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  • Title: Perioperative management of a patient with Bernard-Soulier syndrome for third molar surgery.
    Author: Hartman MJ, Caccamese JF, Bergman SA.
    Journal: Oral Surg Oral Med Oral Pathol Oral Radiol Endod; 2007 May; 103(5):626-9. PubMed ID: 17174124.
    Abstract:
    Bernard-Soulier Syndrome (BSS) is a disease characterized by prolonged bleeding time, thrombocytopenia, and extremely large platelets and has a prevalence of less than 1 in 1,000,000. Patients with disorders of coagulation and bleeding can be among the most difficult surgical patients to manage. Perioperative hemorrhage can contribute to life-threatening complications in even the most routine surgical procedures. Because of the rarity of BSS, there are no well-defined protocols for the management of perioperative bleeding associated with this condition. Treatment with preoperative and intraoperative systemic aminocaproic acid, HLA-matched platelets, and topical gelfoam and thrombin resulted in sustained hemostasis and a durable healing response. For those rare few afflicted with this disease, we present a combined systemic and topical approach that may be helpful in the control and prevention of perioperative hemorrhage in this and other similar platelet disorders.
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