Perioperative management of children with von Willebrand disease and tonsillar hypertrophy in ENT Department of Children Hospital in Warsaw Medical University

*Anna Gorzelnik¹, Monika Jabłońska-Jesionowska¹, Lidia Zawadzka-Głos¹, Anna Klukowska²

Summary
Introduction. The tonsillar hypertrophy are the most common children’s disease. The treatment of choice is surgery. Regardless of surgery procedure perioperative bleeding is still present. The problem with homeostasis occurs in 0.5-2 percent of cases due to bleeding disorders. The most common inherited coagulopaties is von Willebrand disease (vWD). vWD commonly presents with epistaxis, excessive bleeding following soft tissue trauma, ecchymosis. Three type of vWD have been described. The treatment of hemorrhage in patients with vWD is i.v. infusion of Desmopressin (Minirin), concentrate containing factor VIII/vWF or antifibrinolytic drugs.
Material and methods. From January 2008 to December 2011 in the Department of Pediatric Otholaryngology of Medical University of Warsaw we treated by surgery 16 children with von Willebrand disease because of the tonsillar hypertrophy.
Results. 8 children with vWD and tonsillar hypertrophy underwent adenoidectomy and 8 adenoidotonsillotomy. Von Willebrand disease has been recognized because of clinical symptoms and blood tests in preoperative time. We treated by surgery 14 children with type 1 and 2 children with type 2 of vWD. 14 children has received Desmopressin (Minirin) intravenously and 2 children has received factor VIII-vWF concentrate (HaemateP).
8 children has received etamsylat (Cyclonamina), all 16 children has received as antifibrinolytic therapy tranexamicum acidum (Exacyl).
Conclusions. Adenoidectomy with/or without tonsillotomy in children with von Willebrand disease seems to be safe procedure by using adequate treatment of haemostasis.

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