Congenital hypofibrinogenemia in pregnancy: a report of 11 cases.

: To investigate the obstetrical outcomes and plasma concentrations of fibrinogen (FIB) in patients with congenital hypofibrinogenemia in pregnancy, 11 cases with hypofibrinogenemia in pregnancy were analyzed retrospectively. The demographic database, bleeding tendency, plasma levels of FIB throughout pregnancy, peripartum management, as well as, maternal and neonatal outcomes were assessed. FIB levels in our patients remained relatively stable throughout the pregnancy. The mean FIB levels during the three trimesters of pregnancy were 75.7 ± 43.9 (25-148), 67.6 ± 33.7 (22-146), and 77.9 ± 29.2 (32-148) mg/dl, respectively. Twelve full-term deliveries were achieved using FIB replacement therapy only on the group of labor or cesarean deliveries. The postpartum courses were unremarkable without hemorrhagic or thrombotic complications. Five out of twelve neonates were diagnosed with low levels of FIB in 6 weeks after birth. The pregnancies were uneventful with no signs of bleeding in these congenital hypofibrinogenemia women. Vaginal delivery, instead of cesarean section, may be the superior choice. Successful maternal and neonatal outcomes could be achieved by accurate monitoring of the FIB levels and adequate supportive therapy.