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Type II Von Willebrand Disease and Known Difficult Airway in A Twin Pregnancy
Abstract Number: F 65
Abstract Type: Case Report/Case Series
Introduction: Von Willebrand disease (VWD) is the most common inherited bleeding disorder. Type II VWD results from dysfunctional von Willebrand factor (VWF) and replacement therapy is indicated during pregnancy to avoid peripartum hemorrhage and to allow neuraxial anesthesia (1).
Case Presentation: A 39 year old G6P0 presented with preterm contractions at 28 weeks with a twin gestation. Her type II VWD initially presented as heavy menses. Her airway exam was Mallampati class 2, full range of motion, and normal thyromental distance but narrow chin. She had 2 abdominal surgeries complicated by difficult intubation at outside institutions. At our institution she had one awake fiberoptic intubation for a myomectomy and one direct laryngoscopy under general anesthesia with a grade 3 view requiring a bougie for an exploratory laparoscopy.
Prior to CS, hematology recommended replacement therapy with antihemophilic factor/VWF complex (Humate P), which is effective immediately following transfusion. Obstetric anesthesia discussed with MFM the concern for delay if an emergency CS became indicated given the bleeding disorder requiring replacement therapy prior to neuraxial anesthesia and the difficult airway preventing rapid induction of general anesthesia. Four days after admission, twin A developed a category 2 tracing with persistent variable decelerations, and the decision was made to perform a CS at that time. She received Humate P 60 U/kg immediately prior to spinal anesthesia with bupivacaine 12mg, fentanyl 20mcg, and morphine 0.2mg. Supraglottic airways, a video laryngoscope, and a fiberoptic bronchoscope were immediately available. The patient delivered two infants with Apgars 8 and 9. Due to peri-incisional discomfort, she received intravenous midazolam 2mg and fentanyl 80mcg. The estimated blood loss was 1200mL, the surgery was finished in 89 minutes, and she had an uneventful recovery from anesthesia. Humate P was redosed 12 hours post delivery and antifibrinolytic therapy was started with aminocaproic acid. There was no postpartum hemorrhage, and she was discharged home after four days.
Discussion: Type II VWD results from a qualitative defect in VWF. Since desmopressin promotes release of VWF from endothelial storage, it is less likely to be effective in type II VWD. Instead, VWF replacement therapy is used perioperatively. The recommendations for neuraxial anesthesia in the setting of VWD are not well established, but neuraxial technique is thought to be safe when VWF and factor VIII levels are ≥ 0.5 IU/mL (2). Although this patient’s prior abdominal procedures could have prolonged surgical duration, spinal anesthesia instead of CSE was selected to decrease the risk of epidural hematoma. While general anesthesia was ultimately avoided, the choice of spinal anesthesia definitely increased the risk of having to manage a difficult airway intraoperatively.
1) Pacheco LD, Am J Obstet Gynecol 2010
2) Choi S, Anesth Analg 2009