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Perioperative Management of a Pregnant Patient with Factor XI Deficiency and Carrier of Factor IX Deficiency: A Case Report with Literature Review
Abstract Number: F 56
Abstract Type: Case Report/Case Series
Introduction: FXI deficiency (Hemophilia C) is a rare, autosomal recessive trait (1); FIX deficiency (Hemophilia B) is a less rare x-linked recessive trait (2). Our patient represents a “near miss”, a cesarean section (C/S) planned under spinal, but discovered by accident to be both Factors IX and XI deficient. While the incidence of epidural/spinal hematoma is small, the possibility of permanent paralysis merits a clear understanding of the risk.
Case: A 28-y/o G2P0010, at 39 5/7 wks, presented to L & D complaining of contractions. C/S was planned for the next day due to breech presentation. She had no allergies, PMH included hepatitis C, depression, and heroin abuse, currently taking methadone 300mg daily. Admission vitals were stable, physical and airway exam normal. Our patient related no history of abnormal bleeding/bruising, but was a poor historian. Without prior labs, coagulation studies were requested. Unexpectedly, the aPTT was elevated at 49 (rr: 26.7-37.1s)(3). A hematology consult resulted in a normalized aPTT mixing study with deficient FIX (50, rr 80-149 [%])(3) and FXI level assays (28, rr 73-137 [%])(3).
The multidisciplinary care plan (Anesthesia, Hematology and Obstetrics) included transfusion of 4 units FFP to normalize the aPTT for 24 hrs. Was this “good enough” to perform a safe neuraxial block? After a detailed discussion of the risks/benefits with her, our patient agreed to a C/S under GA. Following RSI with 15mg ketamine, 200mg propofol, 100mg succinylcholine, and an uneventful intubation, anesthesia was maintained with propofol, fentanyl boluses, IV acetaminophen and ketorolac. EBL was 450 mL, she was extubated (no postop bleeding) and mother and baby were discharged 4 days later.
Discussion: Our patient has both deficiencies of Factor IX (Hemophilia B carrier) and Factor XI (Hemophilia C), diagnosed by accident. We were unable to find a case report of a patient with both. Additionally, the safe use of neuraxial anesthesia in patients with either deficiency is controversial (4). Although debate exists whether FXI is physiologically low in pregnancy (5), review of the literature suggests that the aPTT is most often shortened in pregnancy (4), not prolonged. Guidelines have been developed for the safe use of neuraxial anesthesia in patients on heparin and LMWH (6), but are less clear with factor deficiencies. Because neuraxial anesthesia is preferred in the pregnant patient, the need for clinical research and guideline development for factor deficiencies is critical.
1. NEJM 1991;325:153-158.
2. Encyclo Med Geno and Proteo 1st ed. 2004; Ch 87. ISBN 9780203997352.
3. Bray, P., Dir, TJUH Lab. Clin Test TJUH Cardeza Fdn, 1015 Walnut St Phila PA 19107.
4. Obstet Anesth Princ and Prac 4th ed. Chestnut D, ed., 2009. Ch 2: 21-23. ISBN: 978-0-323- 05541-3.
5. Best Prac & Research Clin Obstet and Gynae 2010;24:339-52.
6. Reg Anesth & Pain Med 2010;35:64-101.