FVII deficiency is characterized by wide molecular and clinical heterogeneity. The gene encoding FVII, F7, is located on chromosome 13 (13q34) and consists of nine exons and eight introns over a 12.8 kb genomic region. More than 250 mutations of F7 have been reported and missense mutations being the major type [
9]. In congenital FVII deficiency patients, FVII levels are usually less than 10% of the normal range in homozygous or double heterozygous carriers and approximately 20% to 60% in heterozygous carriers. FVII levels usually increase in normal pregnancy, but no increase is seen in homozygous cases and only a moderate increase occurs in heterozygous individuals. Common presenting symptoms are bleeding into joints and muscles, excessive bruising, menorrhagia and epistaxis. The clinical severity of this disorder varies from life-menacing to symptomless [
4]. However, compared to hemophilia A and B, there is a poor correlation between the FVII level and the severity of the bleeding [
10]. This feature makes it difficult to predict which patients may need prophylactic replacement therapy prior to hemostatic challenges. The decision about such therapy should be based on previous bleeding episodes, FVII levels in a prenatal test, and the mode of delivery [
11]. In spite of the poor correlation, the most serious bleeding is linked with low FVII activity levels and surgery [
10]. In our case, the FVII activity of the patient was decreased (7.00%) in the prenatal test. The patient also had a history of successful bleeding control by preventive use of FFP preceding a dental extraction. From our patient's medical history, we decided to use only FFP and antifibrinolytic agents for surgical bleeding prevention. FFP is generally applied in most developing countries to achieve adequate hemostasis and there have been good results in mild cases. However, its application is related with several side effects, such as transmission of blood-borne viruses and blood volume overload. Recombinant activated FVII (rFVIIa, NovoSeven, Novo-Nordisk, Bagsvaerd, Denmark) has many advantages compared to blood products. These include more efficient FVII level elevation and no risk of blood-borne virus transmission. In postpartum hemorrhage, common non-operative management involves the use of uterine stimulants, bimanual compression of the uterus and uterine artery embolization [
12]. More recently, rFVIIa has also been used to prevent or control bleeding in postpartum hemorrhage [
13]. However, it has thrombotic risks, and there are no definitive target FVII levels or optimal treatment regimens to maintain hemostasis [
14]. rFVIIa also has insufficient clinical data in the obstetric field, so its application is still limited. Antifibrinolytic agents are useful as adjunctive therapy in bleeding disorders. Tranexamic acid competitively restricts the activation of plasminogen to plasmin and improves clot stability. It is useful in bleeding control that occurs from mucosal surfaces and skin (oral bleeding, epistaxis, and menorrhagia) [
15]. Vitamin K can be administered in cases of secondary hypoprothrombinemia that occurs due to vitamin K absorption or a synthesis problem. Although our patient seemed to have congenital FVII deficiency, we used vitamin K for prevention because a definitive diagnosis from genotyping was never obtained.
In conclusion, FVII deficiency can make excessive bleeding in surgical interventions, such as postpartum hemorrhage in cesarean sections. Clinicians need to ascertain the expected bleeding risk based on previous bleeding episodes and FVII levels. Replacement blood product therapy is a convenient process and mostly effective in patients with mild bleeding disorders. However, it has some adverse outcomes and rFVIIa can be more effective in elevating FVII levels and controlling bleeding in severe cases. Further study and evaluation are required on rFVIIa application in the obstetric field, and the use of rFVIIa should be considered in severe cases. In addition, careful examination of thrombotic signs and symptoms are required in the outpatient follow-up, as well as during the hospitalization period.