Obstetric and gynaecological aspects of von Willebrand disease

The impact of von Willebrand disease in females is pronounced in terms of m enorrhagia and postpartum haemorrhage. There is a very high proportion of v on Willebrand disease patients with menorrhagia and associated anaemia, imp airment of quality of life, including loss of time from work or school, and a high rate of the use of hysterectomy for ultimate control of the bleedin g. The 'early' detection of von Willebrand disease in females may avert the se complications. Consequently, there have recently been ongoing internatio nal efforts to determine the prevalence of von Willebrand disease in female s presenting with menorrhagia, providing a prevalence of 7-20% combined fro m three studies including a total of 300 patients. Issues remain regarding the optimal dose/schedule of intranasal or subcutaneous desmopressin use fo r menorrhagia and the relative efficacy of anti-fibrinolytic agents. The pr oper role of oral contraceptives deserves further study in von Willebrand d isease patients with menorrhagia as recent studies have paradoxically demon strated a lower response rate in type 1 than type 2 or 3 von Willebrand dis ease. Despite the well-known adage of the 'gestational palliation' of von W illebrand disease, there is also a high proportion of postpartum haemorrhag e in type I patients, especially after the 24 hour post-delivery period. Th is may occur despite a normalization of the factor Ville level in the third trimester, particularly in type 2 and 3 patients. The care-giver must be a ware that haemorrhage can occur up to 5 weeks postpartum. In sum, studies o ver the past decade have documented a substantial impact of menses and chil dbirth on von Willebrand disease patients. These results should serve as a basis for interventional studies to reduce the morbidity of menstruation an d childbirth.