Thompson G H, Bilenker R M
Clin Orthop Relat Res. 1985 Apr(194):6-14.
Arthrogryposis multiplex congenita syndrome, characterized by multiple congenital joint contractures, is a manifestation of a neurogenic or myopathic disorder of unknown etiology. An accurate diagnosis is critical in management strategy. Muscle biopsy is usually the most important diagnostic procedure. Once the diagnosis is established, a team approach to comprehensive care is instituted, and the child is assisted in achieving maximum cognitive, physical, and social development. Physicians, nurse clinicians, therapists, social workers, and others constitute the team and work in coordination to develop an individual habilitation plan. The treatment plan is continually updated and modified as needed. Because the musculoskeletal deformities are generally the most prominent abnormality, the orthopedist usually has the major role in management. The orthopedist may serve as coordinator of the comprehensive care team and therefore must have a fundamental understanding of and global approach to management.
先天性多发性关节挛缩综合征以多发性先天性关节挛缩为特征,是一种病因不明的神经源性或肌病性疾病的表现。准确诊断对管理策略至关重要。肌肉活检通常是最重要的诊断程序。一旦确诊,就会采用团队协作的方式进行全面护理,并帮助患儿实现最大程度的认知、身体和社交发展。医生、护士临床医生、治疗师、社会工作者及其他人员组成团队,协同工作以制定个性化的康复计划。治疗计划会根据需要不断更新和调整。由于肌肉骨骼畸形通常是最突出的异常情况,骨科医生通常在管理中发挥主要作用。骨科医生可能担任综合护理团队的协调员,因此必须对管理有基本的了解并采取整体方法。