Texas Scottish Rite Hospital for Children, Dallas, Texas.
The Turner Scientific Research Institute for Children's Orthopedics, Saint Petersburg, Russia.
Am J Med Genet C Semin Med Genet. 2019 Sep;181(3):363-371. doi: 10.1002/ajmg.c.31722. Epub 2019 Jul 3.
Upper extremity involvement in patients with arthrogryposis multiplex congentia is quite frequent. Treatment initially consists of stretching and splinting as significant gains can be seen in the first years of life. The goal of any surgical procedure is to improve upper extremity function and performance of daily living activities, yet it is important to treat each patient individually and understand that areas do not always need to be addressed surgically. Despite overall lower functioning scores in this patient population, quality of life scores are comparable to the general aged adjusted population. This article will discuss the clinical presentation, treatment procedures and outcomes when addressing the upper extremities of patients presenting with arthrogryposis.
先天性多发性关节挛缩症患者上肢受累较为常见。治疗最初包括伸展和夹板固定,因为在生命的最初几年可以看到明显的改善。任何手术的目的都是改善上肢功能和日常生活活动能力,但重要的是要对每个患者进行个体化治疗,并了解并非所有部位都需要手术治疗。尽管该患者群体的整体功能评分较低,但生活质量评分与一般年龄调整人群相当。本文将讨论先天性多发性关节挛缩症患者上肢的临床表现、治疗方法和治疗结果。