Functional Independence of Children With Arthrogryposis.

BACKGROUND: Arthrogryposis (AMC) is a descriptive term to characterize a child born with multiple joint contractures. Treatment aims to improve functional independence, yet the literature objectively describing functional independence in this population is scarce. This study aimed to describe the functional independence of children with AMC through the lens of the Pediatric Evaluation of Disability Inventory-Computer Adaptive Test (PEDI-CAT) and observational activities of daily living (ADL) tasks. METHODS: Patients with AMC between the ages of 3 and 12 years participated in this prospective study. Parents completed the PEDI-CAT while a trained occupational therapist observed children as they completed a checklist of functional ADL tasks. Patients were grouped according to developmental age groups: "preschoolers" (3 to 5 y), "early school-age" (6 to 9 y), and "late school-age" (10 to 12 y). Patient's PEDI-CAT normative scores were described, comparing the study population to typically developing children, and differences in each domain were examined between developmental age groups. The observed ADL tasks completed were also described, and differences in scores were examined between developmental age groups. RESULTS: Forty-four patients (mean age of 7±2.86 y) were enrolled. The distribution between age groups was nearly even. Mean daily activities T -score for patients with AMC was 25.80±11.98 and the mean mobility T -score was 17.39±9.77. Late school-age children scored significantly lower than preschool-age children in both of these domains ( P <0.01). Observed ADL tasks demonstrated a high level of required assistance for patients (range: 27.3% to 61.4%), although older school-age children did show greater independence with tested activities than preschool-age children ( P =0.05). CONCLUSION: Children with AMC are significantly limited in functional independence, particularly regarding age-appropriate daily activities and mobility. Outcomes from this study provide a reference to help gauge the results of nonoperative and surgical treatment toward improving functional independence in this population. LEVEL OF EVIDENCE: Level III: prognostic study.

Patient-reported Outcomes for Children With Unilateral Congenital Below Elbow Deficiency.

BACKGROUND: The Patient Reported Outcomes Measurement Information System (PROMIS ® ) is a validated tool used to evaluate different domains of function in patients with chronic health conditions. This tool has not been validated in children with unilateral congenital below elbow deficiency (UCBED). The purpose of this study was to determine whether PROMIS discerns functional impairment for children with UCBED and whether children with UCBED differ from the general population with respect to PROMIS outcomes. We hypothesized that children with UCBED report mild impairment in upper extremity function but normal mobility, pain interference and peer relations. METHODS: A retrospective chart review of children aged 5 to 17 years with a diagnosis of UCBED who completed a PROMIS questionnaire at their clinic visit at the [blinded locations] was conducted between April 1, 2017 and March 31, 2020. The mean PROMIS scores of UCBED patients were compared with that of the general reference population. Mann Whitney and ANOVA tests were used to explore the differences across the PROMIS upper extremity function domain by arm length and prosthesis use. RESULTS: Fifty-five children (28 boys) with a mean age of 11±3.6 years met the inclusion criteria. Children with UCBED had similar PROMIS scores as the reference population in mobility (51.9±6.2), peer relations (53.5±9.4), and pain interference (40.1±7.2), with mild impairment in the upper extremity function (44.3±10.7). Compared with the 8 to 17-year-old cohort, the parent-proxy (5 to 7-year-old group) reported significantly more upper extremity function impairment (31.3±5.9) vs (48.0±8.8) ( P =0.000). The two age groups did not differ with respect to mobility, pain interference and peer relations. CONCLUSIONS: Our study confirms previous findings that children with UCBED report upper extremity function, peer relationships, pain interference, and mobility, similar to the reference population. In addition, parents of younger children with UCBED report more upper extremity functional impairment than is self-reported by older children with UCBED. LEVEL OF EVIDENCE: Prognostic Level III (comparison with reference population).

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.

Selective Control of the Upper Extremity Scale: validation of a clinical assessment tool for children with hemiplegic cerebral palsy.

AIM: The ability to determine the relationship between selective motor control and upper extremity function in children with unilateral cerebral palsy (CP), and to measure the functional outcome and efficacy of interventions designed to improve selective motor control, has been limited by the lack of an objective, validated tool. The primary objective of this study is to describe the development of a clinical tool entitled Selective Control of the Upper Extremity Scale (SCUES), and present evidence of its validity and reliability. METHOD: Content validity was established through an expert panel (eight clinicians, mean and median of 17y of clinical experience, range 2-30y). Intra- and interrater reliability was determined by six occupational therapists who scored 10 participant studies. Construct validity of the SCUES was established by comparison to the spontaneous functional analysis section of the Shriners Hospitals Upper Extremity Evaluation, the Manual Ability Classification System, and the Box and Block test for 25 children with unilateral CP. RESULTS: The content validity ratio values were greater than 0 (indicating >50% agreement) for 33 of the 34 items (97%), and equal or greater than 0.5 (indicating ≥75% agreement) for 26 of the 34 items (76%). Intrarater reliability was excellent (intraclass correlation coefficient [ICC] >0.75) for all segments and joints of the affected extremity. Interrater reliability was excellent for all segments and joints of the affected extremity except the shoulder (ICC=0.72). The SCUES was strongly correlated with the SHUEE (Spearman's rho=0.69, p=0.003). The SCUES was not correlated with the Manual Ability Classification System (rho=-0.24, p=0.369) or the Box and Block test (rho=0.47, p=0.066). INTERPRETATION: Psychometric analysis of the SCUES revealed comparable validity to other accepted video-based clinical assessment tools for the upper extremity in children with CP.

Assessment tools and classification systems used for the upper extremity in children with cerebral palsy.

BACKGROUND: Clinicians interested in assessment and outcome measurement of upper extremity (UE) function and performance in children with cerebral palsy (CP) must choose from a wide range of tools. QUESTIONS/PURPOSES: We systematically reviewed the literature for UE assessment and classification tools for children with CP to compare instrument content, methodology, and clinical use. METHODS: We searched Health and Psychosocial Instruments (HaPI), US National Library of Medicine (PubMed), and Cumulative Index to Nursing and Allied Health Literature (CINAHL Plus) databases (1937 to the present) to identify UE assessment and outcomes tools. We identified 21 tools for further analysis and searched HaPI, PubMed, CINAHL Plus, and Google Scholar ( http://scholar.google.com/schhp?tab=ws ) databases to identify all validity and reliability studies, systematic reviews, and original references for each of the 21 tools. RESULTS: The tools identified covered ages birth to adulthood. International Classification of Functioning, Disability and Health domains addressed by these tools included body function, body structure, activities and participation, and environmental factors. Eleven of the tools were patient or family report, seven were clinician-based observations, and three tools could be used in either fashion. All of the tools had published evidence of validity. Nine of the tools were specifically designed for use in subjects with CP. Two of the tools required formal certification before use. Ten of the tools were provided free of charge by the investigators or institution who developed them. CONCLUSIONS: Familiarity with the psychometric and clinometric properties of assessment and classification tools for the UE in children with CP greatly enhances a clinician's ability to select and use these tools in daily clinical practice for both clinical decision-making and assessment of outcome.

Functional outcomes following single-event multilevel surgery of the upper extremity for children with hemiplegic cerebral palsy.

BACKGROUND: Outcomes following single-event multilevel surgery of the upper extremity for children with cerebral palsy have not been well described in the literature. Since 1996, all children with hemiplegic cerebral palsy at our institution thought to be candidates for upper extremity surgery have had serial Shriners Hospital for Children Upper Extremity Evaluation performed for both clinical decision making and outcome assessment. The goal of the current study was to determine the functional outcomes, as described by the Shriners Hospital for Children Upper Extremity Evaluation, following single-event multilevel surgery of the upper extremity in children with hemiplegic cerebral palsy. METHODS: The study design was a retrospective, case-control series. The case group consisted of forty children with hemiplegic cerebral palsy who underwent upper-extremity single-event multilevel surgery. The control group consisted of twenty-six children with hemiplegic cerebral palsy who had not received any upper-extremity interventions. The spontaneous functional analysis, dynamic positional analysis, and grasp-release analysis sections of the Shriners Hospital for Children Upper Extremity Evaluation were compared between the two groups. RESULTS: The operative and nonoperative groups were comparable with respect to age (p = 0.09), sex (p = 0.97), initial spontaneous functional analysis scores (p = 0.37), dynamic positional analysis scores (p = 0.73), and grasp-release analysis scores (p = 0.16). For the single-event multilevel surgery group, significant improvements were noted for the mean spontaneous functional analysis score (p < 0.0001) and the mean dynamic positional analysis score (p < 0.0001), but not the mean grasp-release analysis score (p = 0.75). For the nonoperative control group, no significant changes were noted for the mean spontaneous functional analysis score (p = 0.89), the mean dynamic positional analysis score (p = 0.98), or the mean grasp-release analysis score (p = 0.36). Significant differences were noted between the single-event multilevel surgery and nonoperative control groups for the mean changes in the spontaneous functional analysis score (p = 0.01) and the mean change in the dynamic positional analysis score (p < 0.0001), but not the mean changes in the grasp-release analysis score (p = 0.56). CONCLUSIONS: Children with hemiplegic cerebral palsy showed significantly improved dynamic segmental alignment and, to a lesser degree, spontaneous use of the upper extremity following single-event multilevel surgery compared with a comparable nonoperative control group. However, the grasp-release ability did not significantly improve in either the operative or nonoperative group.

Surgical management of thumb deformity in children with hemiplegic-type cerebral palsy.

BACKGROUND: Evidence-based clinical decision-making for the surgical management of thumb deformities in children with cerebral palsy is difficult, primarily because of a lack of consensus with respect to assessment of thumb alignment and function. We have used the Shriners Hospital Upper Extremity Evaluation, a validated assessment tool, to determine the outcome after thumb reconstruction surgery in these children. The goals of this study were to determine the relationship between static and dynamic assessments of the thumb before surgery, the outcomes with respect to static and dynamic alignments of the thumb after soft tissue and skeletal surgery, and the relationship between preoperative spontaneous functional use of the involved extremity (indicative of the magnitude of neurologic impairment) and the change in thumb dynamic alignment after surgery. METHODS: A retrospective case series was performed, consisting of 33 children with hemiplegic-type cerebral palsy who had undergone surgical reconstruction of the thumb between 1998 and 2006. All children had preoperative and postoperative Shriners Hospital Upper Extremity Evaluation analyses performed (mean time to follow-up was 2 y and 2 mo). Static modified House scale of thumb alignment, dynamic thumb positional analysis, and the spontaneous functional use of the involved extremity were compared and contrasted. RESULTS: Dynamic thumb alignment was significantly worse than static thumb alignment (P=0.0005). Comparable improvements were achieved in both static and dynamic thumb alignment after surgical thumb reconstruction (P=0.6242). Optimal outcome was achieved more frequently in the static alignment (82% of cases) than in the dynamic alignment (61% of cases). There was poor correlation between the spontaneous use of the involved extremity before surgery and the changes in thumb dynamic alignment after surgery (Pearson correlation coefficient 0.1554, P=0.39). CONCLUSIONS: Static thumb alignment is not a good predictor of dynamic function, and interventions designed to improve function should focus on the assessment of dynamic thumb alignment. Improvements in both static and dynamic alignment of the thumb are possible after reconstructive surgery (consisting of muscle release, tendon transfer, and skeletal stabilization), regardless of the degree or density of underlying neurologic impairment.

Prosthetic management of children with unilateral congenital below-elbow deficiency.

BACKGROUND: There is substantial controversy concerning the prosthetic management of children with unilateral congenital below-elbow deficiency. The optimal age at the time of the initial fitting, the value of intensive prosthetic training, and the preferred prosthetic design for these children have not been established. METHODS: The outcomes of prosthetic management for 260 children with unilateral congenital below-elbow deficiency, treated between 1954 and 2004, were analyzed with respect to ongoing clinic attendance and self-reported prosthetic use. A successful prosthetic outcome was defined as a child and parents who continued to attend the limb-deficiency clinic and claimed at the time of the most recent follow-up that the prosthesis had been worn for any period of time. An unsuccessful prosthetic outcome was defined as a child and parents who were lost to follow-up or who claimed at the time of the most recent follow-up that the child never wore the prosthesis. Survival analysis was performed. RESULTS: An unsuccessful prosthetic outcome was noted for 127 children (49%). Initial fitting prior to the age of three years was associated with improved prosthetic outcome (p < 0.001). With the numbers studied, there was no additional benefit noted for fitting before one year of age (p = 0.60). Improved prosthetic outcomes were noted in children who had received intensive training at the time of fitting with an active terminal device (p = 0.005). Provision of a variety of prosthetic designs over the growing years was also associated with improved prosthetic outcome (p < 0.001). CONCLUSIONS: This study supports the initial prosthetic fitting for a child with unilateral congenital below-elbow deficiency prior to the age of three years, the provision of intensive training under the direction of an occupational therapist when an active terminal device is applied, and utilization of a variety of prosthetic designs over the child's years of growth. Further analysis of outcomes for the prosthetic management of these children will require more precise definitions of outcome in multiple domains and the development and validation of specific outcome instruments.

The Unilateral Below Elbow Test: a function test for children with unilateral congenital below elbow deficiency.

The Unilateral Below Elbow Test (UBET) was developed to evaluate function in bimanual activities for both the prosthesis wearer and non-wearer. Nine tasks were chosen for each of four age-specific categories defined by development stages of hand function (2-4y, 5-7y, 8-10y, and 11-21y). Two scales, Completion of Task and Method of Use, were designed to rate performance. To measure reliability, four occupational therapists scored samples of videotaped UBET performances. For Completion of Task, an interval scale, agreement in scoring was measured with interclass correlation coefficients (ICC; n=9; five females, four males). For Method of Use, a nominal scale, chance-adjusted association was calculated with Cohen's kappa coefficients (interobserver n=198; 111 females, 87 males; intraobserver n=93; 56 females, 37 males). For Completion of Task, the average ICC was 0.87 for the prosthesis-on condition, and 0.85 for the prosthesis-off condition. ICCs exceeded 0.80 for eight out of nine tasks for the two older age groups, but for only five out of nine tasks in the younger age groups. Higher inter- and intraobserver kappa coefficients for Method of Use resulted when scoring children with their prostheses on versus off. The oldest age group had lower kappa values than the other three groups. The UBET is recommended for the functional evaluation of Completion of Task in children with unilateral congenital below elbow deficiency with and without their prostheses. Method of Use scoring can evaluate individuals for directed therapy interventions or prosthetic training.

Validation of the Shriners Hospital for Children Upper Extremity Evaluation (SHUEE) for children with hemiplegic cerebral palsy.

BACKGROUND: The Shriners Hospital for Children Upper Extremity Evaluation (SHUEE) is a video-based tool for the assessment of upper extremity function in children with hemiplegic cerebral palsy. This tool includes spontaneous functional analysis and dynamic positional analysis and assesses the ability to perform grasp and release. The purpose of the present study was to assess the reliability, concurrent validity, and construct validity of this instrument. METHODS: The Shriners Hospital for Children Upper Extremity Evaluation studies for eleven subjects with hemiplegic cerebral palsy were selected for the evaluation of intraobserver and interobserver reliability. Concurrent validity was determined through analysis of the Shriners Hospital for Children Upper Extremity Evaluation, Pediatric Evaluation of Disability Inventory, and Jebson-Taylor Test of Hand Function scores for twenty children. Construct validity was determined through analysis of Shriners Hospital for Children Upper Extremity Evaluation scores for eighteen children before and after flexor carpi ulnaris to extensor carpi radialis brevis tendon transfer. RESULTS: The absolute mean differences between the two scoring sessions for three raters were 1.2 and 1.0 for the spontaneous functional analysis and the dynamic positional analysis, respectively. Although the mean differences were significantly different from 0 (p < 0.001 and p = 0.003), the differences were small and not clinically important with regard to the total possible score. There was excellent intraobserver reliability between the two sessions with regard to both spontaneous functional analysis (r = 0.99) and dynamic positional analysis (r = 0.98). Assessment of interobserver reliability revealed absolute mean differences between four raters of 3.8 and 3.7 for the spontaneous functional analysis and the dynamic positional analysis, respectively. These differences were significantly different from 0 (p < 0.001); however, the magnitudes of these differences were not important with regard to total score or clinical interpretation. There was excellent interobserver reliability for both the spontaneous functional analysis (r = 0.90) and the dynamic positional analysis (r = 0.89). There was 100% agreement within and between examiners for the grasp-and-release section. The Shriners Hospital for Children Upper Extremity Evaluation showed fair correlation with the self-care scaled score from the Pediatric Evaluation of Disability Inventory (r = 0.47) and good inverse correlation with the non-dominant total time section of the Jebson-Taylor test (r = -0.76). The Shriners Hospital for Children Upper Extremity Evaluation wrist score improved for all eighteen subjects after the flexor tendon transfer, and the mean improvement was significant (p < 0.001). CONCLUSIONS: The present study establishes the clinical reliability, concurrent validity, and construct validity of the Shriners Hospital for Children Upper Extremity Evaluation for the assessment of upper extremity function in children with hemiplegic cerebral palsy.