2023 FSA Podium and Poster Abstracts
P057: A PARTURIENT WITH VON WILLEBRAND DISEASE (VWD) TYPE 2B FOR EMERGENT CESAREAN DELIVERY
Nicholas Baltera, DO; Reine Zbeidy, MD; University of Miami/Jackson Health System
Introduction: Von Willebrand disease (VWD) is the most common inherited bleeding disorder, with a prevalence of approximately 1-2% of the general population.5 It is characterized by quantitative or qualitative abnormalities of Von Willebrand Factor (VWF).1 Type 2b is a qualitative deficiency, that involves increased platelet binding by VWF due to a gain of function mutation enhancing the binding to Gp1b. This leads to accelerated clearance and sequestration of platelets, which causes thrombocytopenia and decreased VWF.1
Parturient patients with VWD, require careful consideration with attention to their specific subtype and bleeding risk. Although pregnancy can increase procoagulant factors, uncertainty about coagulation status may preclude or delay the use of neuraxial anesthesia in these patients.5 There are no definitive guidelines for the anesthetic management of VWD in parturients, decisions should be made on a case by case basis.
Case Presentation: A 33 year old female at 37 weeks gestation, with history of VWD Type 2b and chronic thrombocytopenia was admitted for pre-delivery workup and a plan for induction of labor after optimization. Her non-pregnant baseline platelets were 30,000. Physical exam was reassuring.
On admission, the patient was seen by Hematology. Initial Platelet count was 13,000. The plan for induction of labor was developed after a multidisciplinary meeting between the Obstetric, Hematology, and Obstetric Anesthesia services. Due to the patient’s specific coagulopathy, regional techniques were deferred for labor analgesia and in the case of emergent C-section.
Induction of labor began five days after admission. Eventually, the patient was called for an emergent cesarean delivery due to fetal tachycardia. The patient received four units of platelets in transit to the OR. General anesthesia with rapid sequence induction was performed. The patient received another four units of platelets on incision, and four units during closure. Her surgery was otherwise uncomplicated; she received 1500mL of crystalloid, with an estimated blood loss of 1000 mL.
Her postoperative course was without major events. She was discharged on postoperative day seven.
Discussion: This case exemplifies the difficulty associated in developing treatment plans for parturient Pt’s with coagulopathies. Due to the risks of post partum hemorrhage and potential complications with neuraxial anesthesia, these patients require careful consideration. VWF activity, VIII activity should be monitored throughout pregnancy, and for patients with subtype 2B, platelet count should also be monitored. Therapies available to treat the coagulopathy seen with VWD include VWF concentrates, antifibrinolytic agents, and DDAVP.4 There are no definitive guidelines that specifically address how to treat these patients. However the American Society of Hematology and the British Committee for Standards in Haematology offered recommendations to aid obstetric anesthesia team’s decisions for individualized care.
The minimal evidence and lack of explicit guidelines for parturients with VWD creates complex decisions for their invasive treatment plans. These should be individualized to each patient. The ultimate decision whether to proceed with invasive procedures is up to the obstetric anesthesiologist. Multidisciplinary discussion between anesthesia, hematology, and obstetrics and with shared decision making with the patient, should be utilized.1