Von Willebrand disease - broadening our vision: a pediatric case report with dental considerations

Von Willebrand disease is an autosomal inherited bleeding disorder caused due to the quantitative or qualitative defects of a multimeric glycoprotein, the Von Willebrand factor (VWF). VWF is a multi-adhesive protein that carries factor VIII in circulation and initiates aggregation of platelets to exposed sub endothelial collagen fibers. The deficiency or abnormality of VWF causes reduction in the levels of factor VIII, as seen in Hemophilia A patients. The common clinical manifestations of Von Willebrand disease include soft tissue and mucosal bleeding. The severity of the disease is determined by the extent of von Willebrand factor and factor VIII reduction in the circulation. Since symptoms are often mild, a significant majority of patients remain undiagnosed. This article presents the ongoing dental management of a seven-year old female diagnosed with Von Willebrand disease. The primary aim of the article is to focus on the diagnosis, management and dental considerations of this common yet overlooked disorder and distinguish it from Hemophilia.