Perioperative Management of Patients with Rare Congenital Factor Deficiency
Ferda Can1, Tekin Guney21Department of Hematology, Ministry of Health Ankara Bilkent City Hospital, Ankara, Türkiye, 2Department of Hematology, University of Health Science Ankara Bilkent City Hospital, Ankara, Türkiye
Objectives: Fibrinogen, prothrombin, factor V, VII, X, XI, XIII and V+VIII deficiencies are defined as rare congenital factor deficiencies (RCD). Due to small number of patients and lack of data, perioperative management can be challeging. We aimed to evaluate the RCD patients who underwent surgery. Methods: Thirteen women, eight men, totally 21 patients was retrospectively analyzed. Results: Ten patients were diagnosed incidentally by investigating the bleeding tests performed before the procedure and routine bleeding tests. Two patients had diagnosed with fibrinogen disorder, one patient factor V deficiency, 9 patients factor VII deficiency, 2 patients factor X deficiency, 4 patients factor XI deficiency, 3 patients combined factor deficiency. Six patients underwent gynecological intervention, 4 patients dental procedures, 2 patients orthopedic surgery, 2 patients appendectomy, 2 patients urological intervention, one patient gastrectomy, one patient rhinoplasty, one retinal surgery, one lipoma excision, one cardiological intervention. Two of the patients were treated with only antifibrinolytic therapy before the procedure/surgery, while 19 patients were treated with replacement therapy appropriate to their disease. Ten patients were continued with replacement therapy after the procedure/surgery. None of the patients had bleeding. Conclusion: Perioperative management of patients with rare factor deficiency is crucial in terms of bleeding and related complications. Keywords: Bleeding, Bleeding disorder, Blood coagulation disorders, Factor deficiency, Surgery
Cite This Article
Can F, Guney T. Perioperative Management of Patients with Rare Congenital Factor Deficiency. EJMI. 2023; 7(4): 401-406
Corresponding Author: Ferda Can