Fifteen Years of Spinal Fusion Outcomes in Children With Cerebral Palsy: Are We Getting Better?

STUDY DESIGN: Retrospective multicenter study. OBJECTIVE: We reviewed 15-year trends in operative factors, radiographic and quality of life outcomes, and complication rates in children with cerebral palsy (CP)-related scoliosis who underwent spinal fusion. SUMMARY OF BACKGROUND DATA: Over the past two decades, significant efforts have been made to decrease complications and improve outcomes of this population. MATERIALS AND METHODS: We retrospectively reviewed a multicenter registry of pediatric CP patients who underwent spinal fusion from 2008 to 2020. We evaluated baseline and operative, hospitalization, and complication data as well as radiographic and quality of life outcomes at a minimum 2-year follow-up. RESULTS: Mean estimated blood loss and transfusion volume declined from 2.7±2.0 L in 2008 to 0.71±0.34 L in 2020 and 1.0±0.5 L in 2008 to 0.5±0.2 L in 2020, respectively, with a concomitant increase in antifibrinolytic use from 58% to 97% (all, P <0.01). Unit rod and pelvic fusion use declined from 33% in 2008 to 0% in 2020 and 96% in 2008 to 79% in 2020, respectively (both, P <0.05). Mean postoperative intubation time declined from 2.5±2.6 to 0.42±0.63 days ( P< 0.01). No changes were observed in preoperative and postoperative coronal angle and pelvic obliquity, operative time, frequency of anterior/anterior-posterior approach, and durations of hospital and intensive care unit stays. Improvements in the Caregiver Priorities and Child Health Index of Life with Disabilities postoperatively did not change significantly over the study period. Complication rates, including reoperation, superficial and deep surgical site infection, and gastrointestinal and medical complications remained stable over the study period. CONCLUSIONS: Over the past 15 years of CP scoliosis surgery, surgical blood loss, transfusion volumes, duration of postoperative intubation, and pelvic fusion rates have decreased. However, the degree of radiographic correction, the rates of surgical and medical complications (including infection), and health-related quality of life measures have broadly remained constant.

Validation of the French version of the Caregivers' Priorities and Child Health Index of Life with Disabilities questionnaire.

INTRODUCTION: The Caregivers' Priorities and Child Health Index of Life with Disabilities (CPCHILD) is a questionnaire that measures the health-related quality of life (HRQL) of children with cerebral palsy (CP). Though measuring HRQL is challenging in these children, it is a valuable help for medical decision-making. There is no questionnaire to assess HRQL in French-speaking children with severe CP. OBJECTIVE: To translate and adapt transculturally the CPCHILD questionnaire into French (CPCHILD-FV). MATERIAL AND METHODS: The CPCHILD was translated from English into French by forward and backward translation by independents translators. The questionnaire was then tested on 32 caregivers of patients with CP classified as GMFCS IV or V, remarks of caregivers were analyzed by an expert committee and, if necessary, modifications were performed. Internal consistency of the CPCHILD-FV was assessed using a sample of 32 parents or caregivers and test-retest reliability was assessed on a random sample of 10 patients. RESULTS: The translation and transcultural process resulted in a French version of the CPCHILD. Some items of the CPCHILD required careful discussion to ensure that items had the same meaning as in the original. Internal consistencies were over 0.70 for each domain except for health, and 0.97 for the total scores. The ICC for the test-retest reliability of the CHILD-FV total score was 0.98 (95% CI: 0.93-0.99) and ranged from 0.59 to 0.99 for the domains. CONCLUSION: The translation and cross-cultural adaptation of the CPCHILD questionnaire provides a French version than can measure the HRQL of children with severe CP. LEVEL OF EVIDENCE: IV; prospective study without control group.

Current Concept and Management of Spastic Hip in Children: A Narrative Review.

Cerebral palsy (CP) is a non-progressive motor condition that hinders the development of movement and posture. One of the common problems faced in CP is spastic hips, which can cause discomfort, deformity, and functional restrictions. This review article seeks to offer a thorough summary of the most recent methods for treating spastic hips in cerebral palsy patients. Additionally, it describes the success and potential risks of various conservative and surgical procedures. It also looks at new treatments and potential avenues for managing this complicated ailment.

[Hungarian adaptation of the Caregiver Priorities and Child Health Index of Life with Disabilities questionnaire]. / Cerebralis paresisben szenvedo gyermekek egészségindexe.

INTRODUCTION: Caregiver Priorities and Child Health Index of Life with Disabilities (CPCHILD) questionnaire is a measurement tool for assessing health status and wellbeing of disabled children, which evaluates children's quality of life from the caregiver point of view. OBJECTIVE: The aim of our work was the Hungarian translation and cultural adaptation of the questionnaire and also validation of the CPCHILD on Hungarian cerebral palsy patients. Further aim was to test the eligibility of the questionnaire for superficial assessment of caregiver's psychological attitudes. METHOD: Translation of the questionnaire was carried out according to the Beaton's guidelines. Test-retest, interrater reliability (ICC) and also internal consistency (Cronbach-alpha) were calculated for reliability. The importance of the questions was assessed for face validity and known group validity test was done to measure construct validity. For examining parental attitudes, the patients were divided into ambulatory and non-ambulatory groups and the 36 quality of life questions of the 7th domain were used to find relations. RESULTS: During test-retest reliability measurements, the ICC was 0.96 (95% CI: 0.88-0.98), and Cronbach-alpha exceeded the minimal expected value of 0.7 (0.74-0.97) except in the 5th domain (0.67), while measuring interrater reliability the ICC was 0.87 (95% CI 0.70-0.94). Face validity was above the 2.0 threshold in every question (2.6-4.5; mean: 3.4 ± 1.34) and the known group validity calculations showed significant differences between the CPCHILD scores of ambulatory and non-ambulatory groups. Examining parental attitudes, a significant difference was also shown among the parents of ambulatory and non-ambulatory children in assessing the importance of sitting in the quality of life of their children (2.89 ± 1.28 vs. 3.51 ± 0.82; p<0.01). CONCLUSION: The final outcome of our study is that CPCHILD questionnaire has become widely accessible in Hungarian language. Our result, that the answers referring to the sitting abilities and the activities should be carried out in sitting position, was significantly different among the caregivers of the ambulatory and non-ambulatory children, showing that the parents of the GMFCS IV and V category children evaluate the importance of sitting ability higher compared to those parents who care for GMFCS I, II and III category children. Furthermore, the results draw attention to the wellbeing and health of the children measurable with CPCHILD as well as that parental caregiver attitudes can be recognized which may give further help in finding the balance between expectations and possibilities during the rehabilitation of cerebral palsy children. Orv Hetil. 2023; 164(16): 610-617.

Reliability of assessing proximal femur geometry with Rutz classification schema in patients with cerebral palsy.

Our investigation aimed to assess the reliability of the femoral head shape classification system devised by Rutz et al . and observe its application in patients with cerebral palsy (CP) at different skeletal maturity levels. Four independent observers assessed anteroposterior radiographs of the hips of 60 patients with hip dysplasia associated with non-ambulatory CP (Gross Motor Function Classification System levels IV and V) and recorded the femoral head shape radiological grading system as described by Rutz et al . Radiographs were obtained from 20 patients in each of three age groups: under 8 years, between 8 and 12 years and above 12 years old, respectively. Inter-observer reliability was assessed by comparing the measurements of four different observers. To determine the intra-observer reliability, radiographs were reassessed after a 4-week interval. Accuracy was checked by comparing these measurements with the assessment of expert consensus. Validity was checked indirectly by observing the relationship between the Rutz grade and the migration percentage. The Rutz classification system's evaluation of femoral head shape showed moderate to substantial intra- and inter-observer reliability (mean κ  = 0.64 for intraobserver and mean κ  = 0.5 for interobserver). Specialist assessors had slightly higher intra-observer reliability than trainee assessors. The grade of femoral head shape was significantly associated with increasing migration percentage. Rutz's classification was shown to be reliable. Once the clinical utility of this classification can be established, it has the potential for broad application for prognostication and surgical decision-making and as an essential radiographic variable in studies involving the outcomes of hip displacement in CP. Level of evidence: III.

Epilepsy and proxy-reported health-related quality of life in children and young people with non-ambulatory cerebral palsy.

AIM: To assess the association between epilepsy characteristics and proxy-reported health-related quality of life (HRQoL) in children and young people with non-ambulatory cerebral palsy (CP) and seizures. METHOD: This was a cross-sectional study of 164 children and young people (74 females, 90 males; mean age 10 years 6 months, range 2-21 years, SD 5 years 5 months). Caregivers completed the Child Health Index of Life with Disabilities (CPCHILD) in an outpatient setting. We utilized univariable linear regression and multivariable modeling to study relationships between variables and CPCHILD scores. RESULTS: Gross Motor Function Classification System levels were 37% IV and 63% V. Sociodemographic factors included the Child Opportunity Index (median 51, interquartile range [IQR] 25-80). A median of 2 (IQR 1-3) antiseizure medications (ASMs) were used, and days with seizures ranged from 0 (30%) to 28 (20%) days in the previous 4 weeks. Total CPCHILD scores decreased 2.3 points for each ASM (95% confidence interval [CI] -4.1 to -0.42). Compared to persons with focal epilepsy, those with generalized epilepsy had lower total CPCHILD scores (-5.7; 95% CI -11 to -0.55). Number of days with seizures was not associated with total CPCHILD scores. INTERPRETATION: Proxy-reported HRQoL was affected by epilepsy-specific features in children and young people with severe CP. WHAT THIS PAPER ADDS: Health-related quality of life (HRQoL) was lower with increasing numbers of antiseizure medications. Overall quality of life (QoL) scores were lower by a similar amount, independent of seizure frequency. HRQoL was lower in persons with recent hospital admissions for epilepsy.

Comparison of intrathecal baclofen pump insertion and selective dorsal rhizotomy for nonambulatory children with predominantly spastic cerebral palsy.

OBJECTIVE: In nonambulatory children with predominantly spastic cerebral palsy (CP), the authors compared care needs, symptom burden, and complications after surgical treatment with either intrathecal baclofen (ITB) pump insertion or selective dorsal rhizotomy (SDR). The patients were treated at two Canadian centers with variability in practice pertaining to these surgical options. METHODS: The authors performed a retrospective analysis of nonambulatory children with predominantly spastic quadriplegic or diplegic CP who underwent treatment with ITB or SDR. These two strategies were retrospectively assessed by comparing patient data from the two treatment groups for demographic characteristics, outcomes, and complications. A partial least-squares analysis was performed to identify patient phenotypes associated with outcomes. RESULTS: Thirty patients who underwent ITB and 30 patients who underwent SDR were included for analysis. Patients in the ITB group were older and had lower baseline functional status, with greater burdens of spasticity, dystonia, pain, deformity, bladder dysfunction, and epilepsy than patients in the SDR group. In addition, children who underwent SDR had lower Gross Motor Function Classification System (GMFCS) levels and were less likely to experience complications than those who underwent ITB. However, children treated with SDR had fewer improvements in pain than children treated with ITB. A single significant latent variable explaining 88% of the variance in the data was identified. CONCLUSIONS: Considerable baseline differences exist within this pediatric CP patient population. Factors specific to individual children must be taken into account when determining whether ITB or SDR is the appropriate treatment.

Test-Retest Reliability and Construct Validity of the German Translation of the Gait Outcome Assessment List (GOAL) Questionnaire for Children with Ambulatory Cerebral Palsy.

The Gait Outcome Assessment List (GOAL) is a patient or caregiver-reported assessment of gait-related function across different domains of the International Classification of Functioning, Disability, and Health (ICF) developed for ambulant children with cerebral palsy (CP). So far, the questionnaire is only available in English. The aim of this study was to translate the GOAL into German and to evaluate its reliability and validity by studying the association between GOAL scores and gross motor function as categorized by the gross motor function classification system (GMFCS) in children with cerebral palsy (CP). The GOAL was administered to primary caregivers of n = 91 children and adolescents with CP (n = 32, GMFCS levels I; n = 27, GMFCS level II; and n = 32, GMFCS level III) and n = 15 patients were capable of independently completing the whole questionnaire (GMFCS level I). For assessing test-retest reliability, the questionnaire was completed for a second time 2 weeks after the first by the caregivers of n = 36 patients. Mean total GOAL scores decreased significantly with increasing GMFCS levels with scores of 71 (95% confidence interval [CI]: 66.90-74.77) for GMFCS level I, 56 (95% CI: 50.98-61.86) for GMFCS level II, and 45 (95% CI: 40.58-48.48) for GMFCS level III, respectively. In three out of seven domains, caregivers rated their children significantly lower than children rated themselves. The test-retest reliability was excellent as was internal consistency given the GOAL total score. The German GOAL may serve as a much needed patient-reported outcome measure of gait-related function in ambulant children and adolescents with CP.

The Gait Outcomes Assessment List for Children With Lower Limb Difference (GOAL-LD): Assessment of Reliability and Validity.

BACKGROUND: The Gait Outcomes Assessment List for children with Lower Limb Difference (GOAL-LD) is a patient and parent-reported outcome measure that incorporates the framework of the International Classification of Functioning, Disability, and Health. This prospective multicenter cohort study evaluates the validity and reliability of the GOAL-LD and the differences between parent and adolescent report. METHOD: One hundred thirty-seven pediatric patients aged over 5 years attending limb reconstruction clinics at the participating sites were assessed at baseline, and a self-selected cohort also completed an assessment 2 to 6 weeks later. Construct and criterion validity were assessed by comparing GOAL-LD scores with a measure of limb deformity complexity (LLRS-AIM) and the Pediatric Outcomes Data Collection Instrument, using Spearman correlation coefficients. Face and content validity were determined through ratings of item importance. Test-retest reliability was reported as an intraclass correlation coefficient and internal consistency using Cronbach α. Adolescent reports were compared with their parents using paired t tests. RESULTS: The GOAL-LD demonstrated a moderate negative correlation with the LLRS-AIM (r=-0.40, P<0.001) and was able to discriminate between deformity complexity groups as defined by the LLRS-AIM (χ2=11.43, P=0.022). Internal consistency was high across all domains (α≥0.68 to 0.97). Like domains of the Pediatric Outcomes Data Collection Instrument and the GOAL-LD were well correlated. Parents reported a lower total GOAL-LD score when compared with adolescents (mean difference 3.04; SE 1.06; 95% confidence interval, 0.92-5.16; P<0.01); however this difference was only significant for body image and self-esteem (Domain F) and gait appearance (Domain D). Test-retest reliability remained high over the study period (intraclass correlation coefficient 0.85; SE 0.03; 95% confidence interval, 0.77-0.91). CONCLUSIONS: The GOAL-LD is a valid and reliable self and parent-reported outcome measure for children with lower limb difference. Parents report a lower level of function and attribute a higher importance to items when compared with their children. The GOAL-LD helps to communicate parent and child perspectives on their function and priorities and therefore has the capacity to facilitate family centered treatment planning and care. LEVEL OF EVIDENCE: Level II-diagnostic. Prospective cross-sectional and a longitudinal cohort design.

Development of the gait outcomes assessment list for lower-limb differences (GOAL-LD) questionnaire: a child and parent reported outcome measure.

BACKGROUND: To develop a priority-based patient/parent reported outcome measure for children with lower-limb differences (LD) by adapting the Gait Outcomes Assessment List (GOAL) questionnaire. METHODS: Guided by a conceptual framework of patient priorities, the GOAL questionnaire was iteratively modified and its sensibility evaluated by field-testing it on children with LD, and their parents. Cognitive interviews were conducted with a subgroup of these children, and an e-survey administered to a multidisciplinary group of health care professionals with expertise in paediatric LD. Findings were integrated to create the final version of the GOAL-LD. RESULTS: Twenty-five children (9-18 years), 20 parents, and 31 healthcare professionals evaluated the content and sensibility of the GOAL, with an emphasis on the relevance and importance of the items to patients' health related quality of life (HRQL). This resulted in the retention of 26 of the original 50 items, elimination of 12, modification of 12, and addition of seven new items. The new 45-item GOAL-LD questionnaire was shown to be sensible, and its content deemed important. CONCLUSIONS: The GOAL-LD questionnaire has a high level of face and content validity, and sensibility. It comprehensively captures the HRQL goals and outcomes that matter to children with LD and their parents. Following further psychometric evaluation, the GOAL-LD may serve as a much needed patient and parent reported outcome measure for this population.

Variability in Postoperative Immobilization and Rehabilitation Following Reconstructive Hip Surgery in Nonambulatory Children With Cerebral Palsy.

BACKGROUND: Despite being a common procedure, there are no standard protocols for postoperative immobilization and rehabilitation following reconstructive hip surgery in children with cerebral palsy (CP). The purpose of this study was to investigate variability in postoperative management and physical therapy (PT) recommendations among orthopaedic surgeons treating hip displacement in children with CP. METHODS: An invitation to participate in an anonymous, online survey was sent to 44 pediatric orthopaedic surgeons. The case of a child undergoing bilateral femoral varus derotation osteotomies and adductor tenotomies was presented. Surgeons were asked to consider their typical practice and the case scenario when answering questions related to immobilization, weight-bearing, and rehabilitation. Recommendations with increasingly complex surgical interventions and different age or level of motor function were also assessed. RESULTS: Twenty-eight orthopaedic surgeons from 9 countries with a mean 21.3 years (range: 5 to 40 y) of experience completed the survey. Postoperative immobilization was recommended by 86% (24/28) of respondents with 7 different methods of immobilization identified. All but 1 (23/24) reported immobilizing full time. Most (20/23) reported using immobilization for 4 to 6 weeks. Return to weight-bearing varied from 0 to 6 weeks for partial weight-bearing and 0 to 12 weeks for full weight-bearing. PT in the first 1 to 2 weeks postoperatively was reported as unnecessary by 29% (8/28) of surgeons. PT for range of motion, strengthening, and return to function was recommended by 96% (27/28) of surgeons, starting at a mean of 2.6 weeks postoperatively (range: 0 to 16 wk). Only 48% (13/27) reported all of their patients would receive PT for these goals in their practice setting. Inpatient rehabilitation was available for 75% (21/28) but most surgeons (17/21) reported this was accessed by 20% or fewer of their patients. CONCLUSIONS: Postoperative immobilization and PT recommendations were highly variable among surgeons. This variability may influence surgical outcomes and complication rates and should be considered when evaluating procedures. Further study into the impact of postoperative immobilization and rehabilitation is warranted.

Differences in responses to English and Korean versions of the Caregiver Priorities & Child Health Index of Life with Disabilities (CPCHILD).

BACKGROUND: The purpose of this study was to identify differences in caregiver responses to Korean-language and English-language versions of the Caregiver Priorities & Child Health Index of Life with Disabilities (CPCHILD) questionnaire. METHODS: Patient data were acquired from the Cerebral Palsy Hip Outcomes Project database, which was established to run a large international multicenter prospective cohort study of the outcomes of hip interventions in cerebral palsy. Thirty-three children whose caregivers had completed the Korean version of CPCHILD were matched by propensity scoring with 33 children whose parents completed the English version. Matching was performed on the basis of 12 covariates: age, gender, gross motor function classification system level, migration percentage of right and hip, seizure status, feeding method, tracheostomy status, pelvic obliquity, spinal deformity, parental report of hip pain and contracture interfering with care. RESULTS: There were no significant differences in CPCHILD scores for section 4 (Communication and Social Interaction), and section 5 (Health) between two groups. Korean-language CPCHILD scores were significantly lower than English-language CPCHILD scores for section 1 (Personal Care/Activities of Daily Living), section 2 (Positioning, Transferring and Mobility), section 3 (Comfort and Emotions) and section 6 (Overall Quality of Life) as well as in terms of total score. CONCLUSIONS: Cultural influences, and the community or social environment may impact the caregivers' perception of the health-related quality of life of their children. Therefore, physicians should consider these differences when interpreting the study outcomes across different countries.

Decision aids available for parents making end-of-life or palliative care decisions for children: A scoping review.

AIM: To identify and assess the quality of decision aids that align the decision, values and information provided for parents making end-of-life or palliative care decisions for children with life-threatening conditions. METHODS: Six databases and the grey literature were searched in December 2018. Two reviewers independently reviewed database citations, and one reviewed grey literature citations. Citation chaining via Scopus was conducted. Quality was assessed using IPDAS Collaboration Criteria. RESULTS: After reviewing 18 671 database citations and 10 988 grey literature citations, 18 citations describing 11 decision aids remained. Decision aids targeted premature infants, children requiring airway management, children with cancer and children with scoliosis. Three aids underwent testing beyond initial development. Quality scores averaged 27 of 50 points. CONCLUSIONS: There are few high-quality decision aids available for use and a lack evidence of widespread clinical use. Additional research is needed to support systematic development and the use of decision aids with families.

Improving Health-related Quality of Life for Patients With Nonambulatory Cerebral Palsy: Who Stands to Gain From Scoliosis Surgery?

INTRODUCTION: It is unclear what factors influence health-related quality of life (HRQOL) in neuromuscular scoliosis. The aim of this study was to evaluate which factors are associated with an improvement in an HRQOL after spinal fusion surgery for nonambulatory patients with cerebral palsy (CP). METHODS: A total of 157 patients with nonambulatory CP (Gross Motor Function Classification System IV and V) with a minimum of 2-year follow-up after PSF were identified from a prospective multicenter registry. Radiographs and quality of life were evaluated preoperatively and 2 years postoperatively. Quality of life was evaluated using the validated Caregiver Priorities and Child Health Index of Life with Disabilities (CPCHILD) questionnaire. Patients who had an increase of 10 points or greater from baseline CPCHILD scores were considered to have meaningful improvement at 2 years postoperatively. 10 points was chosen as a threshold for meaningful improvement based on differences between Gross Motor Function Classification System IV and V patients reported during the development of the CPCHILD. Perioperative demographic, clinical, and radiographic variables were analyzed to determine predicators for meaningful improvement by univariate and multivariate regression analysis. RESULTS: A total of 36.3% (57/157) of the patients reported meaningful improvement in CPCHILD scores at 2 years postoperatively. Preoperative radiographic parameters, postoperative radiographic parameters, and deformity correction did not differ significantly between groups. Patients who experienced meaningful improvement from surgery had significantly lower preoperative total CHPILD scores (43.8 vs. 55.2, P<0.001). On backwards conditional binary logistic regression, only the preoperative comfort, emotions, and behavior domain of the CPCHILD was predictive of meaningful improvement after surgery (P≤0.001). CONCLUSION: Analysis of 157 CP patients revealed a meaningful improvement in an HRQOL in 36.3% of the patients. These patients tended to have lower preoperative HRQOL, suggesting more "room for improvement" from surgery. A lower score within the comfort, emotions, and behavior domain of the CPCHILD was predictive of meaningful improvement after surgery. Radiographic parameters of deformity or curve correction were not associated with meaningful improvement after surgery. LEVEL OF EVIDENCE: Level II-retrospective review of prospectively collected data.

Cerebral Palsy Research Network Clinical Registry: Methodology and Baseline Report.

OBJECTIVE: To apply practice-based evidence to clinical management of cerebral palsy (CP). The process of establishing purpose, structure, logistics, and elements of a multi-institutional registry and the baseline characteristics of initial enrollees are reported. DESIGN: A consensus-building process among consumers, clinicians, and researchers used a participatory action process. SETTING: Community, hospitals, and universities. PARTICIPANTS: More than 100 clinicians, researchers, and consumers and more than 1858 enrollees in the registry. MAIN OUTCOME MEASURES: Not applicable. RESULTS: Consensus was that the purpose of registry was to (1) quantify practice variation, (2) facilitate quality improvement (QI), and (3) perform comparative effectiveness research (CER). Collecting data during routine clinical care using the electronic medical record was determined to be a sustainable plan for data acquisition and management. Clinicians from multiple disciplines defined salient characteristics of individuals and interventions for the registry elements. The registry was central to the clinical research network, and a leadership structure was created. A leading electronic health record platform adopted the registry elements. Twenty-four sites have initiated the data collection process and agreed to export data to the registry. Currently 12 are collecting data. Number of enrollees and characteristics were similar to other population registers. CONCLUSIONS: This is the first multi-institutional CP registry that contains the patient and treatment characteristics needed for QI and CER. The Cerebral Palsy Research Network registry elements are implemented in a versatile electronic platform and minimize burden to clinicians. The resultant registry is available for any institution to participate and is growing rapidly.

Intrathecal baclofen versus selective dorsal rhizotomy for children with cerebral palsy who are nonambulant: a systematic review.

OBJECTIVE: Cerebral palsy (CP) is the most common childhood physical disability. Historically, children with hypertonia who are nonambulatory (Gross Motor Function Classification System [GMFCS] level IV or V) were considered candidates for intrathecal baclofen (ITB) therapy to facilitate care and mitigate discomfort. Selective dorsal rhizotomy (SDR) was often reserved for ambulant children to improve gait. Recently, case series have suggested SDR as an alternative to ITB in selected children functioning at GMFCS level IV/V. The objective for this study was to systematically review the evidence for ITB and SDR in GMFCS level IV or V children. METHODS: Medline, Embase, Web of Science, and Cochrane databases were systematically searched. Articles were screened using the following inclusion criteria: 1) peer-reviewed articles reporting outcomes after SDR or ITB; 2) outcomes reported using a quantifiable scale or standardized outcome measure; 3) patients were < 19 years old at the time of operation; 4) patients had a diagnosis of CP; 5) patients were GMFCS level IV/V or results were reported based on GMFCS status and included some GMFCS level IV/V patients; 6) article and/or abstract in English; and 7) primary indication for surgery was hypertonia. Included studies were assessed with the Risk of Bias in Non-Randomized Studies - of Interventions (ROBINS-I) tool. RESULTS: Twenty-seven studies met inclusion criteria. The most commonly reported outcomes were spasticity (on the Mean Ashworth Scale) and gross motor function (using the Gross Motor Function Measure), although other outcomes including frequency of orthopedic procedures and complications were also reported. There is evidence from case series that suggests that both ITB and SDR can lower spasticity and improve gross motor function in this nonambulatory population. Complication rates are decidedly higher after ITB due in part to the ongoing risk of device-related complications. The heterogeneity among study design, patient selection, outcome selection, and follow-up periods was extremely high, preventing meta-analysis. There are no comparative studies, and meaningful health-related quality of life outcomes such as care and comfort are lacking. This review is limited by the high risk of bias among included studies. Studies of SDR or ITB that did not clearly describe patients as being GMFCS level IV/V or nonambulatory were excluded. CONCLUSIONS: There is a lack of evidence comparing the outcomes of ITB and SDR in the nonambulatory CP population. This could be overcome with standardized prospective studies using more robust methodology and relevant outcome measures.

Should Proximal Femoral Implants be Removed Prophylactically or Reactively in Children With Cerebral Palsy?

BACKGROUND: Implants are commonly used to stabilize proximal femoral osteotomies in children with cerebral palsy (CP). Removal of implants is common practice and believed to avoid infection, fracture, or pain that might be associated with retained hardware. There is little evidence to support a prophylactic strategy over a reactive approach based on symptoms. The aim of this study was to compare the outcomes of prophylactic and reactive approaches to removal of proximal femoral implants in children with CP. METHODS: An intention-to-treat model was used to compare 2 institutions that followed a prophylactic (within ∼1 y) and reactive (following complication/symptoms) approach to hardware removal, respectively. Patients with CP who had femoral implants placed at or before age 16, and had ≥2-year postsurgical follow-up were included. Demographics, surgical details, reasons for removal, and complications were recorded. χ and t tests were used. RESULTS: Six hundred twenty-one patients (prophylactic=302, reactive=319) were followed for an average of 6 years (range, 2 to 17 y). Two hundred eighty-seven (95%) implants were removed in the prophylactic group at 1.2 years. In the reactive group, 64 (20%) implants were removed at an average of 4.2 years. Reasons for removal included pain; infection; fracture; or for repeat reconstruction. The rate of unplanned removals due to fracture or infection was higher in the reactive group (4.7% vs. 0.7%, P=0.002), but there was no difference in the rate of complications during/after removal between the 2 groups (1.7% vs. 3.1%; P=0.616). No specific risk factor associated with unplanned removal could be identified; but children under 8 years old seemed more likely to undergo later removal (odds ratio 1.98; 95% confidence interval, 0.99-3.99). CONCLUSIONS: Eighty percent of patients in the reactive removal strategy avoided surgery. This group did have a 4% higher rate of fracture or infection necessitating unplanned removal but these were successfully treated at time of removal with no difference in complication rates associated with removal between both groups. One would need to remove implants from 25 patients to avoid 1 additional complication, providing some support for a reactive approach to removal of proximal femoral implants in this population. LEVEL OF EVIDENCE: Level III-therapeutic.

Validation of the Caregiver Priorities and Child Health Index of Life with Disabilities (CPCHILD) in a sample of Turkish non-ambulatory children with cerebral palsy.

AIM: The aim of this study was to translate and transculturally adapt the Caregiver Priorities and Child Health Index of Life with Disabilities questionnaire into the Turkish language and test the reliability and validity. MATERIAL AND METHODS: Eighty-two children with cerebral palsy and their parents were included in the study. The majority of children had spastic cerebral palsy. According to the Gross Motor Function Classification System, 26 children were level III, 30 children were level IV, and 26 children were level V. International accepted guidelines were used in the transcultural adaptation and validation process. Reliability was assessed through statistical analysis of the test results for test-retest and internal consistency. To assess construct validity, Caregiver Priorities and Child Health Index of Life with Disabilities was compared with the Child Health Questionnaire Parent Form. Concurrent validity was assessed by examining how Caregiver Priorities and Child Health Index of Life with Disabilities scores changed according to Gross Motor Function Classification System levels. RESULTS: The mean total score of Caregiver Priorities and Child Health Index of Life with Disabilities was 58.34±26.39. The intraclass correlation coefficient for the total questionnaire score was 0.75, ranging from 0.43 to 0.89 for six domains. Cronbach's alpha was above 0.80 in all domains of Caregiver Priorities and Child Health Index of Life with Disabilities, except the health domain. The construct validity was good because there was a positive correlation between total Child Health Questionnaire Parent Form and Caregiver Priorities and Child Health Index of Life with Disabilities scores (r=0.58, p<0.01) according to the Pearson correlation analysis. Caregiver Priorities and Child Health Index of Life with Disabilities scores were found to be different between Gross Motor Function Classification System levels (p<0.05). CONCLUSION: This study showed that the Caregiver Priorities and Child Health Index of Life with Disabilities appears to be easy to administer, seems to have significant validity and reliability, and may be useful in the evaluation of health-related quality of life of children with cerebral palsy.

AMAÇ: Bu çalismanin amaci, Bakim Veren Öncelikleri ve Engelli Çocuk Yasam Saglik Endeksi anketinin Türkçe'ye uyarlanmasi, geçerlilik ve güvenirliligini çalismaktir. GEREÇ VE YÖNTEMLER: Çalismaya 82 beyin felçli çocuk ve ailesi alindi. Ayrica çalismaya alinan çocuklarin büyük çogunlugu spastik tip beyin felci olup, 26'si Kaba Motor Fonksiyon Siniflama Sistemi'ne göre seviye III, 30'u seviye IV ve 26'si seviye V idi. Öncelikle, Bakim Veren Öncelikleri ve Engelli Çocuk Yasam Saglik Endeksi'nin çeviri ve geri çeviri islemi gerçeklestirildi. Güvenirlik, test-tekrar test ve iç tutarlilik ile degerlendirildi. Yapi geçerliligi, Çocuk Sagligi Ebeveyn Formu ile degerlendirildi. Eszamanli geçerlilik ise çocuklarin Kaba Motor Fonksiyon Siniflama Sistemi seviyeleriyle, engelli çocuk yasam saglik endeksi skorlarinin nasil degistigine bakilarak degerlendirildi. BULGULAR: Bakim Veren Öncelikleri ve Engelli Çocuk Yasam Saglik Endeksi için ortalama toplam skor 58,34±26,39 olarak belirlendi. Ölçegin iç tutarlilik degeri her 6 alt ölçüm için 0,43 ile 0,89 arasinda degisen degerlerde olup, toplam skor için 0,75 olarak saptandi. Cronbach alpha degeri saglik alt basligi hariç bütün alt ölçümlerde 0,80 degerinin üstünde bulundu. Yapi geçerliligi için yapilan Pearson korelasyon çözümlemesine göre Çocuk Sagligi Ebeveyn Formu ile Bakim Veren Öncelikleri ve Engelli Çocuk Yasam Saglik Endeksi arasinda pozitif yönde bir iliski bulundu (r=0,58, p<0,01). Bakim Veren Öncelikleri ve Engelli Çocuk Yasam Saglik Endeksi skorlari Kaba Motor Fonksiyon Siniflama Sistemi seviyeleri arasinda farkli bulundu (p<0,05). ÇIKARIMLAR: Bu çalisma, Bakim Veren Öncelikleri ve Engelli Çocuk Yasam Saglik Ölçegi'nin beyin felçli çocuklarda saglikla iliskili yasam kalitesinin degerlendirilmesinde önemli derecede yararli oldugunu, ölçegin oldukça yüksek geçerlilik ve güvenirlige sahip oldugunu göstermistir.

Development and content validation of the Muscular Dystrophy Child Health Index of Life with Disabilities questionnaire for children with Duchenne muscular dystrophy.

AIM: To develop a patient-reported outcome measure that comprehensively captures the health-related priorities of children with Duchenne muscular dystrophy (DMD). METHOD: Children with DMD and their parents completed the iteratively revised versions of the Caregiver Priorities and Child Health Index of Life with Disabilities (CPCHILD), followed by a cognitive interview to develop a pilot version of a new measure. Multidisciplinary health care professionals completed an item-by-item analysis of the measure and a 14-item sensibility questionnaire. Minimum content validity ratio for each item of the new measure and the mean score (0-7) for the items of the sensibility questionnaire were calculated. RESULTS: The CPCHILD underwent changes over 19 interviews with children and their parents, resulting in the pilot Muscular Dystrophy Child Health Index of Life with Disabilities (MDCHILD). The content validity ratio of each MDCHILD item ranged from 0.85 to 1 based on health care professionals' ratings. The mean score exceeded the threshold of four for all items of the sensibility questionnaire. Based on child, parent, and health care professional recommendations, 16 items were added, six eliminated, and 15 items modified from the original CPCHILD. The MDCHILD consists of 47 items over seven domains. INTERPRETATION: The MDCHILD met all sensibility criteria by children with DMD, their parents, and health care professionals, and is ready for psychometric evaluation. WHAT THIS PAPER ADDS: The Muscular Dystrophy Child Health Index of Life with Disabilities (MDCHILD) is a new patient-reported outcome measure for Duchenne muscular dystrophy (DMD). The Priority Framework of Outcomes underpins the content for the MDCHILD. The MDCHILD incorporates the health-related priorities of males with DMD and their parents. The MDCHILD was deemed sensible by children, their parents, and health care professionals.

Reliability of Radiographic Assessments of the Hip in Cerebral Palsy.

INTRODUCTION: Children with cerebral palsy are at risk for progressive hip displacement. Since surveillance for hip displacement uses specific radiographic measurements to guide decision making, it is important to establish the reliability of these measurements, which include Reimer's migration percentage (MP), acetabular index or acetabular angle (AI or AA), and pelvic obliquity (PO). The purpose of this study was to determine the intraobserver and interobserver reliability of these radiographic measures among an international group of pediatric orthopaedic surgeons participating in the prospective international multicenter Cerebral Palsy Hip Outcomes Project (CHOP) currently underway to evaluate the outcomes of hip interventions in cerebral palsy. METHODS: Two compact discs (CDs) containing the same 25 anteroposterior pelvis radiographs in Digital Imaging and Communications in Medicine (DICOM) format were provided to participating surgeons at least 2 weeks apart. To reduce the likelihood of recall or any effects of learning or fatigue, the order of the radiographs varied on the 2 CD versions, and participating surgeons received the 2 CDs in random order. The intraclass correlation coefficients (ICCs) were calculated to assess interobserver and intraobserver reliability. Mean absolute differences of hip measurements obtained at 2 time points were also calculated. RESULTS: The MP had the highest reliability followed by PO, AI, and AA with a mean intrarater ICC (SD; range) of 0.95 (0.04; 0.84 to 0.98); 0.92 (0.03; 0.85 to 0.97); 0.84 (0.05; 0.75 to 0.92); and 0.82 (0.14; 0.51 to 0.98); respectively. The mean interrater ICC (SD; range) for MP, PO, AI, and AA were 0.94 (0.05; 0.78 to 0.99); 0.90 (0.04; 0.76 to 0.99); 0.79 (0.08; 0.52 to 0.93); and 0.69 (0.23; 0.42 to 0.98) for MP, PO, AI, and AA, respectively. The mean (SD; 95% confidence interval) for the absolute difference between the 2 measurements for the raters was 4.9% (2.9%; 3.4%-6.4%); 3. 8 degrees (1.2 degrees; 3.1-4.5 degrees); 2.6 degrees (1.5 degrees; 1.7-3.5 degrees); and 1.3 degrees (0.3 degrees; 1.29-1.31 degrees) for MP, AI, AA, and PO, respectively. CONCLUSIONS: MP is a reproducible measure with excellent intrarater and interrater reliability. However, differences in MP of <7% should be treated with caution as these might be a consequence of measurement error. Although we found a high level of intrarater and interrater reliability of the AI, AA, and PO, these measurements are more variable and not ideal for use as discrete outcome measures. Instead, these parameters might be useful for prognostication and decision making when consistent trends are observed longitudinally over time which might be better indications of true change.