Peripartum Anesthetic Care of a Parturient With Arnold-Chiari Malformation and Factor XI Deficiency: A Case Report

Arnold-Chiari malformation (ACM) and Factor XI (FXI) deficiency are rare conditions that pose distinct anesthetic challenges in the obstetric population. While each condition has been managed individually with varying anesthetic approaches, their coexistence in a single patient significantly complicates peripartum decision-making, particularly regarding the safety of neuraxial anesthesia and the risk of hemorrhage. We report the case of a 26-year-old G2P1011 woman at 39 weeks of gestation with ACM type I and FXI deficiency who presented for induction of labor. Multidisciplinary planning involved hematology, neurology, neurosurgery, obstetrics, and anesthesiology. Given her bleeding history, hematology recommended prophylactic fresh frozen plasma (FFP) transfusion and tranexamic acid (TXA). Due to symptomatic ACM and risk of cerebrospinal fluid leak with neuraxial anesthesia, epidural placement was deferred. Analgesia was managed with remifentanil PCA. As labor progressed, the patient elected for cesarean delivery despite increased bleeding risk. General anesthesia was chosen, and the patient received perioperative FFP and TXA. Intraoperative blood loss was estimated at 2600 milliliters, requiring transfusion of 1 unit of packed red blood cells while maintaining stable hemodynamics. Postoperatively, the patient remained hemodynamically stable with no neurologic complications and was discharged home on postoperative day five. While neuraxial anesthesia can be considered in isolated ACM or FXI deficiency with appropriate precautions, the coexistence of both disorders amplifies the risks of neurologic deterioration and spinal hematoma. General anesthesia provided a controlled and safe alternative in this setting. This case underscores the importance of individualized anesthetic planning, multidisciplinary coordination, and careful hemostatic optimization. The simultaneous presence of ACM and FXI deficiency in an obstetric patient presents unique anesthetic and obstetric challenges. General anesthesia with proactive hemostatic management may be the safest approach when symptomatic ACM coexists with a significant bleeding risk. This case report describes the successful multidisciplinary peripartum management of a patient with both symptomatic ACM and FXI deficiency, highlighting the anesthetic dilemma and the rationale for ultimately selecting general anesthesia for cesarean delivery.