Bleeding prophylaxis in a child with cleft palate and factor VII deficiency: a case report

Area of Science:

• Medical Genetics
• Hematology
• Pediatric Surgery

Background:

• Factor VII deficiency is a rare inherited bleeding disorder.
• Cleft palate is a common congenital condition.
• Surgical correction of cleft palate is standard, but challenging in patients with bleeding disorders.

Observation:

• A pediatric patient presented with both cleft palate and severe factor VII deficiency.
• The patient required surgical intervention for palatoplasty.

Findings:

• A reduced dosage regimen of recombinant factor VIIa (rFVIIa) was administered perioperatively (15 microg/kg every 12 hours).
• This regimen successfully maintained normal prothrombin time and prevented abnormal bleeding during and after palatoplasty.
• The dosage used was lower than typically employed for bleeding prophylaxis or treatment with rFVIIa.

Implications:

• This case demonstrates the efficacy of a reduced-dose rFVIIa regimen for surgical procedures in pediatric patients with factor VII deficiency.
• Lower rFVIIa doses may offer easier administration and comparable efficacy, particularly in pediatric settings.
• This approach could improve surgical outcomes for children with rare bleeding disorders and congenital anomalies.