Perioperative management of tonsilloadenoidectomy and circumcision of a patient with Bernard-Soulier syndrome: case report.

ABSTRACT

Bernard-Soulier syndrome is an autosomal recessive coagulopathy characterized by thrombocytopenia, prolonged bleeding time and large platelets. Because of the rarity of this disease, standard perioperative treatment protocols have not been developed and there are not enough available data for the management of surgical procedures. In this case, we successfully performed adenoidectomy and tonsillectomy and circumcision surgery concurrently under the preventive and intermittent transfusion of platelets. During hospitalization, no intraoperative or postoperative bleeding complications occurred.