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///2018 Abstract Details
2018 Abstract Details2019-08-02T15:57:01-05:00

A Parturient With Hemophilia B Presenting with Eclamptic Seizures and Previously Undiagnosed Pregnancy

Abstract Number: S5C-7
Abstract Type: Case Report/Case Series

Leslie J. Matthews MD, PharmD1 ; Kathleen A. Smith MD2

Case: A 27 year-old G1P0 presented to an outside hospital with altered mental status, where she had multiple prolonged grand mal seizures. She was intubated for airway protection. Head CT showed no focal abnormalities but diminished gray-white matter differentiation, concerning for PRES. Urine pregnancy test was positive, confirming a diagnosis of eclampsia. The fetus was estimated to be 30 weeks gestation by ultrasound. The patient’s family had no knowledge of the pregnancy. Magnesium sulfate infusion was initiated and the patient and was transferred intubated to our tertiary center for management.

Prior to arrival, the anesthesia, obstetric and neonatal teams were notified of patient transfer and preparations were made to proceed with emergent cesarean delivery (CD) upon arrival secondary to maternal status and reported category III fetal tracing. The patient was transported from our emergency department to the OR intubated. The anesthesia team received report, including a patient history of heterozygous hemophilia B, with last known Factor IX (FIX) levels 14%. A multidisciplinary discussion was held, where it was decided to postpone CD to investigate the patient’s coagulation status. Hematology was consulted emergently and FIX was ordered. Arterial line and additional peripheral intravenous access were obtained. Less than 90 minutes after arrival, a FIX bolus was administered. CD commenced under general anesthesia. FIX infusion was continued throughout delivery. Estimated blood loss was <1000ml; no transfusion required. The patient remained hemodynamically stable, and was transferred to the ICU intubated, sedated, and on continuous magnesium sulfate infusion. She was extubated PPD 0, maintained on FIX throughout hospitalization and discharged PPD 7 on anti-hypertensive medication. Postpartum genetic counseling revealed the patient’s father and newborn are both affected by hemophilia B, resulting from 1097C>T mutation on the FIX gene.

Discussion: Clinically significant heterozygous hemophilia B in females is rare. Symptomatic carriers are at risk for surgical bleeding. Prior to surgery, transfusion (50-80U/Kg) is recommended if FIX<60%, with a target of at least 50%1. Unlike hemophilia A, factor levels do not significantly rise during pregnancy. Our patient had both hemophilia and eclampsia, requiring the care team to weigh maternal/fetal risk with respect to delivery timing. Given her last FIX levels were 14%, she was at risk of severe peripartum hemorrhage. Delivery was urgent given eclampsia and non-reassuring fetal status. It was decided to continue maternal/fetal monitoring in the OR while awaiting FIX, prior to CD. Antepartum anesthesia consultation is recommended for patients with bleeding disorders. The patient’s undiagnosed pregnancy and lack of prenatal care made this case particularly challenging.

SOAP 2018