ABSTRACT : Congenital hypofibrinogenemia is an Autosomal Recessive disorder. Fibrinogen is necessary for normal platelet aggregation and functional fibrinogen level should be more than 0.5g/l to prevent microvascular bleeding. Women with hypofibrinogenemia have recurrent pregnancy loss, Ante partum haemorrhage, Postpartumhaemorrhage and Thromboembolism. Ideal Fibrinogen concentrate should be used prophylactically through out the pregnancy, once pregnancy is confirmed.

CASE REPORT : 36 year old female, a known case of Congenital Hypofibrinogenemia with obstetric score G7 A6 and Gestational diabetes on diet control admitted for safe confinement at 35 weeks of gestation. Baseline investigations, coagulation profile and Echocardiography study was normal. Considering Bad obstetric history she was on regular human plasma fibrinogen every 4 to 5 days from the time of conception maintaining fibrinogen level > 100mg/dl. Proceeded for elective LSCS under GA with controlled ventilation. Intraoperatively uterus well contracted but diffuse bleeding was present, managed conservatively with intravenous fluids, misoprostol, methergine, oxytocin, 6 units Cryoprecipitate and Human fibrinogen 2.75 gm. Extubated on table after return of spontaneous circulation, good haemodynamics, adequate urine output and intact airway reflexes.Monitored in postoperative ward with DVT prophylaxis and shifted to ward after 24 hours. Discharged from hospital on 8th day. This case is presented in view of interesting and rare presentation.

REFERENCES:

• Congenital hypofibrinogenemia and pregnancy – case report TUNIS med 1994 Feb ; 72(2) : 107 – 109


• Obstetric anaesthesia and uncommon disorder. David P.Gambling, Robert SF.MFckay 2008