Sagittal spinopelvic alignment in ambulatory persons with cerebral palsy.

PURPOSE: This study aimed to describe the spinopelvic alignment of a cohort of young ambulatory individuals with cerebral palsy (CP) and compare it to published spinopelvic alignment data for the typically developing adolescents. METHODS: Thirty-seven adolescents (18 females) with CP at GMFCS I-III were included in this retrospective case series. Lumbar lordosis and pelvic incidence were measured, and their mismatch was calculated. A model that calculates predicted lumbar lordosis based on pelvic incidence in normative data was utilized to calculate a predicted lumbar lordosis in this cohort with cerebral palsy. RESULTS: At imaging, ages were mean and standard deviation 13.5 ± 3.0 years. Pelvic incidence was 46.2° ± 12.9°, pelvic tilt was 2.8° ± 9.4°, sacral slope was 43.6° ± 10.8°, and measured lumbar lordosis was 59.4° ± 11.6°. There were no differences in pelvic incidence or lumbar lordosis among the GMFCS levels; however, pelvic incidence was higher in females. Pelvic incidence-lumbar lordosis mismatch greater than 10° was found in 67% of the cohort. Mean predicted lumbar lordosis based on the model was 54.7° ± 8.5°, averaging 8° less than measured lordosis. CONCLUSION: PI-LL mismatch was identified in 67% of this cohort of ambulatory adolescents with CP, in part due to greater lordosis than predicted by a model based on data from adolescents without CP. The implications of this finding, such as the correlation between sagittal spinopelvic alignment and quality of life in this population, should be assessed further in ambulatory patients with cerebral palsy. LEVEL OF EVIDENCE: Level IV-retrospective cohort study and literature comparison.

Spinal Deformity Surgery in Pediatric Patients With Cerebral Palsy: A National-Level Analysis of Inpatient and Postdischarge Outcomes.

STUDY DESIGN: Retrospective cohort. OBJECTIVE: To provide a national-level assessment of the short-term outcomes after spinal deformity surgery in pediatric patients with cerebral palsy. METHODS: A national, prospectively collected database was queried to identify pediatric (≤18 years) patients with cerebral palsy, who underwent spinal fusion surgery from 2012 to 2017. Separate multivariate analyses were performed for the primary outcomes of interest including extended length of stay (>75th percentile, >8 days), and readmissions within 90 days after the index admission. RESULTS: A total of 2856 patients were reviewed. The mean age ± standard deviation was 12.8 ± 2.9 years, and 49.4% of patients were female. The majority of patients underwent a posterior spinal fusion (97.0%) involving ≥8 levels (79.9%) at a teaching hospital (96.6%). Top medical complications (24.5%) included acute respiratory failure requiring mechanical ventilation (11.4%), paralytic ileus (8.2%), and urinary tract infections (4.6%). Top surgical complications (40.7%) included blood transfusion (35.6%), wound complication (4.9%), and mechanical complication (2.7%). The hospital cost for patients with a length of hospital stay >8 days ($113 669) was nearly double than that of those with a shorter length of stay ($68 411). The 90-day readmission rate was 17.6% (mean days to readmission: 30.2). The most common reason for readmission included wound dehiscence (21.1%), surgical site infection (19.1%), other infection (18.9%), dehydration (16.9%), feeding issues (14.5%), and acute respiratory failure (13.1%). Notable independent predictors for 90-day readmissions included preexisting pulmonary disease (odds ratio [OR] 1.5), obesity (OR 3.4), cachexia (OR 27), nonteaching hospital (OR 3.5), inpatient return to operating room (OR 1.9), and length of stay >8 days (OR 1.5). CONCLUSIONS: Efforts focused on optimizing the perioperative pulmonary, hematological, and nutritional status as well as reducing wound complications appear to be the most important for improving clinical outcomes.

Gait profile score and gait variable scores in spina bifida.

Quantitative data assessment on the basis of three-dimensional gait analysis has been routinely used in the evaluation of pathological gait of children with cerebral palsy. However, a similar quantitative methodology has not been applied for spina bifida patients in whom atypical gait patterns are thought to correlate with various levels of neurological paralysis. The purpose of this study is to investigate the differences among gait patterns in spina bifida between different levels of neurological lesions using quantitative methods: Gait profile score (GPS) and gait variable scores (GVS), scoring subject's gait deviation from a reference. In this cross-sectional study, 22 children with spina bifida (11 women, 11 men; mean age 9.4 years, SD 3.8 years, range 3-17 years), were examined using three-dimensional gait analysis from 2008 to 2018. Physical examination allowed for classification of each of the 44 limbs as either L4, L5 or S1 and comparison with the GPS and GVS using a linear mixed model. GPS and the GVS of the pelvis and hip range of motion in the coronal plane were significantly higher in the L4 group than in the L5 and S1 groups (GPS, P = 0.041, P = 0.003, respectively; GVS of pelvis, P = 0.001, P = 0.001; GVS of hip, P < 0.001, P < 0.001) GVS (foot progression angle) was significantly lower in the S1 group than in L4 and L5 groups (P < 0.001, P = 0.037). We found that GPS and GVS enable us to quantitatively assess the differences among gait patterns between different neurological levels. The scoring tool showed the potential for detecting individual neurological changes.

Orthopaedic Surgery in Dystonic Cerebral Palsy.

BACKGROUND: Outcomes after orthopaedic interventions in patients with dystonic cerebral palsy (DCP) are historically regarded as unpredictable. This study aims to evaluate the overall outcome of orthopaedic surgery in children with DCP. METHOD: Children with DCP who underwent lower limb orthopaedic surgery with a minimum follow-up of 12 months were included. Data collected included age at time of surgery, surgical procedures performed, Gross Motor Function Classification System (GMFCS) level, and Barry Albright Dystonia Scale (BADS) score. The cohort was divided into 2 groups. Group 1 (GMFCS levels I to III), mean age 12 years 7 months and group 2 (GMFCS levels IV to V), mean age 10 years 7 months. Group 1 had surgery aimed at deformity correction to improve gait and mobility, and group 2 for the management or prevention of hip displacement. Outcome measures analyzed were: the incidence of unpredictable results related to surgery and early recurrence of deformity in both groups. Functional mobility scale scores were evaluated for group 1 and hip migration percentage for group 2. Linear mixed models were used to take into account repeated measures over time and correlations between measurements from the same patient. RESULTS: Group 1 (n=18); had low BADS scores and were considered to have mild dystonia. Three children experienced unpredictable results, 2 had early recurrence of deformity, 3 had a decline, and 1 child improved in the functional mobility scale.Group 2 (n=19); had high BADS scores and were considered to have moderate to severe dystonia. Nine surgical events involved bony procedures and 15 were soft tissue surgery only. One surgical event lead to unpredictable results and 2 children had early recurrence of deformity. Postoperatively, a linear trend of increasing migration percentage [0.49% (95% confidence interval, 0.23-0.74; P=0.0002)] was seen up to 21 months. There was no significant change after 21 months [-0.08% (95% confidence interval, -0.24 to +0.041; P=0.18)]. CONCLUSIONS: This study suggests that unpredictable results and early recurrence of deformity following orthopaedic surgery in children with DCP are not as common as previously regarded. Furthermore, functional mobility and hip morphology can be improved. LEVEL OF EVIDENCE: Level IV-this is a case-series.

PURA syndrome: clinical delineation and genotype-phenotype study in 32 individuals with review of published literature.

BACKGROUND: De novo mutations in PURA have recently been described to cause PURA syndrome, a neurodevelopmental disorder characterised by severe intellectual disability (ID), epilepsy, feeding difficulties and neonatal hypotonia. OBJECTIVES: To delineate the clinical spectrum of PURA syndrome and study genotype-phenotype correlations. METHODS: Diagnostic or research-based exome or Sanger sequencing was performed in individuals with ID. We systematically collected clinical and mutation data on newly ascertained PURA syndrome individuals, evaluated data of previously reported individuals and performed a computational analysis of photographs. We classified mutations based on predicted effect using 3D in silico models of crystal structures of Drosophila-derived Pur-alpha homologues. Finally, we explored genotype-phenotype correlations by analysis of both recurrent mutations as well as mutation classes. RESULTS: We report mutations in PURA (purine-rich element binding protein A) in 32 individuals, the largest cohort described so far. Evaluation of clinical data, including 22 previously published cases, revealed that all have moderate to severe ID and neonatal-onset symptoms, including hypotonia (96%), respiratory problems (57%), feeding difficulties (77%), exaggerated startle response (44%), hypersomnolence (66%) and hypothermia (35%). Epilepsy (54%) and gastrointestinal (69%), ophthalmological (51%) and endocrine problems (42%) were observed frequently. Computational analysis of facial photographs showed subtle facial dysmorphism. No strong genotype-phenotype correlation was identified by subgrouping mutations into functional classes. CONCLUSION: We delineate the clinical spectrum of PURA syndrome with the identification of 32 additional individuals. The identification of one individual through targeted Sanger sequencing points towards the clinical recognisability of the syndrome. Genotype-phenotype analysis showed no significant correlation between mutation classes and disease severity.

Normative reference values for lower limb joint range, bone torsion, and alignment in children aged 4-16 years.

Normative reference values are essential to identify deviation from normal and evaluate response to treatment. As joint range of motion datasets specific to the pediatric population are infrequently reported in the literature, we determined lower limb passive joint range of motion and bone torsion values from 53 typically developing children aged 4-16 years. Our reference values were consistent with previously published norms, although for some measures, large variability in the literature exists. A clear correlation between joint range and age was observed in most measures. Our results highlight the importance of applying age-matched norms when attempting to identify deviation from normal in the growing child.

Lengthening of the gastrocnemius-soleus complex: an anatomical and biomechanical study in human cadavers.

BACKGROUND: Lengthening of the gastrocnemius-soleus complex is frequently performed for equinus deformity. Many techniques have been described, but there is uncertainty regarding the precise details of some surgical procedures. METHODS: The surgical anatomy of the gastrocnemius-soleus complex was investigated, and standardized approaches were developed for the procedures described by Baumann, Strayer, Vulpius, Baker, Hoke, and White. The biomechanical characteristics of these six procedures were then compared in three randomized trials involving formaldehyde-preserved human cadaveric lower limbs. After one of the lengthening procedures was performed, a measured dorsiflexion force was applied across the metatarsal heads with use of a torque dynamometer. Lengthening of the gastrocnemius-soleus complex was measured directly, by measuring the gap between the ends of the fascia or tendon. RESULTS: The gastrocnemius-soleus musculotendinous unit was subdivided into three zones. In Zone 1, it was possible to lengthen the gastrocnemius-soleus complex in either a selective or a differential manner-i.e., to lengthen the gastrocnemius alone or to lengthen the gastrocnemius and soleus by different amounts. The procedures performed in this zone (Baumann and Strayer procedures) were very stable but were limited with regard to the amount of lengthening achieved. Zone-2 lengthenings of the conjoined gastrocnemius aponeurosis and soleus fascia (Vulpius and Baker procedures) were not selective but were stable and resulted in significantly greater lengthening than Zone-1 procedures (p < 0.001). In Zone 3 (Hoke and White procedures), lengthenings of the Achilles tendon were neither selective nor stable but resulted in significantly greater lengthening than Zone-1 or 2 procedures (p < 0.001). CONCLUSIONS: Surgical procedures for the correction of equinus deformity by lengthening of the gastrocnemius-soleus complex vary in terms of selectivity, stability, and range of correction. Procedures for the correction of equinus deformity have different anatomical and biomechanical characteristics. Clinical trials are needed to determine whether these differences are of clinical importance. It may be appropriate for surgeons to select a procedure involving the zone best suited to the clinical needs of a specific patient.

Multilevel surgery for equinus gait in children with spastic diplegic cerebral palsy: medium-term follow-up with gait analysis.

BACKGROUND: In children with spastic diplegia, surgery for ankle equinus contracture is associated with a high prevalence of both overcorrection, which may result in a calcaneal deformity and crouch gait, and recurrent equinus contracture, which may require revision surgery. We sought to determine if conservative surgery for equinus gait, in the context of multilevel surgery, could result in the avoidance of overcorrection and crouch gait as well as an acceptable rate of recurrent equinus contracture at the time of medium-term follow-up. METHODS: This was a retrospective, consecutive cohort study of children with spastic diplegia who had had surgery for equinus gait between 1996 and 2006. All children had distal gastrocnemius recession or differential gastrocnemius-soleus complex lengthening, on one or both sides, as part of single-event multilevel surgery. The primary outcome measures were the Gait Variable Scores (GVS) and Gait Profile Score (GPS) at two time points after surgery. RESULTS: Forty children with spastic diplegia, Gross Motor Function Classification System (GMFCS) level II or III, were included in this study. There were twenty-five boys and fifteen girls. The mean age was ten years at the time of surgery and seventeen years at the time of final follow-up. The mean postoperative follow-up period was 7.5 years. The mean ankle GVS improved from 18.5° before surgery to 8.7° at the time of short-term follow-up (p < 0.005) and 7.8° at the time of medium-term follow-up. The equinus gait was successfully corrected in the majority of children, with a low rate of overcorrection (2.5%) and a high rate of recurrent equinus (35%), as determined by sagittal ankle kinematics. Mild recurrent equinus was usually well tolerated and conferred some advantages, including contributing to strong coupling at the knee and independence from using an ankle-foot orthosis. CONCLUSIONS: Surgical treatment for equinus gait in children with spastic diplegia was successful, at a mean of seven years, in the majority of cases when combined with multilevel surgery, orthoses, and rehabilitation. No patient developed crouch gait, and the rate of revision surgery for recurrent equinus was 12.5%.

Single Event Multilevel Surgery in children with bilateral spastic cerebral palsy: a 5 year prospective cohort study.

BACKGROUND: Single Event Multilevel Surgery (SEMLS) is considered the standard of care to improve gait and function in children with bilateral spastic cerebral palsy (BSCP). We have demonstrated in a randomized controlled trial (RCT) of SEMLS, that gait was improved at 12 months after surgery and gross motor function at 24 months after surgery. The question addressed in this study, was to determine if improvements in gait and function, would be maintained at 5 year follow-up. METHODS: Nineteen children with BSCP, GMFCS levels II (14 children) and III (5 children), mean age 9.7 years (range 7.7-12.2 years) participated in a prospective cohort study following participation in a RCT, with follow-up to 5 years. Outcome measures were Gait Profile Score (GPS), Gillette Gait Index (GGI), Gait Deviation Index (GDI), Gross Motor Function Measure (GMFM66) and Functional Mobility Scale (FMS). RESULTS: Eighteen children have completed follow-up, with interval analysis at 1, 2 and 5 years post SEMLS. One child was excluded because of neurological deterioration and his diagnosis was revised to Hereditary Spastic Paraparesis (HSP). GPS improved by 5.29° and GMFM66 by 3.3% at 5 years post SEMLS. Differences between outcome measures at 1 versus 5 years and 2 versus 5 years (except GMFM66) were not significant, indicating that improvements in gait and gross motor function were stable over time. CONCLUSIONS: SEMLS results in clinically and statistically significant improvements in gait and function, in children with BSCP, which were maintained at 5 years after surgery.

Management of children with ambulatory cerebral palsy: an evidence-based review. Commentary by Hugh Williamson Gait Laboratory staff.

The evaluation of complex interventions, such as Single Event Multilevel Surgery (SEMLS) requires more than randomized controlled trials. Rehabilitation following SEMLS is prolonged and the outcomes of interest may not be apparent for 5 years or more after the surgery. We suggest long term, prospective cohort studies with objective outcome measures be recognized as of equal importance to randomized controlled trials. The evidence in support of instrumented gait analysis (IGA) is also reviewed. We suggest that clinical levels of evidence are not an appropriate method to evaluate a measurement tool. Specifically, IGA should be evaluated in terms of validity, reliability and cost effectiveness. We demonstrate that the use of IGA has improved medium and long term outcomes in ambulant children with cerebral palsy in a center where IGA has been used routinely both for planning SEMLS and for monitoring outcomes.

Adductor surgery to prevent hip displacement in children with cerebral palsy: the predictive role of the Gross Motor Function Classification System.

BACKGROUND: The purpose of this study was to evaluate the relationship between walking ability, as determined with use of the Gross Motor Function Classification System (GMFCS), and the outcome of hip adductor surgery used to prevent hip displacement in children with cerebral palsy. METHODS: We performed a retrospective review of the records of all children with cerebral palsy whose index surgery, performed between January 1994 and December 2004 at one tertiary-level pediatric hospital, was bilateral hip adductor releases. All children had a hip migration percentage of >30% in at least one hip prior to the adductor surgery, and the minimum duration of follow-up was twenty-four months. Kaplan-Meier survivorship curves were generated by determining the time from the index surgery to "failure," defined as either the need for subsequent surgical procedures or a migration percentage of ≥50% in either hip. Hazard ratios were calculated for sex, migration percentage at the time of the index surgery, age at the time of the index surgery, and GMFCS level. RESULTS: Three hundred and thirty children were included in the study; 73% (242) were nonambulatory (GMFCS level IV or V). The mean age at the time of the index surgery was 4.2 years, the mean migration percentage was 43%, and the mean duration of postoperative follow-up was 7.1 years. Surgery consisted of open lengthening of the adductor longus and gracilis muscles in all children, with additional procedures as deemed necessary. "Success" was defined as the absence of subsequent surgical procedures during the study period and a migration percentage of <50% in both hips at the time of follow-up. One hundred and six children (32%) met these criteria for success. The success rate was 94% (thirty-one of thirty-three) in children at a GMFCS level of II, 49% (twenty-seven of fifty-five) in children at a level of III, 27% (twenty-eight of 103) in children at a level of IV, and 14% (twenty of 139) in children at a level of V. CONCLUSIONS: Walking ability, as defined with use of the GMFCS level, is a strong predictor of success or failure after hip adductor surgery in children with cerebral palsy. The paradox of hip adductor surgery for children with cerebral palsy is that the children who are most severely affected and need the surgery the most have the poorest results.

Orthopedic surgery and mobility goals for children with cerebral palsy GMFCS level IV: what are we setting out to achieve?

BACKGROUND: Multilevel orthopedic surgery is considered to be the gold standard treatment for ambulatory children with cerebral palsy (CP), classified at levels I, II, or III according to the Gross Motor Function Classification System (GMFCS). Hip enlocation and stability are the main goals of orthopedic intervention in the GMFCS level IV subgroup and are well researched; however, there is no evidence to date to support or challenge the effectiveness of orthopedic treatment to preserve functional mobility in this patient group. The aim of this study was to evaluate the results of orthopedic surgery to maintain or restore standing transfers and supported walking in children with CP at GMFCS level IV. METHODS: Twenty-two children with CP GMFCS level IV who underwent orthopedic surgery to improve mobility between the years 2004 and 2008 were included in this study. A retrospective chart review was performed and a satisfaction questionnaire sent to all patients. The primary outcome measure was the attainment and maintenance of mobility goals 2 years post-surgery. The secondary outcome measures were family/patient satisfaction, Functional Mobility Scale (FMS), and complications. RESULTS: The two goals identified by the patients and carers were standing transfers and supported walking. At the 2-year post-surgery assessment, 14 children (63.6 %) did not reach their pre-determined goals. In the questionnaire, 21.4 % of the families reported that surgery was not beneficial. The FMS score remained unchanged in 95.4 % of the patients. Fourteen patients (63.6 %) had at least one complication that prolonged their post-operative rehabilitation (e.g., neuropraxia). CONCLUSION: This study suggests that orthopedic surgery in children with CP at GMFCS level IV is unlikely to maintain or restore mobility. Furthermore, it carries a significant risk of complications. LEVEL OF EVIDENCE: Case series, Level IV.

Single-event multilevel surgery in children with spastic diplegia: a pilot randomized controlled trial.

BACKGROUND: Single-event multilevel surgery is considered the standard of care to improve gait and functioning of children with spastic diplegic cerebral palsy. However, the evidence base is limited. This pilot study is the first randomized controlled trial of single-event multilevel surgery, to our knowledge. METHODS: Nineteen children (twelve boys and seven girls with a mean age of nine years and eight months) with spastic diplegia were enrolled. Eleven children were randomized to the surgical group and eight, to the control group. The control group underwent a program of progressive resistance strength training. The randomized phase of the trial concluded at twelve months. The control group then exited the study and progressed to surgery, whereas the surgical group continued to be followed in a prospective cohort study. The primary outcome measures were the Gait Profile Score (GPS) and the Gillette Gait Index (GGI). Secondary outcome measures were gross motor function (Gross Motor Function Measure-66 [GMFM-66]), functional mobility (Functional Mobility Scale [FMS]), time spent in the upright position, and health-related quality of life (Child Health Questionnaire [CHQ]). RESULTS: A total of eighty-five surgical procedures were performed, with a mean of eight procedures per child (standard deviation, four). The surgical group had a 34% improvement in the GPS and a 57% improvement in the GGI at twelve months. The control group had a small nonsignificant deterioration in both indices. The between-group differences for the change in the GPS (-5.5; 95% confidence interval, -7.6 to -3.4) and the GGI (-218; 95% confidence interval, -299 to -136) were highly significant. The differences between the groups with regard to the secondary outcome measures were not significant at twelve months. At twenty-four months after surgery, there was a 4.9% increase in the GMFM-66 score and improvements in the FMS score, time spent in the upright position, and the physical functioning domain of the CHQ in the surgical group. CONCLUSIONS: This study provides Level-II evidence that single-event multilevel surgery improves the gait of children with spastic diplegic cerebral palsy twelve months after surgery. Improvements in other domains, including gross motor function and quality of life, were not observed until twenty-four months after surgery.

Management of the knee in spastic diplegia: what is the dose?

This article discusses the sagittal gait patterns in children with spastic diplegia, with an emphasis on the knee, as well as the concept of the "dose" of surgery that is required to correct different gait pathologies. The authors list the various interventions in the order of their increasing dose. The concept of dose is useful in the consideration of the management of knee dysfunction.

A classification system for hip disease in cerebral palsy.

In population-based studies, hip displacement affects approximately one-third of children with cerebral palsy (CP). Given the extreme range of clinical phenotypes in the CP spectrum, it is unsurprising that hip development varies from normality, to dislocation and degenerative arthritis. Numerous radiological indices are available to measure hip displacement in children with CP; however, there is no grading system for assessing hip status in broad categorical terms. This makes it difficult to audit the incidence of hip displacement, determine the relationship between hip displacement and CP subtypes, assess the outcome of intervention studies, and to communicate hip status between health care professionals. We developed a categorical, radiographic classification of hip morphology based on qualitative indices and measurement of the key continuous variable, the migration percentage of Reimers. One hundred and thirty-four radiographs were reviewed of 52 female and 82 male adolescents with CP who were at, or close to, skeletal maturity (mean age 16y 1mo [SD 1y 4mo] range 14y to 19y 1mo). Twenty-nine were classified at Gross Motor Function Classification System level I, 25 at level II, 27 at level III, 24 at level IV, and 29 at level V. A classification system was developed to encapsulate the full spectrum of hip morphology in CP, with and without intervention.

Botulinum neurotoxin A: an unusual systemic effect.

Systemic effects from Botulinum neurotoxin A are uncommon but can have serious consequences. We report the case of a boy with severe cerebral palsy who developed deterioration in respiratory and oromotor function following repeated injections of Botulinum neurotoxin A. Caution is needed in using this treatment in children with severe cerebral palsy and pseudobulbar palsy.