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Purpose: The article compares physeal recovery after insertion of autologous cartilage and a conventional fat graft in a standardized porcine physeal gap model. Presence of a bone bridge was the primary outcome. Methods: Ten porcines in two groups of five were included in a paired design. A standardized physeal gap in the distal femur was made in all animals. One group (n = 5) was randomized for deposition of autologous cartilage and a Tisseel® or Tisseel® alone. The autologous cartilage was harvested from the femoral articular surface. The other group was randomized for fat grafting or no grafts at all. All animals were housed for 14 weeks. Magnetic resonance imaging (MRI) was performed at 14 weeks prior to euthanasia. The physis was harvested for histology. Results: MRI - Three bone bridges were seen in the fat grafted gaps. All empty gaps formed a bone bridge. No gaps filled with autologous cartilage and Tisseel® resulted in bone bridges. One gap filled with Tisseel® only caused a bone bridge. Histology - The cartilage grafted gaps recovered with physeal-like cartilaginous tissue in histological analysis. Conclusions: Fat grafts seems ineffective in preventing bone bridges. The use of autologous cartilage may be superior to the current treatment. However, donor site complications were not investigated. The study serves as a proof of concept study and requires further investigation. [ABSTRACT FROM AUTHOR]

Introduction: Ludloff's procedure for open reduction of congenital dislocation of the hip (CDH) is recommended for its minimal tissue damage, but is criticised for the risk of late avascular necrosis (AVN) of the femoral head. The aim of present study was primarily to assess the risk of late AVN of the femoral head and secondly the range of motion (ROM) of the hip and the quality of life in children following Ludloff's procedure. Methods and materials: 13 hips in 11 children after Ludloff's procedure due to CDH were included retrospec- tively from 1997 to 2005 at Aarhus University Hospital. Radiographs were evaluated for the presence of AVN of the femoral head and classified according to the Bucholz and Ogden classification, with type 2-4 having clinical relevance. A clinical examination included range of motion (ROM) and leg length discrepancy (LLD) measurements. The HAGOS hip questionnaire evaluated activity, participation and quality of life. Results: No severe type 3-4 AVN was observed. 2 type 2 and 5 type 1 were observed. AVN was observed in 7 of the 13 operated hips (54%). An 8.6° difference in flexion for unilaterally treated hips was observed (p<0.02). 8 of 11 patients had minor LLD (range 0.5-2 cm). Conclusions: Only minor AVN of clinical importance was seen after Ludloff's procedure. [ABSTRACT FROM AUTHOR]

This single group pre and posttest study evaluated the feasibility of a new 10-week group-based motor skills enhancement intervention: “Moving Together,” and associated use of the Challenge assessment and Gait Outcomes Assessment List (GOAL). Participant attendance/completion and satisfaction with the assessments and intervention were evaluated, and a first estimate of associated motor skill-related changes obtained. Ten ambulatory children with cerebral palsy (7–14 years) and their parents participated. Ninety percent of Challenge sessions were attended and 82.5% of GOAL questionnaires completed. Program attendance was 83% overall. Satisfaction with assessments was high for the Challenge and moderate for the GOAL, and intervention satisfaction was high. Mean change scores (95% CI) post-intervention for the Challenge and GOAL were 4.2 (−11.4 to 3.1) and 3.6 (−14.4 to 4.0) points (/100) respectively. Challenge and GOAL use was feasible and appropriate for “MovingTogether” and associated with gains in motor skill performance and functional abilities. [ABSTRACT FROM AUTHOR]

Our novel plate design has been developed for controlled rotation of long bones by guided growth. The objective of this proof‐of‐concept study was to evaluate the precision of the rotation in the femur. Twelve cadaverous femora of six adults (right = 6, left = 6) underwent an osteotomy at the level of the physeal scar. The plates were inserted on each side of the distal femur. Growth was simulated by axial distraction of the bone segments. The femur was stabilized using a unilateral external fixator. Femoral torsion was assessed with computed tomography (CT) and with an electric goniometer before and after distraction. The obtained rotation was compared to the predicted rotation based on the dimension of the plate and the bone. All femora were rotated as intended. The mean obtained rotation was 26.3° (95% confidence interval [CI]: 23.5–29.0) and the mean predicted rotation was 28.2° (95% CI: 26.9–29.5) (p > 0.82). The mean axial distraction was 19.5 mm (95% CI: 17.7–21.3). The predicted rotation of the femora was similar to the obtained values on CT and by goniometer. The obtained rotation occurred as a result of an axial distraction of approximately 2 cm. This suggests a potential for controlled rotation of the femur based on the circumference of the bone and plate dimensions that occurs simultaneously with axial distraction. Clinical significance: These findings suggest a possible clinical application in the treatment of maltorsion in children by guided growth, where theplate design guides the bone into torsional axial growth correcting the deformity. [ABSTRACT FROM AUTHOR]

Aim: We investigated the reliability and validity of the Danish child and parent versions of the Gait Outcomes Assessment List (GOAL) questionnaires for ambulatory children with cerebral palsy (CP). Methods: Translation and cultural adaptations were performed and content validity evaluated. Participants were enrolled between 2016 and 2018 from Aarhus University Hospital, Denmark. Children and parents completed the GOAL questionnaires twice for test–retest reliability. Discriminative validity was evaluated by comparing the child and parent GOAL scores between children with Gross Motor Function Classification System (GMFCS) levels I and II. The concurrent validity of the GOAL questionnaires were investigated by comparing them with Challenge‐20, which assesses motor skills in children with CP. Results: We studied 59 children (57% boys) with CP and GMFCS I‐II at a mean age of 10.6 years. Test–retest intra‐class correlations were excellent for the children (0.91, 95% confidence interval (CI) 0.83–0.96) and good for the parents (0.83, 95% CI 0.67–0.91). GOAL scores decreased with increasing GMFCS (p < 0.05). Both versions correlated well. The mean children's scores were significantly (6.2/100) higher than the parents' (p < 0.001). The GOAL scores correlated positively with Challenge‐20. Conclusion: The Danish GOAL child and parent questionnaires demonstrated good reliability and content and discriminative and concurrent validity. [ABSTRACT FROM AUTHOR]

Aim: We aimed to improve bone health management of children with cerebral palsy (CP) by reviewing studies investigating bisphosphonate therapy in children with CP and other types of secondary osteoporosis. Methods: We included trials on bisphosphonate treatment reporting any direct bone measurement or fracture outcome. All studies of patients with CP were included. We also included all controlled trials of children with secondary bone fragility as well as observational studies with ≥20 participants or at least 3 years of follow‐up. Studies were assessed according to PRISMA guidelines using the RoB2‐tool and the Newcastle‐Ottawa Scale. Results: We reviewed 1104 studies and found 37 eligible. Some studies were sufficiently homogeneous to include in a meta‐analysis, and we found a 1‐year effect on lumbar spine bone mineral density (BMD) Z‐score of +0.65 after oral and + 1.21 after intravenous bisphosphonates in children with secondary osteoporosis. Further, data on adverse events and post‐treatment follow‐up were reviewed. Limitations were heterogeneity and small size of the included studies. Conclusion: Meta‐analysis consistently showed significant BMD increases with bisphosphonates in children with secondary osteoporosis. Direct evidence of the effect of bisphosphonates on reducing fractures is lacking. We found no reports of long‐term adverse events yet longer studies are needed. [ABSTRACT FROM AUTHOR]

Background and purpose -- There is inconsistency in the literature regarding the relationship between increased birthweight and risk of developmental dysplasia of the hip (DDH). We aimed to investigate the correlation between birthweight and pubo-femoral distance (PFD), as well as Graf's α angle in newborns undergoing hip ultrasound examination at 6 weeks of age. Patients and methods -- Basic newborn characteristics and ultrasound measurements were retrospectively collected during a 1-year study period. We excluded multiple births, newborns born at less than 37 gestational weeks, and incomplete information. Simple and multiple linear regression analyses were performed to evaluate the correlation of birthweight and PFD, and, second, birthweight and α angles including a stratified regression analysis investigating the potential effect modification of sex. Results -- 707 newborns (1,414 hips) were included. Mean birthweight was significantly higher for male new-borns (P < 0.001). Increased birthweight was positively correlated to PFD values (crude coefficient 0.21, 95% confidence interval [CI] 0.10-0.32) and the correlation was still present after adjusting for sex, family history, and breech presentation (adjusted coefficient 0.18, CI 0.07-0.29). The stratified α angle model for the males was significant for both the crude coefficient (-0.73, CI -1.28 to -0.19) and the adjusted (-0.59, CI -1.15 to -0.03), and also for the females (crude coefficient -1.14, CI -1.98 to -0.31 and adjusted coefficient -1.15, CI -1.99 to -0.31). Conclusion -- We found that increased birthweight positively correlated to PFD, and negatively correlated to α angle, but this was not of clinical significance. [ABSTRACT FROM AUTHOR]

Background and purpose -- Temporary hemiepiphysiodesis by tension-band devices is commonly applied to correct angular limb deformities in children. We aimed to evaluate knee joint morphology after guided growth using these devices. Patients and methods -- In a retrospective multicenter study we analyzed standardized anteroposterior long-leg radiographs of 222 limbs (285 implants) of patients treated by temporary hemiepiphysiodesis with either eight-Plates or FlexTacks for coronal angular deformities of the knee joint between 2013 and 2019. Femoral floor angle (FFA), femoral notch-intercondylar distance (FNID), and tibial roof angle (TRA) were measured pre- and postoperatively to assess the central knee joint morphology. Statistical exploratory analyses were performed using linear mixed models, t-tests, Wilcoxon signed-rank test, and Mann-Whitney U test. Results -- 217 FlexTacks (femur 106, tibia 111) in 104 children and 68 eight-Plates (femur 61, tibia 7) in 35 children were identified. Median time period under growth guidance was 11 months (range 4-42). No statistically significant change in the FFA was detected (eight-Plate: P = 0.2; FlexTack: P = 0.3). A statistically significant difference of the FNID was found in the eight-Plate group (P = 0.02), but not in the FlexTack group (P = 0.3). While TRA increased in both groups, a statistical significance was observed only in the FlexTack group (P < 0.01). Conclusion -- We found minor but clinically irrelevant changes in knee morphology after the treatment. [ABSTRACT FROM AUTHOR]

Background and purpose -- For correction of leg-length discrepancy or angular deformity of the lower limb in skeletally immature patients temporary or permanent (hemi-)epiphysiodesis can be employed. These are reliable treatments with few complications. Recently, radiographic analysis of treatment-related alterations of the central knee anatomy gained interest among pediatric orthopedic surgeons. To date the comparison and adequate interpretation of potential changes of the central knee anatomy is limited due to the lack of defined standardized radiographic references. We aimed to establish new reference values of the central knee anatomy. Patients and methods -- A retrospective analysis of calibrated longstanding anteroposterior radiographs of 254 skeletally immature patients with a chronological age ranging from 8 to 16 years was conducted. The following radiographic parameters were assessed: (1) femoral floor angle, (2) tibial roof angle, (3) width at femoral physis, and (4) femoral notch-intercondylar distance. Results -- All observed radiographic parameters were normally distributed with a mean age of 12.4 years (standard deviation [SD] 2, 95% confidence interval [CI] 12.2-12.6). Mean femoral floor angle was 142° (SD 6, CI 141.8-142.9), mean tibial roof angle was 144° (SD 5, CI 143.7-144.1), mean width at femoral physis was 73 mm (SD 6, CI 72.8-73.9), and mean femoral notch-intercondylar distance was 8 mm (SD 1, CI 7.5-7.7). The estimated intraclass correlation coefficient values were excellent for all measurements. Conclusion -- This study provides new radiographic reference values of the central knee anatomy for children between 8 and 16 years and we suggest considering values within the range of 2 SD as the physiological range. [ABSTRACT FROM AUTHOR]

To translate and cross-culturally adapt the Challenge, and investigate the reliability and minimal detectable change (MDC) of the Danish Challenge in children with cerebral palsy (CP). A Danish version of the Challenge was created through a standardized translation process. Four physiotherapists evaluated face validity. Independently ambulatory children with CP were tested. Live performance rating was conducted by assessors independently scoring the Challenge. Video-rating was undertaken for a subset of assessments. Same day assessment test–retest reliability was estimated. The Challenge's Best Score Total was of primary interest. Forty-five children (5–18 years: mean 10 years 9 months; 19 girls) in Gross Motor Function Classification System levels I and II were tested. Inter-rater reliability was excellent for live assessments (n = 45) ICC = 0.998 (95% CI 0.998–0.999) and video assessments (n = 15) ICC = 0.991 (95% CI 0.963–0.997) and intra-rater reliability was excellent for live versus video-recorded assessments (n = 10) ICC = 0.977 (95% CI 0.895–0.994). Test–retest reliability (n = 22) was excellent with ICC = 0.991 (95% CI 0.979–0.996) and minimal detectable change (MDC90) of 4.7 points. The Danish Challenge showed excellent reliability in this testing context when physiotherapists scored from live- or video-recorded assessments. The Challenge's ability to detect 4.7 points change seems a clinically realistic target for progress. Clinical trial registration: This trial has been approved by the Data Protection Agency, Central Region Denmark, Ref nr.: 615216, Case nr.: 1-16-02-46-16. Registration date: 01-01-2016. The Challenge remained reliable and maintained a promising minimal detectable change of less than five points after translation and cultural adaptation. The Danish version of the Challenge 20-item version can be used to measure advanced motor skill performance in children with cerebral palsy, GMFCS level I and GMFCS level II. Challenge live scoring is as reliable as the more time-consuming video-recorded scoring, meaning that physiotherapists can choose the method that fits best with their clinical context and preference. [ABSTRACT FROM AUTHOR]

Background and purpose — Physical abuse of children, i.e., nonaccidental injury (NAI) including abusive head trauma (AHT) is experienced by up to 20% of children; however, only 0.1% are diagnosed. Healthcare professionals issue less than 20% of all reports suspecting NAI to the responsible authorities. Insufficient knowledge concerning NAI may partly explain this low percentage. The risk of NAI is heightened during health and socioeconomic crises such as COVID-19 and thus demands increased awareness. This review provides an overview and educational material on NAI and its clinical presentation. Methods — We combined a literature review with expert opinions of the senior authors into an educational paper aiming to help clinicians to recognize NAI and act appropriately by referral to multidisciplinary child protection teams and local authorities. Results — Despite the increased risk of NAI during the current COVID-19 crisis, the number of reports suspecting NAI decreased by 42% during the lockdown of the Danish society. Healthcare professionals filed only 17% of all reports of suspected child abuse in 2016. Interpretation — The key to recognizing and suspecting NAI upon clinical presentation is to be aware of inconsistencies in the medical history and suspicious findings on physical and paraclinical examination. During health and socioeconomic crises the incidence of NAI is likely to peak. Recognition of NAI, adequate handling by referral to child protection teams, and reporting to local authorities are of paramount importance to prevent mortality and physical and mental morbidity. [ABSTRACT FROM AUTHOR]

Background and aims: This prospective study aimed to assess pressure pain thresholds (PPTs) by pressure algometry and the correlation to postoperative pain in children undergoing orthopaedic surgery. We hypothesized, that the PPTs would decline immediately after elective orthopaedic surgery and return to baseline values at follow-up. Methods: Thirty children aged 6–16 years were included. PPTs and intensity of pain (Numerical Rating Scale, NRS) were assessed 3–6 weeks before surgery (baseline), 1–2 h before surgery (Day 0), the first postoperative day (Day 1) and 6–12 weeks after surgery (Follow-up). Results: A significant difference of PPTs between the four assessments was seen using the Friedman test for detecting differences across multiple tests and Wilcoxon signed-rank test with a Bonferroni adjustment. The changes in PPTs between baseline (PPTcrus = 248 kPa, PPTthenar = 195 kPa) and day 1 (PPTcrus = 146 kPa, PPTthenar = 161 kPa) showed a decline of PPTs as hypothesized (Zcrus = 2.373, p = 0.018; Zthenar = 0.55, p = 0.581). More surprisingly, a significant decrease in PPTs between baseline and day 0, just before surgery (PPTcrus = 171 kPa, PPTthenar = 179 kPa), was also measured (Zcrus = 2.475, p = 0.013; Zthenar = 2.414, p = 0.016). PPTs were positively correlated to higher age, weight and height; but not to NRS or opioid equivalent use. Conclusions: Children undergoing orthopaedic surgery demonstrate significant changes in PPTs over time. The PPTs decrease significantly between baseline and day 0, further decreases the first day postoperatively and returns to baseline values at follow-up. This suggests that other factors than surgery modulate the threshold for pain. Implications: Awareness of pressure pain thresholds may help identify children with affected pain perception and hence improve future pain management in children undergoing orthopaedic surgery. Factors as for example anticipatory anxiety, psychological habitus, expected pain, catastrophizing, distraction, physical activity, patient education and preoperative pain medication might play a role in the perception of pain and need further investigation. [ABSTRACT FROM AUTHOR]

Background and purpose — Current selective screening algorithms for developmental dysplasia of the hip (DDH) are insufficient. Universal screening programs have been proposed but so far have been deemed too expensive and time consuming. The pubo-femoral distance may solve this problem as a quick, low-cost, highly sensitive, and specific sonographic measurement for DDH, but this has only been validated in the supine position. Therefore we validated pubo-femoral distance (PFD) in the lateral position as an indicator for instability of the hip. Methods — All participants had undergone ultrasonographic diagnostics using the modified Graf technique. In addition, PFD measurements in lateral position were performed. Results were compared between 25 infants who had been treated for DDH because of dysplastic appearance on ultrasound combined with clinical instability and a control group consisting of 100 untreated infants screened for DDH. Sensitivity, specificity, and cut-off points were determined using Receiver operating characteristics (ROC) analysis. Results — We found a mean PFD of 6.8 mm (6.2-7.4) in the treated group with a control group PFD of 3.4 mm (3.3-3.6) (p < 0.005). A PFD value above a threshold of 4.4 mm yielded a sensitivity of 100% and a specificity of 93% for detecting unstable DDH. Interpretation — PFD measured in lateral position was statistically significantly increased in hips of children treated for DDH with Denis Browne hip brace compared with healthy children with unaffected stable hips. Furthermore, the PFD measurement had a high level of sensitivity and specificity at a cut-off value of 4.4 mm. A cut-off value of 6.00 mm has previously been reported as the gold standard in supine position. We suggest that 4.4 mm is used in lateral position. [ABSTRACT FROM AUTHOR]

Background: Moebius Sequence (MS) is a rare disorder defined by bilateral congenital paralysis of the abducens and facial nerves in combination with various odontological, craniofacial, ophthalmological and orthopaedic conditions. The aetiology is still unknown; but both genetic (de novo mutations) and vascular events in utero are reported. The purpose of present study was through a multidisciplinary clinical approach to examine children diagnosed with Moebius-like symptoms. Ten children underwent odontological, ophthalmological, obstetric, paediatric, orthopaedic, genetic, radiological and photographical evaluation. Five patients maintained the diagnosis of MS according to the diagnostic criteria.Results: All five patients had bilateral facial and abducens paralysis confirmed by ophthalmological examination. Three of five had normal brain MR imaging. Two had missing facial nerves and one had missing abducens nerves. The Strengths and Difficulties Questionnaire (SDQ) showed normal scores in three of five patients. Interestingly, two of five children were born to mothers with uterine abnormalities (unicornuate/bicornuate uterus). In the odontological examination three of five showed enamel hypomineralisation. All five had abnormal orofacial motor function and maxillary prognathism. Two patients had adactyly, syndactyly and brachydactyly. None of the five patients had Poland anomaly, hip dislocation or dysplasia but all had a mild degree of scoliosis. We observed congenital club-feet, calcaneovalgus deformities, macrodactyly of one or more toes or curly toes. Pedobarography showed plantar pressures within normal ranges.Conclusions: Adherence to standard diagnostic criteria is central in the diagnosis of MS. An accurate diagnosis is the basis for correct discussion of other relevant concomitant symptoms of MS, genetic testing and evaluation of prognosis. The multidisciplinary approach and adherence to diagnostic criteria taken in present study increases the knowledge on the relationship between genotype, phenotype and symptomatology of MS. [ABSTRACT FROM AUTHOR]

Background: Seated postural stability can be measured using Tekscan, CONFORMat. Standing postural stability has gained great clinical and, research value by use of different force platforms with mostly good reliability. No reliability testing or biologic variation assessment has been documented regarding seated balance. This study determines the reliability of the parameters of seated balance in healthy children using the Tekscan CONFORMat equipment.Methods: Sixty-six healthy children completed six measurements of seating position the first three with the child seated in a relaxed normal back position and the next three with the child seated in a complete up-right back position. The SAM software calculated five default parameters of balance (area, distance, variability, antero-posterior (AP) excursion and left-right excursion).Results: Reliability parameters were assessed by one-way analysis of variance intra-class correlation (ICC) proving excellent reliability for relaxed and up-right back position with respect to distance (0.75/0.84) and good reliability with respect to variability (0.61/0.62) and area (0.61/0.60). AP excursion (0.41/0.59) and left-right excursion (0.54/0.24) showed fair to poor reliability.Conclusion: In conclusion, two of the five default parameters of balance used in the Tekscan CONFORMat system are direction-independent parameters and have been found reproducible for measuring seated balance in children. This study can be used as reference for comparisons of seated balance in children with affected seated postural control and for evaluating a clinical treatment effect. [ABSTRACT FROM AUTHOR]

Background and purpose — Treatment of postoperative pain in children with cerebral palsy (CP) is a major challenge. We investigated the effect of epidural analgesia, high-volume local infiltration analgesia (LIA), and an approximated placebo control on early postoperative pain in children with CP who were undergoing unilateral hip reconstruction. Patients and methods — Between 2009 and 2014, we included 18 children with CP. The first part of the study was a randomized double-blind trial with allocation to either LIA or placebo for postoperative pain management, in addition to intravenous or oral analgesia. In the second part of the study, the children were consecutively included for postoperative pain management with epidural analgesia in addition to intravenous or oral analgesia. The primary outcome was postoperative pain 4 h postoperatively using 2 pain assessment tools (r-FLACC and VAS-OBS) ranging from 0 to 10. The secondary outcome was opioid consumption over the 21-h study period. Results — The mean level of pain 4 h postoperatively was lower in the epidural group (r-FLACC: 0.7; VAS-OBS: 0.6) than in both the LIA group (r-FLACC: 4.8, p = 0.01; VAS-OBS: 5.2, p = 0.02) and the placebo group (r-FLACC: 5.2, p = 0.01; VAS-OBS: 6.5, p < 0.001). Corrected for body weight, the mean opioid consumption was lower in the epidural group than in the LIA group and the placebo group (both p < 0.001). Interpretation — Epidural analgesia is superior to local infiltration analgesia for early postoperative pain management in children with cerebral palsy who undergo unilateral hip reconstruction. [ABSTRACT FROM PUBLISHER]

A correction is presented to the article "Physical child abuse demands increased awareness during health and socioeconomic crises like COVID-19."

Background and aims Assessment of pain in children with cognitive impairment (CI) including cerebral palsy (CP) is difficult. Several pain assessment tools have been developed and validated for use in children with CI. The revised Face, Legs, Activity, Cry and Consolability score (r-FLACC) includes core behaviours of children with CI and adds an open-ended descriptor for individualisation (5 items assigned 0–2 points, total range 0–10). Other pain assessment tools including individual pain behaviours are the Individualised Numeric Rating Scale (INRS) and the Paediatric Pain Profile (PPP). Both the Noncommunicating Childrens's Pain Checklist – Postoperative version (NCCPC-PV) and the Echelle Douleur Enfant San Salvador (DESS) are developed from core pain behaviours for children with CI but have no possibility for individualisation. For successful clinical application a pain assessment tool should not only be reliable and valid, but also clinically feasible. The aim of this study was to select the most valid and feasible pain assessment tool for children with CI and translate that tool into Danish. Methods A literature review on studies on pain, pain assessment tools, feasibility and CI was performed. Studies were evaluated with focus on children with CI not able to self-report pain and the r-FLACC was chosen for translation. A 10 step translation process guideline was used from the Translation and Cultural Adaptation Group, which describes preparation, forward translation, reconciliation, back translation, back translation review, harmonization, cognitive debriefing, review of cognitive debriefing, finalisation, proofreading, and final report. Results Studies show that the r-FLACC is superior regarding clinical feasibility. The r-FLACC is a useful tool for assessing pain in children with CI due to its ease to use in a clinical setting and its use of both core and individual pain behaviours. In the back translation review discrepancies of words between the original and the back-translated English versions were assessed and in three of nine discrepancies a word was changed. In the cognitive debriefing no issues were found regarding understandability, interpretation or cultural relevance of the translation. No other language translations of the r-FLACC score have been published; hence the harmonisation step with a comparison of the English back translations was not possible. Conclusions The r-FLACC is assessed to have the most preferable profile with use of core pain behaviours, flexibility regarding individualisation, good psychometric properties, and high clinical feasibility; hence suited for use in Danish children with CP. A Danish version of the r-FLACC score now exits and may be used in a clinical setting for the assessment of pain. Implications It is important for health-care professionals to be able to assess pain in hospitalized children with CP who are not able to self-report. The r-FLACC score is clinically feasible and has the potential for becoming the gold standard of pain assessments in children with CP. A standardised approach to pain assessment may lead to an increased focus on pain and help improve the treatment of pain in children with CP in Denmark. [ABSTRACT FROM AUTHOR]

Background and aims Pain in children with cerebral palsy (CP) is difficult to assess and is therefore not sufficiently recognized and treated. Children with severe cognitive impairments have an increased risk of neglected postoperative, procedural and chronic pain resulting in decreased quality of life. The r-FLACC (revised Face, Legs, Activity, Cry and Consolability) pain score is an internationally acclaimed tool for assessing pain in children with CP because of its ease to use and its use of core pain behaviours. In addition the r-FLACC pain score may be superior to other pain assessment tools since it includes an open-ended descriptor for incorporation of individual pain behaviours. The COSMIN group has set up three quality domains, which describe the quality of Health-Related Patient-Reported Outcomes (HR-PROs). These are reliability (internal consistency, reliability and measurement error), validity (content validity, construct validity and criterion validity) and responsiveness. The r-FLACC score has only been assessed for reliability and validity in the original English version by the developers of the score. The aim of this study is to assess reliability and validity of the r-FLACC pain score for use in Danish children with CP. Methods Twenty-seven children aged 3–15 years old with CP were included after orthopaedic surgery. Two methods for assessment of postoperative pain were used. Pain intensity was assessed by r-FLACC, with a 2 min standardized video recording of the child, and the Observational Visual Analogue Score (VAS-OBS) assessed by the parents. The COSMIN checklist was used as a guideline in the reliability and validity testing of the r-FLACC score. Results Reliability was supported by three measurement properties. Internal consistency was excellent with a Cronbachs alpha of 0.9023 and 0.9758 (two raters). A factor analysis of the subgroups in the r-FLACC score showed unidimensionality. A test–retest showed excellent intra-rater reliability with an intraclass correlation (ICC) of 0.97530. Inter-rater reliability was acceptable with an ICC of 0.74576. Validity was supported by three measurement properties. Content validity was tested by the originators of the r-FLACC. Construct validity was supported by a significant increase in r-FLACC scores following surgery ( n = 17; difference 2.23; p = 0.0397). Criterion validity was acceptable with Pearson's correlation coefficients of 0.76 and 0.59 when comparing r-FLACC scores and VAS-OBS scores. Conclusion and implications This study benefits from a systematical approach to the validation and reliability parameters by using the COSMIN checklist as a guideline. It is evident that the r-FLACC pain score maintains its psychometric properties after translation. In conclusion, the r-FLACC pain score is valid and reliable in assessing postoperative pain in children with CP not able to self-report pain. With the r-FLACC pain score clinicians have a valid tool for assessing postoperative pain, hence increasing the quality of pain management in children with CP. In addition the validated r-FLACC score has the potential for use in interventional research regarding pain management in this vulnerable group of patients. Future perspectives include validation of the r-FLACC score for procedural and chronic everyday pain and implementation into daily practice. [ABSTRACT FROM AUTHOR]

Purpose: To analyze the accuracy of growth-plate thickness measurements detected on 1.5-T and 7-T MR images using histology sections as a standard of reference. Materials and methods: Four defrosted pig tibiae were 1.5-T MR scanned and one fresh tibia was 7-T MR scanned. The height of the growth plate was measured and compared to histology. Results: Histology measurements showed a mean growth plate thickness of 467 μm (SD = 82.2). The mean growth plate thickness measured in the 7-T MR images was 465 μm (SD = 62.2) and 1,325 μm (SD = 183.5) on 1.5-T MR measurements. We found a better correspondence between the growth plate thickness measured on the 7-T MR and histology samples compared to 1.5 T. Conclusions: The growth plate can be identified and measured with high accuracy using 7-T MR. 1.5-T MR can only describe some morphological characteristics. [ABSTRACT FROM AUTHOR]

Background and purpose - Current techniques for epiphysiodesis involve opening of cortical windows; use of staples, screws, and tension devices; and fusion with curettes or drills. Complications may have serious consequences. There is a need for a more reliable, precise, and less traumatic procedure that overcomes the known complications from existing techniques. We analyzed a new epiphysiodesis technique using radio-frequency ablation (RFA) in a porcine model. Methods - Six 35-kg and two 25-kg immature pigs were used. 1 hind leg of each animal was randomly selected and the proximal tibia growth plate was ablated laterally and medially. The contralateral leg was used as a control. MR images were obtained immediately after the ablation and 12 weeks later for 6 animals, and 24 weeks later for the other 2 animals. CT was done for the 2 animals that were followed for 24 weeks for proof of bone bridges. Results - Both tibias were equal in length initially. At the 12-week follow-up, there was an average leg length discrepancy of 3.9 mm (95% CI: 3.0-4.8), and at 24 weeks the difference was 8.4 mm and 7.5 mm. No damage to the adjacent tissue was found. Bone bridges and physeal closure were found after 24 weeks. The pigs showed no discomfort after the intervention. Interpretation - We found RFA to be feasible for epiphysiodesis in a pig model. The method is minimally invasive and recovery may be quick compared to conventional methods. We recommend that the method should be tested in larger-scale safety studies before clinical application. [ABSTRACT FROM AUTHOR]

Introduction Hypomelanosis of Ito was originally described as a purely cutaneous disease. Extracutaneous manifestations were described later, forming a neurocutaneous syndrome including skeletal, muscular, ocular and central nervous system symptoms. Hypomelanosis of Ito is characterized by a depigmentation along the lines of Blaschko on the trunk and extremities in certain patterns. The aim of this article was to report another case and give an overview of the related orthopedic symptoms that have been previously described. It was also our wish to contribute with recommendations for consideration with regard to bandages on eczematous rashes, especially on clubfeet. Case presentation A one-and-a-half-month-old boy of Caucasian background born with talipes equinovarus, or clubfoot, on his right foot presented with an eczematous rash after surgical correction and plaster bandaging. Conclusions It is the appearance of hypopigmentation, either alone or in combination with a congenital malformation, particularly central nervous system or musculoskeletal anomalies, which should form the basis of a presumptive diagnosis. This should then lead to further investigations and should always include skin biopsies and a test for chromosomal mosaicism. We report the case of a boy with a clinical picture consisting of a depigmented skin pattern, mental retardation, pes cavus, talipes equinovarus, clinodactyly, eczema, inverted cilia of the eye, strabismus, reduced hearing, ventral hernia, glomerulonephritis, missing testicles, leg length discrepancy with scoliosis, back pain and a syrinx. It is perhaps impossible to make any conclusions about extracutaneous symptoms. However, some symptoms such as retardation, cramps and seizures, delayed development and hypotonia cannot be ignored. Because of the possibility of creating an undesirable and long postoperative period with complications, it is very important to have this diagnosis in mind when deciding to do surgery or not if there are signs of dermatological problems before surgery. In this case, it could also be good clinical practice to test the patient's reaction to plaster or other bandages. [ABSTRACT FROM AUTHOR]

The tension band plating technique for hemiepiphysiodesis is new and advocated because it is believed to reduce the risk of premature closure of the growth plate compared to stapling. The benefit of the tension band plating technique has not yet been proven in experimental or randomized clinical studies. Ten weeks old domestic pigs in two randomized, paired studies were used. Right proximal tibia was randomized to medial epiphysiodesis by either stapling or tension band plating. Left side received the opposite treatment. Study A ( n = 10): 9 weeks treatment. Study B ( n = 8): implant removal after 9 weeks of treatment and 5 weeks of housing. Fractions of the chondrocyte layers were determined using quantitative histomorphometry. Mean heights of the growth plates were measured. No significant changes were observed between histomorphometric parameters in neither study A or B. Areas with disorganized cartilage tissue were abundant in 13/16 samples from study B and observed after both treatment with tension band plating and staples. Chondrocyte zone fractions did not differ between tension band plating and staple treatment in this randomized, paired animal study. The growth plate responded to release of hemiepiphysiodesis with abundant disorganized cartilage tissue in both groups. The histological response to hemiepiphysiodesis by tension band plating and staples appear to be similar. © 2012 Orthopaedic Research Society. Published by Wiley Periodicals, Inc. J Orthop Res 31: 574-579, 2013 [ABSTRACT FROM AUTHOR]

Background In children with angulating deformities of the lower limbs, hemiepiphysiodesis can be used to guide growth to achieve better alignment at skeletal maturity. Traditionally, this has been performed with staples. The tension-band plating technique is new and it has been advocated because it is believed to reduce the risk of premature closure of the growth plate compared to stapling. The benefit of the tension-band plating technique has not yet been proven in experimental or randomized clinical studies. Methods We performed a randomized clinical trial in which 26 children with idiopathic genu valgum were allocated to stapling or tension-band plating hemiepiphysiodesis. Time to correction of the deformity was recorded and changes in angles on long standing radiographs were measured. Pain score using visual analog scale (VAS) was recorded for the first 72 h postoperatively. Analgesics taken were recorded by the parents. Results Mean treatment times for stapling hemiepiphysiodesis (n = 10) and for tension-band plating hemiepiphysiodesis (n = 10) were similar. Postoperative VAS scores and consumption of analgesics were also similar in both groups. No hardware failure or wound-related infection was observed. Interpreatation Treatment time for the 2 treatment modalities was not significantly different in this randomized clinical trial. Tension-band plating and stapling appeared to have a similar effect regarding correction of genu valgum. We cannot rule out type-II error and the possibility that our study was underpowered. ClinicalTrials.gov Identifier: NCT01641354. [ABSTRACT FROM AUTHOR]

Objective. To validate the use of the kernicterus diagnosis in a clinical register in Denmark and to describe occurrence and obstetric outcome in children with a validated kernicterus diagnosis. Design. Population-based cohort study. Setting. Denmark. Population. All children born from 1 January 1994 to 31 December 2003. Methods. We established a national population-based cohort of children with a diagnosis of kernicterus based on data obtained from a mandatory national register and from a clinically established cohort. Information on obstetric outcome and child development was obtained from the registers and from the medical records. Main outcome measures. Validation of the kernicterus diagnosis and description of obstetric and long-term outcomes in children with kernicterus. Results. We found 15 children with a diagnosis of kernicterus in the Danish National Hospital Register and eight children with a diagnosis of kernicterus in a clinically established cohort. A total of nine children had a validated diagnosis of kernicterus which leads to a cumulative incidence of kernicterus in Denmark of 1.3/100.000 newborns. Most of the nine children experienced suboptimal growth but otherwise normal pregnancy and delivery outcomes. All except one child developed severe neurological impairment in childhood. Conclusion. Kernicterus is still an existing disease in Denmark. The children with kernicterus experienced overall normal pregnancy and delivery outcomes but long-term outcomes were affected. Validation of the kernicterus diagnosis in the hospital register was necessary. [ABSTRACT FROM AUTHOR]

Proteoglycans (PGs) are complex macromolecules consisting of a core protein and glycosaminoglycan (GAG) side chains. PGs are important for the constitution and functioning of the connective tissue. The normal composition of the GAG side chains defines the nature of the PGs and a wide range of biological events. Deficiencies of specific enzymes involved in the linkage of GAGs to the core protein to form functional PGs, lead to a heterogeneous disease group called Linkeropathies. This is a group of multisystem conditions characterized by different phenotypes that include skeletal dysplasia and various extra-skeletal features: developmental delay/intellectual disability, ophthalmological abnormalities including blue sclerae, facial characteristics, cardiac defects, abdominal wall defects (hernias), cutis laxa, hypermobility and hypotonia. The conditions show variable severity and often overlapping phenotypes. The enzyme β-1,3-glucuronyltransferase 3, encoded by B3GAT3 , is involved in the linkage process to form functional PGs. Biallelic pathogenic variants in B3GAT3 hence lead to Linkeropathy due to loss of function or decreased activity of this enzyme. We describe a 22-year-old female patient, born of consanguineous parents. The disease history includes congenital severe joint malalignment of elbows, hips, knees and feet, hypermobility, severe kyphoscoliosis, osteoporosis with multiple fractures in childhood, congenital diaphragmatic hernia, minor dental anomalies, digital malformations, and characteristic facial features. Whole exome sequencing was performed, and homozygosity for a novel in-frame deletion in B3GAT3 , (c.61_63delCTC (p.(Leu21del))) was detected. Both unaffected parents (double second cousins) were shown to be heterozygous carriers. This is the first report to describe homozygosity for this specific in-frame deletion in B3GAT3 (p.(Leu21del)). We present a young adult phenotype and a summary of previous reported patients with other biallelic B3GAT3 -variants for comparison. Previously described patients of B3GAT3 -deficiency were, however, all children with phenotypes ranging from prenatal manifestation and early lethality to less severe. We suggest that this novel homozygous in-frame deletion in B3GAT3 may be the cause of a recessive form of Linkeropathy. [ABSTRACT FROM AUTHOR]

Introduction: Hypomelanosis of Ito was originally described as a purely cutaneous disease. Extracutaneous manifestations were described later, forming a neurocutaneous syndrome including skeletal, muscular, ocular and central nervous system symptoms.Hypomelanosis of Ito is characterized by a depigmentation along the lines of Blaschko on the trunk and extremities in certain patterns.The aim of this article was to report another case and give an overview of the related orthopedic symptoms that have been previously described. It was also our wish to contribute with recommendations for consideration with regard to bandages on eczematous rashes, especially on clubfeet.Case Presentation: A one-and-a-half-month-old boy of Caucasian background born with talipes equinovarus, or clubfoot, on his right foot presented with an eczematous rash after surgical correction and plaster bandaging.Conclusions: It is the appearance of hypopigmentation, either alone or in combination with a congenital malformation, particularly central nervous system or musculoskeletal anomalies, which should form the basis of a presumptive diagnosis. This should then lead to further investigations and should always include skin biopsies and a test for chromosomal mosaicism.We report the case of a boy with a clinical picture consisting of a depigmented skin pattern, mental retardation, pes cavus, talipes equinovarus, clinodactyly, eczema, inverted cilia of the eye, strabismus, reduced hearing, ventral hernia, glomerulonephritis, missing testicles, leg length discrepancy with scoliosis, back pain and a syrinx.It is perhaps impossible to make any conclusions about extracutaneous symptoms. However, some symptoms such as retardation, cramps and seizures, delayed development and hypotonia cannot be ignored.Because of the possibility of creating an undesirable and long postoperative period with complications, it is very important to have this diagnosis in mind when deciding to do surgery or not if there are signs of dermatological problems before surgery. In this case, it could also be good clinical practice to test the patient's reaction to plaster or other bandages. [ABSTRACT FROM AUTHOR]