PB0850 - Management of Type 2M Von Willebrand Disease in Lower Limb Surgery

Background: Type 2M Von Willebrand Disease (VWD) is an inherited bleeding disorder, characterised by reduced VWF interactions with platelets or collagen. Whilst there are excellent guidelines regarding the perioperative management of patients with Type 2 VWD, there is very little literature regarding the specific management of these patients undergoing complex reconstructive lower limb surgery, which confers a significant risk of lower limb thrombosis, including in patients with VWD.

Aims: We report the management of a patient with type 2M VWD undergoing complex foot surgery, specifically a left calcaneal osteotomy, tendon transfer and Achille’s tendon lengthening, a procedure which necessitates complete lower limb immobilisation for 2 weeks, followed by 6 weeks of partial weight bearing in a boot.

Methods: This patient’s baseline VWF:RCo levels are 10-20 IU/dl. Pre-operatively, she was given 1500/3600 IU of Voncento (recombinant FVIII/VWF), which increased her VWF:RCo activity from 27 IU/dl to 104 IU/dl. She was given a further 500/1200 IU on the evening after her operation, and 1000/2400 IU the following morning. Prophylactic low molecular weight heparin (LMWH), in the form of Enoxaparin 40mg OD, was started on day 1 post-operatively. Tranexamic acid was also commenced and continued for 5 days. Having established the dose required to maintain her VWF:RCo level >50 IU/dl, our patient was discharged home on day 3 post-op and continued daily Voncento 1000/2400 IU, prior to daily Enoxaparin 40mg, for the duration of her time of immobilisation.

Results: Our patient had an uneventful post-operative course. She had no excess post-operative bleeding and there were no thrombotic complications.

Conclusion(s): This is, to our knowledge, the first detailed report of the management of complex lower limb surgery in a patient with Type 2M VWD. We were able to navigate a safe peri- and post-operative course for this patient, which could be adapted for future similar clinical scenarios.