Current rehabilitation practice for the evaluation and treatment of children with arthrogryposis: an international survey.

PURPOSE: To describe the current practices in rehabilitation for the evaluation and treatment of children with arthrogryposis multiplex congenita (AMC). MATERIALS AND METHODS: Rehabilitation practitioners worldwide with at least 2 years of experience with AMC were invited to complete an electronic survey on the evaluations and treatments used with children with AMC within five areas: muscle and joint function, self-care, mobility, pain, participation and psychosocial wellbeing. RESULTS: Sixty five participants from nine countries completed the survey. Participants completed the sections applicable to their practice. Number of participants within each area varied between 24 and 53. Over 80% of participants used non-standardized evaluations across areas while <50% used patient-reported or standardized measures. Stretching of upper and lower limbs was reported by >80% across ages and clinical presentation severity. Strengthening reported by >70% was mainly used among children >3 years old with less severe contractures. Other interventions used across areas included orthotics (>70%), positioning (>80%), activity-based training (>80%), assistive devices for self-care (>50%) and mobility (>80%), and energy conservation (>70%). Over 70% of participants were involved in perioperative rehabilitation. CONCLUSION: Knowledge of current pediatric rehabilitation practice in AMC, together with empirical evidence, may guide clinical decision-making and identify avenues for future research.

Arthrogryposis multiplex congenita (AMC) is a group of rare conditions and there is currently little empirical evidence on rehabilitation, therefore expert opinion is important to guide best practice.Rehabilitation practitioners should consider the areas of muscle and joint function, self-care and mobility, pain, participation, and psychosocial wellbeing when evaluating and developing a treatment plan for children with AMC.Considering the heterogeneity of AMC, different assessment tools may be selected depending on the clinical presentation of each child.In addition to stretching, orthotic use, and strengthening, the use of activity based training and assistive equipment is important to promote self-care, mobility and participation.

Expert guidance for the rehabilitation of children with arthrogryposis: protocol using an integrated knowledge translation approach.

BACKGROUND: Arthrogryposis multiplex congenita (AMC) is a group of rare congenital disorders characterized by multiple joint contractures present at birth. Contractures can affect different body areas and impact activities of daily living, mobility and participation. Although early rehabilitation is crucial to promote autonomy and participation in children with AMC, empirical evidence to inform best practice is scarce and clinical expertise hard to develop due to the rarity of AMC. Preliminary research involving stakeholders in AMC (youth with AMC, parents, and clinicians) identified priorities in pediatric rehabilitation. Scoping reviews on these priorities showed a lack of high quality evidence related to rehabilitation in AMC. The objective of this project is to provide rehabilitation expert guidance on the assessment and treatment of children with AMC in the areas of muscle and joint function, pain, mobility and self-care, participation and psychosocial wellbeing. METHODS: An integrated knowledge translation approach will be used throughout the project. Current rehabilitation practices in AMC will be identified using a clinician survey. Using the Grading of Recommendations, Assessment, Development and Evaluations framework (GRADE) approach, a panel of interdisciplinary expert clinicians, patient and family representatives, and researchers will develop expert guidance on the assessment and treatment for pediatric AMC rehabilitation based on findings from the scoping reviews and survey results. Consensus on the guidance statements will be sought using a modified Delphi process with a wider panel of international AMC experts, and statements appraised using the Appraisal of Guidelines for Research and Evaluation II (AGREE II) tool. Theoretical facilitators and barriers toward implementing clinical guidance into practice will be identified among rehabilitation clinicians and managers to inform the design of dissemination and implementation strategies. DISCUSSION: This multi-phase project will provide healthcare users and providers with research-based, expert guidance for the rehabilitation of children with AMC and will contribute to family-centered practice.

Arthrogryposis multiplex congenita (AMC) is a group of rare disorders where a child is born with stiff joints. Joint stiffness can be present in different parts of the body, making it difficult for the child to move, to walk and to participate in activities. Rehabilitation interventions starting early in life are very important to maximize autonomy. AMC being rare, it is difficult for clinicians to develop experience with this population and there is little research specific to rehabilitation interventions in AMC. In a previous research project, a group of youth with AMC and their families identified five areas of priority for rehabilitation: muscle and joint function, pain, mobility and self-care, participation and psychosocial wellbeing. The purpose of this project is to provide guidance for the evaluation and treatment of children with AMC, in these priority areas. The project involves multiple steps. First, we will perform an online survey with international rehabilitation practitioners to learn about current evaluation and treatment approaches used with children with AMC. We will then bring together expert clinicians, patient and family representatives, and researchers to develop the guidance statements. Then, we will perform an online survey with a larger group of international experts to validate and finalize the guidance statements. Finally, we will interview rehabilitation clinicians and managers to find out about what could help them or limit them in integrating this guidance into their practice. This project will provide healthcare users and providers with research and expert-based guidance for the rehabilitation of children with AMC.

Changes in postural stability on balance platform in patients after meniscal repair - two years follow up.

PURPOSE: The aim of this work was to evaluate postural stability on the balance platform averagely 2 years following meniscal repair. METHODS: This is a retrospective, case-control comparative analysis of patients who underwent surgical repair for the isolated longitudinal traumatic meniscal tear versus matched healthy controls. The study group consisted of 30 patients (mean age 29.93 years; averagely 2.3 years after surgery) and the control group - of 30 people. Following physical examination and completion of the IKDC, and the Lysholm questionnaires, the evaluation of the postural stability using two single-leg stabilometry tests was performed. In the static test, the analyzed variables included deviations from the horizontal, vertical axes and the length of the balance path travelled. In the dynamic test, the length of the path travelled and the time to complete task were recorded. Between-limb and between-groups comparison of collected stabilometry tests were performed. Additionally, the IKDC and the Lysholm questionnaires scores were compared between the study and heathy groups. RESULTS: No abnormalities were found on clinical examination in the study group nor any differences between the operated and contralateral knee ( p > 0.05). In stabilometry: (1) in the study group, the operated extremity scored worse than the contralateral limb (length of path traveled in: A) static test x = 56.7 cm SD = 37.91 cm vs. x = 21.6 cm SD = 9.06 cm; p = 0.002 and B) dynamic test x = 82.57 cm, SD = 50.43 cm vs. x = 53.32 cm, SD = 13.82 cm; p = 0.003); (2) In the control group, no leg-related differences were noted ( p > 0.05); (3) Between-group comparison revealed that the study group scored worse than the control group (length of path traveled in: A) static test x = 56.7 cm, SD = 37.91 cm vs. x = 17.23 cm, SD = 3.39 cm; p = 0.001 and B) dynamic test x = 82.57 cm, SD = 50.43 cm vs. x = 32.13 cm, SD = 9.41 cm; p < 0.001). Study group scored worse on IKDC scores ( p < 0.001) but not on Lysholm score ( p > 0.05). CONCLUSIONS: Postural stability deficit persists despite a successful meniscal repair.

Treatment and outcomes of arthrogryposis in the lower extremity.

In this multiauthored article, the management of lower limb deformities in children with arthrogryposis (specifically Amyoplasia) is discussed. Separate sections address various hip, knee, foot, and ankle issues as well as orthotic treatment and functional outcomes. The importance of very early and aggressive management of these deformities in the form of intensive physiotherapy (with its various modalities) and bracing is emphasized. Surgical techniques commonly used in the management of these conditions are outlined. The central role of a multidisciplinary approach involving all stakeholders, especially the families, is also discussed. Furthermore, the key role of functional outcome tools, specifically patient reported outcomes, in the continuous monitoring and evaluation of these deformities is addressed. Children with arthrogryposis present multiple problems that necessitate a multidisciplinary approach. Specific guidelines are necessary in order to inform patients, families, and health care givers on the best approach to address these complex conditions.

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.

International multidisciplinary collaboration toward an annotated definition of arthrogryposis multiplex congenita.

Arthrogryposis multiplex congenita (AMC) has been described and defined in thousands of articles, but the terminology used has been inconsistent in clinical and research communities. A definition of AMC was recently developed using a modified Delphi consensus method involving 25 experts in the field of AMC from 8 countries. Participants included health care professionals, researchers, and individuals with AMC. An annotation of the definition provides more in-depth explanations of the different sentences of the AMC definition and is useful to complement the proposed definition. The aim of this study was to provide an annotation of the proposed consensus-based AMC definition. For the annotation process, 17 experts in AMC representing 10 disciplines across 7 countries participated. A paragraph was developed for each sentence of the definition using an iterative process involving multiple authors with varied and complementary expertise, ensuring all points of view were taken into consideration. The annotated definition provides an overview of the different topics related to AMC and is intended for all stakeholders, including youth and adults with AMC, their families, and clinicians and researchers, with the hopes of unifying the understanding of AMC in the international community.

[Baclofen dose in a screening test and in a baclofen pump filling--is there any differences]. / Dawka baklofenu w tescie baklofenowym a rzeczywista w czasie leczenia.

Spasticity is one from many clinical signs appearing in head and spinal cord injury and it is characterized by increased muscle tone with increased tendom reflex and reccurent spasms. Intrathecal baclofen therapy is surgical method for treatment of severe spasticity and a pump implantation is preceded by baclofen screening test. In this study we compared a bolus intrathecal injection given in a screening test with doses uses for filling a baclofen pump. In selected group we observed increased Baclofen dose due to dose given in screening test.

[The parents expectations after baclofen pump implantation in child with severe spasticity in physical rehabilitation and psychological aspects]. / Oczekiwania rodziców po wszczepieniu pompy baklofenowej u dziecka ze spastycznoscia w aspekcie rehabilitacyjnym i psychologicznym.

UNLABELLED: Treatment of spasticity with intrathecal baclofen pump implantation is a chance for clinical improvement in patients with severe spasticity. It results also in improvement of patients and their families quality of life. The aim of study was to assess the effects after baclofen pump implantation due to parents expectations. MATERIAL AND METHODS: The study was performed on a group of 17 parents which children have implantable baclofen pump. According to Assessment of Efficacy in Treatment Spasticity Questionaire was determined if the effects after pump implantation are satisfactory in parents opinion. There were done compartments of results from Questionaire, for which we can say about 1. good effects, 2. satisfactory effects, and 3. unsatifactory effects. RESULTS: In a group of 17 parents of children with implanted baclofen pump 10 assess that the effects after pump implantation are good, 5--effects are satisfactory and 2--effects are unsatisfactory. Parents noted substantial improvement in sleep and rest, in facilitating of body care, in reducing the pain and simplifies followed rehabilitation. Partial dissatifaction comed from excessive expectations due to terapeutical possibilities of the implantable baclofen pump. CONCLUSION: Intrathecal baclofen therapy brings essential benefits to patients and their families.