Among pregnant patients with non-acquired hemophilia, factor VIII or IX levels should be increased to greater than 50% before neuraxial technique implementation and delivery, according to research published in the Journal of Anesthesia.

Hemophilia A and hemophilia B, which are linked with factor VIII and factor IX deficiency, respectively, are rare, inherited, or acquired diseases that occur more frequently in females than in males, although the exact incidence of hemophilia in female patients is unknown. Delayed recognition in pregnant female patients is, furthermore, linked with poor preparation for delivery, as well as increased morbidity and mortality.

Poor preparation for delivery among pregnant patients with hemophilia is also associated with intrauterine growth retardation, antepartum hemorrhage, and postpartum hemorrhage, as well as higher rates of caesarean delivery, preterm delivery, and blood transfusion. Neuraxial anesthesia may also be withheld because of an increased risk of morbidity related to neuraxial hematoma.

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Current guidance, which is based on expert opinion, suggests that patients provided with neuraxial placement and delivery should have factor levels of at least 50%. For this systematic review, researchers evaluated treatment decisions and outcomes among patients with hemophilia with factor levels below and above the factor level benchmark of 50%.

Researchers conducted 2 sets of literature reviews, which were both completed in October 2019. The first set (13 articles) reported neuraxial techniques provided without reference to patient sex or pregnancy status. The second set (19 articles) reported outcomes among pregnant patients.

The first review suggested that 3 of 134 patients had neuraxial hematoma with paraplegia; all 3 patients had a factor level of 1%. The second review showed that, out of 2712 deliveries, postpartum hemorrhage occurred in 193 patients (7.1%); 60% of these patients required blood transfusion.

Overall, postpartum bleeding complications were nearly twice as likely where factor levels were below 50% (51% vs 25.6% in patients with factor levels above this benchmark; P <.001).

“In summary, we found low level evidence (Level 4) that factor VIII and IX levels should be greater than 50% for delivery and neuraxial techniques,” the authors wrote. “In our review of 134 neuraxial placements and 2712 deliveries, neuraxial hematomas were found with a factor level of 1% and hemorrhagic complications were higher when factor activity was <50%.”


Togioka BM, Burwick RM, Kujovich JL. Delivery and neuraxial technique outcomes in patients with hemophilia and in hemophilia carriers: a systematic review. J Anesth. 2021;35(2):288-302. doi:10.1007/s00540-021-02911-1