Shriners Hospital for Children-Canada, Montreal, Canada.
School of Physical and Occupational Therapy, Faculty of Medicine, McGill University, Montreal, Canada.
Am J Med Genet C Semin Med Genet. 2019 Sep;181(3):436-453. doi: 10.1002/ajmg.c.31725. Epub 2019 Jul 26.
Clinical interventions and research have mostly focused on the orthopedic and genetic outcomes of individuals with arthrogryposis multiplex congenita (AMC), and although pain has gained recognition as an important issue experienced by individuals with AMC, it has received little attention within the AMC literature. The aims of this scoping review were to describe the pain experiences of children and adults with AMC, to identify pain assessment tools and management techniques, and document the impact of pain on participation in everyday activities among children and adults with AMC. A search of the literature was conducted in four search engines and identified a total of 89 articles. Once study eligibility was reviewed, 21 studies met the selection criteria and were included in this review. Pain appears to be more commonly experienced in adults with AMC compared with children with AMC, with individuals having undergone multiple corrective procedures self-reporting pain more often. In adult populations, musculoskeletal chronic pain is a significant problem, resulting in restrictions in activities of daily living, mobility, and participation. Researchers and clinicians must agree on the use of validated measures appropriate for evaluating pain in AMC and the use of appropriate pain management techniques to relieve pain. Pediatric studies should focus on determining how commonly pain is experienced in infants, children, and adolescents with AMC. Pain in adults with AMC should be acknowledged to offer proper client-centered interventions throughout the lifespan.
临床干预和研究主要集中在多发性先天性关节挛缩症（AMC）患者的骨科和遗传结果上，尽管疼痛已被认为是 AMC 患者经历的一个重要问题，但在 AMC 文献中却很少受到关注。本范围综述的目的是描述 AMC 儿童和成人的疼痛体验，确定疼痛评估工具和管理技术，并记录疼痛对 AMC 儿童和成人日常活动参与的影响。在四个搜索引擎中进行了文献检索，共确定了 89 篇文章。在审查了研究的资格后，有 21 项研究符合选择标准，并被纳入本综述。与 AMC 儿童相比，疼痛似乎在 AMC 成人中更为常见，经历过多次矫正手术的人更常报告疼痛。在成年人群中，肌肉骨骼慢性疼痛是一个严重的问题，导致日常生活活动、移动性和参与受限。研究人员和临床医生必须就评估 AMC 中疼痛的经过验证的适当措施的使用以及使用适当的疼痛管理技术来缓解疼痛达成一致。儿科研究应侧重于确定 AMC 婴儿、儿童和青少年中疼痛的常见程度。应承认 AMC 成人的疼痛，以便在整个生命周期中提供适当的以客户为中心的干预措施。
