Pediatric Performance-Based Outcome Measures for Upper Extremity Function: A Scoping Review and Linking to the International Classification of Functioning, Disability, and Health.

Background. Performance-based outcome measures (PBOMs) are objective measures that assess physical capacity or performance in specific tasks or movements. Purpose. 1) to identify which PBOMs are most frequently reported to evaluate upper extremity (UE) function in pediatric rehabilitation 2) to determine the link between constructs of the ICF and meaningful concepts extracted from each identified PBOM. Methods. Pediatric UE PBOMs were searched in four databases. The selection of outcome measures included an initial title and abstract screening, followed by full-text review of the articles to be included based on identified selection criteria. Two reviewers were appointed to link the meaningful concepts identified in the outcome measures independently and a third reviewer was consulted in case of ambiguity to make a final decision. Findings. After the initial screening, 1786 full-text articles were reviewed, 1191 met the inclusion criteria, in which 77 outcome measures were identified and 32 were included in the linking process. From the included 32 outcome measures, 538 items were extracted and linked to the ICF. The most commonly cited measures included Assisting Hand Assessment, Jebsen-Taylor Hand Function Test, Melbourne Assessment of Unilateral Upper Limb. The Activity and Participation domain represented 364 codes followed by the Body Functions domain domain which represented 174 codes. Implications. A majority of the outcome measures identified were linked with the Mobility, Fine Hand Use of the ICF. Therefore, when selecting a PBOM, careful considerations need to be made regarding which concept of health is to be assessed.

Stakeholder engagement in the development of an upper extremity outcome measure for children with rare musculoskeletal conditions.

BACKGROUND: Upper extremity (UE) involvement is prevalent in 73% of individuals with arthrogryposis multiplex congenita (AMC), yet no AMC-specific outcome measure exists. When developing a measure specific to a population with a rare musculoskeletal condition, clinicians' and patients' perspectives and involvement is a crucial and necessary step. This study sought to determine the most clinically useful items for an outcome measure of UE function for children with AMC as defined by caregivers and clinicians. METHODS: To ensure the perspectives and needs of caregivers of children with AMC and clinicians were considered in the development of the UE measure for AMC, a Nominal Group technique (NGT) with caregivers of children with AMC (phase 1) followed by a three-round survey with clinicians (phase 2) were carried out. RESULTS: Phase 1: Eleven individuals participated in the nominal group technique and identified 32 items. The most important items were Picking up an object (n = 11), Eating (n = 10), Reaching mouth (n = 10), Getting out of bed (n = 10). Phase 2: Invitations to participate to an online survey was sent to 47 experts in the field of AMC, 20 participants completed round 1, 15 completed round 2 and 13 completed round 3. Throughout the survey, participants were asked about movement required to screen the UE, essential domains to be included in the measure, establishing a scoring guide and identifying tasks associated with joint motion and position. CONCLUSION: A preliminary version of an UE AMC-specific outcome measure was developed with the help of caregivers' perspectives and expert opinions.

Arthrogryposis multiplex congenita (AMC) is a rare musculoskeletal condition affects the joints and muscles of the body. In about 70% of the cases, it affects the upper extremities (UE). However, there is no specific outcome measure for UE function in children with AMC. An outcome measure refers to a tool or method used to assess and measure the results or effects of a particular treatment, intervention, or condition. It helps healthcare professionals and researchers understand the impact or outcome of a specific situation, such as the level of improvement or changes in a person's health or function. The goal of this study was to develop such a measure while accounting for the perspectives of youth with AMC, their caregivers and clinicians. To achieve this, a study was conducted in two phases. In the first phase, a Nominal Group technique (NGT) was used to gather input from caregivers of children with AMC. Eleven individuals participated and identified 32 items, with the most important being picking up an object, eating, reaching the mouth, and getting out of bed. In the second phase, a three-round survey was sent to 47 experts in the field of AMC, with 20 participants completing the first round, 15 completing the second round, and 13 completing the third round. The survey asked participants about screening UE movement, essential domains to include in the measure, establishing a scoring guide, and identifying tasks associated with joint motion and position. With the help of caregivers' perspectives and expert opinions, a preliminary version of an UE AMC-specific outcome measure was developed. This measure will be useful in assessing the UE function in children with AMC and will aid clinicians in developing appropriate treatment plans for this rare condition.

Using Telerehabilitation to Deliver a Home Exercise Program to Youth With Arthrogryposis: Single Cohort Pilot Study.

BACKGROUND: Arthrogryposis multiplex congenita (AMC) is characterized by joint contractures and muscle weakness, which limit daily activities. Youths with AMC require frequent physical therapeutic follow-ups to limit the recurrence of contractures and maintain range of motion (ROM) and muscle strength; however, access to specialized care may be limited because of geographical distance. Telerehabilitation can offer a potential solution for delivering frequent follow-ups for youth with AMC, but research on the use of telerehabilitation in children with musculoskeletal disorders is scarce. OBJECTIVE: The study aims to evaluate the feasibility of delivering a home exercise program (HEP) by using telerehabilitation for youth with AMC. We also aim to explore the effectiveness of the HEP as a secondary aim. METHODS: Youths aged between 8 and 21 years with AMC were recruited at the Shriners Hospitals for Children-Canada. The participants completed baseline and post-HEP questionnaires (the Physical Activity Questionnaire for Adolescents, Pediatrics Outcomes Data Collection Instrument, and Adolescent and Pediatric Pain Tool), and clinicians assessed their active ROM using a virtual goniometer. Clinicians used the Goal Attainment Scale with the participants to identify individualized goals to develop a 12-week HEP and assess the achievement of these goals. Follow-ups were conducted every 3 weeks to adjust the HEP. Data on withdrawal rates and compliance to the HEP and follow-ups were collected to assess the feasibility of this approach. The interrater reliability of using a virtual goniometer was assessed using the intraclass correlation coefficient and associated 95% CI. Nonparametric tests were used to evaluate feasibility and explore the effectiveness of the HEP. RESULTS: Of the 11 youths who were recruited, 7 (median age: 16.9 years) completed the HEP. Of the 47 appointments scheduled, 5 had to be rescheduled in ≤24 hours. The participants performed their HEP 2.04 times per week (95% CI 1.25-4.08) and reported good satisfaction with the approach. A general intraclass correlation coefficient of 0.985 (95% CI 0.980-0.989) was found for the web-based ROM measurement. Individualized goals were related to pain management; endurance in writing, standing, or walking; sports; and daily activities. In total, 12 of the 15 goals set with the participants were achieved. Statistically significant improvements were observed in the pain and comfort domain of the Pediatrics Outcomes Data Collection Instrument (preintervention: median 71; 95% CI 34-100; postintervention: median 85; 95% CI 49-100; P=.08) and Physical Activity Questionnaire for Adolescents (preintervention: median 1.62; 95% CI 1.00-2.82; postintervention: median 2.32; 95% CI 1.00-3.45; P=.046). CONCLUSIONS: The remote delivery of an HEP for youth with AMC is feasible. Promising results were found for the effectiveness of the HEP in helping youths with AMC to achieve their goals. The next step will be to assess the effectiveness of this exercise intervention in a randomized controlled trial. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): RR2-10.2196/18688.

A review of the orthopedic interventions and functional outcomes among a cohort of 114 children with arthrogryposis multiplex congenita.

PURPOSE: Arthrogryposis multiplex congenita (AMC) refers to a large heterogeneous group of conditions involving joint contractures in two or more different areas of the body. Contractures can lead to decreased range of motion and strength, and affect ambulation and autonomy. The aim of this study was to describe the orthopedic interventions and functional outcomes of a large cohort of children with AMC followed in a pediatric orthopedic center. METHODS: A retrospective chart review of all children diagnosed with AMC followed at Shriners Hospital for Children - Canada (SHC) between January 1979 and July 2016 was conducted. One hundred twenty patients were identified, of whom six were excluded due to misdiagnosis or insufficient chart information. One hundred fourteen were retained. Patient demographics, AMC classification, comorbidities, operative and non-operative treatments received as well as community ambulation status, level of autonomy in self-care and transfers at latest follow-up were recorded. RESULTS: There were 54 males and 60 females with a mean age at last clinic visit of 10 years 3 months. Amyoplasia and distal arthrogryposis (DA) were equally represented in our sample, 47 (41.2%) and 49 (43.0%) participants respectively, with the category Other comprising the remaining 18 (15.8%) participants. Children with DA had less involvement of the proximal joints than those in the two other groups. Contractures and deformities of the foot and ankle were the most prevalent, affecting 91.5% with Amyoplasia, 85.7% with DA and 83.3% in the Other category. Contractures of the shoulder and elbow were more common among individuals with Amyoplasia and those categorized Other than those with DA. In terms of walking ability, 98% of participants with DA were independent ambulators. Walking ability varied among the Other participants. Similarly, most children with DA were independent in self-care and transfers at the most recent follow-up. CONCLUSION: The relatively large sample size of this study allowed for a better insight into the challenges associated with AMC management. These findings demonstrated the need for genetic testing to provide accurate diagnosis and classification, along with the use of standardized outcome tools to measure effectiveness of interventions. As AMC is rare, multi-site prospective studies are needed to improve research opportunities, develop functional measures specific to AMC and disseminate findings on a wider scale.

A Telerehabilitation Intervention for Youths With Arthrogryposis Multiplex Congenita: Protocol for a Pilot Study.

BACKGROUND: Arthrogryposis multiplex congenita (AMC) is characterized by joint contractures present in at least two body areas. In addition to these contractures, individuals with AMC can have decreased muscle mass, leading to limitations in activities of daily living. Exercise has the potential to maintain or improve the range of motion and muscle strength. However, this type of intervention necessitates frequent follow ups that are currently difficult to provide for youths with AMC because they often live far from a specialized hospital. To overcome this distance challenge, telecommunication technologies can be used to deliver rehabilitation remotely, which is called telerehabilitation. The study protocol for one such type of rehabilitation will be presented in this paper. OBJECTIVE: This pilot study aims to (1) evaluate the feasibility of using telerehabilitation to provide a home exercise program for youths with AMC, and (2) assess the effectiveness of a home exercise program. METHODS: A total of 10 youths aged 8-21 years with AMC will be recruited. The intervention consists of a 12-week individualized home-based exercise program delivered remotely using telerehabilitation. At baseline, youths will complete the Physical Activity Questionnaire for Adolescents and the Pediatrics Outcomes Data Collection Instrument to assess pain, function, and level of physical activity. During the first telerehabilitation meeting, the rehabilitation therapists will measure range of motion using a virtual goniometer and assess the youth's functional level. The therapists will then use the Goal Attainment Scale to set objectives and develop the individualized intervention. Follow ups will occur every 3 weeks to make sure exercises are performed safely and to progress the exercises when needed. At the end of the 12-week intervention, rehabilitation therapists will re-evaluate the youth using the same outcome measures as the initial evaluation. The youths will be asked to complete the same questionnaires, with the addition of questions about their satisfaction regarding the intervention. Nonparametric and descriptive statistics will be used to evaluate the feasibility and effectiveness. RESULTS: Ethics approval was obtained in October 2018. Recruitment and data collection started in January 2019 and was completed in May 2020. CONCLUSIONS: This pilot study will help us learn how a large-scale project may work in practice to improve outcomes in physical activity, pain, and function, and goal attainment among youths with AMC, thus informing a future clinical trial. INTERNATIONAL REGISTERED REPORT IDENTIFIER (IRRID): DERR1-10.2196/18688.

Participation among Children with Arthrogryposis Multiplex Congenita: A Scoping Review.

AIM: To explore what is currently known regarding participation among children and youth with arthrogryposis multiplex congenita (AMC) using empirical studies, gray literature, and YouTube videos. The secondary objectives included identifying activity types, outcome measures used, interventions provided, and barriers and facilitators to participation. METHOD: Empirical studies and gray literature were searched through electronic databases and videos were searched on YouTube. Articles and videos pertaining to participation and youth with AMC were included by two reviewers. Data regarding activity types, location, outcomes measures, interventions, and barriers and facilitators to participation was extracted. Data was critically appraised using specific evaluation criteria. RESULT: Eleven empirical studies, six gray literature articles and 71 videos met the inclusion criteria. The most common activity types reported in the empirical studies and YouTube videos were active-physical, social, and skill-based activities. Outcome measures included evaluations and questionnaires, none of which were designed to address participation. Interventions did not target participation although the environments could affect participation. CONCLUSION: The paucity of research indicates a need for future studies of participation in this population. Interventions should target participation and address environmental barriers. Videos provide insight for clinicians, youth, and families to help promote participation in the natural environment.

Rehabilitation needs of youth with arthrogryposis multiplex congenita: Perspectives from key stakeholders.

Background: Arthrogryposis multiplex congenita is a term used to describe congenital contractures in at least two body parts with an overall prevalence of 1 in 3000 live births. It is often caused by lack of fetal movement in utero and presents as contractures of varying severity, which may affect the upper and lower extremities, the spine and jaw. Currently, no practice recommendations exist to inform best clinical practice for arthrogryposis multiplex congenita.Purpose: To identify the needs surrounding rehabilitation as experienced by youth with arthrogryposis multiplex congenita, caregivers, and clinicians and to propose solutions to develop family- and client-centred rehabilitation recommendations.Materials and methods: A modified experience-based co-design methodology was used where qualitative interviews were conducted with key stakeholders.Results: Twenty-seven participants completed the interviews and demographic information was collected where early-active rehabilitation began at birth in most cases and became less frequent through adolescence. Three overarching themes were determined for each stakeholder group.Conclusions: All participants reported that early-active rehabilitation is beneficial as it helps determine future treatments. Transition times and participation need to be at the center of interventions to ensure that the needs of youth with arthrogryposis multiplex congenita are being met. The development a condition-specific outcome-measure and rehabilitation practice recommendations will assist clinicians in addressing the needs of youth with arthrogryposis multiplex congenita.Implications for rehabilitationArthrogryposis multiplex congenita presents in at least two different areas of the body as multiple congenital contractures of varying severity which may affect the upper and lower extremities, spine and jaw.Youth with arthrogryposis multiplex congenita identified participation as an essential component of their life, however caregivers and clinicians did not emphasize this need.Gathering information from different stakeholders is important to ensure varying needs are addressed.Rehabilitation was reported to be beneficial from early childhood to late adolescence by youth, caregivers, and clinicians.Frequency of rehabilitation diminished over time, emphasizing the need for continued follow-up into adolescence.

Rehabilitation across the lifespan for individuals with arthrogryposis.

Arthrogryposis multiplex congenita (AMC) can be a perplexing diagnosis that consists of limited range of motion (ROM) and decreased muscle strength in multiple joints. The person with AMC often possesses a certain tenacity and "spunk" that assists them with adjusting and adapting to the realities of daily life. The rehabilitation process assists the individual with AMC in achieving and maintaining the maximal active and passive range of motion and strength in order to participate in activities of daily living (ADL) throughout the developmental stages. The result of this life-long process is greatly impacted by collaboration among the multidisciplinary teams. Ultimately, rehabilitation should focus on three levels of treatment: (a) body structure, (b) activity, and (c) participation. This article describes rehabilitation across the lifespan-focusing on the therapeutic needs in the infant, toddler, school age and teenage/adult years-while also highlighting opportunities for improvement.

Arthrogryposis multiplex congenita definition: Update using an international consensus-based approach.

Arthrogryposis multiplex congenita (AMC) has been described and defined in thousands of articles, but the terminology used has been inconsistent. Some have described it as a diagnosis or syndrome, others as a term or clinical finding. This lack of common language can lead to confusion in clinical and research communities. The aim of this study was to develop a consensus-based definition for AMC using international expert opinion. A consensus-based definition will help harmonize research and clinical endeavors and will facilitate communication among families, clinicians, and researchers. This article describes the methodology used leading to a proposed definition of AMC. First, a literature review was conducted to identify AMC definitions used in included studies. The most commonly used words in the definitions were extracted. Second, a group of eight experts in AMC was selected to identify elements considered critically important to the definition of AMC. Third, based on these critical elements and the literature review, a definition was drafted by the research team. Fourth, a modified Delphi consensus process was conducted using electronic surveys with 25 experts in the field of AMC from eight countries. Survey results were analyzed quantitatively and qualitatively and drafts were modified accordingly. Three rounds of surveys were completed until consensus was reached on a definition of AMC. An annotation of this definition, developed by a panel of international experts, is provided in a separate manuscript in this special issue.

Barriers and facilitators to work reintegration and burn survivors' perspectives on educating work colleagues.

INTRODUCTION: Work reintegration constitutes a major milestone in the rehabilitation process of adults who have sustained a burn. Research studies with other conditions demonstrated that open, explicit communication about the worker's condition and potential limitations may facilitate this transition. However, the best approach to enable this discussion to occur has yet to be described. The aim of this exploratory qualitative study was to investigate burn survivors' and clinicians' perspectives of the barriers and facilitators to work reintegration that could be addressed through education of work colleagues, which information to communicate to the workplace and the most effective method to disseminate this knowledge. METHODS: Five semi-structured focus groups were conducted with three groups of informants including: (1) 13 burn survivors who had already returned to work; (2) 7 who were planning on returning; and (3) 9 burn care professionals. Qualitative data were inductively analyzed employing constant comparative techniques. RESULTS: Key barriers and facilitators that were identified included residual impairments, individual characteristics, support from the social environment, work accommodations and resources from the healthcare and compensation systems. Burn survivors agreed that return to work efforts were not adequately supported and that education should be provided to work colleagues about the burn and rehabilitation process, but that information on residual impairments should be communicated judiciously as it may be used prejudiciously against those seeking new employment. In the latter case, it is preferable to inform the workplace of their strengths and abilities. CONCLUSIONS: Extensive literature demonstrating the benefits of educational programs for the peers and teachers of pediatric burn survivors when they return to school already exists. This study provides evidence that there is a need for a similar process for adult burn survivors returning to work. The educational material must be versatile enough so that it can be customized to individual burn survivor's needs and the environments in which they work.