先天性纤维蛋白原缺乏症是一种罕见的常染色体遗传病，由于机体合成的纤维蛋白原数量减少（甚至缺如）或/和分子结构异常而造成凝血功能障碍，该病临床表现多样，有的患者无出血症状，有症状者表现为自发出血、手术或外伤后出血、颅内出血、血栓栓塞、习惯性流产、胎盘早剥等［1-3］。先天性纤维蛋白原缺乏症产妇往往出现严重的产科并发症，包括自然流产、胎盘早剥、产后出血等。本文报道4例合并先天性纤维蛋白原缺乏症产妇的剖宫产围术期管理，并进行文献回顾。

Congenital fibrinogen deficiency is an autosomal recessive or dominant disorder in which quantitative (afibrinogenaemia or hypofibrinogenaemia) or qualitative (dysfibrinogenaemia) defects in the fibrinogen Aa, Bb or c protein chains that lead to reduced functional fibrinogen. We now report the perioperative management of 4 pregnant women suffering from hypofibrinogenaemia scheduled for elective caesarean section from December 2012 to October 2016 in Peking University First Hospital and review this disease with reference to classification, symptom, replacement therapy, and selection of the modes of pregnancy termination and anesthesia. The four patients were all asymptomatic, whereas there existed recurrent pregnancy loss (case 3), family history (case 2), and offspring heredity (cases 3 and 4). Routine clotting studies revealed low fibrinogen levels and prolonged thrombin time (TT) during pregnancy and on admission .However, the platelet (PLT) count, prothrombin time (PT) and activated partial thromboplastin time (APTT) were normal. All the patients were administered fibrinogen concentrate perioperatively, and underwent uncomplicated combined spinal-epidural anesthesia and uneventful surgical procedure without postpartum hemorrhage. The replacement therapy of fibrinogen or fresh frozen plasma administration was essential to avoid anesthesia and obstetric complications. Regional blockade could safely be offered in the caesarean section, providing that their coagulation defect was corrected by availability of therapeutic products and adequate response to treatment. In addition, the point-of-care rotational thrombelastometry (ROTEM) or thrombelastogram (TEG) could play an important role in an optimal perioperative management for such patients. Management plans must be tailored to each individual, taking into consideration their bleeding risk as well as potential maternal and neonatal complications.