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Surgery in patients with congenital factor VII deficiency - a single center study.

INTRODUCTION
Congenital factor VII deficiency is a rare hemorrhagic disorder inherited in an autosomal recessive pattern. Surgical treatment with insufficient diathesis correction is burdened with high risk of bleeding complications. The aim of the study was evaluation of the surgical outcome in patients with congenital factor VII deficiency and assessment of the efficacy and safety of recombinant activated factor VII (rFVIIa) used for perioperative hemostatic coverage in our two schemas of substitutive therapy.


MATERIAL AND METHODS
In the years 2002-2017 a total of 22 patients with congenital factor VII deficiency were subjected to surgery. Substitution therapy relied on rFVIIa used in two schemas. One involved 15 patients with factor VII activity of<10% of normal value who were injected rFVIIa at a dose of 30 μg/kg b.w. every12 hours on surgery day, 15 μg/kg b.w. every 12 hours on the first postoperative day and 15 μg/kg b.w. every 24 hours on the following days. The second schema involved 7 patients with factor VII activity of 10-25% of normal value who were given rFVIIa at a dose of 15 μg/kg b.w. every 12 hours on surgery day and the first postoperative day; then the same dose was administered every 24 hours on consecutive days. The treatment continued for 4-10 days.


RESULTS
In the 22 patients a total of 26 surgeries were performed; 17 surgeries in 15 patients with factor VII<10% of normal and 9 in 7 patients with factor VII deficiency of 10-25% of normal. The surgeries included: 9 cholecystectomies (8 laparoscopic,1 open), 7 thyroidectomy procedures, 2 exploratory laparotomies, 1 left hemicolectomy, 1 total proctocolectomy, 3 inguinal hernia repairs and 3 excisions of varicose veins. One patient with factor VII activity of 9% required an additional dose of rFVIIa in the intraoperative period due to diathesis bleeding. Intraoperative hemostasis was normal for all other patients; no postoperative hemorrhagic complications were reported. In patients with FVII activity<10% average daily dose of rFVIIa was 31.3(range 20-56) μg/kg b.w., total daily dose 186(136-303) µg/kg b.w., total dose of rFVIIa-15.2(12-112) mg. In patients with FVII activity 10-25% the doses were 21.2(15-31), 117(46-271) µg/kg b.w. and 9.1(6-17) mg respectively.


CONCLUSIONS
Surgery in patients with congenital factor VII deficiency can be safely and efficiently performed with rFVIIa as substitutive treatment securing perioperative hemostasis.