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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.
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).
Background:
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.
Objective:
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.
Methods:
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.
Results:
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.
Conclusions:
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.
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