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Von Willebrand disease for open myomectomy32-year-old woman, 64 kg, for elective open myomectomy of a large fibroid uterus

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Know this first

  • Type 1 is a partial quantitative deficiency and usually responds to desmopressin; type 2B gets thrombocytopenia with it.
  • Desmopressin 0.3 mcg/kg IV 30 minutes before; trial it first unless activity is over 30%.
  • Repeated desmopressin can cause hyponatraemia, so sodium is monitored.
  • Neuraxial bleeding is rare when VWF and factor VIII are above 0.5 IU/mL.

The patient

32-year-old woman, 64 kg, for elective open myomectomy of a large fibroid uterus. Type 1 von Willebrand disease: heavy periods, easy bruising, bleeding after a dental extraction; VWF activity 28%. Drugs: ibuprofen for period pain, ferrous sulphate. Vitals: BP 118/72, HR 84, SpO2 99% on air, RR 14. Hb 10.1 g/dL, platelets 230 × 10⁹/L.

The examiner asks

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  1. Consider

    What are the issues?

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    • A 32-year-old with type 1 von Willebrand disease and anaemia, for open myomectomy with heavy bleeding possible. Patient
    • Her heavy periods, bruising and dental bleeding are the typical mucosal pattern. Disease
    • Type 1 is a partial deficiency and usually responds to desmopressin.
    • Her PT and APTT may well be normal; routine tests often miss this disease.
    • Ibuprofen impairs her platelet function on top of her disease. Stem trigger

  2. Prepare

    How will you prepare her?

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    • Joint plan with her haematologist; multimer testing confirms the type, because treatment differs. Disease
    • A desmopressin trial beforehand, since her activity is below 30%.
    • If she responds: desmopressin 0.3 mcg/kg IV, 30 minutes before surgery.
    • VWF concentrate on hand; major surgery may need it despite desmopressin.
    • Ibuprofen stopped; paracetamol for her period pain. Stem trigger
    • Cross-matched blood and cell salvage, as her loss may exceed 20% of blood volume. Surgery

  3. Plan

    What is your anaesthetic plan?

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    • No spinal or epidural unless her VWF and factor VIII exceed 0.5 IU/mL; general anaesthesia. Disease
    • Two large-bore cannulas and an arterial line before incision. Surgery
    • Tranexamic acid 1 g IV, run across 10 minutes, as an adjunct. Disease
    • Sodium checked through the case; desmopressin can drop it, so I restrict free water.
    • Afterwards: no NSAIDs, paracetamol and opioid, and a close watch for bleeding. After surgery

  4. Crisis

    Two hours in, she has lost 2.5 litres and the whole field oozes. BP 82/48, HR 124, Hb 6.8 g/dL. What do you do?

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    • Massive haemorrhage algorithm: protocol activated; the surgeon compresses and packs. Crisis
    • Red cells, plasma and platelets near 1:1:1, warmed; salvaged blood returned.
    • Her diffuse ooze is her disease: VWF concentrate now, as haematology advise.
    • Tranexamic acid continued: 1 g over 8 hours after the first gram.
    • Cryoprecipitate if fibrinogen falls below 80–100 mg/dL; ionised calcium kept normal.
    • Desmopressin redosed only if her trial showed a response, with sodium monitored.
    • HDU afterwards; VWF concentrate continued until her bleeding risk falls. After surgery

Fatal mistakes

  • Neuraxial block with her VWF uncorrected.
  • Desmopressin in type 2B, where it causes thrombocytopenia.
  • Ibuprofen or ketorolac for her pain.

Checked against: Barash 9e · Miller 10e

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