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

Chaymaa El Messari1, Zineb Isfaoun1, Naoual El Ansari1

  • 1Hemophilia and Inherited Bleeding Disorders Treatment Center, Pediatric Hematology and Oncology Center, Ibn Sina University Hospital Center, Mohammed V University, Rabat, MAR.

Cureus
|May 29, 2026
PubMed

Insights

Perioperative prophylaxis with recombinant activated factor VII (rFVIIa) is effective for managing children with factor VII deficiency undergoing surgery. A single dose showed favorable hemostatic outcomes, minimizing bleeding risks in this rare bleeding disorder.

Area of Science:

  • Pediatric Hematology
  • Hemostasis and Thrombosis
  • Rare Bleeding Disorders

Background:

  • Factor VII (FVII) deficiency is a rare inherited bleeding disorder with unpredictable bleeding risks.
  • Children with FVII deficiency face significant perioperative bleeding risks during surgery.
  • Effective prophylactic management is crucial for surgical safety in these patients.

Purpose of the Study:

  • To evaluate postoperative hemostatic outcomes in children with FVII deficiency.
  • To assess hemorrhagic complications based on surgical type and perioperative prophylaxis.
  • To determine the efficacy of prophylaxis in managing FVII deficiency during surgical procedures.

Main Methods:

  • Retrospective review of medical records for pediatric patients with confirmed FVII deficiency undergoing surgery.
  • Data collection included patient demographics, FVII activity levels, surgical procedures, and perioperative prophylaxis used.
  • Postoperative outcomes, including bleeding events and complications, were analyzed.

Main Results:

  • Eight children (ages 4-6) with FVII deficiency underwent various procedures (e.g., tonsillectomy, dental extractions).
  • Recombinant activated factor VII (rFVIIa) prophylaxis (30 µg/kg) was primarily used, with one patient receiving fresh frozen plasma (FFP).
  • Minimal postoperative bleeding was observed; one FFP-treated patient had a scrotal hematoma, and no thrombotic events occurred.

Conclusions:

  • Perioperative rFVIIa prophylaxis demonstrated favorable hemostatic outcomes in pediatric FVII deficiency patients undergoing surgery.
  • A single preoperative dose of rFVIIa may be sufficient for minor procedures.
  • Larger studies are needed to confirm these findings due to the small sample size (n=8).
Abstract

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