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Hypodysfibrinogenemia in a Pregnant Patient: Peripartum Management and Thromboelastogram Results.
Abstract Number: T-40
Abstract Type: Case Report/Case Series
Introduction: Hypodysfibinogenemia is typically an autosomal dominant disorder characterized by both quantitative and qualitative fibrinogen deficiency with variable degrees of penetrance (1). Adverse outcomes include spontaneous abortion (typically at 8-10 weeks gestation), preterm abruption, postpartum hemorrhage and thrombosis (2). Fibrinogen concentrate is often utilized to prevent these complications throughout pregnancy and labor (3). We report the obstetric anesthetic management and the first reported use of a labor epidural for a patient with hypodysfibrinogenemia.
Case Report: A 30 year old G1P0 at 36 weeks and 0 days with a history hypodysfibrinogenemia presented with absent fetal heart tones. Her pre-pregnancy baseline fibrinogen levels varied between 20 and 60 mg/dl and she had history of abnormal bleeding after a loop electrosurgical excision procedure. During the antenatal period, she received fibrinogen concentrate to maintain her fibrinogen nadir above 50, 100 and 150 mg/dl during 1st, 2nd and 3rd trimester, respectively. Based on multidisciplinary consensus for her labor admission, an arterial line was placed for frequent laboratory draws, including fibrinogen and thromboelastogram. She received an initial fibrinogen concentrate bolus of 2174mg, followed by an infusion 90 mg/hr titrated to maintain a fibrinogen level of approximately 200 mg/dl throughout labor. Fibrinogen levels during labor and fibrinogen infusion rate are shown in Fig 1. A labor epidural was performed when her fibrinogen concentration was greater than 150 mg/dl. She underwent an uncomplicated vaginal delivery with no abnormal bleeding. Fibrinogen concentrate infusion was continued for 24 hours after delivery before being discontinued and allowing to drift to baseline.
Discussion: A threshold fibrinogen level for safe neuraxial block placement is unknown. For this case, we elected to use a threshold of greater than 150 mg/dl. Individualized care for patients with hypodysfibrinogenemia requires early multidisciplinary input from obstetricians, anesthesiologists and hematologists. In order to lessen the potential risk of postpartum hemorrhage and neuraxial hematoma, these patients require fibrinogen supplementation during the peripartum period.
1. Semin Thromb Hemost. 2013 Sep;39(6):585-95.
2. Ann Hematol. 2007 Sep;86(9):693-4.
3. Haemophilia. 2016 Nov;22(6):898-905.