title: Incidence of bleeding complications in pediatric patients with type 1 von Willebrand disease undergoing adenotonsillar procedures.
abstract: OBJECTIVE
  To review the incidence of postoperative bleeding in children with type 1 von Willebrand disease (VWD) who were treated with a single institution protocol.
  
  
  METHODS
  We performed a retrospective study to determine the postoperative hemorrhage rate in pediatric patients with type 1 VWD who were treated via the Children's Hospital of Philadelphia institutional protocol. This protocol utilizes intravenous desmopressin (DDAVP), oral aminocaproic acid, and overnight observation.
  
  
  RESULTS
  Between the years of 2000 to 2006, 41 children with type 1 VWD underwent an adenotonsillar procedure and were treated with this protocol. Seven patients (17%) experienced delayed (>24 hours after surgery) postoperative hemorrhage requiring intervention. Five of the 7 patients required cautery to control the bleeding, and the remaining 2 patients responded to DDAVP and aminocaproic acid alone. Older age and lower VW antigen levels were associated with postoperative hemorrhage (P = .05).
  
  
  CONCLUSIONS
  Despite therapeutic intervention to decrease the risk of postoperative hemorrhage, the incidence of hemorrhage was higher in pretreated patients with type 1 VWD than in children without bleeding disorders. Further prospective studies are necessary to determine the optimal treatment to reduce bleeding complications in these patients.
authors.affiliation: Department of Pediatrics, Division of Hematology, The Children's Hospital of Philadelphia, University of Pennsylvania School of Medicine, Philadelphia, PA 19104, USA. witmer@email.chop.edu
authors.forename: Char M
authors.initials: CM
authors.lastname: Witmer
authors.forename: Lisa
authors.initials: L
authors.lastname: Elden
authors.forename: Regina B
authors.initials: RB
authors.lastname: Butler
authors.forename: Catherine S
authors.initials: CS
authors.lastname: Manno
authors.forename: Leslie J
authors.initials: LJ
authors.lastname: Raffini
mesh_terms: D000233:Adenoidectomy; D000284:Administration, Oral; D000293:Adolescent; D000367:Age Factors; D000614:Aminocaproates / Q000627:therapeutic use; D002425:Cautery; D002648:Child; D002675:Child, Preschool; D003894:Deamino Arginine Vasopressin / Q000627:therapeutic use; D005260:Female; D006490:Hemostatics / Q000627:therapeutic use; D006801:Humans; D007262:Infusions, Intravenous; D008297:Male; D019106:Postoperative Hemorrhage / Q000453:epidemiology* / Q000517:prevention & control*; D020250:Postoperative Nausea and Vomiting / Q000453:epidemiology; D012189:Retrospective Studies; D014068:Tonsillectomy; D014842:von Willebrand Diseases / Q000453:epidemiology* / Q000276:immunology; D014841:von Willebrand Factor / Q000032:analysis / Q000276:immunology
chemical_list: D000614:Aminocaproates; D006490:Hemostatics; C029417:Von Willebrand antigen; D014841:von Willebrand Factor; D003894:Deamino Arginine Vasopressin