Von Willebrand's disease is the most common inherited
bleeding disorder, with an overall prevalence in the general population of 0.8% to 1.3%.
Hemarthrosis occurs mainly in the severest forms of the disease (type III), with a frequency of 3.5% to 11%, and can cause severe
arthropathy similar to that seen in
hemophilia. We retrospectively reviewed our experience with nonsurgical
synovectomy in the treatment of recurrent
hemarthrosis with
arthropathy in patients with
von Willebrand's disease. Four of our six patients had type III disease and the remaining two had type II disease. The age range was 13 to 63 years. The frequency of
hemarthrosis prior to
synovectomy was one to four per month. One (n = 2) or both (n = 1) knees were treated in 4 cases, one (n = 1) or both (n = 1) ankles in 3 cases and an elbow in one case. We used
yttrium 90 in a dose of 5 mCi for one knee,
rhenium 186 in a dose of 2 mCi for two ankles and the elbow and
osmic acid for two knees and one ankle. Clinical and radiological results were evaluated six months after
synovectomy using the World Federation of
Hemophilia score. Radiologic lesions remained stable and clinical manifestations improved in every case (p < 0.05). Five patients achieved a complete remission. Safety was satisfactory. The clinical efficacy of
synovectomy done, using radiocolloids or
osmic acid in
arthropathy due to
von Willebrand's disease, seems similar to that in
hemophilia.