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Congenital factor VII (FVII) deficiency is a rare autosomal recessive bleeding disorder characterized by marked clinical variability. The correlation between plasma FVII activity and bleeding severity is often poor, posing challenges in perioperative management. We report the case of a 25-year-old man with moderate congenital FVII deficiency (FVII:C 39%), diagnosed in infancy. He experienced severe bleeding after a dental extraction in 2008 but later underwent ileocecal resection in 2023 under recombinant activated factor VII (rFVIIa) coverage without complications. Preoperative laboratory investigations showed prolonged prothrombin time (PT 57% activity) with normal activated partial thromboplastin time (aPTT ratio 1.14), confirming isolated moderate FVII deficiency. A personalized perioperative protocol including rFVIIa (15-25 µg/kg IV), tranexamic acid, and local hemostatic measures was implemented. The patient tolerated the procedure well, with no postoperative bleeding or thrombosis. This case highlights that even moderate FVII deficiency can carry significant surgical bleeding risk, and emphasizes the importance of tailored perioperative management combining rFVIIa, antifibrinolytic therapy, and meticulous local hemostasis.
Ansari et al. (Thu,) studied this question.