Radiosynovectomy in Children with Congenital Haemostatic Defects

Abstract

Aims: To evaluate the clinical benefits of radiosynovectomy (RS) in children with congenital coagulative disorders, reduce bleeds and improve progression free survival (PFS). Methods: 35 patients aged 7 to 17 yrs, (mean age 12.6 yrs) were included in the study. 29 had haemophilia A, (4 with inhibitor factor VIII), 3 had haemophilia B (1 with inhibitor factor IX) and 3 had von Willebrand’s disease. There were 43 therapies including injections into the followed joints: knee 21, elbow 13, ankle 6 and shoulder 3. In 30 cases this was initial therapy and in 5 cases repeat therapy due to recurrence or deterioration. Injected activity of 186Re colloid was dependent on type and size of joints, range: 60–180 MBq. Post therapy imaging was performed in each case 1 h after injection of radioisotopes, and then on day 2 and 3. The number of bleedings into joints per month, improvement of joint movements, and other clinical changes were evaluated before and after therapy in 3 month intervals. US images were used to assess changes in joint effusion and synovial overgrowth. PFS in terms of significant clinical improvement within joints was assessed using standard Kaplan Meier methods. The prognostic significance of selected parameters was tested using discriminate function analysis. Results: After injection of 186Re into joints, post-therapeutic images showed intraarticular distribution of tracer and there was no leakage. A significant reduction in bleeds into joints was noted (before therapy mean 1.8 per month and after treatment mean 0.3 per month, p<0.001). Conclusions: This study suggests that use of 186Re in early stages of arthropatic haemophilia reduces the number of bleeds into joints and improves PFS.