Panotopoulos Joannis, Trieb Klemens
Universitätsklinik für Orthopädie, Universität Wien.
Wien Med Wochenschr. 2003;153(9-10):225-31. doi: 10.1046/j.1563-258x.2003.02117.x.
Patients with severe hemophilia A or B and von Willebrand Syndrome have recurrent joint bleedings, which can occur spontaneously or as a result of trauma. Resorption of intraarticular blood induces an inflammation of the synovium and cartilage damage, finally leading to severe changes of the joint with complete destruction. This process is called hemophilic arthropathy. After failure of substitution and intensive conservative therapy after 3 to 6 months, a synovectomy or a synoviorthesis are indicated. In some cases the progression of hemophilic arthropathy cannot be stopped and requires total joint arthroplasty. In some cases joint motion cannot be saved and requires arthrodesis. This paper presents methods and results of different operation concepts in patients (n = 39) with hemophilia or von-Willebrand-syndrome. Especially by implanting total knee (n = 17) and total hip joints (n = 6) we obtained very good functional results after an average follow-up period of 33.3 respectively 56.3 months. In a series of synovectomie of the knee performed before 1990 (n = 7) and followed after an average period of 21.4 years the progression of hemophilic arthropathy could not be stopped. In a second series of synovectomy of the knee performed after 1990 (n = 5) and follow after an average period of 5.6 years, at least the mobility of the knee could be saved. Only one patient of a series of supra- and infracondylar osteotomie (n = 9) achieved the wanted result. Further we performed 6 operations on the hindfoot with satisfying results.
重度甲型或乙型血友病以及血管性血友病患者会反复出现关节出血,出血可能自发发生,也可能由外伤引起。关节内血液的吸收会引发滑膜炎症和软骨损伤,最终导致关节严重变形并完全破坏。这个过程称为血友病性关节病。在替代治疗及强化保守治疗3至6个月失败后,需进行滑膜切除术或滑膜成形术。在某些情况下,血友病性关节病的进展无法阻止,需要进行全关节置换术。在某些情况下,关节活动无法挽救,需要进行关节融合术。本文介绍了对39例血友病或血管性血友病患者采用不同手术方案的方法及结果。特别是通过植入全膝关节(17例)和全髋关节(6例),在平均随访33.3个月和56.3个月后,我们获得了非常好的功能结果。在1990年前进行的一系列膝关节滑膜切除术(7例)中,平均随访21.4年后,血友病性关节病的进展无法阻止。在1990年后进行的第二系列膝关节滑膜切除术(5例)中,平均随访5.6年后,至少膝关节的活动度得以保留。在一系列髁上和髁下截骨术(9例)中,只有1例患者达到了预期效果。此外,我们对后足进行了6例手术,效果令人满意。