title: Perioperative management of a patient with Bernard-Soulier syndrome for third molar surgery.
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.
authors.affiliation: University of Maryland Medical Center, R. Adams Cowley Shock Trauma Unit, Baltimore College of Dental Surgery, Baltimore, MD 21201, USA. mhart002@umaryland.edu
authors.forename: Michael J
authors.initials: MJ
authors.lastname: Hartman
authors.forename: John F
authors.initials: JF
authors.lastname: Caccamese
authors.forename: Stewart A
authors.initials: SA
authors.lastname: Bergman
mesh_terms: D000293:Adolescent; D000614:Aminocaproates / Q000627:therapeutic use; D001606:Bernard-Soulier Syndrome; D016063:Blood Loss, Surgical / Q000517:prevention & control; D017742:Dental Care for Chronically Ill; D006490:Hemostatics / Q000627:therapeutic use; D006801:Humans; D008297:Male; D008964:Molar, Third / Q000601:surgery*; D006472:Oral Hemorrhage / Q000517:prevention & control; D017713:Platelet Transfusion; D014081:Tooth Extraction
chemical_list: D000614:Aminocaproates; D006490:Hemostatics