Ankle-foot orthoses among children with cerebral palsy: a cross-sectional population-based register study of 8,928 children living in Northern Europe.

BACKGROUND: Cerebral palsy (CP) is an umbrella term where an injury to the immature brain affects muscle tone and motor control, posture, and at times, the ability to walk and stand. Orthoses can be used to improve or maintain function. Ankle-foot orthoses (AFOs) are the most frequently used orthoses in children with CP. However, how commonly AFOs are used by children and adolescents with CP is still unknown. The aims of this study were to investigate and describe the use of AFOs in children with CP in Sweden, Norway, Finland, Iceland, Scotland, and Denmark, and compare AFO use between countries and by gross motor function classification system (GMFCS) level, CP subtype, sex, and age. METHOD: Aggregated data on 8,928 participants in the national follow-up programs for CP for the respective countries were used. Finland does not have a national follow-up program for individuals with CP and therefore a study cohort was used instead. Use of AFOs were presented as percentages. Logistic regression models were used to compare the use of AFOs among countries adjusted for age, CP subtype, GMFCS level, and sex. RESULTS: The proportion of AFO use was highest in Scotland (57%; CI 54-59%) and lowest in Denmark (35%; CI 33-38%). After adjusting for GMFCS level, children in Denmark, Finland, and Iceland had statistically significantly lower odds of using AFOs whereas children in Norway and Scotland reported statistically significantly higher usage than Sweden. CONCLUSION: In this study, the use of AFOs in children with CP in countries with relatively similar healthcare systems, differed between countries, age, GMFCS level, and CP subtype. This indicates a lack of consensus as to which individuals benefit from using AFOs. Our findings present an important baseline for the future research and development of practical guidelines in terms of who stands to benefit from using AFOs.

Leisure Time Physical Activity in Children and Young People With Cerebral Palsy: A Population-Based Study.

PURPOSE: To describe leisure time physical activity in children and young people with cerebral palsy and identify barriers and facilitators to participation. METHODS: Leisure time physical activity participation was derived from a national cerebral palsy register and associated factors were analyzed. Barriers and facilitators to participation were investigated through a survey. RESULTS: Leisure time physical activity participation was recorded. Outcomes of participation decreased with increasing Gross Motor Function Classification System level. Leisure time physical activity "not in club" for 11- to 18-year-olds was significantly lower than for those aged 5 to 10 years for Gross Motor Function Classification System level II. The survey supported that disability and disliking help were common barriers and parental encouragement and enjoyment were common facilitators. CONCLUSIONS: Data from the register and survey provide insight into factors influencing leisure time physical activity participation in young people with cerebral palsy and how to increase.

Treatment of spasticity in children and adolescents with cerebral palsy in Northern Europe: a CP-North registry study.

BACKGROUND: Spasticity is present in more than 80% of the population with cerebral palsy (CP). The aim of this study was to describe and compare the use of three spasticity reducing methods; Botulinum toxin-A therapy (BTX-A), Selective dorsal rhizotomy (SDR) and Intrathecal baclofen therapy (ITB) among children and adolescents with CP in six northern European countries. METHODS: This registry-based study included population-based data in children and adolescents with CP born 2002 to 2017 and recorded in the follow-up programs for CP in Sweden, Norway, Denmark, Iceland and Scotland, and a defined cohort in Finland. RESULTS: A total of 8,817 individuals were included. The proportion of individuals treated with SDR and ITB was significantly different between the countries. SDR treatment ranged from 0% ( Finland and Iceland) to 3.4% (Scotland) and ITB treatment from 2.2% (Sweden) to 3.7% (Denmark and Scotland). BTX-A treatment in the lower extremities reported 2017-2018 ranged from 8.6% in Denmark to 20% in Norway (p < 0.01). Mean age for undergoing SDR ranged from 4.5 years in Norway to 7.3 years in Denmark (p < 0.01). Mean age at ITB surgery ranged from 6.3 years in Norway to 10.1 years in Finland (p < 0.01). Mean age for BTX-A treatment ranged from 7.1 years in Denmark to 10.3 years in Iceland (p < 0.01). Treatment with SDR was most common in Gross Motor Function Classification System (GMFCS) level III, ITB in level V, and BTX-A in level I. The most common muscle treated with BTX-A was the calf muscle, with the highest proportion in GMFCS level I. BTX-A treatment of hamstring and hip muscles was most common in GMFCS levels IV-V in all countries. CONCLUSION: There were statistically significant differences between countries regarding the proportion of children and adolescents with CP treated with the three spasticity reducing methods, mean age for treatment and treatment related to GMFCS level. This is likely due to differences in the availability of these treatment methods and/or differences in preferences of treatment methods among professionals and possibly patients across countries.

Point prevalence and motor function of children and adolescents with cerebral palsy in Scandinavia and Scotland: a CP-North study.

AIM: To describe the point prevalence of cerebral palsy (CP) and distribution of gross and fine motor function in individuals registered in a CP-North surveillance programme. METHOD: Aggregate data of individuals with CP aged 6 to 19 years, sex, CP subtype, and gross and fine motor function levels were collected from each programme. Overall and age-specific point prevalence of CP was calculated for each programme using 95% confidence intervals. Logistic regression was used to estimate prevalence and CP subtypes with age as the covariate variable. Pearson χ2 tests were used to compare the distributions of CP subtypes, Gross Motor Function Classification System (GMFCS) levels, and Manual Ability Classification System (MACS) levels by age and between programmes. RESULTS: Among 3 759 138 individuals residing in Scandinavia and Scotland, 8278 had a diagnosis of CP (57-59% were males). The overall point prevalence of CP ranged from 2.13 to 2.32 per 1000 residents. Age-specific prevalence in each programme varied with the exception of Denmark. While the proportions of bilateral spastic CP were similar between programmes, there were variations in all other CP subtypes and in GMFCS and MACS levels. INTERPRETATION: While the results of this study may reflect real differences in CP populations between countries, they may not be clinically relevant. The variations may be attributable to differences in the year when each programme was first established, different data collection methods, and country-specific governmental policies.

Traumatic Glenohumeral Dislocation in Pediatric Patients Is Associated With a High Risk of Recurrent Instability.

BACKGROUND: The natural history of traumatic glenohumeral dislocation is well-established in young adults, but it is less clear in pediatric patients. We aimed to determine the rate of recurrent instability and medium-term functional outcome following shoulder dislocation in patients aged 14 years or younger. METHODS: All patients aged 14 years or younger who sustained a glenohumeral dislocation from 2008 to 2019 presenting to our regional health-board were identified. Patients who had subluxations associated with generalized laxity were excluded. Data was collected regarding further dislocations, stabilization surgery, sporting activity and patient-reported outcomes using the Western Ontario Shoulder Instability (WOSI) Index and Quick Disabilities of the Arm, Shoulder, and Hand score. RESULTS: Forty-one patients with a radiologically confirmed traumatic glenohumeral dislocation were suitable for study inclusion [mean age at injury 12.3 y (range: 7.2 to 14.0 y), male sex 29 (70.7%), median 7.9 y follow-up]. The incidence rate of pediatric glenohumeral dislocation was 2.5 cases per 100,000 population (aged 0 to 14 y) per year. Recurrent dislocation occurred in 43.9% (n=18/41) at a median time of 14.7 months postinjury (range: 1 to 54 mo). Skeletal maturity was associated with significantly higher proportion of recurrent instability (immature 6/24 vs. mature 12/17, P=0.01). One in 5 patients required surgical intervention for recurrent instability [mean 8 (range: 1 to 14) dislocations before surgery]. Twenty-eight patients had completed outcome questionnaires. The median modified WOSI score was 87.1% [270 (interquartile range: 65 to 795)] and the median Quick Disabilities of the Arm, Shoulder, and Hand score was 3.4 (interquartile range: 0 to 9.7). Recurrent shoulder instability was significantly associated with poorer WOSI score (unstable 71.4% vs. stable 94.3%, 95% confidence interval of the difference 6.2-36.9, P=0.04). CONCLUSIONS: Traumatic glenohumeral dislocation in patients aged 14 years or younger occurs rarely but is not a benign event. One in 2 patients experienced recurrent dislocation and 1 in 5 ultimately underwent surgical stabilization. LEVEL OF EVIDENCE: Level IV.

Distribution and motor ability of children with cerebral palsy in Scotland: a registry analysis.

BACKGROUND AND AIMS: Cerebral palsy is the commonest long-term physical disability in children with a prevalence of between 1.77 and 2.11/1000 live births. In 2013, the Cerebral Palsy Integrated Pathway Scotland (CPIPS) surveillance programme was introduced in all 14 Health Boards in Scotland and provides a standardised musculoskeletal examination of the spine and lower limbs. The purpose of this study was to report the prevalence, subtypes, motor classification and motor ability of children with cerebral palsy in Scotland. METHODS AND RESULTS: The family/carer's postal address, the child's neurological classification, motor subtypes, Gross Motor Functional Classification (GMFCS) Level and Functional Mobility Scale of 1972 children at first registration in CPIPS 2013-2018 were analysed. Their mean age at first assessment was 7.6 years. There was an overall prevalence of cerebral palsy in Scotland of 2.02/1000. GMFCS levels and Functional Mobility Scale data and prevalence were reported by Health Board and were comparable to that reported elsewhere. CONCLUSION: For the first time, data are available on the motor abilities of the total population of children with cerebral palsy in Scotland. This information will be highly relevant to resource management of current and future motor needs of these children.

How Critical is Patient Positioning in Radiographic Assessment of the Hip in Cerebral Palsy When Measuring Migration Percentage?

BACKGROUND: Migration percentage (MP) is an accepted method of assessing lateral displacement of the femoral head in children with cerebral palsy (CP). Difficulty in positioning of patients for pelvic radiography remains a concern for the reliability of the MP. METHODS: This 2-part quantitative study examined 100 anteroposterior pelvic radiographs for children with CP. Fifty were from a region that had a positioning protocol for hip surveillance of children with CP and 50 images were from a region without. Images were assessed for acceptability of position in relation to hip abduction/adduction and/or pelvic rotation.Ten images deemed Acceptable or Borderline from the region with no protocol were then randomly selected. MP was measured on 2 separate occasions by 5 children's orthopaedic surgeons and statistically analyzed for intrarater and interrater reliability. RESULTS: There was no statistically significant difference in the acceptability of images between the 2 regions with 60% to 66% of the images meeting the criteria outright. When allowances were made for slight variation of abduction/adduction within 5 degrees, 74% to 80% of the images were acceptable.Reliability was variable with limits of agreement between 4.96% and 15.15%. Observers more familiar with the software measuring package had higher reliability within and between occasions. Variability within and between observers decreased as MP increased. CONCLUSIONS: Poor positioning did not appear to be the main reason for the variation in reliability of MP. Repeat measurements were reliable although standardized technique, training, and familiarity with software measuring programmes did influence outcomes.

Meniscal suturing versus screw fixation for treatment of osteochondritis dissecans: clinical and magnetic resonance imaging results.

PURPOSE: The purpose of this study was to compare meniscal stabilization alone with screw fixation in restoring subjective and objective knee function and structural cartilage integrity in skeletally immature patients with osteochondritis dissecans (OCD) of the knee. METHODS: This study comprised 28 patients (32 knees) with magnetic resonance imaging (MRI)-confirmed OCD. Unstable menisci were treated with either stabilization (15 patients, 16 knees) or screw fixation (13 patients, 16 knees; control group). Patients were assessed by functional scoring (Hughston, International Knee Documentation Committee [IKDC]) and MRI for a minimum follow-up period of 2.5 years. RESULTS: In the meniscus stabilization group, the Hughston score improved from 1.4 to 3.8 (P < .01). There was improvement in the IKDC grade, from grade C preoperatively to grade A in 14 knees and grade B in 2 (P < .01). Of 16 knees, 15 had complete defect resolution on MRI by 1 year. There were 3 complications: 1 wound infection, 1 case of trauma requiring reoperation, and 1 revision because of treatment failure at 6 months. In the screw fixation group, the Hughston score improved from 1.4 to 3.6 (P < .01). There was a significant improvement in IKDC grade, from grade C preoperatively to grade A in 11 knees and grade B in 5 (P < .01). At 2 years postoperatively, no differences in these variables were found between the groups. CONCLUSIONS: Meniscal suturing alone for the treatment of an OCD of the knee shows good or excellent results in terms of clinical scoring and MRI assessment at almost 3 years' follow-up. The results are comparable with those of screw fixation but with a faster recovery in the postoperative course. LEVEL OF EVIDENCE: Level III, retrospective comparative study.

Poor outcome at 7.5 years after Stanisavljevic quadriceps transposition for patello-femoral instability.

INTRODUCTION: Congenital dislocation of the patella and recurrent symptomatic dislocation in adolescents are difficult pathologies to treat. Stanisavljevic described an extensive release procedure essentially involving medializing the entire lateral quadriceps and medial soft tissue stabilization. There are no significant series reporting the success of this method. This procedure has been performed in our institution over several years and we report our results. METHOD: Retrospective case series. Between 1990 and 2007, 20 knees in 13 children and adolescents (mean age 12.8 years; 4-17, 7 female) with recurrent or congenital dislocation of the patella (8 knees) underwent this procedure after failed conservative treatment (mean follow-up 7.5 years; 4-16). All were immobilized in a long leg cast for 6 weeks. RESULTS: Five knees in five patients (20 %, 1 congenital dislocation) reported their knees as improved without further dislocations. Out of the 15 knees with failures (80 %) 12 in six patients (60 %) were revised due to redislocation. Three knees in two patients (15 %) still had dislocations or subluxations, but any revision was refused. Three knees in three patients caused pain and discomfort during daily activity. Redislocation first developed after a mean of 21.3 months (4-72) postoperatively. Only one patient had returned to sport at the 12-month follow-up. DISCUSSION: The Stanisavljevic procedure produces a mediocre success rate with our long-term follow-up series showing a failure rate up to 80 %. We therefore recommend more specific procedures dealing with the anatomical deformity such as trochleaplasty to produce superior success rates.

Chronic osteomyelitis of the pelvis.

The aim of this study was to examine the aetiology and response to treatment of a series of patients with pelvic osteomyelitis. Criteria for selection were multiple positive intra-operative cultures and/or a positive radiological diagnosis. Twenty patients met these criteria (age range: 21-78 years, mean = 46). Data was recorded on host status using the Cierny-Mader classification, neurological status, causative organisms; sensitivities were recorded and the treatment and its outcome. Pelvic osteomyelitis was frequently caused by unusual organisms; a high incidence (45%) of neurologically compromised patients was noted. There were important differences in infective organisms, treatment and outcome in the paraplegic and non-paraplegic population. A high mortality and a high incidence of squamous cell carcinoma was observed. Pelvic osteomyelitis should be managed differently to long bone osteomyelitis as far as the antibiotic therapy is concerned, with a greater need for broad spectrum antibiotics in pelvic osteomyelitis. The response to surgical resection was similar to long bone osteomyelitis with a high chance of success with marginal resection in type A hosts and with wide resection in type B hosts.

Validation of a modified Care and Comfort Score and responsiveness to hip surgery in children with cerebral palsy in Gross Motor Function Classification System levels IV and V.

Aims: The purpose of this study was to assess the reliability and responsiveness to hip surgery of a four-point modified Care and Comfort Hypertonicity Questionnaire (mCCHQ) scoring tool in children with cerebral palsy (CP) in Gross Motor Function Classification System (GMFCS) levels IV and V. Methods: This was a population-based cohort study in children with CP from a national surveillance programme. Reliability was assessed from 20 caregivers who completed the mCCHQ questionnaire on two occasions three weeks apart. Test-retest reliability of the mCCHQ was calculated, and responsiveness before and after surgery for a displaced hip was evaluated in a cohort of children. Results: Test-retest reliability for the overall mCCHQ score was good (intraclass correlation coefficient 0.78), and no dimension demonstrated poor reliability. The surgical intervention cohort comprised ten children who had preoperative and postoperative mCCHQ scores at a minimum of six months postoperatively. The mCCHQ tool demonstrated a significant improvement in overall score from preoperative assessment to six-month postoperative follow-up assessment (p < 0.001). Conclusion: The mCCHQ demonstrated responsiveness to intervention and good test-retest reliability. The mCCHQ is proposed as an outcome tool for use within a national surveillance programme for children with CP.

Distal femoral osteotomy using the LCP pediatric condylar 90-degree plate in patients with neuromuscular disorders.

BACKGROUND: In patients with cerebral palsy and other neuromuscular disorders, correction of a fixed knee flexion deformity is thought to be crucial for the improvement of gait. The distal femoral extension osteotomy (DFO) is one method to achieve this goal. The standard implant for fixation of the 2 fragments in DFO is the conventional AO blade plate. The aim of this study was to report the outcome of using the new LCP Pediatric Condylar 90-Degree Plate for DFO. METHODS: Thirty-eight patients undergoing 63 DFOs were included. The mean age was 16.3±4.4 years (range, 4 to 27 y) at the time of surgery. Thirty-two patients had a diagnosis of cerebral palsy and 6 patients had other neuromuscular disorders including myelomeningocoele and arthrogryposis. Thirteen patients had unilateral procedures and 25 had bilateral procedures. RESULTS: The mean duration of the surgical intervention was 67.9±26.5 minutes (range, 30 to 180 min) and the mean blood loss was 100.0±42.1 mL (range, 50 to 250 mL). In 84% of the cases, large-fragment (5.0 mm) implants were used. The mean extension correction in 84% of the patients (n=53) was 22.8±10.3 degrees (range, 5 to 50 degrees). In this series, there were 2 complications in 63 osteotomies (3%). Radiologic follow-up of the cohort was until the time of plate removal (14.2±4.3 mo; range, 6 to 26 mo). Three months after the index operation, all osteotomies were radiologically consolidated. At this time and at plate removal, there were no malunions or nonunions in this cohort. Clinical follow-up of the cohort was performed until the end of the study (mean 35.5±6.7 mo; range, 22 to 46 mo). At the end of the study, 59 plates (94%) had been removed. CONCLUSIONS: The new LCP Pediatric Condylar 90-Degree Plate provides stable and safe fixation of distal femoral correction osteotomies in patients with neuromuscular disorders. LEVEL OF EVIDENCE: Level IV.

The CPUP Hip Score predicts displacement of the hip in children with cerebral palsy.

AIMS: The Uppföljningsprogram för cerebral pares (CPUP) Hip Score distinguishes between children with cerebral palsy (CP) at different levels of risk for displacement of the hip. The score was constructed using data from Swedish children with CP, but has not been confirmed in any other population. The aim of this study was to determine the calibration and discriminatory accuracy of this score in children with CP in Scotland. METHODS: This was a total population-based study of children registered with the Cerebral Palsy Integrated Pathway Scotland. Displacement of the hip was defined as a migration percentage (MP) of > 40%. Inclusion criteria were children in Gross Motor Function Classification System (GMFCS) levels III to V. The calibration slope was estimated and Kaplan-Meier curves produced for five strata of CPUP scores to compare the observed with the predicted risk of displacement of the hip at five years. For discriminatory accuracy, the time-dependent area under the receiver operating characteristic curve (AUC) was estimated. In order to analyze differences in the performance of the score between cohorts, score weights, and subsequently the AUC, were re-estimated using the variables of the original score: the child's age at the first examination, GMFCS level, head shaft angle, and MP of the worst hip in a logistic regression with imputation of outcomes for those with incomplete follow-up. RESULTS: The discriminatory accuracy of the score in the new population of 367 children was high (AUC 0.78 (95% confidence interval (CI) 0.71 to 0.86)). The calibration of the score was insufficient (slope 0.48 (95% CI 0.31 to 0.65)), and the absolute risks of displacement of the hip in this population were overestimated. The AUC increased with re-estimated weights (0.85 (95% CI 0.79 to 0.91)). CONCLUSION: The CPUP Hip Score had a high ability to discriminate between children at different levels of risk for displacement of the hip. The score overestimated the absolute risks of displacement in this population, which may have resulted from differences in the way children were initially registered in the two programmes. The results are promising, but the score weights may need re-estimation before its clinical application in Scotland. Cite this article: Bone Joint J 2022;104-B(5):640-644.

Hip displacement in children with cerebral palsy.

AIMS: The migration percentage (MP) is one criterion used for surgery in dislocated or displaced hips in children with cerebral palsy (CP). The MP at which a displaced hip can no longer return to normal is unclear. The aim of this paper was to identify the point of no return of the MP through a large population-based study. METHODS: All children registered on the Cerebral Palsy Integrated Pathway Scotland surveillance programme undergo regular pelvic radiographs. Any child who had a MP measuring over 35% since the programme's inception in 2013, in at least one hip and at one timepoint, was identified. The national radiography database was then interrogated to identify all pelvic radiographs for each of these children from birth through to the date of analysis. A minimum of a further two available radiographs following the initial measurement of MP ≥ 35% was required for inclusion. RESULTS: A total of 239 children (346 hips) were identified as suitable for analysis at a mean of 6.5 years (2.0 to 14.8) follow-up. In all, 1,485 radiographs taken both prior to and after a hip had a MP ≥ 35% were examined and the MP measured to identify any progression of displacement. Interrogation of the data identified that hips with a MP up to 46% returned to a MP below 40% without intervention, and all hips with a MP equal to or greater than 46% displaced further and the MP did not return to the normal range. Statistical analysis showed the result to be 98% specific with this degree of certainty that hips reaching a MP ≥ 46% would not spontaneously regress. CONCLUSION: These findings are clinically relevant in showing that it may be reasonable to continue to monitor hips with a MP not exceeding 46%. This threshold will also guide referral for further management of a displacing hip. Cite this article: Bone Joint J 2021;103-B(2):411-414.

Adolescent mid-shaft clavicular fracture displacement does not predict nonunion or inferior functional outcome at long-term follow-up.

AIMS: The aim of this study was to define the complications and long-term outcome following adolescent mid-shaft clavicular fracture. METHODS: We retrospectively reviewed a consecutive series of 677 adolescent fractures in 671 patients presenting to our region (age 13 to 17 years) over a ten-year period (2009 to 2019). Long-term patient-reported outcomes (abbreviated version of the Disabilities of the Arm, Shoulder and Hand (QuickDASH) score and EuroQol five-dimension three-level (EQ-5D-3L) quality of life score) were undertaken at a mean of 6.4 years (1.2 to 11.3) following injury in severely displaced mid-shaft fractures (Edinburgh 2B) and angulated mid-shaft fractures (Edinburgh 2A2) at a minimum of one year post-injury. The median patient age was 14.8 years (interquartile range (IQR) 14.0 to 15.7) and 89% were male (n = 594/671). RESULTS: The majority of fractures were mid-shaft (n = 606) with angulation (Edinburgh 2A2, n = 241/606, 39.8%) or displacement (Edinburgh 2B1/2, n = 263/606, 43.4%). Only 7% of the displaced mid-shaft fractures underwent acute fixation (n = 18/263). The incidence of refracture over ten years following nonoperative management of mid-shaft fractures was 3.2% (n = 19/588) and all united without surgery. Fracture type, severity of angulation, or displacement were not associated with refracture. One nonunion occurred following nonoperative management in a displaced mid-shaft fracture (0.4%, n = 1/245). Of the angulated fractures, 61 had angulation > 30°, of which 68.9% (n = 42/61) completed outcome scores with a median QuickDASH of 0.0 (IQR 0.0 to 0.6), EQ-5D-3L 1.0 (1.0 to 1.0), and 98% satisfaction with shoulder function. For the displaced fractures, 127 had displacement beyond one cortical width of bone for which completed outcome scores were provided in 72.4% (n = 92/127). Of these 15 had undergone acute fixation. Following nonoperative treatment, the median QuickDASH was 0.0 (IQR 0.0 to 2.3), EQ-5D-3L 1.0 (1.0 to 1.0), and satisfaction with shoulder function was 95%. There were no significant differences in the patients' demography or functional outcomes between operative and nonoperative treatments. CONCLUSION: Nonoperative management of adolescent mid-shaft clavicle fractures results in excellent functional outcomes at long-term follow-up. Nonunion is exceptionally rare following nonoperative management and the relative indications for surgical intervention in adults do not appear to be applicable to adolescents. Cite this article: Bone Joint J 2021;103-B(5):951-957.

Hip displacement and dislocation in a total population of children with cerebral palsy in Scotland.

AIMS: The purpose of this study was to compare the prevalence of hip displacement and dislocation in a total population of children with cerebral palsy (CP) in Scotland before and after the initiation of a hip surveillance programme. PATIENTS: A total of 2,155 children with CP are registered in the Cerebral Palsy Integrated Pathway Scotland (CPIPS) surveillance programme, which began in 2013. Physical examination and hip radiological data are collected according to nationally agreed protocols. METHODS: Age, Gross Motor Function Classification System (GMFCS) level, subtype of CP, migration percentage (MP), and details of hip surgery were analyzed for all children aged between two and 16 years taken from a time of census in March 2019 and compared to the same data from the initial registration of children in the CPIPS. Displacement of the hip was defined as a MP of between 40% and 99%, and dislocation as a MP of 100%. RESULTS: A total of 1,646 children were available for analysis at the time of the census and 1,171 at their first registration in CPIPS. The distribution of age, sex, and GMFCS levels were similar in the two groups. The prevalence of displacement and dislocation of the hip before surveillance began were 10% (117/1,171) and 2.5% (29/1,171) respectively, and at the time of the census were 4.5% (74/1,646) and 1.3% (21/1,646), respectively. Dislocation was only seen in GMFCS levels IV and V and displacement seen in 90.5% (67/74) of these levels and 9.5% (7/74) in levels I to III. In total, 138 children had undergone hip surgery during the study period. The hip redisplaced after the initial surgery in 15 children; seven of these had undergone a second procedure and at the time of the census the hips in all seven had a MP < 40. CONCLUSION: Hip surveillance appears to be effective and has reduced the prevalence of hip displacement by over half and dislocation almost by half in these children. Cite this article: Bone Joint J 2020;102-B(3):383-387.

Management of Osteogenesis Imperfecta.

Osteogenesis imperfecta (OI) is the term used to describe a group of rare inherited skeletal disorders characterized by a greatly increased risk of fragility fractures (1). Mutations in several genes can cause OI but the condition is most commonly caused by mutations of COLIA1 or COL1A2 resulting in the production of collagen which is abnormal or present in reduced amounts. Fractures in OI are particularly common during childhood but the elevated fracture risk continues throughout life. Bone mineral density (BMD) can be reduced in OI but the magnitude of increase in fracture risk is far greater than can be accounted for by low BMD, highlighting that a key mechanism of bone fragility is reduced bone quality due to defects of bone matrix and mineralization. A multidisciplinary approach is needed to optimize management of OI, with input from physicians, orthopedic surgeons, physiotherapists, occupational therapists, and other allied health professionals. Orthopedic surgery plays a key role both in the fixation of fractures and in the correction of limb deformities. Bisphosphonates have been widely used in the treatment of children and adults with OI. Although there is good evidence that they increase BMD, it is uncertain to what extent they reduce fracture risk. Clinical trials of bone anabolic drugs such as teriparatide and inhibitors of sclerostin have also been studied; although they increase BMD, studies of these agents have not been powered to look at fracture endpoints. Various other treatment modalities including denosumab, and cell therapy have been explored but haven't gained acceptance in routine clinical practice. There have been huge advances in understanding the pathogenesis of OI but these have not been accompanied by advances in treatment. This signals need for well-designed clinical trials with fracture endpoints in OI, both with existing agents and with the newer therapeutic agents that are now starting to emerge.

The effect of femoral orientation on the measurement of the head shaft angle: an ex-vivo study.

This laboratory study evaluates head shaft angle (HSA) reliability using ranges of simulated femoral orientation often seen in children with cerebral palsy. A dry femur was mounted in a jig that enabled the bone to be positioned in a range of internal and external rotation (-40° to + 40°) and flexion (0°-60°), alone or in combination. A metal wire was placed as a surrogate physis to give two HSA angles of 140° and 160°. Radiographs were taken of the femur in differing combinations of rotation, flexion and the two HSA angles. The HSA was measured by four independent observers on two separate occasions. Intraclass correlation coefficients (ICCs) were used to assess interobserver and intraobserver reliability. The HSA was accurately measured within ± 5° when the femur was positioned between 20° internal rotation and 40° external rotation. Flexion up to 60° did not affect the accuracy of the measurement. The interobserver reliability for the HSA was excellent with an ICC of 0.9970 [95% confidence interval (CI): 0.9995-0.9983] for the first measurement and 0.9988 for the second (95% CI: 0.9979-0.9993, all P < 0.01). The intraobserver reliability was also excellent with an ICC of not less than 0.990 for all four observers (95% CI: 0.9806-0.9986, all P < 0.01). There was excellent interobserver and intraobserver reliability when measuring the HSA in an experimental model provided femoral rotation lay within 20° internal and 40° external rotation and less than 60° of flexion.

Five-year results of the Sigma total knee arthroplasty.

The DePuy Sigma total knee arthroplasty (TKA) was introduced in 1997 as a modification of the Press Fit Condylar Knee (PFC) TKA and has been used extensively in the United Kingdom and worldwide. It is the most commonly used TKA in England and Wales, where it accounts for 36% of all primary TKA. The PFC was well established, with reported 10-year survival rates of 93-97%, but this study reports the first 5-year clinical and radiographic follow-up data for the Sigma TKA. Over a 10-month period, 212 Sigma TKAs were performed in 180 patients. Patients were seen at a specialist nurse-led clinic 7 to 10 days before admission and at 6 months, 18 months, 3 years and 5 years after surgery. Data were recorded prospectively at each visit. Radiographs were obtained at the 5-year follow-up appointment. Of 212 knees, 178 (151 patients) were alive at 5 years. Three were lost to follow up. Six knees (3.0%) were revised, five for infection and one underwent change of polyethylene insert at 4.9 years. Five-year survival with an endpoint of revision for any reason was 97.0%; with an endpoint of revision for aseptic failure it was 99.5%. The median American Knee Society knee rating score was 93 out of 100 at 5 years compared with 25 out of 100 at admission. Of 147 radiographs, none showed radiographic loosening of either component. Seventeen (11.6%) showed radiolucent lines. Twenty-eight (19.0%) had alignment outside the range of 7+/-3 degrees valgus. These results suggest that the Sigma TKA gives acceptable clinical results after 5 years. Further follow-up studies are required to see if this performance is maintained in the long term.