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

Sep 30, 2026

Arnold-Chiari malformation (ACM) and Factor XI (FXI) deficiency are rare obstetric conditions that create distinct anesthetic risks, especially around neuraxial safety and hemorrhage. This case report describes a 26-year-old woman at 39 weeks with ACM type I and FXI deficiency who required induction of labor and presented a complex decision-making scenario. Multidisciplinary planning recommended prophylactic fresh frozen plasma and tranexamic acid; epidural placement was deferred due to symptomatic ACM and CSF leak risk. She later elected cesarean delivery, receiving general anesthesia with perioperative hemostatic optimization, with an estimated 2600 mL blood loss, stable hemodynamics, no neurologic complications, and discharge on postoperative day five.

LanguageEnglish

CategoryHealthcare

FormatPDF

Pages5

Reading Time13 min

AccessFree to download

Abstract

Introduction

Arnold-Chiari malformation (ACM)

Hemophilia C (FXI deficiency)

Case Report (Peripartum anesthetic management)

Review began 10/29/2025 Review ended 12/02/2025 Published 12/09/2025 © Copyright 2025 Monroe et al. This is an open access article distributed under the terms of the Creative Commons Attribution License CC-BY 4.0. , which permits unrestricted use , distribution , and reproduction in any medium , provided the original author and source are credited. DOI: 10.7759/cureus.98806 Open Access Case Report Peripartum Anesthetic Care of a Parturient With Arnold-Chiari Malformation and Factor XI Deficiency: A Case Report Devin G. Monroe 1, Tanner Moore 2, Mohamad El Churafa 1, Taylor Varela 1 1. Anesthesiology, West Virginia University School of Medicine, Morgantown, USA 2. General Surgery, West Virginia University School of Medicine, Morgantown, USA Corresponding author: Devin G. Monroe, [ndmonroe12@gmail.com](ndmonroe12@gmail.com) Abstract 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 G2P 1011 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 ACMand 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. Categories: Anesthesiology Keywords: arnold-chiari malformation, cesarean delivery, epidural, factor xi deficiency, general anesthesia, hemophilia c, neuraxial anesthesia, obstetric anesthesia, spinal anesthesia Introduction Arnold-Chiari malformation (ACM) and Factor XI (FXI) deficiency, also known as hemophilia C, are two distinct medical conditions that highlight the complexities of the neurologic and hematologic systems, respectively. Each of these disease processes presents its own individual and unique challenges when providing obstetric anesthesia. Chiari malformations are defined as morphologic malformations that primarily involve the downward displacement of the cerebellar t

FAQs

Why was neuraxial anesthesia avoided in this case?

Neuraxial anesthesia was deferred because the patient had symptomatic ACM and there was concern about the risk of cerebrospinal fluid leak associated with neuraxial techniques.

What hemostatic measures were recommended and given?

Hematology recommended prophylactic fresh frozen plasma (FFP) and tranexamic acid (TXA). FFP and TXA were provided perioperatively during cesarean delivery.

What anesthesia approach was ultimately selected for delivery and what was the outcome?

General anesthesia was chosen for cesarean delivery. The patient remained hemodynamically stable, had no neurologic complications, and was discharged home on postoperative day five.

Sep 30, 2026

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