Anesthetic Management of Von Willebrand Disease in Pregnancy: A Retrospective Analysis of a Large Case Series

BACKGROUND: Although Von Willebrand disease (vWD) is the most common heritable bleeding disorder, there are limited reports regarding the safety of neuraxial anesthesia in the obstetric population and no definitive guidelines specifying recommended pretreatment or therapies for patients with vWD. The aim of this study is to describe the anesthetic management of pregnant patients with vWD at a large tertiary-care center. METHODS: In this retrospective analysis, the study population was identified from vWD patients evaluated by our high-risk obstetric anesthesia consultation service and by diagnosis codes from our institutional research database registry. We manually reviewed records of patients with vWD in pregnancy who delivered at our institution between January 1, 2000 and January 1, 2019 for demographic characteristics, circumstances of vWD diagnosis, history of bleeding, laboratory studies, and overall management of vWD. Anesthetic management of vWD was at the discretion of individual providers, based on multidisciplinary consensus and the specific circumstances of each patient's disease and obstetric presentation. RESULTS: We identified 106 deliveries among 71 individual vWD patients. Of the unique patients, 54 had vWD type 1, 6 had vWD type 2, and 11 had vWD type unknown. Forty-three cases (40.6%) were cesarean deliveries. Neuraxial techniques were used in 94 of 106 deliveries (88.7%). Treatment with desmopressin or Von Willebrand factor/factor VIII concentrate before neuraxial anesthesia occurred in 27 of 94 neuraxial anesthetics (28.7%). Eleven deliveries (10.4%) were complicated by postpartum hemorrhage (PPH), defined as estimated blood loss of >= 1000 mL. There were no noted adverse anesthetic outcomes (0 of 106; 0% [95% confidence interval, 0-3.4]), including neuraxial hematoma or thromboembolic events. CONCLUSIONS: In this large case series, the majority of vWD patients received neuraxial anesthesia for labor and delivery, with no noted adverse events. This suggests that neuraxial anesthesia can be safely performed with the peripartum management that we describe. Pretreatment was dictated by the type and severity of vWD. Multidisciplinary planning is important to optimize the coagulation status of patients with vWD and facilitate options for analgesia and anesthesia.