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.