Title : Interesting and a rare case of incidental diagnosis of dysfibrogenemia in an uncomplicated pregnancy- A case report
Abstract:
Objective: To present an interesting case of diagnosis and management of undiagnosed dysfibrinogenemia in an otherwise uncomplicated pregnancy and its outcome.
Case Report: A 29/F, G1, BMI 38kg/m2 with a previous history of surgical intervention without any bleeding or complications, family history of thrombosis but an uncomplicated pregnancy and a normal grown baby admitted for IOL at 39+2 weeks due to spontaneous rupture of membranes. IOL commenced as per protocol and patient monitored for normal progress of labor. Later on, decision for a Category 2 Caesarean section (CS) taken for failure to progress and suspected sepsis secondary to prolonged SROM. Anesthetic review and Bloods done that showed preop fibrinogen level of 0.9 (repeated twice) followed by 2g fibrinogen transfusion and Cat 2 CS done with a Bakri Balloon and 1 vaginal pack in situ. No intra-operative complications otherwise. Postop plan for HDU care was to repeat bloods in 6 hours. If normal then for routine postop thromboprophylaxis and to continue with sepsis protocol. Postop repeated fibrinogen level still 0.8 but uneventful recovery. Referral to Hematology team as in patient and provisional of diagnosis of either Dysfibrinogenemia or hypofibrinogenemia made. As per their advice, fibrinogen level and fibrinogen ELISA done, patient discharged home (as no other concerns) with thromboprophylaxis for 6 weeks and a follow up in Hematology Clinic. Patient seen by Consultant Hematologist at 3 and 7 weeks postnatal and a diagnosis of Dysfibrinogenemia was confirmed after thorough investigations and a genetic link was established and a Plan for further routine follow up with Hematology made as per protocol.
Discussion: A case report by Yamanaka10 and Miesbach11 et al suggests that an asymptomatic pregnancy with dysfibrinogenemia but no obstetric complications doesn’t warrant treatment unless complications such as bleeding or thrombosis occur. Literature suggests that Women with Congenital dysfibrinogenemia (CD)can experience similar obstetric complications as those with afibrinogenemia. However, these patients might not be diagnosed as CD can be asymptomatic.
Conclusion: CD is a rare but potentially serious condition that puts the pregnant women at high risk of obstetric complications such as bleeding, blood clots and pregnancy loss. The complex management requires a multidisciplinary approach so that timely detection and appropriate management with Fibrinogen replacement therapy can help prevent the complications and result in an uneventful delivery.
Audience Take Away:
- CD is a rare but potentially dangerous condition with limited information on its management. And can cause serious complications such as Hemorrhage, Pregnancy losses and thrombosis. By discussing these cases and literature reviews we can bring awareness and insight into managing such complex rare conditions in a much better way that can help prevent any complications aiming for uneventful deliveries.
- CD requires a multidisciplinary approach that involves Obstetrician and Gynecologist, Hematologist, Anesthetist, Maternal-fetal medicine, Blood bank services and Pharmacists as well.
- CD can be very difficult to diagnose and equally challenging to manage in pregnancy and a standard guideline needs to be established for its management.
- By gaining Knowledge and working on such cases some serious complications in an otherwise uncomplicated pregnancies can be prevented.
