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[Recurrent intra-articular bleeding episodes in haemophiliacs. Treatment outcomes in the patients at the university hospital motol in 1985-2005].

[Article in Czech]

Author information

  • 1Klinika dětské a dospělé ortopedie a traumatologie 2. LF UK a FN v Motole, Praha.

Abstract

PURPOSE OF THE STUDY:

Chronic synovitis is a common finding in people with haemophilia. It regularly appears after recurrent episodes of intra-articular bleeding. The bleeding originates from the subsynovial venous plexus underlying the capsule where a lack of thromboplastic activity has been demonstrated. Therefore, the changed synovium appears to be a treatment target. There are several methods which can be used to remove the synovial layer from the joint. The aim of our study was to asses the efficacy of different treatment approaches used in a group of haemophiliacs between 1985 and 2005 in our hospital.

MATERIAL AND METHODS:

A group of 30 patients with bleeding disorders was evaluated in the study. There were 29 men with haemophilia and one woman with von Wilebrandt factor deficiency. Their age ranged from 6 to 18 (median 13) years. They underwent a total of 68 interventions including surgical synovectomy (n=28), radionuclide synovectomy (n=33) and corticosteroid instillation (n=7). The necessity of a repeat intervention was used as a criterion of successful treatment.

RESULTS:

In the group of surgical synovectomies, 22% of the patients required repeat operations, in the group of radiation synovectomy, this was 9% and, in the group treated with corticosteroids, this was 43%. The average hospitalisation time was 50 days for surgical procedures (19-133 days) and 7 days for radiation synovectomy procedures (4-13 days).

DISCUSSION:

In 1994 Merchan presented seven excellent or good results in a group of 10 knees evaluated 1 year after treatment with methylprednisolone. Six years later he reported that "five years after completion of treatment, all results of the observed patients were poor". Generally, corticosteroids will reduce synovitis in the majority of patients but the effect is temporary. A complete remission is a very rare situation under corticosteroid treatment. The experience with surgical synovectomies is not recent and this method is described as carrying a high risk of complications and requiring a high amount of coagulating factor consumption. There are several recent reports on the application of Yttrium-90: in Madrid they evaluated treated joints (knees, ankles and elbows, n = 66) in 44 patients aged from 9 to 39 years. The results were good in less than half of the knees and ankles. The treatment of elbows was more successful. It was recommended to perform synoviorthesis at the early stages of synovitis. In Israel, they reported that a decrease in the number of bleeding episodes was achieved in 80% of 115 patients treated with Yttrium-90; in 15% of them, bleeding in the treated joints stopped completely. In Izmir, Yttrium was used in the treatment of knees, elbows, ankles and also shoulders in children and young adults (3-25 years). The method was found to be safe and effective. Brazilian authors have experience with the treatment of knees, ankles, elbows and shoulders too; they have concluded that this method represents an important resource for the treatment of chronic haemophilic synovitis and markedly reduces joint bleeding frequency and pain, irrespective of the radiographic stage and inhibitor status. While the European Association of Nuclear Medicine (EANM) recommend using 186Re-sulfide for treatment in medium-sized joints, Chinese authors have published a study comparing the effect of using three different doses of 186Re-sulfide in the treatment of chronic synovitis in knees. Their patients have received an amount of radionuclide according to the thickness of their synovial layer measured on MRI, with the result that 22 patients exhibited significant reduction in synovial thickness. A reduction in the number of bleeding episodes was reached in 71% of the patients within an 18-month period. No significant differences were found among the groups receiving different radioactivity doses. In Turkey, 35 elbows, 26 ankles and two shoulders in 49 patients aged between 3 and 30 years were treated with 186Re. The patients were followed up from 6 months to 3 years. At 6 months after the procedure, 81% of the elbows and 86% of the ankles with grade II synovitis were free from bleeding, as well as 53% and 44% of the elbows and ankles with grade III synovitis, respectively.

CONCLUSIONS:

Radiation synovectomy appears to be the method of choice in the treatment of recurrent bleeding in the joint cavity in people with haemophilia. The efficacy of surgical synovectomy is lower in comparison with radiation synovectomy. Risks associated with surgery and anaesthesia, the need of hospitalisation and a prolonged period of rehabilitation are bothering. On the contrary, the application of corticosteroids cannot be recommended as a good method to treat recurrent haemarthroses.

PMID:
24750967
[PubMed - in process]
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