Abstract
BackgroundArthrogryposis multiplex congenita (AMC) is a group of congenital conditions characterized by joint contractures in two or more body areas. Management of AMC starts early in life and focuses on improving mobility and function through intensive rehabilitation and surgical interventions. Psychosocial wellbeing is an important determinant of health and the psychosocial experience of individuals with AMC should be considered in the management of this condition. The aim of this scoping review was to explore what is known about the psychosocial wellbeing of children and adults with AMC, to identify the outcome measures used and to explore the factors associated with psychosocial outcomes in this population.MethodsA comprehensive search in four databases was conducted. Articles discussing psychosocial outcomes and outcome measures used with children or adults with AMC were included. Data on the measures used, psychosocial outcomes, and factors associated with psychosocial outcomes, were extracted and analyzed descriptively and synthesized narratively.ResultsSeventeen articles were included in this scoping review, ten including the pediatric population, six including adults and one article including both children and adults with AMC. The most commonly used outcome measures were the PODCI in the pediatric studies, and the SF-36 in studies on adults. In the pediatric studies, psychosocial outcomes were often secondary, compared to the studies on adults. Results showed that in both children and adults, psychosocial outcomes are comparable with the levels of the general population. Qualitative studies reflected the affective needs of this population and issues with emotional wellbeing. Factors such as fatigue and pain were associated with poorer psychosocial outcomes in adults with an impact on social relationships, intimacy and family planning.ConclusionValidated outcome measures, qualitative approaches and longitudinal studies are needed to better understand the psychosocial outcomes in AMC over time. Psychosocial support should be part of the multidisciplinary management of AMC throughout the lifespan.
Highlights
Arthrogryposis multiplex congenita (AMC) is a group of congenital conditions characterized by joint contractures in two or more body areas
Arthrogryposis multiplex congenita (AMC) is a term that describes a group of congenital conditions characterized by joint contractures in two or more body areas [1] and affects 1 in 3000 live births [2, 3]
Three hundred and nine articles were excluded at screening of titles and abstracts, and 29 articles were excluded at screening of full texts
Summary
Arthrogryposis multiplex congenita (AMC) is a group of congenital conditions characterized by joint contractures in two or more body areas. Arthrogryposis multiplex congenita (AMC) is a term that describes a group of congenital conditions characterized by joint contractures in two or more body areas [1] and affects 1 in 3000 live births [2, 3]. Depending on the severity and distribution of joint contractures, individuals with AMC may experience limitation in mobility and activities of daily living [5,6,7]. Other body systems such as the respiratory, gastro-intestinal, and central nervous system may be affected, depending on the underlying diagnosis [1, 3]. The management of AMC starts early in life with intensive rehabilitation, such as splinting and orthosis, range of motion exercises, strengthening programs, and surgical interventions to correct deformities, all of which aim to improve function [8,9,10,11]
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