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Journal of Obstrectic Anaesthesia and Critical Care
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Year : 2021  |  Volume : 11  |  Issue : 1  |  Page : 49-50

Anesthesia for cesarean section in a patient with von willebrand's disease

Department of Anaesthesia and Critical Care, Amrita Institute of Medical Sciences, Amrita Vishwa Vidyapeetham, Kochi, Kerala, India

Date of Submission24-Jun-2020
Date of Acceptance21-Dec-2020
Date of Web Publication16-Apr-2021

Correspondence Address:
Dr. Nitu Puthenveettil
Department of Anaesthesia and Critical Care, Amrita Vishwa Vidyapeetham, Kochi, Kerala
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/joacc.JOACC_52_20

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How to cite this article:
Puthenveettil N, Mathew J, Rajan S, Kumar L. Anesthesia for cesarean section in a patient with von willebrand's disease. J Obstet Anaesth Crit Care 2021;11:49-50

How to cite this URL:
Puthenveettil N, Mathew J, Rajan S, Kumar L. Anesthesia for cesarean section in a patient with von willebrand's disease. J Obstet Anaesth Crit Care [serial online] 2021 [cited 2021 Jun 14];11:49-50. Available from: https://www.joacc.com/text.asp?2021/11/1/49/313912


Von Willebrand's disease (vWD) is an autosomal dominant hereditary coagulation disorder, affecting up to 1% of the population.[1] It is characterized by a deficiency or defect of von Willebrand's factor (vWF). vWF aids in platelet adhesion and preventing degradation of coagulation factor VIII.[2],[3] There are three types of vWD. Type 1 with quantitative deficiency, type 2 with a qualitative defect, and type 3 with a total absence of VWF. Type 2 is further subdivided into 2A, 2B, 2M, and 2N.[3],[4] Women face hemostatic challenges during menstruation and childbirth.[4] Preoperatively hemoglobin, prothrombin time, thromboplastin time, bleeding time, factor VIII, and vWF antigen levels should be evaluated. A multidisciplinary team including obstetricians, hematologist, and anesthetists are required for a good obstetric outcome. We present two cases of von Willibrand's disease that were referred to our obstetric department for further management.

A 29-year-old G2P1L1, at 37 weeks of gestation, a known case of von Willibrand's disease type 2B was admitted for safe confinement. She gave a history of multiple platelet transfusions in her previous pregnancy. On the day of surgery, her platelet count was 32,000/μL. One unit of single donor platelet (SDP), 2000 U of factor VIII, and vWF concentrate were transfused before the surgery. In the operation theatre, in addition to standard monitors, an invasive arterial line and two large-bore IV access were secured. General anesthesia with rapid sequence induction was performed, and a live baby was extracted. Oxytocin infusion was started. However, she had a bout of atonic bleeding, which was managed with additional oxytocin, prostaglandin F2α, tranexamic acid, bilateral uterine artery ligation, and per rectal prostaglandin E1 (PGE1). A point of care thromboelastogram was performed (R = 3.5 min, k = 2.7 min, Angle = 58.9, MA = 61.6 mm, CI = 0.9). Two units of packed red blood cells, 10 units of cryoprecipitate, 1 unit of SDP, 2 units of fresh frozen plasma, and 2000 U of factor VIII were transfused. The postoperative period was uneventful.

A 28-year-old G2P1A1, at 37 weeks of gestation, a known case of vWD Type 2 was admitted for safe confinement. The investigations showed normal factor VIII and vWF antigen levels, but the platelet count was 45,000/μL. On the day of surgery, a half unit of SDP was transfused. General anesthesia with rapid sequence induction was performed. She was given intravenous oxytocin, tranexamic acid, and per rectal PGE1. The uterus was well contracted, and the postoperative period was uneventful.

Patients with vWD have an increased risk of postpartum hemorrhage due to uterine atonicity.[3],[4] Caesarean sections are performed for obstetric indications. Successful use of regional anesthesia has been reported.[5] But general anesthesia is safer when coagulation parameters are deranged. A platelet count below 50,000 has to be corrected before the caesarean section. Desmopressin, which raises plasma levels of endogenous vWF is preferred in type 1 vWD and factor concentrates in type 2 and 3. Tranexamic acid is used as it inhibits fibrinolysis.[6] Intraoperative bleeding can be managed with blood transfusion, fresh frozen plasma, and cryoprecipitate. Cryoprecipitate has 5 to 10 times more factor VIII and vWF than fresh frozen plasma. Factor VIII and vWF are preferred to avoid viral transmission.[1] A thromboelastogram can be used to guide transfusion.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form the patient(s) has/have given his/her/their consent for his/her/their images and other clinical information to be reported in the journal. The patients understand that their names and initials will not be published and due efforts will be made to conceal their identity, but anonymity cannot be guaranteed.

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Conflicts of interest

There are no conflicts of interest.

  References Top

Galante D. Anesthesia management for emergency cesarean section in a patient affected by von Willebrand's disease with perinatal distress. Pediatr Anesth Crit Care J 2013;1:39-42.  Back to cited text no. 1
Roberson MC, Wigley MD, Austin PN. Anesthetic management of a patient with type 1 von Willebrand disease and uterine placental abruption: A case report. AANAJ 2018 86:209-12.  Back to cited text no. 2
Peyvandi F. Diagnosis and management of patients with von Willebrand's disease in Italy: An expert meeting report. Blood Transfus 2018;16:326-8.  Back to cited text no. 3
Govorov I, Löfgren S, Chaireti R, Holmström M, Bremme K, Mints M. Postpartum hemorrhage in women with von Willebrand disease – A retrospective observational study. PLoS One 2016;11:e0164683.  Back to cited text no. 4
DelbrückC, Miesbach W. The course of von Willebrand factor and factor VIII activity in patients with von Willebrand disease during pregnancy. Acta Haematol 2019;142:71-8.  Back to cited text no. 5
Castaman G, James PD. Pregnancy and delivery in women with von Willebrand disease. Eur J Haematol 2019;103:73-9.  Back to cited text no. 6


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