lastname: Laros-van Gorkom
authors.affiliation: Department of Haematology, Erasmus University Medical Center Rotterdam, Rotterdam, The Netherlands.
authors.forename: F W G
authors.initials: FWG
authors.lastname: Leebeek
authors.affiliation: Van Creveldkliniek, University Medical Center Utrecht, Utrecht, The Netherlands.
authors.forename: E P
authors.initials: EP
authors.lastname: Mauser-Bunschoten
keywords: arthropathy; joint bleeds; orthopaedic surgery; von Willebrand disease