Haemophilic pseudotumours of the limbs and their percutaneous treatment.

The percutaneous treatment of limb pseudotumours is a nonaggressive method of treating haemophilic pseudotumours. However, efforts should be directed to the prevention of such pseudotumours by ensuring that all patients receive adequate treatment of their bleeding episodes through education and the elimination of geographical or social barriers that prevent access to such treatment. Prevention of pseudotumours by means of early substitution treatment of muscular bleeding episodes is the best treatment.

Chemical synoviorthesis with rifampicin in haemophilia.

Rifampicin is an antibiotic that has been currently used for the treatment of noninfectious articular lesions with satisfactory results. The first experience was performed with patients who presented rheumatoid arthritis, and later with haemophilic patients. The clinical experience of three haemophilia centres which used rifampicin for the treatment of chronic haemophilic synovitis is presented here. The protocols were different. It was observed that rifampicin is more effective when it is used in small joints (elbows and ankles), than when used in bigger ones (knees), and that a high number of injections predicts failure. Mention is also made of experimental studies in animals where it was shown that the healing pattern of rifampicin is similar to that of NSAIDs.

Extensor supracondylar femoral osteotomy as treatment for flexed haemophilic knee.

The experience with extensor supracondylar femoral osteotomy as treatment for the flexed haemophilic knee is presented with the description of 19 patients treated during a 30-year period (1968-98). The average age of the patients was 16 (8-35 years), and the average age follow-up was 13 years (3-30 years). Six patients had flexion fixed deformity while the rest presented 40 degrees average range of motion (10-75 degrees). In 13 patients a single osteotomy without internal fixation was performed, in one an osteosynthesis with a condylar plate and in five stabilization was achieved by means of Blount staples. Previous surgery was performed in two patients with patello-femoral ankylosis. The osteotomy site was consolidated in every patient and the deformity was corrected. Two bleeding complications were observed: one haemarthrosis and one psoas haematoma. Flexion relapsed in one patient who underwent another procedure after 12 years. One patient presented with a peroneal nerve paralysis; another one a genu recurvartum; which required flexor osteotomy. The extensor supracondylar femoral osteotomy is a procedure that aligns the limb with scarce modification of articular mobility.

Intraarticular dexamethasone in advanced chronic synovitis in hemophilia.

From 1988 to 1966, 34 patients with advanced chronic hemophilic synovitis (25 Grade III and nine Grade IV) were treated with intraarticular injections of long acting dexamethasone (sodium phosphate of dexamethasone plus acetate of dexamethasone) in cycles of three injections with 3-week intervals between each injection with 6-month rest intervals between cycles for as many as three cycles, depending on the evolution of each case. All patients had chronic severe synovitis, axial deformity, muscular atrophy, and diminution of range of movement. There were 31 knees, two ankles, and two shoulders. Subjective and objective evaluations were done grouping the results in good, fair, and poor according to grade of patient satisfaction, presence of synovitis and pain, range of movement, and limitation of activities of daily living. In the subjective results there were 19 good results, 12 fair results, and four poor results, and in the objective evaluation there were 22 good results, nine fair results, and four poor results at an average followup of 1.5 years. The use of intraarticular dexamethasone as an alternative in the short to medium term for treatment of advanced chronic hemophilic synovitis with pain and limitation of function before doing an invasive surgical treatment is proposed.

Chemical synoviorthesis for hemophilic synovitis.

For many years, Rifampicin has been used empirically for the treatment of hemophilic chronic synovitis with encouraging results. A study was performed in which Rifampicin was shown to reduce the inflammation of joints affected by hemophilic synovitis. A clinical study was performed on 48 hemophilic patients (48 joints). Seventeen elbows, eight knees, and 23 ankles were treated. The mean age of the patients was 6 years (range, 4-23 years) and the mean followup was 29 months (range, 24-53 months). Overall, 40 excellent results and eight good results were obtained. The average number of weekly injections of Rifampicin was 3.06 (range, 1-10 injections). Eight patients experienced pain on the first injection, which subsided gradually with the subsequent procedures. Synoviorthesis with Rifampicin seems to be a good method for the treatment of hemophilic synovitis, especially in small joints (elbows and ankles) and in younger children.

Chemical synovectomy vs. radioactive synovectomy for the treatment of chronic haemophilic synovitis: a prospective short-term study.

Between January 1992 and December 1994, a prospective study was performed in two centres comparing chemical synovectomy using repeated weekly injections (range, 1-17) of Rifampicin (Group R) vs. radioactive synovectomy using 1-2 injections of Yttrium-90 (Group Y). The study was performed on 38 joints in a total of 35 haemophilic patients suffering from chronic haemophilic synovitis unresponsive to 3 months of conservative treatment (substitution therapy with the deficient coagulation factor plus a rehabilitation protocol). Group R included 18 patients with an average age of 9 years, with an average follow-up time of 25 months. In Group R, 19 joints were treated: nine knees, four elbows and six ankles. Group Y included 17 patients with an average age of 18 years, with an average follow-up time of 23 months. In Group Y, 19 joints were treated: 14 knees, two elbows and three ankles. The joints were evaluated according to the frequency of bleeding episodes and the Pettersson radiographic score. On the basis of the radiographic scale at the time of the synoviortheses, the joints were categorized as stage I (0 points), stage II (1-2 points), stage III (3-7 points), stage IV (8-10 points) and stage V (11-13 points). The overall results showed that in Group R, bleeding decreased in 12 joints, remained the same in six and worsened in one. In Group Y, bleeding decreased in 11 joints, remained the same in seven and worsened in one. In both Groups, bleeding was controlled best if joints were in stages I and II. In stages III, IV and V, synoviorthesis should not be carried out in haemophilia because it is not effective. Use of radioactive substances in persons under the age of 9 years has been discouraged in the past and therefore only Rifampicin was used in this age group. However, in patients over 9 years of age, this study showed similar decrease of bleeding in both treatment groups. Taking into account the advantages and disadvantages of both agents, we suggest the use of Rifampicin in elbows and ankles but prefer Yttrium-90 for the knees.