Variability of joint hypermobility in children: a meta-analytic approach to set cut-off scores.

Current international consensus of the appropriate Beighton score cut-off to define if a child has generalised joint hypermobile or not is based upon expert opinion. Our aim was to determine the prevalence of Beighton scores of children worldwide to provide a recommendation for establishing the Beighton score cut-off to identify generalised joint hypermobility in children. We used AMED, OVID Medline, Embase and CINAHL to find published articles from inception to April 2024 describing Beighton scores of children up to and including 18 years from the general population. We extracted study demographics including country of publication, total number of participants, summary data about the age and sex of participant, Beighton scores and any cut-off used where authors deemed children hypermobile and how many children were rated at the corresponding Beighton scores. There were 37 articles reporting on the prevalence or incidence of hypermobility at cut-off scores from 28,868 participants. Using the cut-off of ≥ 6 resulted in a prevalence of 6% for studies reporting male data and 13% for studies reporting female data. Limited data reporting availability precluded further sub-analysis at a Beighton score of ≥ 7, age, pubertal status and ethnicity.    Conclusion: The working threshold for identifying generalised joint hypermobility in children should be a Beighton score of 6 or more. Our analysis also suggests a Beighton score of 7 or greater may be appropriate in childhood, particularly for females. What is Known: • The working threshold for identifying generalised joint hypermobility in children previously was set based on expert opinion. What is New: • The threshold to identify hypermobility in children should be at a minimum of ≥ 6 on the Beighton score.

Conservative management of hand impairment in children and adolescents with heritable disorders of connective tissue: A scoping review.

AIMS: To synthesize and critically appraise available interventions in the conservative management of hand impairment for children and adolescents with heritable disorders of connective tissue (HDCT). METHODS: A search of peer-reviewed literature and online platforms were included with data regarding hand impairment and function, conservative management and outcome measures extracted and appraised. Levels of evidence were applied to published literature. RESULTS: Ten peer-reviewed papers, eleven webpages and YouTube videos met the inclusion criteria. Reported interventions included: strengthening, orthoses, assistive equipment, education and pacing. Evidence of intervention effectiveness and evidence-based guidance on dosage were absent, with no consistency of outcome measures monitoring intervention effectiveness. Online platforms posted by health professionals predominantly provided advice for families without clinical detail of interventions. CONCLUSIONS: There is a consistent suite of interventions identified in both peer-reviewed literature and online platforms used by clinicians and families to manage hand impairment for children and adolescents with HDCT. Clear dosage parameters and outcome measures are needed in future intervention studies to determine the effectiveness of interventions and guide clinicians in how best to treat hand impairment. Increasing accountability and quality of online resources posted by health professionals for families is warranted to ensure dosage details and precautions are provided.

Scar outcomes for conservatively managed children post burn injury: A retrospective study.

Hypertrophic scarring is a significant complication post burn injury, especially for delayed healing after 3 weeks. Burn injuries healing prior to 3 weeks also have the potential to develop hypertrophic scarring, even when prescribed prophylactic conservative scar interventions. A retrospective chart audit reviewed 326 burn patients treated at a paediatric tertiary hospital from 2014 to 2019 who sustained a partial thickness burn, healed >14 days and did not receive skin grafting. A scar was deemed hypertrophic if >1 mm in height. Early hypertrophic scar prevalence was defined as 3-6 months post burn, while persistent hypertrophic scarring was defined as 12-18 months post burn. Median days to wound closure was 18. The prevalence of early and persistent hypertrophic scarring was 56.1% and 16.3%, respectively. Seventeen (5.2%) children underwent medical interventions for scar modulation. Early signs of hypertrophic scarring were seen in just over half the patients presenting to burn therapy and despite scar intervention, persistent hypertrophic scarring was seen in 16.3%. At both time points, just over half of the children presenting healed between 14 and 21 days. Therefore, children healing prior to 21 days have potential to develop hypertrophic scarring.

Australian guidelines for the management of children with achondroplasia.

Achondroplasia is the most common form of skeletal dysplasia. In addition to altered growth, children and young people with achondroplasia may experience medical complications, develop and function differently to others and require psychosocial support. International, European and American consensus guidelines have been developed for the management of achondroplasia. The Australian focused guidelines presented here are designed to complement those existing guidelines. They aim to provide core care recommendations for families and clinicians, consolidate key resources for the management of children with achondroplasia, facilitate communication between specialist, local teams and families and support delivery of high-quality care regardless of setting and geographical location. The guidelines include a series of consensus statements, developed using a modified Delphi process. These statements are supported by the best available evidence assessed using the National Health and Medicine Research Council's criteria for Level of Evidence and their Grading of Recommendations Assessment, Development and Evaluation (GRADE). Additionally, age specific guides are presented that focus on the key domains of growth, medical, development, psychosocial and community. The guidelines are intended for use by health professionals and children and young people with achondroplasia and their families living in Australia.

Subgroups of Idiopathic Clubfoot Can Predict Short-term Outcomes.

BACKGROUND: The Pirani scale is used for the assessment of Ponseti-managed clubfoot. Predicting outcomes using the total Pirani scale score has varied results, however, the prognostic value of midfoot and hindfoot components remains unknown. The purpose was to (1) determine the existence of subgroups of Ponseti-managed idiopathic clubfoot based on the trajectory of change in midfoot and hindfoot Pirani scale scores, (2) identify time points, at which subgroups can be distinguished, and (3) determine whether subgroups are associated with the number of casts required for correction and need for Achilles tenotomy. METHODS: Medical records of 226 children with 335 idiopathic clubfeet, over a 12-year period, were reviewed. Group-based trajectory modeling of the Pirani scale midfoot score and hindfoot score identified subgroups of clubfoot that followed statistically distinct patterns of change during initial Ponseti management. Generalized estimating equations determined the time point, at which subgroups could be distinguished. Comparisons between groups were determined using the Kruskal-Wallis test for the number of casts required for correction and binary logistic regression analysis for the need for tenotomy. RESULTS: Four subgroups were identified based on the rate of midfoot-hindfoot change: (1) fast-steady (61%), (2) steady-steady (19%), (3) fast-nil (7%), and (4) steady-nil (14%). The fast-steady subgroup can be distinguished at the removal of the second cast and all other subgroups can be distinguished at the removal of the fourth cast [ H (3) = 228.76, P < 0.001]. There was a significant statistical, not clinical, difference in the total number of casts required for correction across the 4 subgroups [median number of casts 5 to 6 in all groups, H (3) = 43.82, P < 0.001]. Need for tenotomy was significantly less in the fast-steady (51%) subgroup compared with the steady-steady (80%) subgroup [ H (1) = 16.23, P < 0.001]; tenotomy rates did not differ between the fast-nil (91%) and steady-nil (100%) subgroups [ H (1) = 4.13, P = 0.04]. CONCLUSIONS: Four distinct subgroups of idiopathic clubfoot were identified. Tenotomy rate differs between the subgroups highlighting the clinical benefit of subgrouping to predict outcomes in Ponseti-managed idiopathic clubfoot. LEVEL OF EVIDENCE: Level II, prognostic.

The prevalence and impact of orthostatic intolerance in young women across the hypermobility spectrum.

Orthostatic intolerance (OI) is frequently reported in young women with generalized hypermobility spectrum disorder (G-HSD) and hypermobile EDS (hEDS). However, it remains currently unclear whether OI is a comorbidity or fundamental part of the pathophysiology of G-HSD or hEDS. This study investigated the prevalence and impact of OI in young women across the hypermobility spectrum. Forty-five women (14-30 years, 15 controls, 15 G-HSD, and 15 hEDS) undertook a head-up tilt (HUT) and active stand test. Postural Orthostatic Tachycardia Syndrome (POTS) and Orthostatic Hypotension (OH) were assessed using age-related criteria. Autonomic dysfunction and quality-of-life questionnaires were also completed. The prevalence of POTS was higher in women with G-HSD than hEDS and control groups during HUT (43% vs. 7% and 7%, respectively, p < 0.05), but similar between groups during the active stand (47%, 27%, and 13% for G-HSD, hEDS, and control, respectively). No participants had OH. hEDS and G-HSD participants reported more severe orthostatic symptoms and poorer quality of life than controls. Although POTS was observed in hypermobile participants, there is no conclusive evidence that its prevalence differed between groups due to differences between the HUT and active stand assessments. Nevertheless, OI and broader autonomic dysfunction impacted on their quality of life.

Medical complications in children with achondroplasia.

AIM: To determine the rates of medical investigations, complications, interventions, and outcomes in children with achondroplasia. METHOD: Children and adolescents with achondroplasia born between 2000 and 2019, aged between 0 and 18 years of age, and seen at The Children's Hospital at Westmead skeletal dysplasia clinic were included. Data were collected retrospectively from clinical records. Standard descriptive statistics were used for analysis. RESULTS: The study included 108 participants, 58 males and 50 females. Ninety-nine participants (91.7%) entered the study at birth. The other nine (8.3%) participants entered the study after birth (mean age = 2 years 4 months, SD = 1 year 8 months). The median age of exit from the study was 8 years 8 months (IQR = 8 years 9 months) with a median follow-up of 8 years 8 months (IQR = 8 years 9 months). Fifty-two (48%) participants presented with craniocervical stenosis, 15 (13.9%) with hydrocephalus, 66 (61.1%) with hearing impairment, 44 (40.7%) with sleep-disordered breathing, 46 (42.6%) with lower-limb malalignment, 24 (22.2%) with thoracolumbar kyphosis, 10 (9.3%) with symptomatic spinal stenosis, 12 (11.1%) with obesity, and 16 (14.8%) who had at least one admission for respiratory illness. Two children died during the study period. INTERPRETATION: We report contemporary rates of medical complications in an Australian population of children with achondroplasia. Recommendations for surveillance in clinical practice are discussed. This information will help guide clinicians with their expectant management of achondroplasia and provide prognostic information to the families of children with achondroplasia.

Muscle Strength, Muscle Mass and Physical Impairment in Women with hypermobile Ehlers-Danlos syndrome and Hypermobility Spectrum Disorder.

OBJECTIVES: To evaluate differences in physical impairment, muscle strength, muscle mass and muscle density between patients with hypermobile Ehlers Danlos Syndrome (hEDS), hypermobile spectrum disorder (HSD), and healthy controls. METHODS: Female adults with hEDS (n=20) and HSD (n=23), diagnosed to the most recent criteria, and age-matched healthy controls (n=28) completed the Arthritis Impact Measurement Scale (physical functioning) and performed maximal muscle strength and strength endurance tests of lower and upper limbs (hand grip, posture maintenance, 30 seconds chair rise and isokinetic tests). Muscle mass and density were evaluated by dual-energy X-ray absorptiometry and peripheral quantitative computed tomography. RESULTS: No differences in physical functioning and muscle strength were found between adults with hEDS and HSD. Furthermore, no differences in muscle mass and density were observed between the three groups. Nevertheless, when both patient groups were compared to controls, physical functioning, maximal muscle strength and muscle strength endurance were significantly lower (all p<0.001), except for the hand flexors. CONCLUSION: Physical functioning, muscle strength, density and mass did not significantly differ between individuals with hEDS and HSD. Compared to controls, physical functioning and muscle strength (maximal and endurance) were significantly lower. Consequently, (functional) strength training in individuals with hEDS and HSD is necessary.

Reliability of a novel technique to assess palmar contracture in young children with unilateral hand injuries.

BACKGROUND: Palmar contracture in young children can have significant developmental consequences. Despite this, objective techniques to measure palmar range of movement and quantify contracture in young children are limited. PURPOSE: The purpose of this study was to determine the reliability of hand span and hand length measures in young children and to establish whether there is any association with age, sex and presence of a palmar burn injury in the reliability of these measures. The study also sought to determine the normative difference and establish a cut off value for the between-hand difference to identify loss of movement in 1 hand. STUDY DESIGN: Cross sectional METHODS: Forty-four children aged 0 to <5 years were recruited. Twenty-two children had a unilateral palmar burn injury and 22 did not have a palmar burn injury. Each child's hand span and hand length were measured 3 times. This was performed twice by the first assessor and once by the second assessor. Intraclass correlation coefficients were calculated to determine the intra-rater and inter-rater reliability. The largest of the 3 values for both hand span and hand length from the first assessor's first assessment were used to determine the normative between-hand difference. Outliers were removed prior to determining the normative difference. Children were considered outliers if their between-hand difference in hand span and/or hand length was in the top 5% of values. RESULTS: Excellent reliability was established for hand span and hand length measures for the whole group (intra-rater ICC2,1 ≥0.95, inter-rater ICC2,1 ≥0.94). The mean normative between-hand difference for both measures was 2 mm. The cut-off for the normative difference in hand span was <9 mm and hand length was <6 mm. CONCLUSION: This measurement technique has excellent reliability and could be a useful method to quantify palmar range of movement and identify contracture in young children with unilateral hand injuries.

Hand Impairment and Function in Children and Adolescents With Heritable Disorders of Connective Tissue.

IMPORTANCE: Heritable disorders of connective tissue (HDCTs) affect hand function and participation in daily activities for children and adolescents. OBJECTIVE: To describe hand impairment and function and determine the extent to which hand impairment and function explain the variation in self-reported functional performance. DESIGN: Cross-sectional observational study. SETTING: Specialist tertiary hospital. PARTICIPANTS: Children and adolescents ages 8-18 yr with HDCTs (N = 73). INTERVENTION: None. OUTCOMES AND MEASURES: Hand function outcomes included grip strength (digital dynamometer), manipulation and dexterity (Functional Dexterity Test, Nine-Hole Peg Test), and fine motor skills (Bruininks-Oseretsky Test of Motor Proficiency). Upper limb hypermobility was assessed using the Upper Limb Hypermobility Assessment Tool. Hand pain and fatigue were recorded for a timed button test and 3- and 9-min handwriting tasks. Functional performance was measured using the Childhood Health Assessment Questionnaire. RESULTS: Scores on all hand function measures were below expected norms. Pain and fatigue were significantly worse after the writing tasks (p < .001) but not the button test (p > .40). Secondary students had significantly lower handwriting scores than primary students (p = .03) but similar grip strength z scores (p = .95). Variation in self-reported functional performance was explained by grip strength (6%) and upper limb hypermobility and dexterity (16%). CONCLUSIONS AND RELEVANCE: Young people with HDCTs have poor hand function attributable to poor grip strength and hand pain and fatigue. Comprehensive upper limb evaluation and ongoing monitoring throughout the school years are warranted to inform timely intervention. What This Article Adds: Children and adolescents with heritable disorders of connective tissue have difficulty with hand function that affect their participation in daily activities. The results of this study can help clinicians identify, assess, and monitor daily activities, performance skills, and symptoms of children and adolescents with HDCTs to promote their participation in all aspects of daily life.

International Perspectives on Joint Hypermobility: A Synthesis of Current Science to Guide Clinical and Research Directions.

ABSTRACT: There is exponential clinical and research interest in joint hypermobility due to recognition of the complexity of identification, assessment, and its appropriate referral pathways, ultimately impacting management. This state-of-the-science review provides an international, multidisciplinary perspective on the presentation, etiology, and assessment of joint hypermobility, as it presents in those with and without a systemic condition. We synthesize the literature, propose standardizing the use of terminology and outcome measures, and suggest potential management directions. The major topics covered are (i) historical perspectives; (ii) current definitions of hypermobility, laxity, and instability; (iii) inheritance and acquisition of hypermobility; (iv) traditional and novel assessments; (v) strengths and limitations of current assessment tools; (vi) age, sex, and racial considerations; (vii) phenotypic presentations; (viii) generalized hypermobility spectrum disorder and hypermobility Ehlers-Danlos syndrome; and (ix) clinical implications and research directions. A thorough understanding of these topics will equip the reader seeking to manage individuals presenting with joint hypermobility, while mindful of its etiology. Management of generalized joint hypermobility in the context of a complex, multisystem condition will differ from that of acquired hypermobility commonly seen in performing artists, specific athletic populations, posttrauma, and so on. In addition, people with symptomatic hypermobility present predominantly with musculoskeletal symptoms and sometimes systemic symptoms including fatigue, orthostatic intolerance, and gastrointestinal or genitourinary issues. Some also display skeletal deformities, tissue and skin fragility, and structural vascular or cardiac differences, and these warrant further medical follow-up. This comprehensive review on the full spectrum of joint hypermobility will assist clinicians, coaches/sports trainers, educators, and/or researchers in this area.

Childhood prevalence of achondroplasia in New South Wales and the Australian Capital Territory, Australia.

The aim of this study was to estimate the childhood prevalence of achondroplasia, trends over time in birth prevalence, and age at diagnosis in Australia. Children born between 1990 and 2019 with a clinical and radiological and/or molecular diagnosis of achondroplasia were identified from a tertiary hospital servicing New South Wales (NSW) and the Australian Capital Territory (ACT) and compared with population data from the Australian Bureau of Statistics. Childhood prevalence of achondroplasia, based on children ≤19 years of age and resident in NSW/ACT on June 30, 2019 (n = 109), was 5.2 per 100,000. A total of 127 individuals with achondroplasia were born in 1990-2019 in NSW/ACT. Birth prevalence rates increased across birth decades, from 3.3 per 100,000 live births in 1990-1999 to 5.3 per 100,000 in 2010-2019 (p < 0.0001). Median age at diagnosis decreased to 17 days in 2010-2019 compared with 30 days in 1990-1999 (p = 0.035), although the overall decreasing trend across consecutive decades did not reach statistical significance. This is the first study to show a rising birth prevalence rate for achondroplasia in Australia with a concurrent decreasing age at diagnosis, both of which were statistically significant after 2 decades.

Outcome measures for assessing change over time in studies of symptomatic children with hypermobility: a systematic review.

BACKGROUND: Generalised joint hypermobility (GJH) is highly prevalent among children and associated with symptoms in a fifth with the condition. This study aimed to synthesise outcome measures in interventional or prospective longitudinal studies of children with GJH and associated lower limb symptoms. METHODS: Electronic searches of Medline, CINAHL and Embase databases from inception to 16th March 2020 were performed for studies of children with GJH and symptoms between 5 and 18 years reporting repeated outcome measures collected at least 4 weeks apart. Methodological quality of eligible studies were described using the Downs and Black checklist. RESULTS: Six studies comprising of five interventional, and one prospective observational study (total of 388 children) met the inclusion criteria. Interventional study durations were between 2 and 3 months, with up to 10 months post-intervention follow-up, while the observational study spanned 3 years. Three main constructs of pain, function and quality of life were reported as primary outcome measures using 20 different instruments. All but one measure was validated in paediatric populations, but not specifically for children with GJH and symptoms. One study assessed fatigue, reporting disabling fatigue to be associated with higher pain intensity. CONCLUSIONS: There were no agreed sets of outcome measures used for children with GJH and symptoms. The standardisation of assessment tools across paediatric clinical trials is needed. Four constructs of pain, function, quality of life and fatigue are recommended to be included with agreed upon, validated, objective tools.

Impact of heritable disorders of connective tissue on daily life of children: Parent perspectives.

AIM: The aim of this study was to investigate parent perspectives on how heritable disorders of connective tissue (HDCT) affect a child's everyday life. In addition, this study aimed to determine if parents seeking health professional services perceive their children with HDCT to have difficulties with activities reliant on hand function. METHODS: This cross-sectional study used a questionnaire for parents to explore the impact of an HDCT on a child's ability to carry out everyday activities. Parents of children (8-18 years) attending a tertiary connective tissue dysplasia clinic, over a 12-month period, were invited to participate. RESULTS: We analysed 100 surveys completed by parents. Children with Ehlers-Danlos syndrome-hypermobile type, joint hypermobility syndrome (48%) and osteogenesis imperfecta (42%) were the largest diagnostic groups represented. Pain (73%) and fatigue (68%) were the most common symptoms parents perceived to affect day-to-day activities. More parents were satisfied with their child's self-care (61%) than school participation (33%). Keeping up with handwriting (71%) and gross motor activities (70%) were the most frequently reported difficulties at school. Most parents (65%) reported leisure activity difficulties, with pain (64%) and fatigue (60%) as the main contributing factors. CONCLUSIONS: This study has provided new knowledge about the concerns of parents with their child's engagement in everyday life including the impact of HDCT on hand function. Further research is needed on effective management strategies to reduce symptoms and improve hand function for these children.

Neonates as intrinsically worthy recipients of pain management in neonatal intensive care.

One barrier to optimal pain management in the neonatal intensive care unit (NICU) is how the healthcare community perceives, and therefore manages, neonatal pain. In this paper, we emphasise that healthcare professionals not only have a professional obligation to care for neonates in the NICU, but that these patients are intrinsically worthy of care. We discuss the conditions that make neonates worthy recipients of pain management by highlighting how neonates are (1) vulnerable to pain and harm, and (2) completely dependent on others for pain management. We argue for a relational account of ethical decision-making in the NICU by demonstrating how an increase in vulnerability and dependence may be experienced by the healthcare community and the neonate's family. Finally, an ethical framework for decisions around neonatal pain management is proposed, focussing on surrogate decision-making and the importance of compassionate action through both a reflective and an affective empathy. As empathy can be highly motivating against pain, we propose that, in addition to educational programs that raise awareness and knowledge of neonatal pain and pain management, healthcare professionals must cultivate empathy in a collective manner, where all members of the NICU team, including parents, are compassionate decision-makers.

Interventions for congenital talipes equinovarus (clubfoot).

BACKGROUND: Congenital talipes equinovarus (CTEV), also known as clubfoot, is a common congenital orthopaedic condition characterised by an excessively turned-in foot (equinovarus) and high medial longitudinal arch (cavus). If left untreated it can result in long-term disability, deformity and pain. Interventions can be conservative (such as splinting or stretching) or surgical. Different treatments might be effective at different stages: at birth (initial presentation); when initial treatment does not work (resistant presentation); when the initial treatment works but the clubfoot returns (relapse/recurrent presentation); and when there has been no early treatment (neglected presentation). This is an update of a review first published in 2010 and last updated in 2014. OBJECTIVES: To assess the effects of any intervention for any type of CTEV in people of any age. SEARCH METHODS: On 28 May 2019, we searched the Cochrane Neuromuscular Specialised Register, CENTRAL, MEDLINE, Embase, CINAHL Plus, AMED and Physiotherapy Evidence Database. We also searched for ongoing trials in the WHO International Clinical Trials Registry Platform and ClinicalTrials.gov (to May 2019). We checked the references of included studies. SELECTION CRITERIA: Randomised controlled trials (RCTs) and quasi-RCTs evaluating interventions for CTEV, including interventions compared to other interventions, sham intervention or no intervention. Participants were people of all ages with CTEV of either one or both feet. DATA COLLECTION AND ANALYSIS: Two review authors independently assessed the risks of bias in included trials and extracted the data. We contacted authors of included trials for missing information. We collected adverse event information from trials when it was available. When required we attempted to obtain individual patient data (IPD) from trial authors for re-analysis. If unit-of-analysis issues were present and IPD unavailable we did not report summary data, MAIN RESULTS: We identified 21 trials with 905 participants; seven trials were newly included for this update. Fourteen trials assessed initial cases of CTEV (560 participants), four trials assessed resistant cases (181 participants) and three trials assessed cases of unknown timing (153 participants). The use of different outcome measures prevented pooling of data for meta-analysis, even when interventions and participants were comparable. All trials displayed high or unclear risks of bias in three or more domains. Twenty trials provided data. Two trials reported on the primary outcome of function using a validated scale, but the data were not suitable for inclusion because of unit-of-analysis issues, as raw data were not available for re-analysis. We were able to analyse data on foot alignment (Pirani score), a secondary outcome, from three trials in participants at initial presentation. The Pirani score is a scale ranging from zero to six, where a higher score indicates a more severe foot. At initial presentation, one trial reported that the Ponseti technique significantly improved foot alignment compared to the Kite technique. After 10 weeks of serial casting, the average total Pirani score of the Ponseti group was 1.15 points lower than that of the Kite group (mean difference (MD) -1.15, 95% confidence interval (CI) -1.32 to -0.98; 60 feet; low-certainty evidence). A second trial found the Ponseti technique to be superior to a traditional technique, with mean total Pirani scores of the Ponseti participants 1.50 points lower than after serial casting and Achilles tenotomy (MD -1.50, 95% CI -2.28 to -0.72; 28 participants; very low-certainty evidence). One trial found evidence that there may be no difference between casting materials in the Ponseti technique, with semi-rigid fibreglass producing average total Pirani scores 0.46 points higher than plaster of Paris at the end of serial casting (95% CI -0.07 to 0.99; 30 participants; low-certainty evidence). We found no trials in relapsed or neglected cases of CTEV. A trial in which the type of presentation was not reported showed no evidence of a difference between an accelerated Ponseti and a standard Ponseti treatment in foot alignment. At the end of serial casting, the average total Pirani score in the accelerated group was 0.31 points higher than the standard group (95% CI -0.40 to 1.02; 40 participants; low-certainty evidence). No trial assessed gait using a validated assessment. Health-related quality of life was reported in some trials but data were not available for re-analysis. There is a lack of evidence for the addition of botulinum toxin A during the Ponseti technique, different types of major foot surgery or continuous passive motion treatment following major foot surgery. Most trials did not report on adverse events. Two trials found that further serial casting was more likely to correct relapse after Ponseti treatment than after the Kite technique, which more often required major surgery (risk differences 25% and 50%). In trials evaluating serial casting techniques, adverse events included cast slippage (needing replacement), plaster sores (pressure areas), and skin irritation. Adverse events following surgical procedures included infection and the need for skin grafting. AUTHORS' CONCLUSIONS: From the evidence available, the Ponseti technique may produce significantly better short-term foot alignment compared to the Kite technique. The certainty of evidence is too low for us to draw conclusions about the Ponseti technique compared to a traditional technique. An accelerated Ponseti technique may be as effective as a standard technique, but results are based on a single small comparative trial. When using the Ponseti technique semi-rigid fibreglass casting may be as effective as plaster of Paris. Relapse following the Kite technique more often led to major surgery compared to relapse following the Ponseti technique. We could draw no conclusions from other included trials because of the limited use of validated outcome measures and the unavailability of raw data. Future RCTs should address these issues.

Exploring health professionals' understanding of evidence-based treatment for idiopathic toe walking.

BACKGROUND: Idiopathic toe walking (ITW) is an exclusionary diagnosis resulting in a child walking on the balls of their feet. Preferred treatment options may be due to the severity of the toe or the health professional preference There are limited guidelines supporting consistent treatment recommendations for this condition. This research aimed to understand agreement between health professionals' knowledge of evidence for common treatment strategies for ITW and if health professionals supported these strategies being used in clinical practice. METHODS: An international online survey was opened to registered health professionals who treat children with ITW between July 2017 and March 2018. The survey had two components: (a) demographic variables and variables relating to knowledge of evidence about ITW treatments and (b) support for common treatment strategies. Additional data on strategy use, referrals, and preference were collected. Kappa statistics described intra-rater agreement between evidence knowledge and support. Multivariable regression analyses identified factors associated with the 10 most commonly preferred treatments. RESULTS: There were 908 international responses. Kappa agreement for paired correct responses determined a fair agreement for evidence support knowledge for four strategies including watch and wait (Kappa = 0.24), stretching (Kappa = 0.30), sensory integration strategies (Kappa = 0.40), and motor control strategies (Kappa = 0.24) and moderate responses for 13 others. No strategies had greater than moderate agreement between correct knowledge of evidence and strategy support. Profession, location, number of children seen in practice, and not correctly identifying the evidence factored into many of the most commonly used strategies for ITW (p < .05). CONCLUSIONS: The results from this study, which confirm a variety of interventions, are utilized in the management of ITW around the world. Furthermore, there remains a disconnection between paediatric health professionals' understanding of the evidence of common treatment strategies of ITW and a consensus for the treatment of this condition.

Interventions for idiopathic toe walking.

BACKGROUND: Idiopathic toe walking (ITW) is an exclusionary diagnosis given to healthy children who persist in walking on their toes after they should typically have achieved a heel-toe gait. The literature discusses conservative and surgical interventions using a variety of treatment modalities. Young children and children without a limitation in ankle dorsiflexion (the upwards movement of the foot towards the shin of the leg) are commonly treated with conservative interventions. Older children who continue toe walking and present with limitations in ankle dorsiflexion are sometimes treated with surgical procedures. This systematic review is needed to evaluate the evidence for any intervention for the treatment of ITW. The conclusions of this review may support decision making by clinicians caring for children with ITW. It may also assist families when deciding on treatment options for their children with ITW. Many of the treatments employed have financial implications for parents or healthcare services. This review also aims to highlight any deficits in the current research base. OBJECTIVES: To assess the effects of conservative and surgical interventions in children with ITW, specifically effects on gait normalisation, ankle range of motion, pain, frequency of recurrence, and any adverse effects. SEARCH METHODS: On 29 April 2019, we searched the Cochrane Neuromuscular Specialised Register, CENTRAL, MEDLINE, Embase, CINAHL Plus, and PEDro. We searched the following registers of clinical trials for ongoing and recently completed trials: the World Health Organization (WHO) International Clinical Trials Registry Platform (ICTRP, apps.who.int/trialsearch), and ClinicalTrials.gov (clinicaltrials.gov). We searched conference proceedings and other grey literature in the BIOSIS databases and System for Information on Grey Literature in Europe (OpenGrey, opengrey.eu). We searched guidelines via the Turning Research Into Practice database (TRIP, tripdatabase.com) and National Guideline Clearinghouse (guideline.gov). We did not apply language restrictions. SELECTION CRITERIA: We considered randomised or quasi-randomised trials for inclusion in the review if they involved participants diagnosed with ITW gait in the absence of a medical condition known to cause toe walking, or associated with toe walking. As there is no universally accepted age group for ITW, this review includes ITW at any age, who have been toe walking for more than six months, who can or cannot walk with a heel-toe gait, and who may or may not have limited dorsiflexion of the ankle joint. DATA COLLECTION AND ANALYSIS: We used standard Cochrane methodological procedures. The primary outcome was improvement in toe walking (defined as greater than 50% of time spent heel-toe walking). Secondary outcomes were active and passive range of motion of the ankle joint, pain, recurrence of ITW after treatment, and adverse events. We assessed the certainty of the evidence using the GRADE framework. MAIN RESULTS: Four studies, comprising 104 participants, met the inclusion criteria. One study did not report data within the appropriate follow-up timeframe and data from two studies were insufficient for analysis. The single study from which we extracted data had 47 participants and was a randomised, controlled, parallel-group trial conducted in Sweden. It tested the hypothesis that combined treatment with serial casting and botulinum toxin type A (BTX) was more effective than serial casting alone in reducing ITW gait.This study found that more participants treated with BTX improved (defined as toe walking less than 50% of the time, as reported by parents) (risk ratio (RR) 1.21, 95% confidence interval (CI) 0.57 to 2.55; 1 trial, 46 participants; very low-certainty evidence). However, there was little or no difference between groups in passive ankle joint dorsiflexion range of movement on the right with the knee extended (mean difference (MD) -1.48º, 95% CI -4.13 to 1.16; 1 trial, 47 participants), on the right with the knee flexed (MD -0.04º, 95% CI -1.80 to 1.73; 1 trial, 46 participants), on the left with the knee flexed (MD 1.07, 95% CI -1.22 to 3.37), or on the left with the knee extended (MD 0.05, 95% CI -0.91 to 1.91). Nor was there a clear difference between the groups in recurrence of toe-walking gait (assessed via severity of toe walking (graded 1 (mild), 2 (moderate), or 3 (severe)) on gait analysis, analysed as continuous data: MD 0.34 points, 95% CI -0.09 to 0.78; 46 participants). In principle, MDs greater than zero (i.e.) positive values) would favour BTX and casting and negative values would favour casting alone. We have not reported effects as better or worse because all results were from evidence of very low certainty. We downgraded the certainty of evidence because of study limitations (outcome assessment was not blinded) and imprecision. Outcomes of pain and active range of motion were not reported in the included study.In terms of adverse events, calf pain was reported twice in the casting-only group and three times in the BTX group. There were three minor skin problems in each group and one reported case of pain directly after BTX injection. The report did not state if calf pain and skin irritation were from the same or different participants. The study authors reported that adverse events did not alter treatment adherence. AUTHORS' CONCLUSIONS: The certainty of evidence from one study, which compared serial casting with serial casting with BTX for ITW in children, was too low for conclusions to be drawn. A further three studies reported outcomes relating to BTX, footwear, exercises, and different types of orthoses as interventions, however the outcome data were too limited to assess their effects.

Systematic review and meta-analysis suggest that varying prevalence of non-acute pain in critically ill infants may be due to different definitions.

AIM: Our aim was to quantify the prevalence of non-acute pain in critically ill infants and to identify how non-acute pain was described, defined and assessed. METHODS: This systematic review and meta-analysis used multiple electronic databases to search for papers published in any language to March 2018: 2029 papers were identified, and 68 full texts were screened. Studies reporting the prevalence of non-acute pain in infants younger than 2 years and admitted to critical care units were included. The extracted data included the use of non-acute pain descriptions, definitions and pain assessment tools. RESULTS: We included 11 studies published between 2002 and 2018 that comprised 1204 infants from Europe, the USA, Canada and India. They were prospective observational (n = 7) and retrospective observational (n = 1) studies and randomised controlled trials (n = 3). The prevalence of non-acute pain was 0%-76% (median 11%). Various pain assessment tools were used, and only two could be pooled. This gave a pooled prevalence of 3.7%-39.8%. A number of different descriptors were used for non-acute pain, and all of these were poorly defined. CONCLUSION: The prevalence of non-acute pain in infants admitted to critical care units varied considerably. This could have been because all the studies used different definitions of non-acute pain.

Which Patients With Chronic Pain Are More Likely to Improve Pain Biology Knowledge Following Education?

BACKGROUND: Pain education is an important part of multidisciplinary management of chronic pain. The characteristics of people likely to have more improvement in pain biology knowledge following pain education are unknown. OBJECTIVE: To identify the baseline factors predicting changes in pain biology knowledge in adults with chronic pain following a 2-hour multidisciplinary pain education session. METHODS: Fifty-five adults with chronic pain attended a 2-hour pain education session prior to a multidisciplinary assessment at a pain clinic. Patients completed the 12-item revised Neurophysiology of Pain Questionnaire (rNPQ, score/12) before and after the pain education session. The primary outcome was change in pain biology knowledge, evaluated with the change in rNPQ score. Ten preselected predictors were investigated using univariate models followed by multivariable models with a manual forwards-building process. RESULTS: Education level and age were significantly associated with change in rNPQ score in the univariate models. Participants with higher levels of education had, on average, 1.96 (0.68 to 3.23) points more improvement in rNPQ score than those with lower levels of education. For every 10 years older a participant was, his or her rNPQ score changed on average by 0.5 (0.1 to 0.8) points less. In the multivariable model, only the education level remained significant, explaining 17% of the variance (R2  = 0.17). The clinical variables that were assessed (pain severity, pain interference, pain self-efficacy, depression, anxiety, and pain catastrophizing) did not predict any knowledge change. CONCLUSION: This study suggests that, of those patients with chronic pain who choose to attend pain education, more educated patients are more likely to improve their pain biology knowledge after a pain education session.