RUPTURED HEMORRHAGIC OVARIAN CYST IN A PATIENT WITH WILLEBRAND DISEASE: A CASE REPORT AND REVIEW OF LITTERATURE

Abstract

Willebrand Disease (WD) is a familial haemorrhagic disease described in 1926 by Erik von Willebrand, a Finnish physician (1), and is related to a deficiency of Willebrand Factor (VWF) leading to a disorder of primary haemostasis and secondary coagulation. The clinical and biological expression of WD varies according to the form. The common feature is the existence of a constitutional quantitative and/or qualitative VWF deficiency. A classification of the different forms of the disease exists to guide the diagnosis, treatment and genetic information of patients. While isolated biological abnormalities are relatively common with a prevalence of nearly 1%, symptomatic forms of WD are more rare. The prevalence of WM type 3, the most severe form (autosomal recessive), has been estimated at 1 per million population. While autosomal genetic inheritance predicts that both sexes are affected in the same proportion, symptomatic forms are more common in women because of the haemostatic challenge of menstruation, pregnancy and childbirth. Ovarian cysts are fluid-filled sacs that lie on the surface of or within the ovary(2). Roughly 20% of women develop at least one pelvic mass in their lifetime. Therapeutic management is based on surgical haemostasis by total cystectomy and medical haemostasis by exogenous supply of Willebrand factor and finally resuscitation of any state of haemorrhagic shock starting with vascular filling with crystalloids, the introduction of vasoactive amines and finally massive transfusion.We report here the case of a 24 year old female patient, treated for Willebrands disease since birth and who presented to the emergency department with haemorrhagic shock due to a ruptured haemorrhagic ovarian cyst with internal bleeding.