NEURAXIAL ANALGESIA IN A PATIENT WITH FACTOR XII DEFICIENCY
Jacqueline Bogan DO, Aditi Saraswat MD,
Mohamed-Rida Alsaden MB.Ch.B, FRCPC, Jayakar Guruswamy MD
Department of Anesthesiology, Henry Ford Health System, Detroit, Michigan
Factor XII (Hageman factor) deficiency is a rare genetic blood disorder that causes prolonged coagulation in vitro without the presence of prolonged clinical bleeding tendencies. Studies show that individuals who are deficient in factor XII can have normal hemostasis and are able to undergo major surgeries without plasma replacement therapy despite dramatic PTT elevation. The ability to maintain a normal coagulation cascade may be accredited to the direct activation of factor IX by the tissue factor and VIIa complex, which bypasses the defective step and thus compensates for the deficiency of factor XII.
This report presents the case of a 74 year old male with factor XII deficiency who underwent extensive abdominal surgery with neuraxial analgesia for post-operative pain control without hemostasis sequelae. Pre-operatively, the patient had PTT > 200 and factor XII < 2%. No history of bleeding was documented. Series of tests showed partial correction of PTT on mixing study and possible minor and nonspecific inhibitors to factors of intrinsic pathway. In view of normal activities of factors VIII, IX and XI, patient was deemed not at risk of bleeding. Given the fact that the patient did not have a bleeding diathesis and there is substantial evidence in the literature stating that despite the in vitro PTT abnormality, there is no clinically significant coagulopathy in patients with factor XII deficiency, decision was made to proceed with epidural placement for post-operative pain management. Coagulation labs were checked prior to the procedure - INR 1.34, PT 16.4, PTT 66, platelets 173.
Epidural was placed via left paramedian approach at the T10-T11 interspace by regional anesthesia staff using sterile techniques and standard placement method. Epidural was secured at 12cm with a loss of resistance at 7cm. There was no excessive bleeding noted other than what was expected from insertion and removal of the Tuohy needle. Patient tolerated the procedure without any complications. Epidural remained in place for a duration of 5 days without adverse events. Patient received scheduled neurological assessments every 4 hours while epidural was in place and epidural site was checked daily for signs of bleeding, hematoma or infection. Subcutaneous Heparin injections were given to the patient every 8 hours for DVT prophylaxis with the epidural in place; no bleeding issues were noted. Coagulation panel checked prior to epidural removal was within normal limits with the exception of an elevated PTT of 80. Catheter was removed from the epidural space uneventfully without bleeding and neurological sequelae. Epidural site remained clean, dry and intact without hematoma and bleeding on 24-hour follow up after removal.
To our knowledge, this is the first case report in literature documenting a patient with factor XII deficiency receiving a neuraxial block for postoperative analgesia. Factor XII deficiency does not appear to lead to abnormal bleeding clinically, even with major surgical procedures. The lack of bleeding manifestations is not fully understood but may be attributed to activation of other clotting factors to bypass the defective step in the coagulation cascade. One of the main concerns related to factor XII deficiency is the unnecessary testing, worry of interventions that may be prompted by the abnormal laboratory result, and delay in patient care. There have been case reports of patients with factor XII deficiency undergoing obstetric procedures, major surgeries and even cardiac surgery with cardio-pulmonary bypass without major hemostasis issues. Few case reports suggest the use of FFP to correct the lab abnormality, but accepted the futility of doing so. In addition, some older medical reports suggest that factor XII deficiency may predispose affected individuals to developing blood clots which may increase their risk for deep vein thrombosis or acquired thrombotic disorders. However, such an association remains unproven. Therefore, factor XII deficiency is thought to be benign and is usually only accidentally discovered through pre-operative blood tests that are required by hospitals.