Anesthetic and perioperative management of a patient with Bernard-Soulier syndrome

We describe a patient with Bernard-Soulier syndrome who underwent emergency laparotomy for intraabdominal bleeding. Presentation of this recent case highlights some of the anesthetic and perioperative implications of the disease. Briefly, perioperative and anesthetic management of patients with Bernard-Soulier syndrome should include platelet transfusions and choice of appropriate anesthetic drugs and techniques. The potential benefit of desmopressin and corticosteroids should be considered. Thrombelastography, if available, is also useful.