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Safe and successful: labor analgesia for a rare case of congenital factor XIII deficiency
Abstract Number: SUN-38
Abstract Type: Case Report/Case Series
Case: 33 y/o G5P0130 with a history of congenital factor XIII-B (FXIII) deficiency presented for induction of labor (IOL) at 39w1d gestational age (GA). She was followed closely by hematology and obstetrics given multiple miscarriages and a 25-week fetal demise likely due to her FXIII deficiency. Given this history, the frequency of her FXIII infusions were increased from monthly to every 2 weeks during IVF treatments and pregnancy (see table). Monthly FXIII trough levels were followed until 36 weeks GA, and the FXIII concentrate dose was adjusted based on these results (see table). At 36 weeks GA, FXIII dosing was increased to weekly intervals and weekly FXIII peak levels were assessed so that any necessary dosing adjustments could be made prior to IOL.
IOL was scheduled at 39 weeks GA to ensure that a dose of FXIII could be administered the day before delivery. An epidural catheter was placed uneventfully and provided effective labor analgesia. Estimated blood loss at delivery was 400mL. She began oral tranexamic acid on postpartum (PP) day 1 for one week and resumed pre-pregnancy FXIII infusion at 4 weeks PP.
Discussion: FXIII has a long half-life of 9-10 days, promotes fibrin crosslinking and is essential for clot stability, resistance to fibrinolysis, placental adherence and angiogenesis (1,2). Deficiency of FXIII has an estimated incidence of one in a million, and can result in bleeding complications, impaired wound healing, and frequent miscarriage secondary to poor placental adhesion (2-4).
Prophylactic FXIII infusions are recommended for to ensure successful pregnancy in these patients. Given the rarity of the disease, the optimal type, dose, and frequency of factor infusion are unclear and often rely on individual clinicians’ experiences. Literature suggests to maintain FXIII trough levels >10% during pregnancy and >20% for delivery (3,4). Given her obstetric history, higher FXIII levels were targeted (>20% during pregnancy; >30% for delivery) and ultimately this approach led to a successful outcome.
With recent peak FXIII levels ~100%, it was felt that her bleeding risk was minimal and safe epidural placement could be accomplished. To our knowledge, only one other case of neuraxial anesthesia in a parturient with FXIII deficiency has been reported (5).
Thromb Haemost 1983;49:102-5
Obstet Gynecol Surv 2007;62:255-60