Of congenital coagulation factor deficiencies, hemophilia A and hemophilia B are among the most common, and will be encountered in the oral and maxillofacial surgery office. Both are X-linked disorders resulting in a deficiency of either factor VIII (hemophilia A) or factor IX (hemophilia B). Hemophilia A occurs in approximately 1:5000 male births and hemophilia B occurs in approximately 1:30,000 male births. There is no specific geographic or racial predilection. Classic symptoms include soft tissue bleeding and hemarthroses, which can result in debilitating arthropathy.
Patients with mild hemophilia typically will not have spontaneous bleeding, and will only have bleeding in response to trauma, surgical procedures, or dental extractions. Those with moderate hemophilia may experience excess bleeding after trauma, surgery, or dental extractions and may also sustain joint or muscle bleeding after minor injury. Patients with severe hemophilia may experience spontaneous bleeding into joints and muscles and severe bleeding after injuries or surgery
Surgical management of the hemophilia patient requires knowledge of the patient’s level of severity, presence and responsiveness of inhibitors, and the history of how surgery has been managed in the past and the success of postoperative hemostasis. The appropriate management of these patients requires close consultation with the patient’s hematologist and usually the patient and their family member will be able to provide a significant amount of helpful historical information concerning their disease state. In general, the goal is to raise factor (VIII or IX) concentrations to 80% to 100% just before surgery and a level of at least 50% should be maintained 5 to 14 days postoperatively. For dental extractions, slightly less aggressive correction has been supported, with recommendations to achieve factor levels of 50% to 70% preoperatively and maintained at 50% for 5 to 7 days postoperatively.
Several adjuncts are available to assist with hemostasis during oral surgery procedures. Certainly, one may wish to use absorbable gelatin sponges, collagen plugs, absorbable fibrillary hemostatic agents, topical thrombin, and sutures at the surgical site to directly enhance hemostasis. Antifibrinolytics such as tranexamic acid or epsilon-aminocaproic acid are recommended additions to increase the efficacy of haemostasis. Antifibrinolytics competitively inhibit plasminogen and decrease clot breakdown. Both tranexamic acid and epsilon-aminocaproic acid can be used topically via soaked gauze for the patient to bite down on, as well as systemically. Ideally, the antifibrinolytic should be started before the surgery and continued for 7 days postoperatively.
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