NONSURGICAL SYNOVECTOMY IN THE TREATMENT OF ARTHROPATHY IN VON-WILLEBRANDS-DISEASE

Von Willebrand's disease is the most common inherited bleeding disorde r, with an overall prevalence in the general population of 0.8% to 1.3 %, Hemarthrosis occurs mainly in the severest forms of the disease (ty pe III), with a frequency of 3.5% to 11%, and can cause severe arthrop athy similar to that seen in hemophilia. We retrospectively reviewed o ur experience with nonsurgical synovectomy in the treatment of recurre nt hemarthrosis with arthropathy in patients with von Willebrand's dis ease. 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 frequen cy 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 i n 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, Clin ical and radiological results were evaluated six months after synovect omy 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 satisf actory. The clinical efficacy of synovectomy done, using radiocolloids or osmic acid in arthropathy due to von Willebrand's disease, seems s imilar to that in hemophilia.