Factor XI Deficiency and Obstetrical Anesthesia

Abstract

Factor XI (FXI) deficiency is a rare inherited coagulation disorder associated with prolonged activated partial thromboplastin time. The severity of bleeding often does not correlate with plasma factor levels. We reviewed the medical and anesthetic records of 13 parturients with FXI deficiency that presented for delivery. Nine cases were managed with neuraxial anesthesia. (epidural, seven; spinal, one; combined spinal-epidural, one). Three received general anesthesia for cesarean delivery, and one had an unmedicated vaginal delivery. Baseline factor levels ranged from severe (<15%) to mild (near 50%) deficiency. Fresh frozen plasma was administered to correct activated partial thromboplastin time in most, but not all, cases. Hematology consultation was obtained for all. No hematological or anesthetic complications were noted. FXI deficiency is not an absolute contraindication to neuraxial anesthesia, provided appropriate hematology consultation has been obtained, and factor replacement is provided as guided by clinical and laboratory hemostatic evaluation.

Congenital Factor XI Deficiency: An Update

“…In a series of 13 pregnant women with FXI deficiency, four with severe deficiency were treated with FFP with the aim to correct the aPTT before general, epidural, and spinal anesthesia. 36 In another study, low dose of rFVIIa (30 µg/kg) was given in three pregnant women before regional block. 37 In regard to women with partial deficiency of FXI and history of prolonged menorrhagia, we would recommend the use of antifibrinolytic agents, such as tranexamic acid or epsilon aminocaproic acid, once von Willebrand disease has been ruled out.…”

Peri-delivery Analgesia and Anesthesia in Women with Hemostatic or Thrombotic Disorders

“…Patients with hemophilia B may require FIX replacement to increase levels. A case series [ 73 ] reported nine women with FXI defi ciency who were safely managed with a range neuraxial techniques (spinal, epidural and CSE) during labor and delivery. Most received factor replacement with FFP.…”

Postpartum Hemorrhage on Labor and Delivery

“…Von Willebrand disease, hemophilia A and B, and factor XI deficiency account for approximately 90% of inherited bleeding disorders. [29][30][31] Inherited platelet disorders (e.g., Bernard Soulier syndrome, Glanzmann thrombasthenia) are rare. Given the clinical heterogeneity within each diagnosis, consultation with a hematologist and blood bank personnel will help to clarify optimal management.…”