Von Willebrand Disease-Associated Angiodysplasia: Presentation of a Paediatric Case

Abstract

Von Willebrand disease (VWD) is a bleeding disorder, resulting from a quantitative or qualitative defect in von Willebrand factor (VWF). A regulatory role for VWF in angiogenesis was postulated upon the clinical observation that qualitative or quantitative VWF defects are associated with the frequent occurrence of neoangiogenesis, particularly in the gastrointestinal (GI) tract. Vascular malformations of the GI tract are a cause of digestive bleeding in the form of either acute or chronic haemorrhage and represent a heterogeneous group of lesions, including angiodysplasias and telangiectasias. The management of these patients is challenging due to recurrent and severe episodes of GI bleeding. The mainstay of treatment of angiodysplasia is replacement therapy when this abnormality causes GI bleeding in patients with congenital VWD. When bleeding episodes recur frequently, regular prophylaxis should be implemented, leading to an acceptable degree of prevention of bleeding. The authors had a difficult experience in a 14-year-old adolescent with Type 3 VWD, who had presented with extremely serious recurrent bleeding secondary to duodenal angiodyplasia.