RESUMEN
Perioperative management of patient with severe von willebrand disease [vWD] especially from major surgery is always a challenging task for the anesthesiologists. Wer report a case of a 17 year old female, diagnosed as a case of vWD at 8 years of age, who was scheduled for exploratory laparotomy for ruptured ovarian cyst with hemoperitonium. She was successfully managed with factor VIII, fresh frozen plasma [FFP] and tranexamic acid administration. Intraoperatively, more than 2 liters of clotted blood was removed and 1.5 liters of fresh blood was lost, which was replaced by red cell concentrate [RCC], colloid and crystalloid solutions. Post-operatively, cryoprecipitates and RCC were transfused. Postoperative serum coagulation studies remained within normal limits. She was transfused with cryoprecipitates and factor VIII till 5 th day. Patients with vWD do not carry an increased operative risk during elective procedures if appropriate prophylactic and corrective therapy is administered. Apart from the administration of cryoprecipitate and other blood products; factor VIII is needed for patients with severe vWD