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A case of cesarean section of a pregnant woman with congenital dysfibrinogenemia
Abstract Number: F5C-8
Abstract Type: Case Report/Case Series
Congenital dysfibrinogenemia(CD) is a rare disease characterized by qualitative congenital fibrinogen disorder despite of normal antigen levels and there are few reports on delivery of women diagnosed with CD. We report a case of anesthetic management using a portable fibrinogen measuring device against a caesarean section of a woman diagnosed with CD.
The patient is a 40-year-old, one parous pregnant woman and her father had previously been diagnosed with CD. Although she had not presented bleeding tendency herself, she was also diagnosed with CD by genetic examination at 20 years old in consideration of her family history. Subsequently, she got pregnant and delivered her first child transvaginally at the age of 38 at the other hospital and bleeding was within normal range. This time, she underwent cesarean section at our hospital due to DD twin pregnancy.
There was no bleeding tendency despite the fibrinogen concentration was continuously low at around 0.6 g/L during the course of pregnancy. However, it was predicted to be difficult for selection of the mode of anesthesia and the determination of indication of blood transfusion during the perioperative period. Therefore, we decided to measure fibrinogen concentration at POC using a portable fibrinogen measuring device.
The fibrinogen concentration in the morning on the day of surgery was 0.69 g/L, hence 4g of fibrinogen concentrate preparation was preoperatively administered. The fibrinogen value after administration was 1.17 g/L, which was not as high as expected, but we judged that spinal anesthesia was possible because the platelet count was within normal range (226,000/L) and bleeding tendency was not observed as well. The anesthesia was performed at Single-Shot-Spinal with bupivacaine added fentanyl and morphine. The surgery progressed uneventfully, and the operation time was 62 minutes and the bleeding volume was 1560 g.
During surgery, the fibrinogen concentration was repeatedly measured with a portable fibrinogen measuring device, and the lowest value was 0.91 g / L, thus no further replenishment of fibrinogen was necessary. She was discharged on the fifth postoperative day without any complications.
We performed anesthetic management using a portable fibrinogen measuring device in caesarean section of a woman diagnosed with CD. It has been well recognized that serum concentration of fibrinogen is important as an indicator of transfusion in the perinatal period. However, there are few reports of delivery of women diagnosed with CD, and no consensus on adequate target levels of fibrinogen concentration has been obtained in pregnant women with CD. In addition, the portable fibrinogen measuring device was useful for selection of the mode of anesthesia and the determination of transfusion (fibrinogen supplementation) since it showed us the fibrinogen concentration quickly, but further investigation is necessary to establish its reliability in special disease conditions such as CD.