STUD DESIGN: Clinical prospective. MATERIAL AND METHOD: A 10-year prospective research was conducted in 20 patients with hemophilia or von Willebrand disease (vWD). They were submitted to a total of 25 elective otolaryngological surgical events. The average age of the patients was 23.75 years (2-62 years). The study group consisted of 14 hemophiliacs, 11 with severe hemophilia A (1 female), 1 female with 30% of VIII factor (VIIIF) level, 1 male with hemophilia B and 1 female with severe factor X deficiency; and 6 with vWD, 4 type 1 (3 females), 1 male type 2 A and 1 male type 3. Acquired immunodeficiency syndrome was present in 13 hemophilic patients. The mean duration of the surgical events was 1 hour and 37 minutes (15 min-12 hours). The coagulation defect was corrected with desmopressin (DDAVP), intermediate purity VIIIF concentrate 8Y, cryoprecipitated or non-activated prothrombinic complex (PPSB), according to factor levels and the severity of the surgery. Epsilon aminocaproic acid was used in association. In 1 severe hemophiliac A patient, excessive bleeding was observed in the second day of the postoperative period which ceased with elevation of the minimal level of VIIIF to 80%. In another patient, with type 3 vWD, severe postoperative bleeding occurred because of difficulty to identify the best reposition blood coagulation factor for him. After the use of intermediate purity VIIIF concentrate 8Y, the bleeding was controlled. RESULTS: The hemostatic effect in the other patients was rated as normal or excellent. CONCLUSION: It was concluded that patients with vWD or hemophilia do not have an increased operative risk if appropriate therapy is given.
haemophilia; von Willebrand; otolaryngology surgery
