Hip Surveillance and Management of Hip Displacement in Children with Cerebral Palsy: Clinical and Ethical Dilemmas.

Hip displacement is the second most common musculoskeletal deformity in children with cerebral palsy. Hip surveillance programs have been implemented in many countries to detect hip displacement early when it is usually asymptomatic. The aim of hip surveillance is to monitor hip development to offer management options to slow or reverse hip displacement, and to provide the best opportunity for good hip health at skeletal maturity. The long-term goal is to avoid the sequelae of late hip dislocation which may include pain, fixed deformity, loss of function and impaired quality of life. The focus of this review is on areas of disagreement, areas where evidence is lacking, ethical dilemmas and areas for future research. There is already broad agreement on how to conduct hip surveillance, using a combination of standardised physical examination measures and radiographic examination of the hips. The frequency is dictated by the risk of hip displacement according to the child's ambulatory status. Management of both early and late hip displacement is more controversial and the evidence base in key areas is relatively weak. In this review, we summarise the recent literature on hip surveillance and highlight the management dilemmas and controversies. Better understanding of the causes of hip displacement may lead to interventions which target the pathophysiology of hip displacement and the pathological anatomy of the hip in children with cerebral palsy. We have identified the need for more effective and integrated management from early childhood to skeletal maturity. Areas for future research are highlighted and a range of ethical and management dilemmas are discussed.

Foot drop after gastrocsoleus lengthening for equinus deformity in children with cerebral palsy.

BACKGROUND: Gastrocsoleus lengthening (GSL) is the most common surgical procedure to treat equinus deformity in ambulant children with cerebral palsy (CP). Foot drop, where the ankle remains in plantarflexion during swing phase, can persist in some children post-operatively. There is currently limited understanding of which children will demonstrate persistent foot drop after GSL. RESEARCH QUESTION: Which children develop persistent foot drop after GSL surgery for equinus? METHODS: We conducted a retrospective cohort study on ambulant children with CP who had GSL surgery for fixed equinus deformity. The aims of the study were: to determine the frequency of persistent foot drop post-operatively and to compare outcome parameters from physical examination and three-dimensional gait analysis for children with hemiplegia or diplegia. RESULTS: One hundred and ten children functioning at GMFCS Levels I/II/III of 28/75/7 met the inclusion criteria for this study. There were 71 boys and mean age was 9.1 years at time of GSL surgery. The overall frequency of persistent foot drop was 25%, with a higher frequency of persistent foot drop in children with hemiplegia (42%) than children with diplegia (19%). There were significant improvements in dorsiflexor strength and in selective motor control in children with diplegia but not in children with hemiplegia. Mean (SD) pre-operative mid-swing ankle dorsiflexion for children with hemiplegia was - 14.0° (9.9°) and improved post-operatively to - 1.6° (5.5°). For children with diplegia, the pre-operative mid-swing ankle dorsiflexion was - 12.1° (12.9°) and improved post-operatively to + 4.2° (6.9°). SIGNIFICANCE: Foot drop is present following GSL surgery for fixed equinus deformity in a significant number of children with hemiplegia and to a lesser extent in children with diplegia, which may reflect a difference in the central nervous system lesion between these groups. New management approaches are required for this important and unsolved problem.

Australian hip surveillance guidelines at 10 years: New evidence and implementation.

Optimum management of hip displacement in children with cerebral palsy (CP) is facilitated by an approach that focuses on anticipatory and preventive measures. Hip surveillance programs for children with CP were developed at the beginning of the new millennium, with the purpose of identifying hip displacement sufficiently early to permit a choice of effective management options. In the early years, hip surveillance was guided by epidemiological analysis of population-based studies of prevalence. In Australia, a National Hip Surveillance in CP Working Group was first convened in 2005. This resulted in a 2008 Consensus Statement of recommendations published and endorsed by Australasian Academy of Cerebral Palsy and Developmental Medicine (AusACPDM). The group undertook that the recommendations should be reviewed every 5 years to ensure currency and congruency with the emerging evidence base. As new evidence became available, hip surveillance guidelines developed, with the most recent 2020 Australian Hip Surveillance Guidelines endorsed by the AusACPDM. Implementing comprehensive hip surveillance programs has now been shown to improve the natural history of hip dislocations and improve quality of life. Standardised hip surveillance programs can also facilitate planning for multicentre research through harmonisation of data collection. This, in turn, can help with the identification of robust new evidence that is based on large cohort or population studies. Here a review of evidence informing the updated 2020 Hip Surveillance Guidelines is presented.

Ankle Dorsiflexor Function after Gastrocsoleus Lengthening in Children with Cerebral Palsy: A Literature Review.

Background and Objectives: Ambulant children with cerebral palsy can demonstrate persistent "foot drop" after successful gastrocsoleus lengthening (GSL) surgery for equinus deformity. This may be due to inadequate strength and/or selective motor control of the ankle dorsiflexor muscles. A procedure has been developed to reduce foot drop-Tibialis Anterior Tendon Shortening (TATS), to be performed in conjunction with GSL. However, it is currently unclear how ankle dorsiflexor function changes after surgery and which children could benefit from TATS. This review summarises changes in ankle dorsiflexor function after GSL for equinus, as reported in the literature. Methods: A search was performed of the Medline, Embase and PubMed databases from 1980 to 5 March 2021. Keywords included "cerebral palsy", "equinus deformity", "orthopedic procedures" and "gait analysis". The search identified 1974 studies. Thirty-three cohort studies met the inclusion criteria for this review. Results: Twenty-two studies reported improvement in swing phase ankle dorsiflexion kinematics, after GSL. There was also evidence that clinical measures of ankle dorsiflexor strength improved after surgery. Four studies reported changes in selective motor control, with mixed results across the studies. Conclusions: There is good evidence that swing phase ankle dorsiflexion improves after GSL surgery. Although, there is limited evidence that this correlates with reduced foot drop or diminished need for an ankle-foot orthosis. Future research should be prospective, randomised, include a large sample size, and should focus on identifying the optimal candidates for TATS.

Epidemiology of scoliosis in cerebral palsy: A population-based study at skeletal maturity.

AIM: This study investigated the prevalence of scoliosis in a large, population-based cohort of individuals with cerebral palsy (CP) at skeletal maturity to identify associated risk factors that may inform scoliosis surveillance. METHODS: Young people with CP born between 1990 and 1992 were reviewed through routine orthopaedic review or a transition clinic. Classification of CP was recorded by movement disorder, distribution, gross and fine motor function. Clinical examination was undertaken and those with clinical evidence of scoliosis or risk factors had radiographs of the spine. Scoliosis severity was measured and categorised by Cobb angle. RESULTS: Two hundred and ninety-two individuals were evaluated (78% of the birth cohort) at a mean age of 21 years, 4 months (range 16-29 years). Scoliosis (Cobb angle >10°) was found in 41%, with strong associations to the Gross Motor Function Classification System (GMFCS), Manual Abilities Classification System (MACS) and dystonic/mixed movement disorders. Those at GMFCS V were 23.4 times (95%CI 9.9-55.6) more likely to develop scoliosis than those at GMFCS I. Severe curves (Cobb >40°, 13% of the cohort) were found almost exclusively in those functioning at GMFCS IV and V, and were 18.2 times (95%CI 6.9-48.5) more likely to occur in those with dystonia than those with spasticity. CONCLUSIONS: Scoliosis was very common in young people with CP, with prevalence and severity strongly associated with GMFCS and MACS level and dystonic movement disorder. Severe curves were almost exclusively found in non-ambulant children. Clinical screening for scoliosis should occur for all children with CP, with radiographic surveillance focusing on those functioning at GMFCS IV and V.

Musculoskeletal Pathology in Cerebral Palsy: A Classification System and Reliability Study.

This article presents a classification of lower limb musculoskeletal pathology (MSP) for ambulant children with cerebral palsy (CP) to identify key features from infancy to adulthood. The classification aims to improve communication, and to guide referral for interventions, which if timed appropriately, may optimise long-term musculoskeletal health and function. Consensus was achieved by discussion between staff in a Motion Analysis Laboratory (MAL). A four-stage classification system was developed: Stage 1: Hypertonia: Abnormal postures are dynamic. Stage 2: Contracture: Fixed shortening of one or more muscle-tendon units. Stage 3: Bone and joint deformity: Torsional deformities and/or joint instability (e.g., hip displacement or pes valgus), usually accompanied by contractures. Stage 4: Decompensation: Severe pathology where restoration of optimal joint and muscle-tendon function is not possible. Reliability of the classification was tested using the presentation of 16 clinical cases to a group of experienced observers, on two occasions, two weeks apart. Reliability was found to be very good to excellent, with mean Fleiss' kappa ranging from 0.72 to 0.84. Four-stages are proposed to classify lower limb MSP in children with CP. The classification was reliable in a group of clinicians who work together. We emphasise the features of decompensated MSP in the lower limb, which may not always benefit from reconstructive surgery and which can be avoided by timely intervention.

The impact of symptomatic femoral neck anteversion and tibial torsion on gait, function and participation in children and adolescents.

BACKGROUND: Torsional deformities of the lower limbs in children and adolescents are a common cause of in-toeing gait and cause gait deviations. The purpose of this study was to examine the relationship of children and adolescents with suspected Idiopathic Torsional Deformities (ITD) and pain, gait function, activity and participation. METHODS: A retrospective review of all children and adolescents who attended our Centre over a 5-year period for evaluation of the effect of ITD. All children completed three-dimensional gait analysis (3DGA), standardized physical examination, medical imaging and the Pediatric Outcomes Data Collection Instrument (PODCI). Statistical analysis was completed using two sample t-tests, Pearson's Correlation and linear regression. RESULTS: Fifty children and adolescents, 40 females and 10 males with a mean age of 13.5 years were included. Children reported a high prevalence of pain(86%), had increased internal hip rotation(p = 0.002) and decreased external hip rotation(p < 0.001) on physical examination when compared to published normative data. Medical imaging showed a mean(SD) femoral neck anteversion (FNA) of 38°(13°) and external tibial torsion of 39°(12°). Mean(SD) PODCI score was 32(16), indicating these children are functioning below their typically developing peers. The 3DGA kinematics show deviations from typical data including hip rotation, foot progression, pelvic tilt, hip flexion and knee extension. Observed mild kinetic deviations were within typical limits. The relationship between FNA and gait parameters, FNA and PODCI and gait and PODCI were weak. SIGNIFICANCE: These children and adolescents have altered gait and experience pain leading to impaired function and diminished participation. Therefore, ITD is not purely a cosmetic issue.

Three-Dimensional Gait Analysis in Children Undergoing Gastrocsoleus Lengthening for Equinus Secondary to Cerebral Palsy.

Background and Objectives: Equinus is the most common deformity in children with cerebral palsy, and surgical lengthening of the gastrocsoleus muscle-tendon unit is the most commonly performed operation for children with cerebral palsy. Treatment outcomes of orthopaedic surgery can be measured objectively with three-dimensional gait analysis. This study examined the quality of evidence for gastrocsoleus lengthening surgery based on objective measures. Materials and Methods: A search was performed with Medline, Embase and PubMed from 1990 to 25 August 2020 using the keywords "cerebral palsy", "equinus", "surgery" and "gait analysis". Only studies of gastrocsoleus lengthening surgery using three-dimensional gait analysis were included, yielding 34 studies. Results: Fourteen studies reported swing phase kinematics and all studies reported a significant improvement. Rates of recurrent equinus and calcaneus were reported in 21 studies and varied widely based on follow-up period and surgical technique. Conclusions: Poor study quality and marked variability in study samples and interventions made comparison difficult. Future studies should consider prospective design, controls or comparison groups and more detailed breakdowns of outcomes by cerebral palsy subtype, sagittal gait pattern, and equinus type in order to allow more rigorous treatment recommendations to be made.

A Decision Support System to Facilitate Identification of Musculoskeletal Impairments and Propose Recommendations Using Gait Analysis in Children With Cerebral Palsy.

The identification of musculoskeletal impairments from gait analysis in children with cerebral palsy is a complex task, as is formulating (surgical) recommendations. In this paper, we present how we built a decision support system based on gait kinematics, anthropometrics, and physical examination data. The decision support system was trained to learn the association between these data and the list of impairments and recommendations formulated historically by experienced clinicians. Our aim was 2-fold, train a computational model that would be representative of data-based clinical reasoning in our center, and support new or junior clinicians by providing pre-processed impairments and recommendations with the associated supportive evidence. We present some of the challenges we faced, such as the issues of dimensionality reduction for kinematic data, missing data imputations, class imbalance and choosing an appropriate model evaluation metric. Most models, i.e., one model for each impairments and recommendations, achieved a weighted Brier score lower than 0.20, and sensitivity and specificity greater than 0.70 and 0.80, respectively. The results of the models are accessible through a web-based application which displays the probability predictions as well as the (up to) 5 best predictors.

Australian children undergoing selective dorsal rhizotomy: protocol for a national registry of multidimensional outcomes.

INTRODUCTION: Selective dorsal rhizotomy (SDR) is a neurosurgical intervention intended to permanently reduce spasticity in the lower limbs and improve mobility in selected children with cerebral palsy (CP). Despite SDR having been performed worldwide for the past 30 years, there is moderate quality of evidence that SDR is effective in reducing spasticity with low to very low evidence of its effectiveness in improving gait, function and participation, using the Grading of Recommendations, Assessment, Development and Evaluations (GRADE) system. Published studies have described outcomes for groups that differ in selection, surgical technique and postoperative rehabilitation making it difficult for clinicians to use this information to advise families on best management. There is substantial community interest in SDR. A small number of children with CP undergo SDR in Australia each year and some families seek the intervention at international sites. Capturing clinical outcomes and adverse event (AE) data for Australian children undergoing SDR will provide clinicians with information to help guide families considering SDR. METHODS AND ANALYSIS: The Australian SDR Research Registry is a national registry of multidimensional outcomes for Australian children undergoing SDR in an Australian or overseas centre. Data will be collected for up to 10 years following the surgery, to include surgery and admission details, surgical and long-term AEs, and outcome measures across the body structure and functions, activity and participation domains of the International Classification of Functioning, Disability and Health. Data will be collected at baseline, during inpatient admission and at 1, 2, 5 and 10 years post. The aim of collecting these data is to improve understanding of short-, medium- and long-term outcomes and adverse effects of the intervention. ETHICS AND DISSEMINATION: This study was approved by the individual Human Research and Ethics committees at the five Australian tertiary hospitals involved. Results will be disseminated via peer-reviewed publications and conference presentations. TRIAL REGISTRATION NUMBER: ACTRN12618000985280; Pre-results.

Classifying Adverse Events Following Lower Limb Orthopaedic Surgery in Children With Cerebral Palsy: Reliability of the Modified Clavien-Dindo System.

BACKGROUND: The modified Clavien-Dindo (MCD) system is a reliable tool for classifying adverse events (AEs) in hip preservation surgery and has since been utilized in studies involving lower limb surgery for ambulant and nonambulant children with cerebral palsy (CP). However, the profile of AEs recorded in children with CP compared with typically developing children is different, and the reliability of the MCD in CP is unknown. This study aimed to evaluate the interrater and intrarater reliability of the MCD system for classifying AEs following lower limb surgery in children with CP. METHODS: Eighteen raters were invited to participate, including clinicians from surgical, nursing, and physical therapy professions, and individuals with CP. Following a MCD familiarization session, participants rated 40 clinical scenarios on 2 occasions, 2 weeks apart. Fleiss' κ statistics were used to calculate interrater and intrarater reliability. RESULTS: The overall Fleiss' κ value for interrater reliability in the first rating was 0.70 (95% confidence interval, 0.61-0.80), and increased to 0.75 (95% confidence interval, 0.66-0.84) in the second rating. The average Fleiss' κ value for intrarater reliability was 0.78 (range, 0.48 to 1.00). Grading of more severe AEs (MCD III to V) achieved near perfect agreement (κ, 0.87 to 1.00). There was a lower level of agreement for minor AEs (MCD I-II) (κ, 0.53 to 0.55). A κ score of 0 to 0.2 was deemed as poor, 0.21 to 0.4 as fair, 0.41 to 0.6 as good, 0.61 to 0.8 as very good, and 0.81 to 1.0 as almost perfect agreement. CONCLUSIONS: The MCD System demonstrates a very good interrater and intrarater reliability following lower limb surgery in children with CP. The MCD can be used by clinicians from different health care professions with a high level of reliability. The MCD may improve standardization of AE recording with a view to accurate audits and improved clarity in outcome studies for CP. LEVEL OF EVIDENCE: Level II-diagnostic.

The Gait Outcomes Assessment List (GOAL): validation of a new assessment of gait function for children with cerebral palsy.

AIM: We investigated the validity of the Gait Outcomes Assessment List (GOAL), as an assessment of gait function in children with cerebral palsy (CP). METHOD: We studied a prospective cohort of 105 children with CP (Gross Motor Function Classification System [GMFCS] levels I-III; 65 males, 40 females; mean [SD] age 11y 11mo [3y 5mo], range 6-20y), who attended gait assessment over a 10-month period. Parents completed the GOAL, Functional Mobility Scale (FMS), and Functional Assessment Questionnaire (FAQ) during their child's gait evaluation. Ninety children completed instrumented gait analysis (IGA). Total GOAL and domain scores, Gait Profile Score (GPS), and Gait Variable Scores were calculated. RESULTS: The total GOAL discriminated between GMFCS levels (mean [SD] GMFCS level I, 72.5 [12.7]; GMFCS level II, 61.4 [13.0]; GMFCS level III, 38.8 [10.6]; [F2,97 =42.4, p<0.001]). Moderate correlations were found between total GOAL and FMS (5m and 50m r=0.59; 500m r=0.66) and FAQ walking (r=0.77) and activities list (r=0.75, p<0.01). There was a moderate negative correlation between total GOAL and GPS (r=-0.59) and gait appearance domain and GPS (r=-0.52, p<0.01). INTERPRETATION: The GOAL is a valid assessment of gait function in ambulant children with CP. It has the potential to improve understanding of the child's and parents' priorities and thus, in conjunction with IGA, provide a more balanced assessment across the domains of the World Health Organization's International Classification of Functioning, Disability and Health. WHAT THIS PAPER ADDS: The Gait Outcomes Assessment List (GOAL) can discriminate between Gross Motor Function Classification System levels. The GOAL correlates with standard functional assessments and gait analysis. Used with gait analysis, the GOAL provides comprehensive assessment across all International Classification of Functioning, Disability and Health domains.

Long-term development of gait after multilevel surgery in children with cerebral palsy: a multicentre cohort study.

AIM: We investigated the long-term efficacy and safety of multilevel surgery (MLS) in ambulatory children with bilateral spastic cerebral palsy (CP). METHOD: Two hundred and thirty-one children were evaluated at short term (1.1y, SD 0.4) and long term (9.1y, SD 3.0) follow-up using clinical examination and gait analysis. MLS was investigated by studying changes in the Gait Profile Score (GPS) referenced to the minimally important clinical difference. RESULTS: Ambulatory children aged 10 years and 7 months (SD 2y 11mo) at MLS in Gross Motor Function Classification System levels I (19), II (144), and III (68) showed a decrease (improvement) in preoperative GPS from 16.3° (SD 4.8) to 11.3° (SD 3.2) at short-term follow-up, an improvement of 5°. At long-term follow-up, GPS was maintained at 11.4° (SD 3.1). Overall, 177 (76.6%) children maintained their improvement in GPS after 9 years. INTERPRETATION: Multilevel surgery is a safe and effective surgical intervention, which leads to a significant improvement in gait kinematics in children with bilateral spastic CP. This study improves our understanding of MLS in the long term and will help to inform families and children when planning for MLS. WHAT THIS PAPER ADDS: Largest study of multilevel surgery (MLS) for children with bilateral spastic cerebral palsy, with longest follow-up. MLS resulted in significant long-term improvements in gait function. Minor adverse events were common, while events requiring intervention were uncommon (4% of children). Thirty-nine per cent of children required additional surgery during follow-up. 'Single-event multilevel surgery' was changed to the more realistic term 'multilevel surgery'.

Screening and referral for children with physical disabilities.

The clinical care of children with physical disabilities is a major priority for paediatricians and paediatric orthopaedic surgeons. Cerebral palsy (CP) is the prototypical condition and remains the most common cause of physical disability in developed countries. The incidence is approximately 2 per 1000 live births, translating to between 600 and 700 new children per annum in Australia, with approximately 34 000 children and adults currently living with CP. This figure is predicted to rise inexorably over the next 20 years. The care of children with physical disabilities, including those with CP, is usually coordinated by paediatricians, general practitioners and allied health teams including physiotherapists, with input from paediatric orthopaedic surgeons when appropriate. The emphasis in care for children with CP has moved from 'reactive' to 'proactive'. In the past, children are often referred when symptomatic, for example when a hip dislocation had occurred and became painful. The emphasis now is on coordinated, multidisciplinary care in which musculoskeletal manifestations of disability are identified by screening programmes. Systematic screening, especially when population-based and linked to a register, avoids children getting 'lost in the system'. Early and more effective interventions may be offered for the prevention of contractures, dislocation of the hip and spinal deformities. In this review, we will focus on the assessment of gait in children with physical disabilities, and monitoring for hip and spine deformity.

Hip health at skeletal maturity: a population-based study of young adults with cerebral palsy.

AIM: We studied 'hip health' in a population-based cohort of adolescents and young adults with cerebral palsy to investigate associations between hip morphology, pain, and gross motor function. METHOD: Ninety-eight young adults (65 males, 33 females) from the birth cohort were identified as having developed hip displacement (migration percentage >30) and were reviewed at a mean age of 18 years 10 months (range 15-24y). Hip morphology was classified using the Melbourne Cerebral Palsy Hip Classification Scale (MCPHCS). Severity and frequency of pain were recorded using Likert scales. Gross motor function was classified by the Gross Motor Function Classification System (GMFCS). RESULTS: Hip pain was reported in 72% of participants. Associations were found between pain scores and both hip morphology and GMFCS. Median pain severity score for MCPHCS grades 1 to 4 was 2 (interquartile range [IQR] 1.0-3.0) compared to 7 (IQR 6.0-8.0) for grades 5 and 6 (severe subluxation or dislocation). Hip surveillance and access to surgery were associated with improved hip morphology and less pain. INTERPRETATION: Poor hip morphology at skeletal maturity was associated with high levels of pain. Limited hip surveillance and access to surgery, rather than GMFCS, was associated with poor hip morphology. The majority of young adults who had access to hip surveillance, and preventive and reconstructive surgery, had satisfactory hip morphology at skeletal maturity and less pain.

Body mass index in ambulatory children with cerebral palsy: A cohort study.

AIM: Children with cerebral palsy (CP) have reduced levels of physical activity compared with children without physical disability and experience risk factors for becoming overweight or obese. In the Australian CP population, there is little information available about the weight status of children with CP. The aims of this study were to compare the distribution of body mass index (BMI) in a cohort of ambulant children with CP with the BMI distribution of Australian children and explore the relationship between BMI and gross motor function. METHODS: A retrospective cohort study of 587 children with CP Gross Motor Function Classification System (GMFCS) levels I-III who attended a Gait Laboratory between July 1995 and January 2012 was carried out. The BMI and Z-score were calculated at each assessment. Data were grouped into the categories of underweight, healthy, overweight and obese according to age-specific and sex-specific percentiles. RESULTS: There were 348 boys and 240 girls with a mean age 11.2 (standard deviation 3.2) years. Mean BMI Z-score was 0.11 (standard deviation 1.33). Seven percent of children were underweight, 73.6% healthy, 7.3% overweight and 12.1% obese. This was similar to the distribution of children without disability. The largest percentage of children in the healthy group were classified GMFCS I. The largest percentage of children in the obese group were classified GMFCS III. CONCLUSIONS: In this cohort, 19.4% of ambulant children with CP were overweight or obese. This is of concern as BMI may impact on the outcomes of surgical intervention and rehabilitation. Further research is needed to determine the consequences of obesity for children with CP.