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. Cite this article: Bone Jt Open 2023;4(8):580–583

Aims. Hip displacement, common in patients with cerebral palsy (CP), causes pain and hinders adequate care. Hip reconstructive surgery (HRS) is performed to treat hip displacement; however, only a few studies have quantitatively assessed femoral head sphericity after HRS. The aim of this study was to quantitatively assess improvement in hip sphericity after HRS in patients with CP. Methods. We retrospectively analyzed hip radiographs of patients who had undergone HRS because of CP-associated hip displacement. The pre- and postoperative migration percentage (MP), femoral neck-shaft angle (NSA), and sphericity, as determined by the Mose hip ratio (MHR), age at surgery, Gross Motor Function Classification System level, surgical history including Dega pelvic osteotomy, and triradiate cartilage status were studied. Regression analyses using linear mixed model were performed to identify factors affecting hip sphericity improvement. Results. A total of 108 patients were enrolled. The mean preoperative MP was 58.3% (SD 31.7%), which improved to 9.1% (SD 15.6%) at the last follow-up. NSA and MHR improved from 156.5° (SD 11.5°) and 82.3% (SD 8.6%) to 126.0° (SD 18.5°) and 89.1% (SD 9.0%), respectively. Factors affecting the postoperative MHR were preoperative MP (p = 0.005), immediate postoperative MP (p = 0.032), and history of Dega osteotomy (p = 0.046). Conclusion. We found that hip sphericity improves with HRS. Preoperative MP, reduction quality, and acetabular coverage influence femoral head remodelling. We recommend that surgeons should consider intervention early before hip displacement progresses and that during HRS, definite reduction and coverage of the femoral head should be obtained. Cite this article: Bone Joint J 2021;103-B(1):198–203

This retrospective study was to investigate radiographic and clinical outcomes in treatment of hip instability in children and young adults undergoing periacetabular osteotomy (PAO) with or without femoral osteotomy. 19 patients (21 hips) with CP were treated with PAO with or without femoral osteotomy The mean age was 16.2 years old (7 to 28 years). Five patients (5 hips) received PAO, Six patients (7 hips) PAO with femoral derotation osteotomy, Eight patients (9 hips) PAO with varus derotational osteotomy (VDRO). Anteroposterior pelvic radiographs and CT were taken to assess the migration percentage (MP), lateral center-edge angle (LCEA), Sharp angle, femoral neck anteversion, neck-shaft angle. Gross Motor Function Classification System (GMFCS) was assessed pre- and post-surgery. Complications were recorded. The mean follow-up time was 41.2 months (range, 24 to 86 months). All hips but one were pain free at final visit. The GMFCS improved by one level in 10 of 19 patients. MP improved from a mean of 76.6% to 18.6% at the final follow-up(p<0.001). The mean pre-operative LCEA and Sharp angle were −33.5 ? and 35 ? respectively, improved to 21.5 ? and 11.8 ? at the final follow-up (p < 0.001). There were six patients (7 hips) had re-subluxation at latest follow-up. Nervus cutaneus femoris lateralis was impaired in four patients after surgery. There was no re-dislocation, AVN, or infections in this group. Satisfactory clinical and radiologic results can be obtained by PAO with or without femoral osteotomy minor complications

Abstract. Objectives. Single-event multilevel surgery (SEMLS) is the standard orthopaedic treatment for gait abnormalities in children with diplegic cerebral palsy (CP). The primary aim of this study was to report the long-term functional mobility of these patients after surgery. The secondary aim was to assess the relationship between functional mobility and quality of life (QoL). Methods. Patients were included if they met the following criteria: 1) diplegic CP; 2) Gross Motor Function Classification System (GMFCS) I to III; 3) SEMLS at age ≤ 18. A total of 61 patients, mean age at surgery 11 years eight months (SD 2y 5m), were included. A mean of eight years (SD 3y 10m) after SEMLS, patients were contacted and asked to complete the Functional Mobility Scale (FMS) questionnaire over the telephone and given a weblink to complete an online version of the CP QOL Teen. FMS was recorded for all patients and CP QOL Teen for 23 patients (38%). Results. Of patients graded GMFCS I and II preoperatively, at long-term follow-up the proportion walking independently at home, school/work and in the community was 71% (20/28), 57% (16/28) and 57% (16/28), respectively. Of patients graded GMFCS III preoperatively, at long-term follow-up 82% (27/33) and 76% (25/33) were walking either independently or with an assistive device at home and school/work, respectively, while over community distances 61% (20/33) required a wheelchair. The only significant association between QoL and functional mobility was better ‘feelings about function’ in patients with better home FMS scores (r=0.55; 95% confidence interval 0.15 to 0.79; p=0.01). Conclusion. The majority of children maintained their preoperative level of functional mobility at long-term follow-up after SEMLS. Despite the favourable functional mobility, there was little evidence to establish a link between functional status and quality of life

Treatment for hip displacement in children and youth with cerebral palsy (CP) is dependent upon when the problem is detected. Hip surveillance aims to identify hip displacement early through systematic screening and, together with timely orthopaedic intervention, can eliminate the need for salvage hip procedures. Here we report the impact on surgical practice of 1) hip surveillance program advocacy and knowledge translation efforts and 2) initial population-based program implementation. A retrospective review was completed of all children with CP undergoing surgery for hip displacement at a provincial tertiary pediatric hospital in the years 2004 to 2018. Date and type of surgery, age at surgery, Gross Motor Function Classification System (GMFCS) level, and pre-operative migration percentages (MP) were collected. Surgeries were categorized as soft-tissue release, reconstructive, and salvage procedures. Results were collected for three time periods: historical (September 2004- June 2010), hip surveillance advocacy and knowledge translation (July 2010- August 2015), and post provincial hip surveillance program implementation (September 2015 – December 2018). A total of 261 surgeries on 321 hips were reviewed. The rate of salvage procedures dropped from 29% (24) of surgeries performed in the historical period to 12% (14) during the period that included targeted hip surveillance knowledge translation and development of provincial guidelines. Since implementation of the surveillance program, salvage procedures have accounted for 7% (4) of interventions performed; all of these were in patients new to the province or existing patients. During the three study periods, reconstructive surgeries accounted for 64%, 81%, and 80% of the interventions performed. The mean initial MP at time of reconstructive surgery has decreased from 66% (SD 20%) in the historical time period to 57% (SD 25%) and 57% (SD 22%) in the subsequent time periods. There were a greater number of children at GMFCS levels III and IV in the two more recent time periods suggesting surveillance may assist in identifying children at these levels of impairment. The rate of soft-tissue surgeries was low during all time periods with the number trending higher since program implementation (7%, 7%, 10%). Knowledge translation and use of standardized surveillance guidelines can have a significant impact on the prevalence of salvage hip surgeries. Centers should utilize existing surveillance guidelines and educate key stakeholders about the importance of hip surveillance in the absence of a formal hip surveillance program

Botulinum toxin A (BoNT-A) is a substance that requires repeated application due to its effectiveness being lost 12–16 weeks post application. Performing these intra-muscular injections under anesthesia reduces pain and distress during applications, ensuring effective and successful functional results. This study evaluates motor development of patients undergoing 3 or more repeated BoNT-A application in a tertiary pediatric hospital and the safety as well as effect of 3 different types of anesthesia. 75 children with cerebral palsy who underwent BoNT-A application at least three times consecutively with 6-month intervals and a total of 320 procedures admitted between January 2008 and January 2018 were retrospectively examined. Gross Motor Function Classification System (GMFCS) was employed in motor development evaluation. To observe the improvement in motor development, those with 2-1-0 level decreases in GMFCS classes were grouped and compared in terms of birth time, birth weight, cerebral palsy type and first BoNT-A application age. The 3 types of anesthesia methods (sedation analgesia, larengeal mask anesthesia (LMA) and inhalation mask anesthesia) applied during the procedures were compared in terms of sedation, procedure, recovery and total operation room time. The mean age of the children for all procedures was 45.51 ± 22.40 months. As a result of procedures, significant motor development was observed in 60 (80%) patients (p <0,000∗). No significant difference was observed when the children with cerebral palsy whose GMFCS declined in the form of level 2, 1 and unchanged were compared in terms of first application age, birth weight and gestational age. It was found that 106 (33.1%) were applied sevoflurane with anesthesia mask, 103 (32.1%) were administered sevoflurane with laryngeal mask, and 111 (34.6%) were sedation-analgesia. Only 10 out of 320 procedures were seen to develop side effects (8 vomiting, 2 bronchospasm). In the patients who underwent sedation analgesia during the first 3 BoNT-A procedures, the duration of recovery and total operating room time was seen to be significantly shorter than the others, while there was no difference between the anesthesia methods in the 4th and subsequent procedures. Regardless of the type of anesthesia, the recovery and total operating room times of those having undergone 6 or more procedures were longer than those with less than 6 procedures (p <0.009, p <0.016, respectively). As conclusion, repeated BoNT-A applications in children with CP provides progress in motor steps, it can be applied safely and effectively under anesthesia. Sedation analgesia application provides easier recovery compared to general anesthesia with LMA and mask only in the first three applications. However, recovery time increases with 4 and more repeated applications as the number of applications increases

Purpose. The purpose of this study was to analyse inter- and intra-rater reliability of the Head-Shaft angle (HSA) on radiographs from a surveillance programme for children with cerebral palsy (CP). Method. A high HSA is a risk factor for hip displacement in children with CP. To evaluate inter- and intra-rater reliability of the HSA, hip radiographs from the CP surveillance programme CPUP in the southern part of Sweden during the first half of 2016 were included in this study. Results. We analysed 50 radiographs from children in Gross Motor Function Classification System (GMFCS) levels II-V with a mean age of 6.6 (SD 3.2) years from 11 different radiology departments. Three raters measured the HSA of one hip at baseline and after four weeks. Intra-Class Correlation Coefficient (ICC) showed excellent inter- and intra-rater reliability of the HSA with ICC 0.92 (95% CI 0.87–0.96) and ICC 0.99 (95% CI 0.98–0.99) respectively. Conclusion. We conclude that the HSA, important when predicting hip displacement in CP, showed high inter-and intra-rater reliability when analysed on radiographs from various radiology departments in a surveillance program for CP

This study evaluates outcomes of hip adductor surgery in children with cerebral palsy in preventing hip displacement. This review is from the perspective of an extended follow-up (beyond 3 years in contrast to currently available literature) and the Gross Motor Function Classification System (GMFCS). A retrospective audit was performed of children with cerebral palsy aged 2 to 10 years who had primary adductor surgery at the Royal Children’s Hospital Melbourne between January 1994 and December 2004. These children had hip migration percentages (MP) greater than 30% and been followed up for a minimum 12 months post-operatively

Intramuscular injections of botulinum neuro toxin A (BoNT-A) have been a cornerstone in the treatment of spasticity for the last two decades. In India, the treatment is now offered to children with spastic cerebral palsy (CP). However, despite its use, the evidence for its functional effects is limited and inconclusive. The objective of this study is to determine whether BoNT-A makes walking easier in children with CP. We hypothesize that injections with BoNT-A will not reduce energy cost during walking, improve walking capacity, reduce pain or improve self-perceived performance and satisfaction. Between the period of 2012 and 2014, 35 children with spastic CP less than 10 years of age were included. The patients were classified according to their gross motor function classification system (GMFCS) and their pre-and post-injection gait analysis were performed. Spasticity assessed by Modified Ashworth Score [MAS]. Trained parents were utilised for the post injection physiotherapy as these children will be more complaint to them. GMFCS and MAS scoring done every three months till one year follow up. Therapeutically, effect was found in 90% of the patients, an average duration of the medical effect was 6–12 months. The improvement in GMFC functional score in serial measurements was seen in these patients though some deterioration in spasticity scores at one year. Despite mild recurrence in spasticity, majority maintained independent (42%) or assisted ambulation (48%) at one year. No major side effects occurred. Botox may prove a useful adjuvant in conservative management of the spasticity of cerebral palsy. Apart from being very cost effective in these financially deprived populations, successful management with these injections may allow delay of surgical intervention until the child is older and at less risk of possible complications, including the need for repeated surgical procedures

Aim. In an earlier study we identified severe Vitamin D deficiency as a problem in institutionalised children with cerebral palsy (CP), which resulted in rickets and a high incidence of fractures. The purpose of this study was to establish whether a cohort of non-ambulatory children with CP, living at home, presented with Vitamin D deficiency. Method. The participants were a consecutive sample (N=100) of non-ambulatory children with CP attending a CP outpatient clinic. Their ages ranged from 2 to 15 years (mean 5.8, SD 3.3 years). There were 57 males and 43 females. Nineteen were on Level IV of the Gross Motor Function Classification System (GMFCS), and 81 were on Level V. 66% were on anticonvulsant therapy (ACT). Basic demographic data was collected, and measurements included blood sample analysis and wrist radiographs. There was radiographic evidence of osteopenia and delayed ossification of the carpal bones. Results. Three participants had Vitamin D deficiency rickets confirmed by wrist changes and serology. There was a significantly higher level of Alkaline Phosphatase (p=0.04) in children on ACT than in those who did not receive ACT. Preliminary results show that one third of the children had Vitamin D deficiency. Conclusion. Non ambulatory children with CP are at risk of developing rickets. We recommend regular exposure to sunlight or Vitamin D supplementation as preventative measures. NO DISCLOSURES

Purpose. The authors have used the Edinburgh Visual Gait Score (VGS) in the management of diplegic cerebral palsy patients treated with Botulinum toxoid injections into their hamstrings muscles. Video-filmed gait episodes were recorded before and after Botox injections over a treatment period of 6 years from 2007 to 2012. Method. The video-recordings of 32 patients were available for VGS analysis. Ages ranged from 5 to 22 years, with 17 patients under the age of 12 years, and 15 over 12 years. Gross Motor Function Classification System (GMFSC) levels were accorded to: 8 patients level 1, 10 patients level 2, and 14 patients level 3; 15 were boys and 17 girls. The indication for hamstring injections were a GMFCS level of 1 to 3, age five years or older, no previous hamstring surgery, and a patient keen to have the Botox treatment. Video-recordings were taken with a Sony and a Canon Digital camera in a back-front-back view and a lateral left-right view; walking distance ap was 10 metres, lateral camera distance 5 metres. All injections of Botox were done by the senior author without anaesthetic or sedation. All assessments were done by the junior authors; they did not know the patients, the status before or after injections or repeat injections, or the dates of injections and filming of the episodes. Results. After the Botox injections into the hamstrings bilaterally, no patient changed GMFCS level status, nobody deteriorated; all could be classified in the GMFCS. Conclusion. The Edinburgh VGS is a reliable analysis method for classifying GMF levels in diplegic walking cerebral palsy patients. Video-recordings are permanent and can repeatedly be re-assessed in future; different visual parameters may be chosen for assessment. ONE DISCLOSURE

Aim. To assess the risk of hip dislocations in children with cerebral palsy (CP) attending special schools, and the effects of preventative soft tissue surgery (psoas and adductor tenotomy). Method. 110 children were reviewed from 1985-2000. Severity of CP was graded according to the Gross Motor Function Classification System (GMFCS). Hemiplegics - grade 1, Diplegics grades 2 & 3, Quadriplegics grades 4 & 5. Patients were grouped into age groups 5, 10 and 15 years and the number of hip dislocations in each group were noted. Numbers of patients who had preventative soft tissue surgery was also noted. Indication for surgery, >35% femoral head uncovering. Results. Percentage of dislocations increased with CP severity. At 15 years, no dislocations in GMFCS 2, 6% GMFCS 3, 19% GMFCS 4 and 40% in GMFCS 5. The number of patients with at least 1 hip dislocation increased with age at all age groups. GMFCS 4 (age 5 yrs 9%, age 10 yrs 20% and 15 yrs 30%). GMFCS 5 (age5 yrs 22%, age 10 yrs 48% and age 15 yrs 50%). By 15 years, 54 hips in GMFCS 2 & 3 (diplegics), preventative soft tissue surgery had not reduced chances of hip dislocation; only 1 dislocated (2%). In GMFCS 4 & 5 (quadriplegics), dislocations reduced from 40% in those without surgery to 26% in those who had preventive soft tissue surgery. This was particularly so in GMFCS 5 where there was a reduction from 83% dislocation without surgery to 33% with soft tissue preventive surgery. Conclusions. Preventive soft tissue surgery was effective in reducing the rate of hip dislocation in quadriplegic CP especially GMFCS 5

Proximal femoral deformity is common in children with cerebral palsy (CP), contributing to hip instability and ambulation difficulties. This population-based cohort study investigates the prevalence and significance of these deformities in relation to Gross Motor Function Classification System (GMFCS) level. Children with a confirmed diagnosis of CP born within a three-year period were identified from a statewide register. Motor type, topographical distribution and GMFCS level were obtained from clinical notes. Neck Shaft Angle (NSA) and Migration Percentage (MP) were measured from an anteroposterior pelvis x-ray with the hips internally rotated. Measurement of FNA was by the Trochanteric Palpation Test (TPAT) or during fluoroscopic screening of the hip with a guide wire in the centre of the femoral neck. Linear regression analysis was performed for FNA, NSA and MP according to GMFCS level. 292 children were eligible. FNA was increased in all GMFCS levels. The lowest measurements were at GMFCS levels I and II p<0.001. GMFCS levels III, IV, and V were uniformly high p<0.001. Neck shaft angle increased sequentially from GMFCS levels I to V (p<0.001). This study confirms a very high prevalence of increased FNA in children with CP in all GMFCS levels. In contrast, NSA and MP progressed step-wise with GMFCS level. We propose that increased FNA in children with CP represents failure to remodel normal fetal alignment because of delay in ambulation and muscle imbalance across the hip joint. In contrast, coxa valga is an acquired deformity and is largely related to lack of weight bearing and functional ambulation. The high prevalence of both deformities at GMFCS levels IV and V explain the high rate of displacement in these hips and the need for proximal femoral realignment surgery in the prevention and management of hip displacement

Serial casting (SC) and Botulinum toxin-A (Btx-A) have been used to treat ankle equinus contractures in cerebral palsy. Previous studies that examined the effects of combining Btx-A injections with SC and either one of the treatments alone in terms of passive ankle range of motion (PROM) have shown mixed results [two-four]. Therefore, the goal of this study is to examine PROM and gait characteristics in children with CP who have undergone SC, either with or without Btx-A injections to the plantarflexors. Patients who underwent SC +/− Btx-A injections were evaluated for improvement in PROM at the end of treatment. The participants’ age at beginning of SC treatment, Gross Motor Function Classification System (GMFCS) level, treatment duration, PROM, and gait characteristics observed by the treating physiotherapist were obtained from the charts. Only one side per treatment is included in this study (treated side for unilateral treatments, randomly chosen side for bilateral treatments). Table One shows the characteristics of the two groups. Independent samples t-tests showed that the two groups are similar in terms of age, treatment duration and pre PROM. A repeated measures ANCOVA, using the pre- and post- treatment range of motion as the within subject variables, treatment type as the between subject variable, and GMFCS and age as the covariates showed that the PROM changed significantly regardless of treatment type (p< 0.001). However, the treatment type does not influence the outcome (p=0.411). The changes in range of motion obtained from the two types of treatments were not significantly different using the independent t-test (p=0.957). Based on these results, it appears that both types of interventions resulted in significant changes in ankle passive range of motion, which is in agreement with Kay et al [4]. Similar ranges of motion at the end of the treatment were obtained from both treatments, and the treatments have similar success rates. Future work is needed to further explore the outcomes associated with serial casting only and Btx-A and serial casting treatments

This study describes the development and initial validation of a new disease-specific outcome measure of health status and burden of care in children with severe cerebral palsy. The instrument, Caregiver Priorities and Child Health Index of Life with Disabilities(CPCHILD), has thirty-six items spanning six domains:. Personal care/ADLs,. Positioning/Mobility,. Communication/Social interaction,. Comfort/Emotions & Behaviour,. Health,. Quality of life and additionally. Caregiver’s perspective on the Importance of these items to the child’s quality of life. The CPCHILD is a reliable and valid measure of caregivers’ perspectives on the health status, functional limitations, and well-being of children with severe cerebral palsy. Develop and validate a disease-specific measure of health status and well-being of children with severe cerebral palsy. The Caregiver Priorities and Child Health Index of Life with Disabilities (CPCHILD) was constructed from recommendations of caregivers, health care providers, and review of other measures. The CPCHILD has thirty-six items spanning six domains. Items are rated on an ordinal scale. Standardized scores (0–100) are reported for each domain and in total. Primary caregivers(n=77) of children(5–18yrs) with cerebral palsy, categorized by the Gross Motor Function Classification System (GMFCS) level completed the CPCHILD. Caregivers of children with severe cerebral palsy(GMFCS-IV & V), also completed the PEDI, the CHQ, and a second administration of the CPCHILD two weeks after the first. The mean CPCHILD scores for children with severe cerebral palsy(GMFCS-V) was fifty-nine (Range:35–93) and did not demonstrate the floor effects of the PEDI and CHQ. The mean CPCHILD scores for children in GMFCS levels I to V were 22.0, 38.2, 23.0, 44.5.8 and 59.3 respectively (p < 0.0001 by ANOVA). Reliability was tested in the 41/52 caregivers who reported no health status change between the twoadministrations of the CPCHILD. The intraclass correlation coefficient(ICC) was 0.94(95% CI: 0.90–0.97). Children with severe cerebral palsy often undergo interventions to improve quality of life and ease burden of care. The CPCHILD seems to be a reliable and valid measure of caregivers’ perspectives on the health status, functional limitations, and well-being of these children. Further validation in a larger mutli-centred study is planned. Funding:. Paediatric Orthopaedic Society of North America (POSNA) research grant award. Bloorview MacMillan Foundation

Aims

Reimers migration percentage (MP) is a key measure to inform decision-making around the management of hip displacement in cerebral palsy (CP). The aim of this study is to assess validity and inter- and intra-rater reliability of a novel method of measuring MP using a smart phone app (HipScreen (HS) app).

The development of spinal deformity in children with underlying neurodisability can affect their ability to function and impact on their quality of life, as well as compromise provision of nursing care. Patients with neuromuscular spinal deformity are among the most challenging due to the number and complexity of medical comorbidities that increase the risk for severe intraoperative or postoperative complications. A multidisciplinary approach is mandatory at every stage to ensure that all nonoperative measures have been applied, and that the treatment goals have been clearly defined and agreed with the family. This will involve input from multiple specialities, including allied healthcare professionals, such as physiotherapists and wheelchair services. Surgery should be considered when there is significant impact on the patients’ quality of life, which is usually due to poor sitting balance, back or costo-pelvic pain, respiratory complications, or problems with self-care and feeding. Meticulous preoperative assessment is required, along with careful consideration of the nature of the deformity and the problems that it is causing. Surgery can achieve good curve correction and results in high levels of satisfaction from the patients and their caregivers. Modern modular posterior instrumentation systems allow an effective deformity correction. However, the risks of surgery remain high, and involvement of the family at all stages of decision-making is required in order to balance the risks and anticipated gains of the procedure, and to select those patients who can mostly benefit from spinal correction.

The February 2015 Children’s orthopaedics Roundup³⁶⁰ looks at: Hip dislocation in children with CTEV: two decades of experience; Population-based prevention of DDH in cerebral palsy: 20 years’ experience; Shoulder derotation in congenital plexus palsy; Back pain in the paediatric population: could MRI be the answer?; Intercondylar fracture of the humerus in children; The Dunn osteotomy in SUFE; Radiocapitellar line a myth!; Do ‘flatfooted’ children suffer?