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Management of the Term Parturient Patient with Factor XI Deficiency
Abstract Number: T-39
Abstract Type: Case Report/Case Series
Introduction: Factor XI deficiency is an inherited coagulopathy which is typically seen in the Ashkenazi Jewish population with an incidence of up to 9%. However, it has also been described in individuals with other ethnic backgrounds. There is limited definitive information currently available regarding the safety and standard practice of labor analgesic and anesthetic neuraxial techniques in the parturient patient who presents with this coagulopathy. We describe a case series of seven patients with factor XI deficiency who presented to our academic tertiary care hospital labor and delivery ward for delivery over the past 36 months.
Cases: All patients presented with documented factor XI deficiency and were under the care of a hematologist during pregnancy. Six of the 7 patients were otherwise healthy, whereas one patient was also heterozygous for a prothrombin gene mutation and factor V Leiden mutation. All patients had documented endogenous factor XI levels with a range between 4% and 48% at the time of delivery. Only two patients reported a history of bleeding, including heavy menses and hemorrhage after tooth extraction. Of the seven patients, three received a neuraxial anesthetic technique for labor and delivery, three had a vaginal delivery with a fentanyl PCA for analgesia, and one underwent a general anesthetic for scheduled cesarean section. Two patients received fresh frozen plasma prior to delivery, one patient received intraoperative tranexamic acid during cesarean delivery, and one patient received aminocaproic acid postpartum. No patients exhibited excessive postpartum hemorrhage or neurologic complications.
Discussion: Factor XI deficiency is an inherited coagulation disorder that presents challenges for the anesthesia provider, particularly when these patients present to the labor and delivery ward. Specific challenges include the rarity of the disease, lack of data on the safety of neuraxial anesthesia techniques, and the poor correlation between measured serum factor XI levels and severity of the clinical bleeding diathesis. At our institution, we have found considerable heterogeneity in the clinical management of these patients by obstetricians, hematologists, and anesthesiologists. More data is required to identify risk factors that better correlate with disease severity and predict the likelihood of adverse maternal outcomes such as spinal hematoma and postpartum hemorrhage, especially given the well-described benefits of spinal and epidural anesthesia in laboring women. Furthermore, a standard or protocol-driven approach regarding the use and timing of neuraxial anesthesia, factor replacement, and appropriate utilization of antifibrinolytic therapy would help provide consistent care to these patients who are frequently presented with conflicting information from multiple providers regarding labor and delivery management and anesthetic options.