Epidural Anesthetic Management of a Parturient with Von Willebrand Disease Type 1C: A Case Report

Document Type

Conference Proceeding - Restricted Access

Publication Date

5-9-2025

Abstract

Spinal epidural hematoma (SEH) is a rare, potentially lethal, and often neurologically devastating complication of epidural anesthetic delivery that necessitates emergent surgical decompression1. In healthy populations, incidence of SEH following epidural anesthesia is estimated at 1:150,0002. However, pregnancy and coagulopathies are thought to significantly increase the risk of SEH1,2. Among inherited bleeding disorders, Von Willebrand disease (vWD) is the most common, with estimated prevalence as high as 1%3. A rare variant, vWD type 1c is characterized by accelerated clearance of structurally normal vWF3. While epidural anesthetic management of classical vWF is well-described, there is only a single published case4 known to the authors addressing the type 1c variant. Here, we report a case of uncomplicated epidural anesthetic care of a parturient with vWD type 1c.

A 30 year-old primigravida with a history of vWD type 1c, menorrhagia, recurrent epistaxis, bleeding from minor trauma, and ventricular septal defect presented at 37 weeks gestation for induction of labor due to gestational hypertension. The patient previously underwent extensive workup for vWD as a child given her history of recurrent bleeding and known paternal history of vWD. Subsequently, a formal diagnosis of the type 1c variant was made using D-amino D-arginine vasopressin (DDAVP) stimulation testing, which demonstrated a partial, unsustained response in vWF antigen, consistent with vWF type 1c5. Coagulation studies at 29 weeks gestational age were remarkable for vWF activity of 15% (ref: 50-200%), vWF antigen of 37% (ref: 50-160%), and Factor VIII activity of 62% (ref: 55-180%). A complete blood count obtained at 37 weeks prior to induction demonstrated RBC of 4.18 (ref: 4.20-5.40 x106/µL), Hgb of 12.6 (ref: 12.0-16.0 g/dL), and platelet count of 282 (ref: 140-400 x103/µL).

A loading dose of 3670 units of Humate-P (human antihemophilic factor/vWF complex) was administered 30-60 minutes prior to epidural placement. The dose was calculated at 50 U/kg using vWF activity of 15% with a goal of maintaining >50%. The epidural catheter was introduced at the L3-L4 interspace using a midline approach. The catheter was aspirated and was negative for blood or CSF. A test dose of lidocaine 1.5%-epinephrine 1:200,000 was incrementally administered, with resulting heart rate increase <10 bpm, indicating successful cannulation of the epidural space. The patient underwent vaginal delivery without complication and minimal bleeding. One gram of tranexamic acid was administered postpartum, and the patient was continued on Humate-P 2000 units (30 U/kg) every 12 hours for 5 days. She was discharged five days later on oral tranexamic acid (1300 mg TID for 5 days) without evidence of bleeding or spinal compression. The patient remained healthy and asymptomatic at 40-day follow-up post-discharge.

This case demonstrates the importance of careful planning and interdisciplinary collaboration in managing neuraxial anesthesia for parturients with rare coagulopathies like vWD type 1c. Administration of Humate-P, combined with careful monitoring and stepwise epidural placement, enabled safe labor and delivery without complications. This report adds to the limited literature on epidural management in vWD type 1c, demonstrating that neuraxial anesthesia can be safely performed with appropriate hemostatic optimization and close follow-up.

Comments

2025 Research Day Corewell Health West, Grand Rapids, MI, May 9, 2025. Abstract 1674

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