Perioperative Management of von Willebrand's Disease in Otolaryngologic Surgery

Authors

  • Saurabh B. Shah MD,

    1. Department of Otolaryngology-Head and Neck Surgery, University of California, San Francisco, San Francisco, California.
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  • Anil K. Lalwani MD,

    Corresponding author
    1. Department of Otolaryngology-Head and Neck Surgery, University of California, San Francisco, San Francisco, California.
    • Anil K. Lalwani, MD, Division of Otology, Neurotology and Skull Base Surgery, Department of Otolaryngology-Head and Neck Surgery, UCSF, 350 Parnassus Avenue, Suite 210, San Francisco, CA 94117.
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  • Marion A. Koerper MD

    1. Division of Pediatric Hematology, Department of Pediatrics, University of California, San Francisco, San Francisco, California.
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  • Presented at the Meeting of the Western Section of the American Laryngological, Rhinological and Otological Society, Inc., Tucson, Arizona, January 11, 1997.

Abstract

von Willebrand's disease (VWD) is the most common hereditary bleeding disorder. Unchecked or improperly managed, VWD-associated hemorrhage can lead to catastrophic surgical outcome. Based on the authors' recent experience with 21 procedures in 12 patients, a contemporary protocol for successful perioperative management of VWD in otolaryngologic surgery is presented. In patients with VWD type 1 or 2a, desmopressin, a synthetic vasopressin analog, is administered both pre- and postoperatively to release von Willebrand factor (VWF) from storage sites. In type 2b or 3, a factor VIII concentrate rich in VWF is administered. In addition, a 10- to 14-day course of intravenous and/or oral Amicar (Immunex Corp., Seattle, WA) may be prescribed postoperatively. Intraoperatively, the surgical laser is used to further decrease blood loss and augment hemostasis. This medical and surgical protocol minimizes the risk of hemorrhage and of transfusion-related complications through the judicious use of preoperative and postoperative coagulation replacement products. Using these guidelines in a variety of otolaryngologic cases, the authors have had no bleeding complications at their institution.

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