Management of von Willebrand Disease of three cases
Gözde İnan, Naciye Türk Özterlemez, Melis Altuğ, Berrin Günaydın
Background and aim: Von Willebrand disease (vWD) is the most challenging inherited bleeding disorder resulting from an impairment of von Willebrand Factor (vWF) for anesthesiologists. We aimed to present and share management in three parturients with vWD.
Case report: Informed consents were obtained from patients.
Case 1; a 24 year-old term parturient with previous abortus imminens history underwent cesarean section (CS) under general anesthesia after receiving vWF/factor VII concentrate. Postpartum period was uneventful, and no hemorrhage was observed.
Case2; a 33 year-old women had vaginal birth with vWF/F VIII C and then was admitted on the postpartum 13th day because of abnormal vaginal bleeding and epistaxis. Three units (U) of erythrocyte concentrate (EC), vWF/factor VIII and tranexamic acid (TXA) as given to the patient because of low hemoglobin. One week later the patient was re-admitted to the ICU due to vaginal bleeding. She received vWF/factor VIII, TXA, contraceptive, metil ergonovin and 1 U of EC and discharged after 6 days. Four years later, because of abnormal vaginal bleeding again, cervical cauterisation was performed along with infusing vWF/factor VII. After pathology report, abdominal hysterectomy was planned under general anesthesia. Preoperatively, vWF/factor VII was administered and followed by 3 U of EC and FFP.
Case3; a 29 year-old term parturient with vWD was admitted for CS under hematology supervision. She underwent general anesthesia using thiopentone, succinylcholine, sevoflurane in 50% oxygen-air mixture. After delivery, uterotonics (oxytocine and carbetocine) and TXA were given intravenously. Intravenous paracetamol and morphine with PCA were administered for analgesia. Perioperative period were uneventful without need for blood or factor transfusion.
Conclusion: VWD parturients with serious bleeding symptoms can be admitted repetitively, therefore any parturient with VWD scheduled for delivery needs careful evaluation and follow up in tertiary care centers where blood and/or factor concentrates readily available.
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