Södergard J
Rev Chir Orthop Reparatrice Appar Mot. 1996;82(5):403-9.
In arthrogryposis Multiplex Congenita (AMC) multiple joint contractures and deformities are present. Hips are often affected. In this follow-up study the outcome after primary treatment and secondary surgical procedures was analyzed with clinical and radiological features.
52 patients with AMC were treated at Children's Hospital, Helsinki. 34 patients had Hip joint involvement. The most common type of Hip joint involvement was a flexion contracture (40 per cent). Thirty-five per cent of patients had rigid dislocations. Ten hips with rigid dislocations and four hips with flexion contractures was primarily operated on. Two failures of the primary operative procedures were noted. Thirty-one dislocated hips were treated non-operatively.
In fourteen cases, reduction could not be achieved. Nineteen delayed surgical procedures were done. Acetabuloplasty was made in 6 hips, femoral osteotomy in 8 hips and total hip replacement in 3 hips. Aseptic necrosis of the femoral head encountered for 4 poor results in two cases of acetabuloplasty and 3 hips had a poor range of motion. In flexion contractures of the hips, 6 corrective osteotomies were done and in four cases the results were good. In all cases of total hip replacement the results were good and no complications were seen.
The inefficiency to achieve reduction of dislocated hips in AMC-patients by conservative methods was confirmed in this study. Although it has been claimed that the majority of patients with flexion contractures can be managed by conservative treatment there were ten patients in this series who did not respond to this form of treatment.
The primary form of treatment should be operative. Only in selected cases with bilateral dislocations should it be considered to leave the hips untreated. Double femoral osteotomy in recurrent flexion contractures or resistant flexion contractures and total hip replacement in adult patients with hip dislocations are useful methods.
先天性多发性关节挛缩症（AMC）存在多个关节挛缩和畸形。髋关节常受影响。在这项随访研究中，通过临床和放射学特征分析了初次治疗和二次手术的结果。
52例AMC患者在赫尔辛基儿童医院接受治疗。34例患者髋关节受累。髋关节受累最常见的类型是屈曲挛缩（40%）。35%的患者有僵硬性脱位。对10例僵硬性脱位的髋关节和4例屈曲挛缩的髋关节进行了初次手术。记录到2例初次手术失败。31例脱位髋关节采用非手术治疗。
14例未能实现复位。进行了19次延迟手术。6例髋关节进行了髋臼成形术，8例进行了股骨截骨术，3例进行了全髋关节置换术。2例髋臼成形术出现股骨头无菌性坏死，导致4例效果不佳，3例髋关节活动范围不佳。对于髋关节屈曲挛缩，进行了6次矫正截骨术，4例效果良好。所有全髋关节置换术病例效果良好，未出现并发症。
本研究证实了AMC患者采用保守方法复位脱位髋关节无效。尽管有人声称大多数屈曲挛缩患者可通过保守治疗，但本系列中有10例患者对这种治疗方式无反应。
主要治疗方式应为手术。仅在双侧脱位的特定病例中，才应考虑不治疗髋关节。对于复发性屈曲挛缩或顽固性屈曲挛缩进行双股骨截骨术，以及对髋关节脱位的成年患者进行全髋关节置换术是有用的方法。
