Severe postadenoidectomy bleeding revealing congenital alpha(2) antiplasmin deficiency in a child

Most coagulation disorders are detected preoperatively, provided that complete history of bleeding tendency as well as adequate preoperative blood testing are obtained (1-3). However, standard preoperative blood tests only detect platelet and coagulation factor disorders and fail to detect rare disorders, such as factor XIII deficiency and fibrinolysis disorders, which may be responsible for severe hemorrhage. We report a case of severe bleeding after adenoidectomy in a child with a positive history of previous surgical bleeding in whom extensive preoperative blood testing was normal. This severe bleeding was due to an cu, plasmin inhibitor (alpha(2) PI) deficiency, a rare congenital disease, resulting in activation of fibrinolysis and requiring specific treatment with antifibrinolytic agents.