Adenotonsillectomy in children with von Willebrand's disease: how and when. A case report with review of the literature

Abstract

The presence of coagulation pathology in children who are candidates for adenotonsillectomy (AT) is a challenge to the otolaryngologist. von Willebrand's disease (vWD) is the most common hereditary coagulopathy and is due to a quantitative and/or qualitative deficiency of von Willebrand's factor (vWF). In recent years, the administration of 1-deamino-8- d-arginine vasopressin (DDAVP) has been recommended as coadjuvant therapy for surgical procedure. This synthetic hormone promotes the release of vWF and factor VIII from endothelial cells. In this report, the authors describe the history of a child with vWD undergoing successful AT after administration of DDAVP. Furthermore, a review of the literature with particular emphasis on the use of DDAVP is made.