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Anesthestic and Pain Management in a Parturient with Type 2B von Willebrand Disease and Severe Thrombocytopenia
Abstract Number: S-05
Abstract Type: Case Report/Case Series
Introduction: Patients with bleeding disorders present unique challenges in anesthetic management. This case describes a patient with Von Willebrand Disease (vWD) Type 2B and severe thrombocytopenia requiring Cesarean delivery.
Case: A 30 year old G1 at 39 weeks gestation was admitted for labor induction due to vWD Type 2B and severe thrombocytopenia. A platelet count at 8 weeks gestation was 183k. By 37 weeks, this count decreased to 17k and platelets were transfused with minimal response. Induction was planned at 39 weeks given persistent severe thrombocytopenia. The plan by her hematologist was to administer Wilate, a vWF/Factor VIII complex, during labor and 5 days post-partum. Platelets would be transfused to maintain a count >50k.
Induction of labor was initiated with oxytocin after 2 units of platelets were transfused and an appropriate response to Wilate was seen. On day 2, she began having regular contractions with appropriate cervical dilation. Adequate analgesia was achieved with patient administered inhaled nitrous oxide for a period of time until membranes ruptured; at this time, her pain was intolerable. IV opioids, hydrotherapy, and TENS unit were all utilized with little effect. Cesarean delivery was planned for arrest of dilation at 7cm and suspected fetal occiput posterior position.
vWF panel showed Factor VIII levels of 192%, vWF antigen level of 217%, and vWF activity 200% compared to 67%, 43%, and 4% on admission. She received 2 units of platelets prior to the OR given a recent count of 20k. Given her previous poor response to platelet transfusion, spinal anesthesia was not considered. General anesthesia was induced uneventfully. The obstetricians noted the incision to be “oozy” and one unit of platelets was transfused. A vigorous female in the OP position was delivered 4 minutes after anesthetic induction. A right uterine artery laceration was repaired and no significant bleeding was noted. She remained stable, intraoperative platelet count was 54k and a thromboelastography (TEG) showed normal clot formation. She was extubated and taken to recovery in stable condition. During pregnancy, labor and delivery a total of 11 units of platelets were administered.
Discussion: Type 2 vWD is characterized by a qualitative defect in plasma vWF accounting for 20-30% of vWD cases. Type 2B is characterized by increased affinity of vWF for platelet glycoprotein Ib, resulting in spontaneous binding and clearance of large vWF multimers and platelets (1). This is exacerbated in pregnancy when levels of dysfunctional vWF are elevated. This patient required 11 platelet transfusions; neuraxial anesthesia was not considered safe given this severe persistent thrombocytopenia. Alternative methods of analgesia were offered with little effect; fetal malpresentation likely contributed to length of labor, pain and need for Cesarean delivery (2).
1. Am J Obstet Gynecol. 2010 Sep;203(3):194-200
2. Obstet Gynecol. 2001 Dec;98(6):1027