Perioperative management of a patient with Fechtner syndrome

Fechtner syndrome is a rare type of familial thrombocytopenia associated wi th large platelets, leukocyte inclusions, and features of Alport's syndrome . The bleeding tendency is usually mild, but severe hemorrhages have been r eported. This is the case of a patient with Fechtner syndrome who was sched uled to undergo tonsillectomy. The patient had a history of easy bruising i n childhood and a markedly prolonged bleeding time. Administration of DDAVP led to normalization of the bleeding time, and the patient underwent surge ry without complications. With this approach the use of platelet concentrat es could be avoided.