Title: Anaesthetic management of a pregnant patient with von Willebrand's disease

Authors: Alves DR, Franco S, Saldanha L

Institution: Centro Hospitalar de Lisboa Ocidental, Dept of Anaesthesiology, Lisboa, Portugal

Text:
Background: Being the most frequent inherited bleeding disorder (1-2% prevalence in the general population1, von Willebrand's disease (vWD) is likely to be encountered clinically by anaesthesiologists. It is characterized by either quantitative or qualitative abnormalities in von Willebrand Factor (vWF) and encompasses 3 types2, the most common of which is type 1, where there is essentially a quantitative deficit of vWF.

Case report: 29-year-old, ASA 2 patient presenting in labour with known type 1 vWD, diagnosed at the age of 13 (study of menorrhagia), with no treatment. There was no clinical history of either spontaneous or provoked mucocutaneous bleeding during pregnancy.
The patient had been followed closely as an outpatient by Haematology, with dosing of vWF showing supranormal levels in the third trimester, no changes in clotting times nor in platelet levels. Haematology considered there to be no contraindication for neuroaxial techniques nor indication for desmopresin use. A combined spinal epidural was performed, with the patient eventually proceeding to caesarean section for non-progression of labour, with anaesthesia provided via the previously placed epidural catheter. There were no excessive blood losses either intra- or postoperatively. The epidural catheter was removed 24h after surgery, and the patient discharged nome uneventfully on the 3rd postoperative day. Close neurologic monitoring evidenced no changes.

Discussion: During late pregnancy the levels of several clotting factors tend to increase as a preparation for labour, namely fibrinogen, FVII, FVIII, FX and vWF3. In type 1 vWD, where there is a lower level of normally functioning vWF, this means that the disease can actually normalize towards the peripartum period, with temporary correction of the increased bleeding tendency. Appropriate haematological follow-up is instrumental, and in our case it allowed a timely characterisation of our patient's profile, revealing that neuroaxial techniques were not contraindicated by the disease. 

Learning points: While bleeding risk may constitute a contraindication for neuroaxial techniques, in the particular case of type 1 vWD blood levels of clotting factors may become transiently normal during late pregnancy. Naturally, it is fundamental to make sure these patients are appropriately followed by a specialist to timely assess available therapeutic options.