Shriners Hospital Research Articles

The SHOnet learning health system: Infrastructure for continuous learning in pediatric rehabilitation.

To describe the development and implementation of learning health system (LHS) infrastructure for a pediatric specialty care health system to support LHS research in pediatric rehabilitation settings. An existing pediatric common data model (eg, PEDSnet) of standardized medical terminologies for research was expanded and leveraged for this stud, and applied to SHOnet, a clinical research data resource consisting of deidentified data extracted from the electronic health record (EHR) from the Shriners Hospitals for Children speacialty pediatric health care system. We mapped EHR data for laboratory, procedures, drugs, and conditions to standardized vocabularies including ICD-10, CPT, RxNorm, and LOINC to the common data model using an established extraction-transformation-loading process. Rigorous quality checks were conducted to ensure a high degree of data conformance, completeness, and plausibility. SHOnet data elements from all sources are de-identified and the server is managed by the SHC Information Systems Department. SHOnet data are refreshed monthly and data elements are continually expanded based on new research endeavors. Not applicable. The Shriners Health Outcomes Network (SHOnet) includes data for over 10 000 distinct observational data elements based on over two million patient encounters between 2011 and present. The systematic process to develop SHOnet is replicable and flexible for other pediatric rehabilitation research settings interested in building out their LHS capabilities. Challenges and facilitators may arise for building such LHS infrastructure for rehabilitation in areas of (a) data capture, curation, query, and governance, (b) generating knowledge from data, and (c) dissemination and implementation of new institutional knowledge. Further research studies are needed to evaluate these data resources for scalable system-learning endeavors.SHOnet is an exemplar of an LHS for rehabilitation and specialty care settings. The success of an LHS is dependent on engagement of multiple stakeholders, shared governance, effective knowledge translation, and deep commitment to long-term strategies for engaging clinicians, administration, and families in leveraging knowledge to improve clinical outcomes.

Comparison of outcome between operative treatment and constraint-induced movement therapy for forearm and wrist deformities in cerebral palsy. A randomized controlled trial

Several treatment strategies for upper extremity deformities in cerebral palsy have been studied for their effectiveness in terms of function and quality of life. There are a few comparative studies between nonoperative and operative management for upper extremity deformities. This study aimed to compare the outcomes between conservative methods using constraint-induced movement therapy (CIMT) and operative methods using pronator teres rerouting, flexor carpi ulnaris transfer to extensor carpi radialis brevis (FCU transfer to ECRB) and fractional lengthening in cerebral palsy patients with forearm pronation and wrist flexion contracture. A total of 19 participants aged 7-15 years were randomized to either CIMT or surgery. The primary endpoint was changes in Shriners Hospital Upper Extremity Evaluation (SHUEE) functional score. Secondary endpoints comprised range of motion, Volkmann’s angle, and the Pediatric Outcomes Data Collection Instrument (PODCI). At 1-year follow-up, there was no significant difference between the nonoperative and operative groups in any SHUEE dimensions, range of motion or PODCI. However, there was significant improvement in Volkmann’s angle in the operative group compared to the CIMT group: CIMT = 11.11 ± 9.92, surgery = -13.00 ± 4.72: p = 0.04 (95% CI = 1.68-46.54). Operative treatment provided more improvement in wrist posture, represented by Volkmann’s angle, in cerebral palsy patients. However, SHUEE, range of motions and PODCI were comparable between the CIMT and operative groups.

Comparison of the kinematics, repeatability, and reproducibility of five different multi-segment foot models

BackgroundMulti-segment foot models (MFMs) for assessing three-dimensional segmental foot motions are calculated via various analytical methods. Although validation studies have already been conducted, we cannot compare their results because the experimental environments in previous studies were different from each other. This study aims to compare the kinematics, repeatability, and reproducibility of five MFMs in the same experimental conditions.MethodsEleven healthy males with a mean age of 26.5 years participated in this study. We created a merged 29-marker set including five MFMs: Oxford (OFM), modified Rizzoli (mRFM), DuPont (DFM), Milwaukee (MiFM), and modified Shriners Hospital for Children Greenville (mSHCG). Two operators applied the merged model to participants twice, and then we analysed two relative angles of three segments: shank-hindfoot (HF) and hindfoot-forefoot (FF). Coefficients of multiple correlation (CMC) and mean standard errors were used to assess repeatability and reproducibility, and statistical parametric mapping (SPM) of the t-value was employed to compare kinematics.ResultsHF varus/valgus of the MiFM and mSHCG models, which rotated the segment according to radiographic or goniometric measurements during the reference frame construction, were significantly more repeatable and reproducible, compared to other models. They showed significantly more dorsiflexed HF and plantarflexed FF due to their static offset angles. DFM and mSHCG showed a greater range of motion (ROM), and some models had significantly different FF points of peak angle.ConclusionsUnder the same conditions, rotating the segment according to the appropriate offset angle obtained from radiographic or goniometric measurement increased reliability, but all MFMs had clinically acceptable reliability compared to previous studies. Moreover, in some models, especially HF varus/valgus, there were differences in ROM and points of peak angle even with no statistical difference in SPM curves. Therefore, based on the results of this study, clinicians and researchers involved in the evaluation of foot and ankle dysfunction need an understanding of the specific features of each MFM to make accurate decisions.

Cause of Death Trends Among Persons With Spinal Cord Injury in the United States: 1960-2017

ObjectiveTo identify trends in causes of death after spinal cord injury (SCI) that could enhance understanding of why life expectancy after SCI has not improved in the last 3 decades. DesignCohort study. SettingTwenty-nine SCI Model Systems and 3 Shriners Hospitals. ParticipantsIndividuals with traumatic SCI (N=49,266) enrolled in the SCI Collaborative Survival Study Database between 1973 and 2017. InterventionsNot applicable. Main Outcome MeasuresAge-standardized cause-specific SCI mortality rates and 95% confidence intervals were calculated for 5 time intervals (1960-1979, 1980-1989, 1990-1999, 2000-2009, and 2010-2017). ResultsA total of 17,249 deaths occurred in 797,226 person-years of follow-up. Since 2010, the highest mortality rate was for respiratory diseases, followed by heart disease, cancer, infective and parasitic diseases (primarily septicemia), and unintentional injuries. Mortality rates for respiratory diseases, cancer, stroke, urinary diseases, and digestive diseases, initially decreased significantly but remained relatively stable since 1980, whereas essentially no progress occurred for infective and parasitic diseases. Mortality rates for heart disease, pulmonary embolus, and suicide decreased significantly throughout the entire study period, but were offset by increases in mortality rates for endocrine (primarily diabetes), nutritional, and metabolic diseases, as well as unintentional injuries. From 2010 to 2017, the overall age-standardized mortality rate was 3 times higher for individuals with SCI than the general population, ranging from 27% higher for cancer to 9 times higher for infective and parasitic diseases. ConclusionImproving life expectancy after SCI will require: (1) reducing mortality rates from respiratory diseases and septicemia that have remained high, (2) reversing current trends in diabetes and unintentional injury deaths, and (3) continuing to reduce mortality from heart disease and other leading causes.

Editorial introductions

Editorial introductions

Massive Pediatric Burn Injury: A 10-Year Review.

Advances in the care of burn-injured pediatric patients have improved mortality over the last 20 years. However, massive burn injuries (50% TBSA or greater) in pediatric patients, while overall rare, have a significant morbidity and mortality. The primary aim for this study is to analyze treatment and outcomes in massive pediatric burn injuries. A retrospective study of children with burn injuries 50% TBSA or greater who were admitted to Shriners Hospital for Children Northern California, from May 1, 2009 to May 22, 2020, was conducted. Data were collected from the electronic health records through a comprehensive chart review that included: patient demographics, past medical history, treatment interventions, and outcomes. This study included 69 patients (59.4% male) with a mean age of 8.7 ± 6 years. The median time from injury to admission was 2 (1-4) days. In this study, 63.8% of patients were from Mexico, 34.8% were from the United States, and 1% patient was from American Samoa. The median time from injury to admission was 2 (1-4) days. Mean TBSA was 66% ± 12%. The median TBSA of second-degree burns was 0 (0-6) %, and the mean TBSA of third-degree burns was 60% ± 16%. Forty percent of patients suffered an inhalation injury and 83% of patients received a tracheostomy. The median number of days requiring ventilator assistance was 26 (12-58) days. Mean length of hospitalization was 90 ± 60 days, with 61 ± 41 days spent in the intensive care unit. The mean number of surgical procedures was 6 ± 4. The time between surgical procedures was 12 ± 6 days. The median time from admission to the first surgical procedure was 1 (0-2) day. At the first procedure, a mean of 42% ± 15% TBSA of the burn injury was excised. Sixty-two percent of patients received autografting (22% ± 11% TBSA) and 52% of patients received allografting (27% ± 17% TBSA) during the first procedure. For survivors, the median number of inpatient occupational therapy encounters was 143.5 (83-215) and inpatient physical therapy encounters was 139.5 (81-215). Twenty-five percent of the patients included in this study died as a result of their burn injury. Multivariate regression revealed that sustaining an inhalation injury was a significant and independent predictor of death (odds ratio: 3.4, 95% confidence interval: 1.05 to 11, P = .04). Massive burn injuries in children required a very high number of surgical procedures and hospital resources. Most children who died as a result of their massive burn injury died within the first month of admission. Inhalation independently increases the risk of dying in pediatric patients with a massive burn injury.

The Global Research Trends and Hotspots on Developmental Dysplasia of the Hip: A Bibliometric and Visualized Study.

Background: Developmental dysplasia of the hip (DDH) is a common musculoskeletal disorder in newborns and also one of the most common causes of hip arthritis in women. Many topics concerning DDH still remain controversial, and the global research trend in this field has not been well-studied yet. The aim of the present study was to illustrate the overall knowledge structure, development trends, and research hotspots of DDH.Methods: The publications related to DDH from 1998 to 2020 were identified from the Web of Science Core Collection (WOSCC). Three bibliometric tools were used to conduct visualization and knowledge maps. Annual trends of publications, contributions of countries, institutions, authors, funding agencies and journals, and clustering of keywords were analyzed.Results: A total of 2,691 publications were included. The annual number of DDH publications showed an increasing trend worldwide. The United States has made the greatest contribution, with the largest number of publications and the highest H-index. The most prolific institutions were Shanghai Jiao Tong University, Children's Hospital of Philadelphia, and Shriners Hospital for Children. Professors Tönnis D, Harris WH, Crowe JF, Graf R, and Salter RB have made great achievements in this field. However, the collaboration between international institutions or researchers was relatively low and mainly conducted in European and American countries. All the keywords could be divided into five clusters: hip osteoarthritis study, hip replacement study, hip ultrasound study, osteotomy surgery study, and etiology study. A trend of balanced and diversified development existed in these clusters. Keywords with the ongoing bursts, including clinical outcome, risk factor, femoroacetabular impingement, predictor, arthroscopy, morphology, and anteversion may continue to be the research hotspots in the near future.Conclusions: There will be an increasing number of publications on DDH research, and the United States stay ahead in this field. International collaboration needs to be further strengthened. The information can provide helpful references for researchers to explore hot issues or target a specific field of DDH.

Autoencoder-derived Single Summary Metric to Assess Gait Quality

Research Objectives To improve holistic interpretation of quantitative gait analysis data through a deep learning autoencoder with a single-valued reduced representation. The tailored single value is another strategy to quantify overall gait quality in individuals with movement disorders. Such overall summary metrics provide a holistic assessment of gait quality for assessing outcomes of therapeutic interventions. Design Observational Study. Setting The data was collected at the Motion Analysis Centers (MACs) at the Shriners Hospitals for Children - Chicago. Participants 412 subjects with cerebral palsy under the age of 18 and 86 normal subjects (498 total) with collected gait data from the MAC at Shriners Hospitals for Children - Chicago over 20 years. Interventions Not applicable. Main Outcome Measures A single summary metric generated by an autoencoder model quantifies the quality of an individual's gait. This metric can be used as a measure of improvement of gait after surgery to improve mobility in conditions such as Cerebral Palsy. Results The model is trained using high-quality data at the Shriners MAC in Chicago from 2004 to 2020, which includes temporo-spatial parameters (7), lower extremity kinematic (64), and lower extremity kinetic (43) data - a total of 114 features. The learned single metric captures more than 86% variance, which was established using subject-wise cross-validation. Conclusions This preliminary model can produce an overall summary value for the gait of individuals by incorporating the temporo-spatial parameters, lower extremity kinematics, and lower extremity kinetic data. Such summary metrics can be used to assess the impact of therapeutic interventions. Author(s) Disclosures None. To improve holistic interpretation of quantitative gait analysis data through a deep learning autoencoder with a single-valued reduced representation. The tailored single value is another strategy to quantify overall gait quality in individuals with movement disorders. Such overall summary metrics provide a holistic assessment of gait quality for assessing outcomes of therapeutic interventions. Observational Study. The data was collected at the Motion Analysis Centers (MACs) at the Shriners Hospitals for Children - Chicago. 412 subjects with cerebral palsy under the age of 18 and 86 normal subjects (498 total) with collected gait data from the MAC at Shriners Hospitals for Children - Chicago over 20 years. Not applicable. A single summary metric generated by an autoencoder model quantifies the quality of an individual's gait. This metric can be used as a measure of improvement of gait after surgery to improve mobility in conditions such as Cerebral Palsy. The model is trained using high-quality data at the Shriners MAC in Chicago from 2004 to 2020, which includes temporo-spatial parameters (7), lower extremity kinematic (64), and lower extremity kinetic (43) data - a total of 114 features. The learned single metric captures more than 86% variance, which was established using subject-wise cross-validation. This preliminary model can produce an overall summary value for the gait of individuals by incorporating the temporo-spatial parameters, lower extremity kinematics, and lower extremity kinetic data. Such summary metrics can be used to assess the impact of therapeutic interventions.

Assessment of Longitudinal Changes in Manual Wheelchair Handrim Forces and Stroke Patterns in Children with Spinal Cord Injury

<h3>Research Objectives</h3> To identify changes in handrim kinetics and propulsive stroke patterns over time in pediatric manual wheelchair users. <h3>Design</h3> Longitudinal, prospective. <h3>Setting</h3> Motion Analysis Lab at Shriners Hospital for Children- Chicago. <h3>Participants</h3> Six pediatric manual wheelchair users with spinal cord injury. To be eligible, participants were required to be under 21 years of age during their first visit and use a manual wheelchair as their primary mode of mobility. <h3>Interventions</h3> Participants were evaluated in two separate data collection sessions, approximately one year apart. Participants propelled their wheelchair along a 15-meter, tile walkway at a self-selected speed and stroke pattern for multiple trials while kinetic and kinematic data were collected using a Vicon motion capture system and a Smartwheel affixed to their personal wheelchair. <h3>Main Outcome Measures</h3> Handrim peak force, variance of force, and stroke patterns. <h3>Results</h3> Over half of the participants increased their maximum rate of rise (F/t) of resultant force between visits. No trends were observed in the change in average peak resultant force per stroke cycle between visits, though one participant had a large decrease and another had a large increase. The variance of peak resultant force decreased notably in the three oldest participants and decreased in the three youngest participants. The number of types of stroke patterns used for propulsion and the frequency with which each pattern was used changed for all but one participant between visits. <h3>Conclusions</h3> Changes in handrim kinetics and stroke patterns were observed in most participants between visits. This is concerning, as some of these changes, such as decreased variance of peak resultant force and increased maximum rate of rise, have been correlated with shoulder pain and pathology in adults. Additional research using a larger cohort of pediatric manual wheelchair users is indicated to observe changes that could lead to musculoskeletal pain and pathology. <h3>Author(s) Disclosures</h3> None.

The Effects of Biological Sex on Glenohumeral Joint Motion, Force, and Moment Variability during Pediatric Manual Wheelchair Propulsion

Research Objectives To assess the biological sex-related differences in three-dimensional glenohumeral joint motion, force, and moment variability in pediatric manual wheelchair users with spinal cord injury. Design Cohort, prospective, cross-sectional. Setting Motion Analysis Lab at Shriners Hospital for Children - Chicago. Participants Nine female and 11 male pediatric manual wheelchair users with spinal cord injury (mean±SD age: 14±4.4 years). To be eligible, participants were required to be under 21 years of age and use a manual wheelchair as their primary mode of mobility. Interventions Participants propelled their personal wheelchair equipped with a Smartwheel using a self-selected speed and stroke pattern. Upper extremity kinematics and kinetics data were measured in a single experimental session. Main Outcome Measures Variance of three-dimensional glenohumeral joint motions, forces, and moments. Results Several biological sex-related differences in kinematic and kinetic variance were found, although no differences in glenohumeral joint kinematics or kinetics were observed. Male participants exhibited greater variance in glenohumeral joint adduction/abduction and flexion/extension ranges of motion, peak joint adduction and external rotation angles, and peak maximum anterior force and superior force (all p≤0.001). Conversely, females exhibited greater variance in peak glenohumeral joint flexion and abduction moments (both p < 0.001). Conclusions Biological sex-related differences in variability of glenohumeral joint motions, forces, and moments exist in pediatric manual wheelchair users with spinal cord injury. Female pediatric manual wheelchair users may be at an increased risk of shoulder pain and pathology due to the combination of decreased variability in glenohumeral joint motions and forces, potentially leading to increased repetitive strain injuries. Author(s) Disclosures No conflicts of interest to declare. To assess the biological sex-related differences in three-dimensional glenohumeral joint motion, force, and moment variability in pediatric manual wheelchair users with spinal cord injury. Cohort, prospective, cross-sectional. Motion Analysis Lab at Shriners Hospital for Children - Chicago. Nine female and 11 male pediatric manual wheelchair users with spinal cord injury (mean±SD age: 14±4.4 years). To be eligible, participants were required to be under 21 years of age and use a manual wheelchair as their primary mode of mobility. Participants propelled their personal wheelchair equipped with a Smartwheel using a self-selected speed and stroke pattern. Upper extremity kinematics and kinetics data were measured in a single experimental session. Variance of three-dimensional glenohumeral joint motions, forces, and moments. Several biological sex-related differences in kinematic and kinetic variance were found, although no differences in glenohumeral joint kinematics or kinetics were observed. Male participants exhibited greater variance in glenohumeral joint adduction/abduction and flexion/extension ranges of motion, peak joint adduction and external rotation angles, and peak maximum anterior force and superior force (all p≤0.001). Conversely, females exhibited greater variance in peak glenohumeral joint flexion and abduction moments (both p < 0.001). Biological sex-related differences in variability of glenohumeral joint motions, forces, and moments exist in pediatric manual wheelchair users with spinal cord injury. Female pediatric manual wheelchair users may be at an increased risk of shoulder pain and pathology due to the combination of decreased variability in glenohumeral joint motions and forces, potentially leading to increased repetitive strain injuries.

Multicenter Study of Pain in Osteogenesis Imperfecta

<h3>Research Objectives</h3> To investigate the frequency and type of pain by disease severity and the treatment of pain in individuals with osteogenesis imperfecta (OI), a genetic disorder and member of the rare disease family. <h3>Design</h3> Cross-sectional analysis. <h3>Setting</h3> Subjects were enrolled in a longitudinal multicenter study of OI. <h3>Participants</h3> The study included 558 individuals with a clinical or molecular diagnosis of OI, classified as OI type I (44%), type III (20%), type IV (27%), and other (9%). Median age was 12.4 years (0.0 - 67.2 years). <h3>Interventions</h3> N/A. <h3>Main Outcome Measures</h3> Rare Diseases Clinical Research Network, Brittle Bone Disorders Consortium: Clinical Assessment Instrument: presence, type of pain and pharmacological treatment used for pain relief. <h3>Results</h3> 62% of subjects reported pain (25% acute; 26% chronic; 11% acute & chronic). Acute pain did not change significantly across age groups (p=0.13), but the frequency of chronic pain increased with age, being highest in adults (p < 0.01). Acute pain was significantly more frequent in OI type III when compared with OI type I (p < 0.05). The prevalence of chronic pain did not vary significantly (p=0.69) across OI types. Pain was identified as a limitation to walking by 38% of individuals. Bisphosphonates (BPs) were the pharmacological treatment most frequently used (54% BP; 13% NSAIDs; 28% No treatment). Those with OI types III and IV receiving intravenous BPs treatment had less chronic pain compared with non-treated peers (p < 0.01). <h3>Conclusions</h3> A majority of individuals with OI experience pain. Chronic pain increases with age across all disease types while acute pain is more frequent in OI type III. The study underscores the need for additional research to address causes of pain, treatment effectiveness, and the impact of pain on mobility for individuals with OI. <h3>Author(s) Disclosuress</h3> The study was supported by 1) BBDC (1U54AR068069), a part of the NCATS RDCRN. BBDC is funded through ORDR, NCATS, NIAMS, NIDCR, and NICHD, 2) HHS/NIDILRR 90ARHF0006-01, 3) OREC at Marquette University, and 4) Shriners Hospitals for Children.

Survey of Functional Deficits in Pediatric Patients with Osteogenesis Imperfecta to Determine Rehabilitation Needs: A Retrospective Analysis

<h3>Research Objectives</h3> To investigate components of self-described functional deficits in pediatric patients with osteogenesis imperfecta to understand areas of need for rehabilitation in this population. <h3>Design</h3> A cohort of pediatric patients receiving ongoing OI care in outpatient settings or parents were surveyed using pediatric PROMIS scales across multiple domains. Children too young or unable had parents complete a proxy form. Surveys were repeated within one year to assess for consistency. Data were analyzed retrospectively. <h3>Setting</h3> Patients and families were surveyed during in Shriners Hospitals for Children ambulatory settings. This sample was of the Shriners Chicago patients who completed the PROMIS measures. <h3>Participants</h3> Patients with OI of varied phenotypes seeking care at Shriners Chicago able to complete a form in English or Spanish utilizing a provided tablet were surveyed. Initial results for 102 patients were obtained. <h3>Interventions</h3> Not applicable. <h3>Main Outcome Measures</h3> PROMIS pediatric scales for mobility, upper extremity function, pain interference and peer relations. Repeat surveys were conducted within one year. <h3>Results</h3> Mobility was moderate to severely impaired in 16/60 children, 18/30 parent proxy. Twelve children didn't have a mobility score, as they use a wheelchair for primary mobility. Pain interference was not severe in any patients, but moderate in 25/65 (38%) children and 6/32 (19%) parent proxy. Half of children self-reported mild or no pain interference. Peer relations were mildly or not affected in over 88% (90/102). Upper extremity impairment was moderately to severely affected in about half of the respondents (53/102) with higher impairment by parent proxy than child self-report. <h3>Conclusions</h3> OI is typically characterized by bone fragility, however muscle weakness is a common presenting symptom and muscle abnormalities in this population have been widely described. As 45% of children have reported issues in our cohort with gait/mobility issues or primarily use a wheelchair, anticipatory guided approach to rehabilitation and planning in conjunction with bone-modifying treatment, may improve strength and mobility outcomes. Further study across other Shriners centers is planned. This data may serve as a baseline to assess outcomes of developed programs. <h3>Author(s) Disclosures</h3> N/A.

Validity and reliability of "Shriners Hospital for Children Upper Extremity Evaluation" in children with rheumatic diseases.

The aim of this study was to investigate the validity and reliability of "Shriners Hospital for Children Upper Extremity Evaluation (SHUEE)" for children with rheumatic diseases. The study was carried out after obtaining the necessary permissions and retrospectively registered. The psychometric properties evaluated were reliability and concurrent validity. Reliability was determined by intra- and inter-observer agreement. Concurrent validity was performed using the Jebsen Taylor Hand Function Test (JTHFT), Abilhand-Rheumatoid Arthritis (Abilhand-RA), and Children Health Assessment Questionnaire (CHAQ). The validity and reliability of the evaluation were determined after the retest 1week later. Twenty children with rheumatic diseases were participated in to study. Intraclass coefficients ranged from 0.82 to 0.97 and the intraobserver reliability for SHUEE total and subscales were considered "excellent." Interobserver reliability was considered "excellent" for the SHUUE total score, spontaneous functional analysis and dynamic positional analysis, and "moderate" for grasp-release. A moderate negative correlation was determined between Spontaneous Functional Analysis and JTHFT (r = - 0.63; p = 0.003). SHUEE is a valid and reliable evaluation for children with rheumatic diseases. ClinicalTrials.org NCT04685434/21.12.2020 Key Points • SHUEE tends to be appropriate and acceptable to children with rheumatic diseases. • SHUEE can be used safely in the pediatric rheumatology group and it is beneficial in the clinical decision-making process. • SHUEE is a pioneering performance test that evaluates the quality of movement in pediatric rheumatology on a joint basis.

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

BackgroundArthrogryposis 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.ObjectiveThe 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.MethodsYouths 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.ResultsOf 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).ConclusionsThe 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

Evaluation of a Customized 3D Printed ORGAN-Hand Orthotic Device for Unilateral Cerebral Palsy: a Pilot Study.

To achieve intensive activity-based and goal-directed rehabilitation for unilateral cerebral palsy (UCP), several static and functional upper limb orthoses have been used but with limited robust evidence-base. The current pilot study evaluated the feasibility and efficacy of a customized 3D-printed orthotic device in children with UCP. The attainment of a prespecified goal and Shriners Hospital Upper Extremity Evaluation (SHUEE) at 3 and 6 mo were the efficacy measures. Of the 14 screened children, 5 (median age: 7.9 y; 3 boys) were included. The 3-mo follow-up could be completed for 3 children while 6-mo follow-up could be completed for 1 child. Rest could not be assessed due to pandemic restrictions. Although none attained set goals till the last follow-up, all 3 children (at 3-mo follow-up) showed improvement in SHUEE scores without any significant safety concerns. Further studies on 3D-printed orthosis in UCP are the need of the hour.

Missing and unerupted teeth in osteogenesis imperfecta.

Osteogenesis imperfecta (OI) is a genetic disorder characterized by bone fragility and craniofacial and dental abnormalities such as congenitally missing teeth and teeth that failed to erupt which are believed to be doubled in OI patients than normal populations and were associated with low oral health quality of life. However, the etiology of these abnormalities remains unclear. To understand the factors influencing missing and unerupted teeth, we investigated their prevalence in a cohort of OI patients as a function of the clinical phenotype (OI type), the genetic variant type, the tooth type and the onset of bisphosphonate treatment. A total of 144 OI patients were recruited from The Shriners Hospital, Montreal, Canada, between 2016 and 2017. Patients were evaluated using intraoral photographs and panoramic radiographs. Missing teeth were evaluated in all patients, and unerupted teeth were assessed only in patients ≥15years old (n=82). On average, each OI patient had 2.4 missing teeth and 0.8 unerupted teeth, and the most common missing and unerupted teeth were the premolars and the upper second molars, respectively. These phenomena were more prominent in OI type III and IV than in OI type I, and were not sex or age-related. Missing teeth were significantly more common in patients with C-propeptide variants than all other variants (p-value <0.05). Unerupted teeth were significantly more common in patients with α1 and α2 glycine variants or substitutions than in those with haploinsufficiency variants. Early-onset of bisphosphonate treatment would significantly increase the risk of unerupted teeth in patients with OI types III and IV (OR=1.68, 95% CI (1.15-1.53)). The prevalence of missing and unerupted teeth at the tooth type level in OI patients varies according to the nature of the collagen variants and the OI type. These findings highlight the role of collagen in tooth development and eruption.

The modified Shriners Hospitals for Children Greenville (mSHCG) multi-segment foot model provides clinically acceptable measurements of ankle and midfoot angles: A dual fluoroscopy study

BackgroundSeveral multi-segment foot models have been developed to evaluate foot and ankle motion using skin-marker motion analysis. However, few multi-segment models have been evaluated against a reference standard to establish kinematic accuracy. Research questionHow accurately do skin-markers estimate foot and ankle motion for the modified Shriners Hospitals for Children Greenville (mSHCG) multi-segment foot model when compared against the reference standard, dual fluoroscopy (DF), during gait, in asymptomatic participants? MethodsFive participants walked overground as full-body skin-marker trajectory data and DF images of the foot and shank were simultaneously acquired. Using the mSHCG model, ankle and midfoot angles were calculated throughout stance for both motion analysis techniques. Statistical parametric mapping assessed differences in joint angles and marker positions between skin-marker and DF motion analysis techniques. Paired t tests, and linear regression models were used to compare joint angles and range of motion (ROM) calculated from the two techniques. ResultsIn the coronal plane, the skin-marker model significantly overestimated ROM (p = 0.028). Further, the DF model midfoot ROM was significantly positively related to differences between DF and skin-marker midfoot angles (p = 0.035, adjusted R2 = 0.76). In the sagittal plane, skin-markers underestimated ankle angles by as much as 7.26°, while midfoot angles were overestimated by as much as 9.01°. However, DF and skin-marker joint angles were not significantly different over stance. Skin-markers on the tibia, calcaneus, and fifth metatarsal had significantly different positions than the DF markers along the direction of walking for isolated portions that were less than 10 % of stance. Euclidean distances between DF and skin-markers positions were less than 9.36 mm. SignificanceAs the accuracy of the mSHCG model was formerly unknown, the results of this study provide ranges of confidence for key angles calculated by this model.

The Dynamic Thumb-in-Palm Pattern in Children with Spastic Cerebral Palsy and Its Effects on Upper Limb Function.

The thumb-in-palm (TIP) pattern is one of the most common upper limb deformities in cerebral palsy (CP). This study was designed to investigate the effect of the dynamic TIP pattern on upper limb function in children with spastic CP. This prospective observational study included a total of 106 children with CP with dynamic TIP. The House TIP classification while grasping small or large objects, Melbourne Assessment of Unilateral Upper Limb Function (MUUL), Shriners Hospital Upper Extremity Evaluation (SHUEE), Zancolli classification for wrist–finger flexor deformity, and degree of swan neck deformity were assessed. Type I was the most common and highest functioning House TIP classification type. However, there were no significant differences in upper arm function between types II, III, and IV. The three components of the SHUEE showed stronger association with MUUL than House TIP and Zancolli classifications. After multivariable analysis, functional use of the wrist–finger and the thumb played a more significant role than the dynamic alignment of the thumb. In conclusion, the House TIP classification is useful to describe the TIP pattern. The SHUEE thumb assessment is a useful tool for reflecting upper arm function. The upper arm function was related more with the associated wrist flexor deformity than dynamic TIP.

Validity and reliability of the Turkish versions of Assessment of Children's Hand Skills and Children's Hand-Skills Ability Questionnaire in children with hemiplegic cerebral palsy.

Assessment of Children's Hand Skills (ACHS) and Children's Hand-Skills Ability Questionnaire (CHSQ) are interconnected hand skills assessment tools that together contain parent questionnaire and observational assessment. With this quality, ACHS and CHSQ enable the therapist to obtain information about the child's real-life performances as well as to conduct a clinical observational assessment. The purpose of this study was to investigate the validity and reliability of the Turkish versions of ACHS and CHSQ in children with hemiplegic cerebral palsy (HCP). A total of 112 children with HCP between 2 and 12 years of age were included. All participants were subjected to ACHS, CHSQ, Shriners Hospital Upper Extremity Evaluation (SHUEE) and ABILHAND-Kids. Convergent construct validity was investigated through analysing the relationship between ACHS and SHUEE and between CHSQ and ABILHAND-Kids. Discriminative construct validity was investigated through analysing the differences between genders for CHSQ and ACHS. For reliability, test-retest interclass correlation coefficient (ICC) and internal consistency Cronbach's alpha were calculated and analysed. ACHS showed very strong relationships with SHUEE's spontaneous functional analysis (r = 0.86) and grip-release function (r = 0.86) parameters with a strong relationship with positional dynamic analysis (r = 0.78). CHSQ's leisure (r = 0.80), school/education (r = 0.75) and activities of daily living (r = 0.76) domains showed strong relationships with the ABILHAND-Kids. There was no difference between genders for ACHS and all domains of CHSQ (p > 0.05). All domains and total score of ACHS and CHSQ had perfect test-retest reliability (ICC > 0.90). ACHS had perfect internal consistency (Cronbach's α = 0.98); CHSQ had very high internal consistency (Cronbach's α = 0.84). The Turkish versions of ACHS (ACHS-TR) and CHSQ (CHSQ-TR) are valid and reliable hand skills assessment tools in children with HCP.

Analysis of dynamic elbow flexion deformity in children with hemiplegic cerebral palsy.

BackgroundCerebral palsy affects 1 per 1.000 children, and in 83% of the cases upper extremity is involved. Dynamic elbow flexion deformity is a movement disorder observed in individuals with hemiparesis secondary to cerebral palsy. We sought to determine whether children with hemiplegic cerebral palsy exhibit dynamic elbow flexion deformity during daily activities and its influence to reaching function. MethodsSixteen children with upper limb hemiparesis and cerebral palsy (age 11y 7mo (SD 3y 2mo); 11 boys, 5 girls; Gross Motor Function Classification System level I or II) were included in this observational descriptive study. Manual Ability Classification System, Children's Hand-use Experience Questionnaire and Shriners Hospital for Children Upper Extremity Evaluation were used to evaluate affected upper extremity function. Spasticity was assessed with Modified Ashworth scale. Involuntary elbow flexion was recorded in eight daily activities. Elbow motion during reach function was measured. FindingsFifteen out of 16 individuals showed dynamic elbow flexion deformity. There was a significative increase of involved median elbow flexion in all the activities studied, except for “high speed stairs climbing” evaluation. Children's Hand-use Experience Questionnaire showed that children were independent in most of the daily activities (21 out of 29). Correlation between dynamic elbow flexion deformity and Shriners Hospital for Children Upper Extremity Evaluation was observed only in sitting-standing activity (Spearman's ρ 0.549, P = 0.028). InterpretationDynamic elbow flexion deformity is very common in hemiplegic cerebral palsy and occurs proportionally to the degree of the effort demanded by the activities. Despite of presenting this movement disorder, it does not affect in children participation in daily activities.