Surgery on patients with hemostatic disorders

Patients with hemostatic disorders about to undergo surgery present a special challenge for the otolaryngologist. The most common coagulation disorders constituting more than 90% of all patients seen with severe inherited bleeding defects are hemophilia and von Willebrand's disease (pseudohemophilia). Neither of these is an absolute contraindication for surgery. Hemophilia, a sex-linked disorder, can be divided into two distinct classes: hemophilia A (classic hemophilia), a deficiency of factor VIII production, and hemophilia B (Christmas disease), a deficiency of factor IX production. Von Willebrand's disease is an autosomal dominant disorder consisting of a decrease in factor VIII activity plus a prolonged bleeding time and abnormal platelet aggregation with ristocetin. Careful preoperative evaluation and early hospitalization are important for these patients. The close cooperation of a hematologist, monitoring availability and proper replacement of these factors, is essential. Careful postoperative attention should be directed at the maintenance of sufficient therapeutic levels of replacement therapy for a minimum of 7–10 days. We shall present cases of patients with inherited bleeding diatheses who underwent successful surgery at our institution. The use of Amicar® and Avitene® will also be presented.