Perioperative Prophylaxis in Pediatric Factor VII Deficiency: Experience From Rabat Children's Hospital

Factor VII (FVII) deficiency is a rare inherited bleeding disorder characterized by heterogeneous clinical manifestations and unpredictable bleeding risk. Surgical procedures in affected children may expose them to significant perioperative bleeding, highlighting the importance of appropriate prophylactic management.

The objective of this study is to evaluate postoperative hemostatic outcomes and hemorrhagic complications according to the type of surgery and perioperative prophylaxis in children with FVII deficiency.

We conducted a retrospective review of the medical records of patients with confirmed FVII deficiency who underwent surgical procedures. Collected data included demographic characteristics, baseline FVII activity levels, type of surgery, perioperative prophylaxis, and postoperative outcomes.

Eight children aged 4-6 years were included. Baseline FVII activity levels ranged from 1.6% to 22%. Surgical procedures included circumcision, hernia repair, tonsillectomy, and dental extractions. Prophylaxis consisted mainly of recombinant activated factor VII at 30 µg/kg administered 30 minutes before surgery, while one patient received fresh frozen plasma (FFP) at 20 mL/kg. Postoperative bleeding was minimal overall. Two patients developed complications: a scrotal hematoma in the patient who received FFP prophylaxis and a bleeding episode revealing previously undiagnosed FVII deficiency. No thrombotic events were observed.

Perioperative prophylaxis with rFVIIa was associated with favorable hemostatic outcomes in children with congenital FVII deficiency undergoing surgery. A single preoperative dose appeared sufficient for minor procedures in this cohort; however, these findings should be interpreted with caution, given the small sample size (n=8), and require confirmation in larger studies.