Intracranial Hemorrhage as a Life-Threatening Complication of Congenital Factor VII Deficiency Associated With...

Congenital factor VII (FVII) deficiency is an uncommon autosomal recessive bleeding disorder with highly variable clinical expression. Although most patients present with mild to moderate bleeding symptoms, severe cases may manifest with life-threatening hemorrhage, particularly intracranial bleeding. The emergence of inhibitors against exogenous FVII is exceedingly rare but poses a significant therapeutic challenge because of treatment refractoriness and an increased risk of major bleeding. We describe a five-year-old Saudi girl with severe congenital FVII deficiency diagnosed in the neonatal period who developed inhibitory antibodies while on regular recombinant activated FVII (rFVIIa) prophylaxis. She presented with acute intracranial hemorrhage and was successfully managed with rFVIIa despite the presence of a high-titer inhibitor. This report highlights the importance of early recognition of inhibitor development in FVII deficiency and the need for individualized therapeutic approaches in rare bleeding disorders.