BACKGROUND AND OBJECTIVES: von Willebrand's disease is secondary to a mutation on chromosome 12, and is characterized by a qualitative and quantitative deficiency of the von Willebrand's factor. The diversity of the mutations is responsible for several different clinical manifestations, enabling the classification of several types and subtypes. The coagulopathy is manifested basically through a platelet dysfunction associated with a reduction in the serum levels of factor VIII. The objective of this review was to present the perioperative care of patients with von Willebrand's disease. CONTENTS: The physiopathology, classification, laboratorial diagnosis, and current treatment of von Willebrand's disease, as well as the perioperative management of these patients are discussed. CONCLUSIONS: von Willebrand's disease is the most common hereditary coagulopathy, but it is underdiagnosed due to the complexity of the disease itself. The right classification, proper use of desmopressin, and transfusion of von Willebrand's factor are fundamental for a successful anesthesia.
BACKGROUND AND OBJECTIVES:von Willebrand's disease is secondary to a mutation on chromosome 12, and is characterized by a qualitative and quantitative deficiency of the von Willebrand's factor. The diversity of the mutations is responsible for several different clinical manifestations, enabling the classification of several types and subtypes. The coagulopathy is manifested basically through a platelet dysfunction associated with a reduction in the serum levels of factor VIII. The objective of this review was to present the perioperative care of patients with von Willebrand's disease. CONTENTS: The physiopathology, classification, laboratorial diagnosis, and current treatment of von Willebrand's disease, as well as the perioperative management of these patients are discussed. CONCLUSIONS:von Willebrand's disease is the most common hereditary coagulopathy, but it is underdiagnosed due to the complexity of the disease itself. The right classification, proper use of desmopressin, and transfusion of von Willebrand's factor are fundamental for a successful anesthesia.