A 10-year prospective study of invasive ENT procedures or surgery in patients with hemophilia

Abstract

Problem: To evaluate the viability and safety of invasive ENT procedures or surgery in patients with hemophilia or von Willebrand’s disease (vWD) by preventing bleeding. Methods: Between August 1991 and July 2001, 22 patients with vWD type 1 (3 cases, 1 male), vWD type 2A (1 female), vWD type 2B (1 male), vWD type 3 (1 male), haemophilia A-severe type (12 cases, 11 males), 1 female with 30% of factor-VIII level, haemophilia B (2 males, low type), and a female with severe factor X deficiency were studied. Human immunodeficiency virus (HIV) was present in 14, all of them with AIDS. Replacement therapy: cryoprecipitate, activated prothrombin-complex concentrates (APCCs) and/or factor VIII/von Willebrand factor concentrate. Antifibrinolytic agents: Tranexamic acid and/or DDAVP intravenously were used. Results: Puncturing through the anterior wall of maxillary to lavage and/or to obtain specimens for culture was performed on 10 patients, breaking down as follows: adenoidectomy-4, myringotomy-3, turbinectomy-2, adenotonsillectomy-1, rhinoplasty-1, uvulectomy-1, ethmoidectomy-1, ethmoidectomy and polypectomy by FESS-1, and resection of a large ethmoidal osteoma with skull base invasion-1. One patient was treated with Tranexamic acid, 5 with DDAVP and Tranexamic acid (2 hemophiliacs B, 1 vWD Type 1, 1 vWD type 2 A, 1 with 30% of factor VIII level). One patient received APCCs-severe factor X deficiency. Three patients with vWD were treated with 1 vWD type 1 (high risk surgery), 1 patient with vWD type 2B, and 1 with vWD type 3 need substitution therapy with factor VIII/von Willebrand factor concentrate. Cryoprecipitate and/or factor concentrate were used in all 12 patients with haemophilia A. Those procedures were able to maintain clinical haemostasis. None of the patients had an uncontrolled bleeding, thromboembolic complication, or developed inhibitor--serious complications in patients with hemophilia or vWD. Conclusion: Meticulous attention to haemostasis, careful preoperative assessment, and postoperative follow-up minimize complications in patients with coagulation deficiencies who require invasive ENT procedures or surgery. Significance: Hemophiliacs and vWD patients could be submitted to ENT procedures when necessary, but they need special care. Support: None reported.