Isolated Factor X Deficiency, A Rare Occurrence: Anesthetic Concerns And Perioperative Management

Abstract

Purpose: Congenital factor X (FX) deficiency is a rare inherited coagulation disorder. Anesthesiologists have limited experience in the prevention of the severe hemorrhagic diathesis caused by FX deficiency before surgical procedures. Clinical features:A 20 year old, ASA grade III female patient was admitted with complaints of pain in the abdomen, polymenorrhagia and an abdominal mass. She was diagnosed as FX deficiency earlier. Presently, at the time of admission she had grossly deranged coagulation [prothrombin time (PT)-55.6 seconds (normal 12 seconds), activated partial thromboplastin time (aPTT)-52.7 seconds (normal 30 seconds)]. She was scheduled for exploratory laparotomy to confirm the diagnosis. Preoperatively, FFP 15ml/kg was transfused. On the morning of surgery in spite of receiving recommended dose of FFP as planned, PT was 20 seconds (normal 12 seconds). Further 4 FFP had to be transfused prior to induction of general anesthesia. After induction laryngoscopy and tracheal intubation was performed when adequate muscle relaxation was achieved to avoid any airway trauma. Injection Trenaxemic acid 2 gm intravenously was administered on the day of surgery and continued postoperatively. The postoperative plan was to transfuse 4 units of FFP 12 hourly for next 4-7 days with PT, aPTT monitoring. Postoperative analgesia was provided with IV tramodol (50 mg) 8 hourly and IV pethidine (37.5mg), phenargan (12.5 mg) for breakthrough pain.Conclusion: In patients with FX deficiency, coagulation should be optimized preoperatively and continuously assessed in the perioperative period as in severe deficiency; the coagulation parameters may vary and be deranged in spite of seemingly adequate FFP transfusion.Implication Statement: Patients with factor X deficiency require multidisciplinary approach including hematologist, gynecologist and anesthesiologist along with meticulous homeostasis during surgery and careful monitoring of blood coagulation in the postoperative period.