Identifying and Evaluating Young Children with Developmental Central Hypotonia: An Overview of Systematic Reviews and Tools.

Children with developmental central hypotonia have reduced muscle tone secondary to non-progressive damage to the brain or brainstem. Children may have transient delays, mild or global functional impairments, and the lack of a clear understanding of this diagnosis makes evaluating appropriate interventions challenging. This overview aimed to systematically describe the best available evidence for tools to identify and evaluate children with developmental central hypotonia aged 2 months to 6 years. A systematic review of systematic reviews or syntheses was conducted with electronic searches in PubMed, Medline, CINAHL, Scopus, Cochrane Database of Systematic Reviews, Google Scholar, and PEDro and supplemented with hand-searching. Methodological quality and risk-of-bias were evaluated, and included reviews and tools were compared and contrasted. Three systematic reviews, an evidence-based clinical assessment algorithm, three measurement protocols, and two additional measurement tools were identified. For children aged 2 months to 2 years, the Hammersmith Infant Neurological Examination has the strongest measurement properties and contains a subset of items that may be useful for quantifying the severity of hypotonia. For children aged 2-6 years, a clinical algorithm and individual tools provide guidance. Further research is required to develop and validate all evaluative tools for children with developmental central hypotonia.

Supported standing and stepping device use in young children with cerebral palsy, gross motor function classification system III, IV and V: a descriptive study.

This study described and compared use of supported-standing and stepping devices by young children with cerebral palsy, Gross-Motor Function Classification System (GMFCS) levels III-V following power mobility introduction. Data was collected at two time-points, 5-6 months apart, for 42 participants, aged 18-80 months, using the Home Use of Technology for CHildren (HUTCH). Supported-standing and stepping device choice, and time in each device remained stable over 6 months. Associations between device use and three functional classifications were examined. Children with more impaired motor, postural and manual abilities were more likely to use a supine stander rather than a prone/upright stander or no stander. Children at GMFCS V tended to use hands-free stepping devices, while support-arms stepping devices were more common for children at GMFCS IV. Only children at GMFCS III used convertible stepping devices. Using power mobility, standers and supported-stepping devices was feasible and 19/34 classified at GMFCS IV/V used all three devices over 6 months. A key finding was that introduction of power mobility did not reduce use of supported-stepping devices at any GMFCS level. Use of multiple upright positioning and mobility devices may assist children with limited mobility to be actively engaged and participate in daily life.

Supported-standing interventions for children and young adults with non-ambulant cerebral palsy: A scoping review.

AIM: To describe the evidence, outcomes, and lived experience of supported standing for children and young adults with cerebral palsy aged 25 years or younger, classified in Gross Motor Function Classification System levels IV and V. METHOD: This scoping review included searches in eight electronic databases and manual searching from database inception to May 2020 and updated on 21st February 2022. Two of three reviewers independently screened titles and abstracts and extracted and appraised data. Methodological quality and risk of bias were appraised using tools appropriate to study type. Content analysis and frequency effect sizes were calculated for qualitative and descriptive evidence. RESULTS: From 126 full-text references, 59 citations (one study was reported over two citations) were included: 16 systematic reviews, 17 intervention studies reporting over 18 citations, eight analytical cross-sectional studies, five descriptive cross-sectional/survey studies, five qualitative studies, and one mixed-methods study were identified, along with six clinical guidelines. Maintenance of bone mineral density and contracture prevention outcomes were supported by the most experimental studies and evidence syntheses, while evidence supporting other outcomes was primarily quasi-experimental or descriptive. Qualitative evidence suggests that programmes are influenced by attitudes, device, child, and environmental factors. INTERPRETATION: Individualized assessment and prescription are essential to match personal and environmental needs. Although experimental evidence is limited due to many factors, lived-experience and cohort data suggest that successful integration of standing programmes into age-appropriate and meaningful activities may enhance function, participation, and overall health. WHAT THIS PAPER ADDS: Supported-standing interventions may provide an important psychosocial and physical change of position. Supported standing is not passive for those classified in Gross Motor Function Classification System level IV or V. Supported standing may enhance social participation, functional abilities, and fitness. Children need choice in where and when to stand.

Tool Use for Early Detection of Cerebral Palsy: A Survey of Spanish Pediatric Physical Therapists.

PURPOSE: The purpose of this study was to assess the use of diagnostic assessment tools in pediatric physical therapy practice in Spain. Best practice recommendations indicate the timely use of key assessment tools to reduce the age of diagnosis of cerebral palsy (CP). METHODS: Pediatric physical therapists currently working in Spain in early intervention were recruited through targeted physical therapy entities. They were invited to complete the purpose-developed electronic survey, consisting of 45 multiple-choice questions, with 5 thematic blocks. RESULTS: Results from 140 anonymous respondents were analyzed. The average reported age when CP was suspected was 12.6 months. Most used the child's clinical history (88.1%), the Alberta Infant Motor Scale (41.3%), and Vojta Assessment Procedure (32.1%) to assess and detect CP. General Movements Assessment (25.7%) and Hammersmith Infant Neurological Examination (28.4%) were used infrequently. CONCLUSIONS: Currently, pediatric physical therapists in Spain rely on clinical history and outdated tools to identify children with CP.Digital Abstract available at: http://links.lww.com/PPT/A361 (English).Digital Abstract available at: http://links.lww.com/PPT/A362 (Spanish).

Evidence-informed clinical perspectives on postural management for hip health in children and adults with non-ambulant cerebral palsy.

Postural management is a multi-disciplinary approach incorporating a comprehensive schedule of daily and night-time positions, equipment and physical activity to help maintain or improve body structures and function and increase activity and participation. Postural management may play a role in preventing contracture, deformity, pain, and asymmetry. This article provides an overview of the evidence supporting use of postural management to positively influence hip health in individuals with cerebral palsy, functioning as Gross Motor Classification System (GMFCS) levels IV or V. Sitting or lying without changing position for more than 8 hours, unsupported supine lying and asymmetrical or windswept postures are associated with pain and hip subluxation/dislocation. Although high-quality experimental research is still limited by many factors, there is limited evidence of harm, and most individuals at GMFCS IV or V require positioning supports to enable participation and function and ease caregiving. Clinical recommendations combining research and clinical opinion support the early use of comfortable positioning routines and/or equipment to reduce time spent in sustained asymmetrical or potentially harmful sitting and lying positions. Supported standing, active weightbearing and stepping are recommended to promote active movement and position change when possible, depending on individual, family and caregiver routines and preferences.

Inclination, hip abduction, orientation, and tone affect weight-bearing in standing devices.

PURPOSE: With children who are unable to stand or walk independently in the community, therapists commonly use standing devices to assist lower-extremity weight-bearing which is important for bone and muscle health. In addition, positioning in hip abduction may improve hip stability and range of motion. This is the first study to explore the effect of angle of inclination, hip abduction, body orientation, and tone on weight-bearing in pediatric standing devices. METHODS: This descriptive exploratory study used a convenience sample of 15 participants (2 with normal tone, 5 with generalized hypotonia, and 8 with hypertonia) (mean age of 5 years and 10 months, range of 3 years 4 months to 9 years 7 months); 13 of whom used standing devices at home, as well as 2 typically developing siblings (normal tone). Each child stood in 36 positions to measure the amount of weight-bearing through footplates. RESULTS: Weight-bearing was highest with 60 degrees of abduction and no inclination (upright) in supine positioning for children with low and normal tone. Children with high muscle tone bore most weight through their feet with no abduction (feet together) and no inclination (upright) in prone positioning. Overall, supine positioning resulted in more weight-bearing in all positions for children with low and normal tone. Prone positioning resulted in slightly more weight-bearing in all positions for children with high tone. CONCLUSIONS: Weight-bearing was affected by all three of the variables (inclination, abduction, and orientation) for participants with high, normal, and low tone. To determine optimal positioning, all standers should include a system to measure where and how much weight-bearing is occurring in the device.

Stander Use for an Adolescent with Cerebral Palsy at GMFCS Level with Hip and Knee Contractures.

Aims: Adolescents with cerebral palsy (CP) report high rates of pain and contractures. Standing may positively affect these outcomes, yet there are no published studies on how to accommodate contractures or when a stander is no longer beneficial. This is the first study that answers the question: Can an adolescent with CP and 40-degree knee and/or hip flexion contractures benefit from a standing program, after being non-weight bearing for many years? Methods: A 16-year-old male with spastic tetraplegic CP, Gross Motor Function Classification System (GMFCS) level V, used a stander with a "knee and hip contracture system." Knee and hip extension, as well as upright inclination, were increased weekly. Knee and hip passive range of motion (PROM) measurements, pain scale, and a parent survey were completed at baseline, 7 and 15 months. Results: After 15 months of stander use, both hips and left knee PROM improved. The subject's activity and participation increased, while pain, suppository use, and time spent on bowel care decreased. Conclusion: An adolescent with 40-degree knee and hip flexion contractures may be positioned well in a stander with a specialized contracture system. PROM, bowel function, pain, activity, and participation may improve for some adolescents at GMFCS level V, through use of a stander.

Interventions to improve sensory and motor outcomes for young children with central hypotonia: A systematic review.

OBJECTIVE: To evaluate evidence supporting physical and occupational therapy interventions used to improve sensory and motor outcomes for children 0-6 years with central hypotonia. METHODS: Four electronic databases were searched from 1996 to March 2017. Level of evidence and study conduct was evaluated using American Academy of Cerebral Palsy and Developmental Medicine criteria. Traffic lighting classification identified interventions that were green (proven effective), yellow (possibly effective) or red (proven ineffective or contraindicated). RESULTS: Thirty-seven articles were included. Nine studies measured orthotic interventions while four distinct studies published over nine articles measured treadmill interventions. Remaining studies measured impact of compression garments, massage, motor and sensori-motor interventions, positioning and mobility interventions. CONCLUSIONS: Green light evidence supports treadmill training (to promote ambulation and gait characteristics) and massage (to positively affect muscle tone, motor development and use of vision) for infants with Down syndrome. These interventions are considered Yellow (possibly effective) for other populations. Green light evidence supports impact of orthoses on foot alignment for ambulatory children with hypotonia, while impact on gait characteristics is Yellow light and motor development may be negatively impacted (Red light) in pre-ambulatory children. All other interventions rated Yellow (possibly effective) and therapists should monitor using sensitive outcome measures.

Use of a dynamic gait trainer for a child with thoracic level spinal cord injury.

Paediatric spinal cord injury (SCI) can result in permanent mobility impairment with consequences for activity, participation and quality of life. This case documents the effect of an overground supported stepping intervention using a dynamic gait trainer. To our knowledge, there are no published studies on this intervention for children with SCI and similar interventions have only been reported in children at American Spinal Injury Association Impairment Scale (AIS) levels B and C.A child with a T10 (thoracic level, vertebra 10), AIS level A injury, sustained at 2 years of age, continued to make gains in all areas including participation, activity, body structure and function over the following 4 years. Use of a dynamic gait trainer improved the participant's ability to be active and participate despite lack of further neuromuscular recovery. This novel approach with a commonly available device allowed the child to be active and participate in the absence of neural recovery.

Physical risk factors influencing wheeled mobility in children with cerebral palsy: a cross-sectional study.

BACKGROUND: There is a lack of understanding of the factors that influence independent mobility and participation in meaningful activities. The purpose of this study was to analyse physical factors influencing independent use of manual and power wheelchairs in a total population of children with cerebral palsy (CP). METHODS: A cross-sectional study based on the most recent examination of all children with CP, born 2002-2013, reported into the Swedish cerebral palsy registry (CPUP), from January 2012 to June 2014. There were 2328 children (58 % boys, 42 % girls), aged 0-11 years, at all levels of gross motor function and hand function. Hazard ratios adjusted for age and sex were used to calculate the risk for not being able to self-propel based on Gross Motor Function Classification System (GMFCS) levels, upper extremity range of motion and hand function including Manual Ability Classification System (MACS), House functional classification system, Thumb-in-palm deformity, Zancolli (spasticity of wrist/finger flexors) and bimanual ability. RESULTS: In total 858 children used wheelchairs outdoors (692 manual, 20 power, 146 both). Only 10 % of the 838 children self-propelled manual wheelchairs, while 90 % were pushed. In contrast 75 % of the 166 children who used power mobility outdoors were independent. Poor hand function was the greatest risk factor for being unable to self-propel a manual wheelchair, while classification as GMFCS V or MACS IV-V were the greatest risk factors for not being able to use a power wheelchair independently. CONCLUSIONS: The majority of children with CP, aged 0-11 years did not self-propel manual wheelchairs regardless of age, gross motor function, range of motion or manual abilities. Power mobility should be considered at earlier ages to promote independent mobility for all children with CP who require a wheelchair especially outdoors.

Comparison of the inertial properties and forces required to initiate movement for three gait trainers.

The purpose of this study was to evaluate the inertial properties and forces required to initiate movement on two different surfaces in a sample of three commonly prescribed gait trainers. Tests were conducted in a laboratory setting to compare the Prime Engineering KidWalk, Rifton Pacer, and Snug Seat Mustang with and without a weighted anthropometric test dummy configured to the weight and proportions of a 4-year-old child. The Pacer was the lightest and the KidWalk the heaviest while footprints of the three gait trainers were similar. Weight was borne fairly evenly on the four casters of the Pacer and Mustang while 85% of the weight was borne on the large wheels of the mid-wheel drive KidWalk. These differences in frame style, wheel, and caster style and overall mass impact inertial properties and forces required to initiate movement. Test results suggest that initiation forces on tile were equivalent for the Pacer and KidWalk while the Mustang had the highest initiation force. Initiation forces on carpet were lowest for the KidWalk and highest for the Mustang. This initial study of inertia and movement initiation forces may provide added information for clinicians to consider when selecting a gait trainer for their clients.

Systematic review and clinical recommendations for dosage of supported home-based standing programs for adults with stroke, spinal cord injury and other neurological conditions.

BACKGROUND: Sitting for more than 8 h a day has been shown to negatively impact health and mortality while standing is the recommended healthier alternative. Home-based standing programs are commonly recommended for adults who cannot stand and/or walk independently. The aim of this systematic review is to review effectiveness of home-based standing programs for adults with neurological conditions including stroke and spinal cord injury; and to provide dosage guidelines to address body structure and function, activity and participation outcomes. METHODS: Eight electronic databases were searched, including Cochrane Library databases, MEDLINE, CINAHL and EMBASE. From 376 articles, 36 studies addressing impact of a standing intervention on adults with sub-acute or chronic neurological conditions and published between 1980 and September 2015 were included. Two reviewers independently screened titles, reviewed abstracts, evaluated full-text articles and rated quality and strength of evidence. Evidence level was rated using Oxford Centre for Evidence Based Medicine Levels and quality evaluated using a domain-based risk-of-bias rating. Outcomes were divided according to ICF components, diagnoses and dosage amounts from individual studies. GRADE and the Evidence-Alert Traffic-Lighting system were used to determine strength of recommendation and adjusted in accordance with risk-of-bias rating. RESULTS: Stronger evidence supports the impact of home-based supported standing programs on range of motion and activity, primarily for individuals with stroke or spinal cord injury while mixed evidence supports impact on bone mineral density. Evidence for other outcomes and populations is weak or very weak. CONCLUSIONS: Standing should occur 30 min 5 times a week for a positive impact on most outcomes while 60 min daily is suggested for mental function and bone mineral density.

Outcomes of gait trainer use in home and school settings for children with motor impairments: a systematic review.

OBJECTIVE: To summarize and critically appraise evidence regarding use of gait trainers (walkers providing trunk and pelvic support) at home or school with children who are unable to walk independently or with hand-held walkers. DATA SOURCES: Searches were performed in seven electronic databases including EBM Reviews, CINAHL, Medline and EMBASE for publications in English from database inception to November 2014. REVIEW METHODS: Included studies involved at least one child with a mobility limitation and measured an outcome related to gait trainer use. Articles were appraised using American Academy of Cerebral Palsy and Developmental Medicine criteria for group and single-subject designs and quality ratings completed for studies rated levels I-III. The PRISMA statement was followed with inclusion criteria set a priori. Two reviewers independently screened titles, abstracts and full-text articles. RESULTS: Seventeen studies involving 182 children were included. Evidence from one small randomized controlled trial suggests a non-significant trend toward increased walking distance while the other evidence level II study (concurrent multiple baseline design) reports increased number of steps. Two level III studies (non-randomized two-group studies) report statistically significant impact on mobility level with one finding significant impact on bowel function and an association between increased intervention time and bone mineral density. Remaining descriptive level evidence provides support for positive impact on a range of activity outcomes, with some studies reporting impact on affect, motivation and participation with others. CONCLUSIONS: Evidence supporting outcomes for children using gait trainers is primarily descriptive and, while mainly positive, is insufficient to draw firm conclusions.

Practice considerations for the introduction and use of power mobility for children.

AIM: The aim of the study was to support clinicians in recommending and justifying power mobility for children of different ages and abilities, and with different needs. The study comprised three distinct parts: a literature review; a Delphi consensus; and clinical practice considerations. METHOD: A scoping review of eight electronic databases and manual searches carried out in February 2011 identified 15 themes or transferable messages among 27 articles meeting initial inclusion criteria and these formed the basis of a draft paper. Informal consensus at two international conference presentations refined and modified the paper to include 10 messages supported by 24 articles. The literature review was updated in May 2012 and a modified Delphi process sought to formalize the consensus process with an international panel of 16 expert clinicians and researchers using a priori criteria of 80% agreement. RESULTS: Evidence from studies was classified using the American Academy of Cerebral Palsy and Developmental Medicine guidelines, with evidence from most studies being classified as either level IV or level V, apart from one study each with evidence classified as level II and level III. Expert consensus on the content and wording of nine transferable messages may raise evidence overall to level III. INTERPRETATION: This paper suggests that power mobility may reasonably be considered as an effective and appropriate intervention for children lacking efficient, independent mobility from around 12 months of age including children who may never become competent drivers and children lacking independent mobility only in early childhood.

Systematic review and evidence-based clinical recommendations for dosing of pediatric supported standing programs.

PURPOSE: There is a lack of evidence-based recommendations for effective dosing of pediatric supported standing programs, despite widespread clinical use. METHODS: Using the International Classification of Functioning, Disability, and Health (Child and Youth Version) framework, we searched 7 databases, using specific search terms. RESULTS: Thirty of 687 studies located met our inclusion criteria. Strength of the evidence was evaluated by well-known tools, and to assist with clinical decision-making, clinical recommendations based on the existing evidence and the authors' opinions were provided. CONCLUSIONS AND RECOMMENDATIONS FOR CLINICAL PRACTICE: Standing programs 5 days per week positively affect bone mineral density (60 to 90 min/d); hip stability (60 min/d in 30° to 60° of total bilateral hip abduction); range of motion of hip, knee, and ankle (45 to 60 min/d); and spasticity (30 to 45 min/d).

A systematic review of supported standing programs.

OBJECTIVE: The routine clinical use of supported standing in hospitals, schools and homes currently exists. Questions arise as to the nature of the evidence used to justify this practice. This systematic review investigated the available evidence underlying supported standing use based on the Center for Evidence-Based Medicine (CEBM) Levels of Evidence framework. DESIGN: The database search included MEDLINE, CINAHL, GoogleScholar, HighWire Press, PEDro, Cochrane Library databases, and APTAs Hooked on Evidence from January 1980 to October 2009 for studies that included supported standing devices for individuals of all ages, with a neuromuscular diagnosis. We identified 112 unique studies from which 39 met the inclusion criteria, 29 with adult and 10 with pediatric participants. In each group of studies were user and therapist survey responses in addition to results of clinical interventions. RESULTS: The results are organized and reported by The International Classification of Function (ICF) framework in the following categories: b4: Functions of the cardiovascular, haematological, immunological, and respiratory systems; b5: Functions of the digestive, metabolic, and endocrine systems; b7: Neuromusculoskeletal and movement related functions; Combination of d4: Mobility, d8: Major life areas and Other activity and participation. The peer review journal studies mainly explored using supported standers for improving bone mineral density (BMD), cardiopulmonary function, muscle strength/function, and range of motion (ROM). The data were moderately strong for the use of supported standing for BMD increase, showed some support for decreasing hypertonicity (including spasticity) and improving ROM, and were inconclusive for other benefits of using supported standers for children and adults with neuromuscular disorders. The addition of whole body vibration (WBV) to supported standing activities appeared a promising trend but empirical data were inconclusive. The survey data from physical therapists (PTs) and participant users attributed numerous improved outcomes to supported standing: ROM, bowel/bladder, psychological, hypertonicity and pressure relief/bedsores. BMD was not a reported benefit according to the user group. CONCLUSION: There exists a need for empirical mechanistic evidence to guide clinical supported standing programs across practice settings and with various-aged participants, particularly when considering a life-span approach to practice.