FVII has a pivotal role in coagulation in vivo. Vascular injury leads to binding of FVII to Tissue Factor (TF), initiating coagulation and generating a concentration of thrombin at the place of vascular damage .
FVII deficiency was first recognized in 1951 by Alexander . Patients with heterozygous genotype are typically asymptomatic whereas homozygous or compound heterozygotes develop bleeding manifestations. FVII levels are generally less than 10% of the normal values in homozygous or double heterozygous carriers in inherited FVII deficiency however may be approximately 20–60% in heterozygous carriers . FVII level was less than 1% in first & 17.1% in second case with no haemorrhagic manifestations following delivery. FVII deficiency may be seen in chronic liver disease, warfarin users, vitamin K deficiency secondary to long term antibiotic use, bile duct obstruction or poor intestinal absorption . FVII deficiency was probably congenital, since no other aetiology was apparent in present cases which were managed with FFPs and FVII concentrates.
In reported cases, PT and APTT were performed multiple times as was done in our cases also and each time PT was prolonged and APTT was normal and hence, a deficiency of factor VII was suspected.
Women with heterozygous FVII deficiency have significantly higher FVII levels  with insignificant increase in FVII levels during pregnancy in women with homozygous (severe) FVII deficiency [12–15]. The risk of bleeding might be more in early pregnancy as compared to at term because of insufficient rise in FVII levels in early pregnancy . In our cases, FVII level was estimated at term or near-term gestation with very low levels. This suggests that probably these patients were homozygous or double heterozygous for a mutation in the FVII gene.
Management of inherited FVII deficiency includes replacement therapy with FFPs, factor VII concentrates, prothrombin complex concentrates, and/ or recombinant F VIIa (rFVIIa) . Previous bleeding episodes, recent FVII levels, mode of delivery and anticipated surgical intervention determine the decision about the treatment . Despite a poor correlation of low FVII levels, the most serious bleeding has been linked with postpartum hemorrhage (PPH) and surgery [7, 16]. Disadvantages of FFP are volume overload and the possibility of blood-borne infections. The treatment of choice is recombinant factor VIIa; however, the major issue with factor VII concentrate is a risk of disseminated intravascular coagulation and thromboembolic complications . As our first case had a history of postpartum hemorrhage in previous pregnancy and had severe FVII deficiency in index pregnancy, 2 units of rFVIIa were arranged but could be managed with FFPs. Prolonged second stage of labour and instrumental delivery which increases the risk of neonatal hemorrhage should be avoided .
66 pregnant women have been reported so far with factor VII deficiency [5, 7, 8, 9]. The median FVII activity was 5.5%. 76% women had level less than 10% and 82% had level less than 20%. Similar FVII level were found in women with or without a history of bleeding. Hemostatic prophylaxis was used in 30 deliveries with recombinant FVII in 17 deliveries, FFP and FVII concentrate in 6 deliveries each and both FVII concentrate and FFP in one delivery . Similar to first index case, case series included 8 women with FVII level < 1 %. 5 women were delivered vaginally and 3 by C.S. All 3 women who had cesarean sections, received rFVIIa as prophylaxis and in vaginal delivery group, one woman received FFP. Out of these eight patients, one had postpartum hemorrhage during cesarean despite receiving rFVIIa . Rate of hemorrhage was similar in the women who received prophylaxis and those who didn’t. Hence, hemostatic prophylaxis is not necessary in all cases and should be individualized . Yazicioglu et al  reported a case of FVII deficiency presenting as abruptio placentae at 29 weeks POG and was managed without any replacement therapy. Similar to first case, recently a case report of pregnancy in a known patient of FVII deficiency was successfully managed with antifibrinolytics and FFPs . Pinar HU et al reported dilatation and curettage at 9 weeks POG in a patient with FVII deficiency after prophylactic administration of FVII .
Prenatal diagnosis should be offered to parents by Chorionic villus sampling/ amniocentesis/ cordocentesis to those parents who had severely affected child or when both parents are heterozygous carriers. Physiologically low levels in newborn make it difficult to diagnose FVII deficiency in neonatal period but FVII levels should be determined from cord blood at time of delivery if severe deficiency is suspected .