Perioperative Anaesthetic Management of Emergency Caesarean Delivery in a Patient with Von Willebrand Disease

Perioperative Anaesthetic Management of Emergency Caesarean Delivery in a Patient with Von Willebrand Disease

 

Dr. Parmanand Mandawaria *1, Dr. Aariz Javed Ansari 2, Dr. Sony Baby 3, Nazzal Tahena 4

 

  1. Consultant, Department of Anesthesia and Pain Medicine, Sheikh Shakhbout Medical City, Abu Dhabi, United Arab Emirates

 

  1. Specialist, Department of Anesthesia and Pain medicine, Sheikh Shakhbout Medical City, Abu Dhabi, United Arab Emirates

 

  1. Consultant, Department of Anesthesia and Pain Medicine, Sheikh Shakhbout Medical City, Abu Dhabi, United Arab Emirates

 

  1. Technologist, Department of Anesthesia and Pain Medicine, Sheikh Shakhbout Medical City, Abu Dhabi, United Arab Emirates.

 

*Correspondence to: Dr. Parmanand Mandawaria, Consultant Anaesthetist, Sheikh Shakhbout Medical City, Abu Dhabi, United Arab Emirates.

 

Copyright.

© 2026 Dr. Parmanand Mandawaria, This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Received: 01 October 2026

Published: 13 October  2026

DOI: https://doi.org/10.5281/zenodo.23336503

 

Abstract

Background: Because von Willebrand disease causes haemostatic problems during childbirth, anaesthetic planning should take into account the patient's bleeding history, the results of the laboratory tests and the response to treatment.

Case presentation: A 34-year-old woman who was gravida 2 and para 1 came in at 38 weeks of gestation having been diagnosed previously with von Willebrand disease and having gestational diabetes mellitus that was controlled with insulin. She had previously undergone a vaginal delivery which was complicated by postpartum haemorrhage and for which three units of blood were required. Her preoperative haemoglobin level was 97 g/L and her factor VIII activity was 43%. The clinical records showed low levels of von Willebrand factor antigen and activity as well as no reaction to a desmopressin challenge. Since labour did not progress after induction, an emergency caesarean section was carried out. Factor replacement was given about 30 minutes before the operation. General anaesthesia was induced using propofol and rocuronium and then maintained with sevoflurane. Tranexamic acid and uterotonic agents were given after delivery. The estimated blood loss was 500 mL. After surgery she was given, every 12-hour (O'Donnell & Lavin, 2019)s for three days, a combination of von Willebrand factor and factor VIII concentrate while she (Nordic guidelines for diagnosis and management of von Willebrand disease (VWD) – Management of Bleeds or Invasive Procedures with FVIII/VWF or VWF Concentrates, n.d.) was in the high dependency unit. She stayed clinically stable and was discharged on day 4 following the operation. Later, specific testing confirmed that she had type 2 von Willebrand disease.

Conclusion: The case shows a favourable short-term result for the mother after an emergency caesarean section under general anaesthesia, with perioperative replacement of the factors and subsequent postoperative monitoring. However, the specific factor preparation used, the factor levels that were achieved, and the reasons for choosing the anaesthetic technique need to be confirmed before the report can be published.

     Keywords: von Willebrand disease; caesarean delivery; general anaesthesia; factor replacement; postpartum haemorrhage.

Perioperative Anaesthetic Management of Emergency Caesarean Delivery in a Patient with Von Willebrand Disease

Introduction

Von Willebrand disease (VWD) is an inherited condition characterised by either a quantitative or qualitative abnormality of von Willebrand factor (VWF); in Type 2 there is a qualitative defect. (Molecular genetics of type 2 von Willebrand disease, 2002, pp. 249-258) For diagnosis and classification a specialised evaluation is required [1]. The provision of obstetric care is made more difficult by the risk of bleeding and by the need to choose an appropriate anaesthetic technique [2].

We describe the course of the woman with von Willebrand's disease who had an emergency caesarean section due to failure of labour to progress. The fact that she had previously experienced postpartum haemorrhage and that she had reported not responding to desmopressin were important factors in planning the procedure. The report centres on the anaesthetic record provided, the haemostatic support given and the early outcome for the mother.

 

Case Presentation

Patient history and assessment

The woman, who was 34 years of age, had two pregnancies and one live birth, and came in during the early stage of labour at 38 weeks of gestation. Her weight was 61 kg and her height 150 cm, giving her a body mass index of 27.11 kg/m². She had a previous history of von Willebrand disease and gestational diabetes mellitus, the latter being well controlled with insulin. She was rated as American Society of Anesthesiologists physical status IIIE. (DJ et al., 2022)

She had a history of bleeding that lasted a long time after minor injuries, with bleeding from her gums and heavy menstrual bleeding. When she was previously delivered vaginally 16 years ago, this occasion was complicated by a postpartum haemorrhage for which three units of blood had to be transfused. At the time of evaluation for the present delivery, her vital signs were said to be stable and there were no signs of active bleeding.

The results obtained before the operation are given in Table 1; the haematology assessment also noted low levels (O'Donnell & Lavin, 2019) of VWF antigen and activity, even though the actual figures were not provided. It had previously been reported that a Minirin (desmopressin) challenge had yielded no response; however, the dose used, when the challenge was administered, and the response that was measured were not available.

 

The value for the platelet count has been adjusted to read "305.000" according to the source notation; the unit should however be checked with the laboratory report. The reference intervals were not given.

 

Delivery decision and haemostatic preparation

The birth was at first intended to be by vaginal delivery. After induction of labour, since there was no progress the obstetric team decided to carry out an emergency caesarean section. The haematology department recommended the administration of combined VWF and factor VIII concentrate at a dose of 50 units per kg every 12 hours, together with tranexamic acid and red blood cell transfusion as required on a clinical basis.

Factor replacement was given about 30 minutes before the surgery; the source refers to this preoperative therapy as 'factor VIII concentrate', the haematology plan and the postoperative treatment however specify a combination of VWF and factor VIII concentrate. The product used, the actual dose given and the unit basis must be checked against the medication record.

 

Anaesthetic and intraoperative management

In the right upper extremity an 18-gauge cannula was used to obtain peripheral intravenous access and in the left one a 20-gauge cannula was employed. A quantity of 500 mL of crystalloid was given at the start. Following three minutes of preoxygenation with 100% oxygen via face mask, general anaesthesia was induced using propofol 150 mg and rocuronium 50 mg. Intubation of the trachea was carried out with a cuffed endotracheal tube having an internal diameter of 7.0 mm. Controlled ventilation was started with an inspired oxygen fraction of 0.4 and a positive end-expiratory pressure of 5 cm H?O. Anaesthesia was then maintained with sevoflurane at 2% in an air–oxygen mixture.

Before the surgical incision, cefazolin at a dose of 2 g and metronidazole 500 mg were given by intravenous injection. After the live baby had been delivered, fentanyl 100 µg, carbetocin 100 µg and tranexamic acid 1 g were administered intravenously and misoprostol 1,000 µg was given rectally. The newborn's birth weight, its Apgar scores and its subsequent neonatal course were not provided.

Antiemetic prophylaxis was given at the end of the operation in the form of ondansetron 4 mg and dexamethasone 4 mg. To reverse the neuromuscular blockade, sugammadex 200 mg was administered intravenously. Since the patient fulfilled the extubation criteria as reported, extubation was carried out without any complications. For postoperative pain relief, an intravenous dose of morphine 6 mg and paracetamol 1 g were given.

The procedure took about 60 minutes, with an estimated blood loss of 500 mL and a urine output of 150 mL. The amount of crystalloid given during the operation was 1,000 mL; it is not yet known whether this figure included the initial 500 mL. The patient was haemodynamically stable the whole time the surgery was going on. The source does not say if any blood products were given during this admission.

 

Postoperative course and follow up

She was moved to a high dependency unit so that she could be closely monitored after the operation. On the advice of the haematology department, she was given a combination of VWF and factor VIII concentrate at a dose of 50 IU/kg every 12 hours for three days. Her complete blood counts and coagulation values were checked each day, and no excessive vaginal bleeding, bleeding at the surgical site or any other bleeding was noted during the period of observation.

On the third day after the operation, the haemoglobin level was 93 [the unit is to be confirmed]. She was discharged on the fourth day after the operation. Further haematology follow-up and special coagulation tests are said to have confirmed a diagnosis of type 2 VWD. However, the subtype, the supporting results and the length of the follow-up were not provided. It was recommended that she should have an evaluation regarding the abnormal bleeding and arrange for a haematological review as well as for perioperative haemostatic support before any future surgery or invasive procedure.

 

Discussion

The fact that the mother had suffered a postpartum haemorrhage previously meant that her bleeding history was especially important in this instance. Although her condition was stable before surgery, this did not eliminate the need for haemostatic planning. The haematology team took into account both the reported factor VIII activity of 43% and the low VWF measurements. However, since there were no numerical VWF results, it was not possible to assess the initial defect or the response to replacement.

The 2021 multidisciplinary guidelines recommend that both VWF and factor VIII activity should be kept at or above 0.50 IU/mL for at least three days following major surgery, with the targets and duration to be determined on an individual basis [2]. While this report includes three days of replacement it does not provide the achieved factor levels; it is therefore impossible to assess biochemical adequacy.

General anaesthesia was given, although the source does not explain why neuraxial anaesthesia was not chosen. Guidelines recommend that a VWF activity target of 0.50–1.50 IU/mL should be aimed for when neuraxial anaesthesia is considered appropriate; however, suitability still has to be assessed on an individual basis [2]. The fact that VWD exists should not be taken as evidence that neuraxial anaesthesia was contraindicated in this patient.

A large number of patients suffering from type 2 von Willebrand's disease do not respond well to desmopressin and might therefore have to receive replacement therapy [2]. While the negative challenge result is relevant, its interpretation still depends on the initial test results. A classification as type 2 von Willebrand's disease should be based on the diagnostic report and not merely inferred from the challenge alone [1].

The estimate of blood loss, the stable clinical condition and the fact that she was discharged on day 4 all point to a favourable early outcome for the mother. However, they do not prove the independent benefit of any particular intervention nor do they show that general anaesthesia is superior. Furthermore, since there are no detailed postoperative factor assays, no confirmed record of a transfusion, and no longer follow-up, it is not possible to draw any conclusions regarding haemostatic targets or about delayed bleeding.

 

Conclusion

The woman, who had von Willebrand's disease and a previous history of postpartum haemorrhage, experienced an emergency caesarean section under general anaesthesia and then had a stable early period after the operation. The method of management involved the use of haematology-directed replacement, tranexamic acid, uterotonics and high dependency monitoring. In order to strengthen the report prior to submission, accurate records should be kept of the products and laboratory results as well as the reason for choosing general anaesthesia.

Reference

  1. James PD, Connell NT, Ameer B, et al. 2021 ASH ISTH NHF WFH guidelines for the diagnosis of von Willebrand disease. Blood Advances, 2021;5(1):280–300. DOI: 10.1182/bloodadvances.2020003265.
  2. Connell NT, Flood VH, Brignardello-Petersen R, et al. ASH, ISTH, NHF, WFH 2021 guidelines on the management of von Willebrand disease. Blood Adv. 2021;5(1):301, 325. doi:10.1182/bloodadvances.2020003264.
  3. O'Donnell, J. S. & Lavin, M. (2019). Perioperative management of patients with von Willebrand disease.           Hematology     Am      Soc                          Hematol                    Educ            Program. https://doi.org/10.1182/hematology.2019000065
  4. (n.d.). Nordic guidelines for diagnosis and management of von Willebrand disease (VWD) – Management of Bleeds or Invasive Procedures with FVIII/VWF or VWF Concentrates. Nordic Hemophilia Council. https://guidelines.nordhemophilia.org/vwd/Management.html (2002). Molecular genetics of type 2 von Willebrand disease. Blood 100(1), pp. 249-258. https://doi.org/10.1182/blood-2002-01-0010
  5. DJ, D., JM, H. & EH, G. (2022). American Society of Anesthesiologists Physical Status Classification System. StatPearls [Internet]. Treasure Island (FL): StatPearls Publishing; 2022 Jan-.. https://doi.org/10.1056/NEJMra1512610
  6. O'Donnell, J. S. & Lavin, M. (2019). Perioperative management of patients with von Willebrand disease. Hematology Am Soc Hematol Educ Program 2019(1). https://doi.org/10.1182/hematology.2019000065.