Asian Hematology Research Journal

Background: Perioperative hemostasis for major surgery in Hemophilia A has been cumbersome with clotting factor concentrate (CFC) administration based on half-life and factor activity to minimize fluctuation. Availability of newer agents like FVIII mimetic Emicizumab with steady state levels in blood and long half-life as an adjunct has helped to reduce fluctuations of hemostasis.

Case report: A 11-month-old male with Moderate Hemophilia A, a previously untreated patient with sagittal craniosynostosis was started on Emicizumab prophylaxis. Elective anterior 2/3^rd calvarial remodeling surgery was undertaken while continuing Emicizumab prophylaxis with additional FVIII clotting factor concentrate.Chromogenic assays were used to confirm factor activity levels for hemostasis. The patient underwent surgery without perioperative bleeding complications, with FVIII support continued for eight days postoperatively. Emicizumab prophylaxis was continued subsequently, with ongoing monitoring to ensure optimal surgical wound healing.

Conclusion: This case highlights the complexity of managing moderate hemophilia A in a pediatric patient requiring major surgery while using Emicizumab, which, along with FVIII CFC, ensured adequate hemostasis and reduced hospitalization by allowing Factor VIII infusion to stop after Day 8. Limited data exist on Emicizumab use in congenital hemophilia A patient undergoing neurosurgery, emphasizing the importance of careful preoperative planning, including arranging factor products and laboratory reagents. Unlike traditional prophylaxis with CFCs, which requires frequent monitoring and venous access, Emicizumab offers advantages of subcutaneous administration, reduced hospital visits, and effective hemostasis with a lower risk of spontaneous or recurrent bleeding.