The Role of Tissue Expansion Before Baclofen Pump Insertion in the Pediatric Population.

BACKGROUND: Tissue expansion is a well-established technique for soft tissue reconstruction in the pediatric population. We present a case series of this technique to create a safe pocket for baclofen pump insertion to minimize risk of complications including pump migration, extrusion, wound dehiscence and infection. METHODS: A case series of 3 pediatric patients undergoing tissue expansion prior to baclofen pump insertion at a single center in Melbourne. The expansion procedure was performed by senior surgeon N Low in all cases, and patients followed up 6 months after expander-pump exchange. The study was conducted over a 4-year period 2019 to 2023. RESULTS: We suffered 2 minor complications with the tissue expansion process including cellulitis and pump deflation. Neither complication precluded further tissue expansion nor led to premature removal of the expander. All patients proceeded to safely complete expander-pump exchange. One patient suffered a small (6 mL) abdominal seroma associated with pump insertion, which required no intervention. All patients have had a successful outcome 6 months after pump insertion. CONCLUSIONS: We describe a reliable and reproducible approach in creating a safe abdominal wall pocket to better accommodate baclofen pump insertion. Our series has overcome the imbalance between device size and abdominal wall pocket, now offering an alternative approach to achieve the safe delivery of intrathecal baclofen in the pediatric population.

Comparison of two modeling approaches for the identification of predictors of complications in children with cerebral palsy following spine surgery.

BACKGROUND: Children with non-ambulatory cerebral palsy (CP) frequently develop progressive neuromuscular scoliosis and require surgical intervention. Due to their comorbidities, they are at high risk for developing peri- and post-operative complications. The objectives of this study were to compare stepwise and LASSO variable selection techniques for consistency in identifying predictors when modelling these post-operative complications and to identify potential predictors of respiratory complications and infections following spine surgery among children with CP. METHODS: In this retrospective cohort study, a large administrative claims database was queried to identify children who met the following criteria: 1) ≤ 25 years old, 2) diagnosis of CP, 3) underwent surgery during the study period, 4) had ≥ 12-months pre-operative, and 5) ≥ 3-months post-operative continuous health plan enrollment. Outcome measures included the development of a post-operative respiratory complication (e.g., pneumonia, aspiration pneumonia, atelectasis, pleural effusion, pneumothorax, pulmonary edema) or an infection (e.g., surgical site infection, urinary tract infection, meningitis, peritonitis, sepsis, or septicemia) within 3 months of surgery. Codes were used to identify CP, surgical procedures, medical comorbidities and the development of post-operative respiratory complications and infections. Two approaches to variable selection, stepwise and LASSO, were compared to determine which potential predictors of respiratory complications and infection development would be identified using each approach. RESULTS: The sample included 220 children. During the 3-month follow-up, 21.8% (n = 48) developed a respiratory complication and 12.7% (n = 28) developed an infection. The prevalence of 11 variables including age, sex and 9 comorbidities were initially considered to be potential predictors based on the intended outcome of interest. Model discrimination utilizing LASSO for variable selection was slightly improved over the stepwise regression approach. LASSO resulted in retention of additional comorbidities that may have meaningful associations to consider for future studies, including gastrointestinal issues, bladder dysfunction, epilepsy, anemia and coagulation deficiency. CONCLUSIONS: Potential predictors of the development of post-operative complications were identified in this study and while identified predictors were similar using stepwise and LASSO regression approaches, model discrimination was slightly improved with LASSO. Findings will be used to inform future research processes determining which variables to consider for developing risk prediction models.

Intrathecal baclofen therapy as treatment for spasticity and dystonia: Review of cases in a pediatric palliative care unit.

INTRODUCTION: Patients managed in the Pediatric Palliative Care Integral Unit (PPCIU) have serious neurological conditions that involve significant damage at central nervous system level. The movement disorder is a very common clinical problem and for the patients where an adequate control of muscle tone is not achieved with usual techniques or drugs, intrathecal baclofen therapy (IBT) should be considered. MATERIALS AND METHODS: Descriptive retrospective study based on the review of clinical records of patients who received IBT being followed by the PPCIU of Madrid Autonomous Region in the timeframe between September 2012 and February 2021. RESULTS: IBT was implanted in 8 patients affected by infantile cerebral palsy (ICP) with a Gross Motor Function Scale (GMFCS) IV-V, 3 patients was a Pantothenate kinase deficit-associated neurodegeneration (PKAN), 2 had Acquired Brain Damage, and the remaining 3 had, respectively, 2 glutaric aciduria type I (GA-1), and poly-malformative syndrome. In all patients we observed a period of clinical stability after IBT, we call this period "honeymoon". Two patients died while in the honeymoon period, at 24.9 and 19.6 months from implantation of the pump; the median of duration of the honeymoon period in the remaining 14 was 14.4 months (IQ: 8.3-25.8). CONCLUSIONS: IBT was not only used in patients with non-progressive diseases, but also in the group of patients with neurodegenerative or progressive diseases. In all of them, after implantation of the device, we have objectified a period of clinical stability and a better control of muscle tone disorders.

Exploring the Relationship between Cerebral Palsy and Hip Dysplasia: Insights from the National Inpatient Sample.

Background and Objective: Cerebral palsy (CP) significantly impacts quality of life globally. Hip dysplasia (HD) is a common musculoskeletal issue in CP patients. This study investigates the prevalence, risk factors, and impact of HD on CP patients using a large national database. Materials and Methods: Data from the National Inpatient Sample (NIS) database (2016-2019) were used, identifying CP and HD diagnoses through ICD-10 codes. Baseline characteristics were tabulated. Univariate and multivariate logistic regression analyses examined predictors of HD development in CP patients, presenting data as odds ratios (ORs) with 95% confidence intervals (CIs). Results: Among 3,951,040 pediatric patients, 28,880 had CP (27,466 without HD, and 1414 with HD), and 3,922,160 did not have CP. CP significantly increased the likelihood of developing HD in univariate (OR = 35.03, 95% CI [33.01, 37.17], p < 0.0001) and multivariate (OR = 26.61, 95% CI [24.94, 28.40], p < 0.0001) analyses. Among patients with CP, race was significantly associated with HD, with ORs below 1 for all racial categories compared to Whites. Females had nearly twice the odds of HD compared to males (OR = 1.96, 95% CI [1.86, 2.05], p < 0.0001). Age was significantly associated with HD, with each additional year increasing the odds (OR = 1.03, 95% CI [1.026, 1.034], p < 0.0001). Individuals in the high 51st-75th income quartile had a 17% increase in the odds of HD (OR = 1.17, 95% CI [1.09, 1.25], p < 0.0001) compared to the low 1st-25th income quartile. Conclusions: This study reinforces the strong association between CP and HD, highlighting the need for further research and prospective studies to validate these findings.

In-Home Intravenous Dexmedetomidine Infusion for Treatment of Dystonia: A Pediatric Case Report.

The off-label use of an intravenous dexmedetomidine infusion is an option for refractory symptoms in pediatric palliative care with a few published cases of in-home use, most limited to end-of-life care. After an intrathecal baclofen pump malfunction with meningitis and medication withdrawal, a 17-year-old adolescent with quadriparetic cerebral palsy and paroxysmal autonomic instability experienced relief of refractory pain and dystonia with an in-home intravenous dexmedetomidine infusion. His interdisciplinary care team collaborated to establish safety measures and adjust his home medication regimen to further improve quality of life well before his end of life.

Constraint-Induced Movement Therapy (CIMT) and Neural Precursor Cell (NPC) Transplantation Synergistically Promote Anatomical and Functional Recovery in a Hypoxic-Ischemic Mouse Model.

Cerebral palsy (CP) is a common neurodevelopmental disorder characterized by pronounced motor dysfunction and resulting in physical disability. Neural precursor cells (NPCs) have shown therapeutic promise in mouse models of hypoxic-ischemic (HI) perinatal brain injury, which mirror hemiplegic CP. Constraint-induced movement therapy (CIMT) enhances the functional use of the impaired limb and has emerged as a beneficial intervention for hemiplegic CP. However, the precise mechanisms and optimal application of CIMT remain poorly understood. The potential synergy between a regenerative approach using NPCs and a rehabilitation strategy using CIMT has not been explored. We employed the Rice-Vannucci HI model on C57Bl/6 mice at postnatal day (PND) 7, effectively replicating the clinical and neuroanatomical characteristics of hemiplegic CP. NPCs were transplanted in the corpus callosum (CC) at PND21, which is the age corresponding to a 2-year-old child from a developmental perspective and until which CP is often not formally diagnosed, followed or not by Botulinum toxin injections in the unaffected forelimb muscles at PND23, 26, 29 and 32 to apply CIMT. Both interventions led to enhanced CC myelination and significant functional recovery (as shown by rearing and gait analysis testing), through the recruitment of endogenous oligodendrocytes. The combinatorial treatment indicated a synergistic effect, as shown by newly recruited oligodendrocytes and functional recovery. This work demonstrates the mechanistic effects of CIMT and NPC transplantation and advocates for their combined therapeutic potential in addressing hemiplegic CP.

Retrograde Insertion Approach for Anterior Distal Femoral Hemiepiphysiodesis Procedure: A Case Report.

CASE: A 13-year-old adolescent boy with spastic diplegic cerebral palsy (CP) presented with crouch gait and bilateral knee flexion contractures (KFCs). After failure of conservative treatments, the patient was referred to orthopaedics for surgical intervention. Anterior distal femoral hemiepiphysiodesis (ADFH), using a retrograde approach, and Strayer gastrocnemius recession were performed bilaterally. No complications were reported during surgery/follow-up. Full-active knee extension and improved gait were achieved. CONCLUSION: KFC is prevalent in ambulatory patients with CP, reducing gait function. Retrograde ADFH is an effective surgical intervention to reduce KFC and improve gait, and offers simpler insertion/removal than the traditional antegrade technique.

Reliability and Accuracy of Ultrasound Measurement of Hip Displacement in Children with Cerebral Palsy.

OBJECTIVE: Hip migration percentage (MP) measured on anteroposterior pelvis radiographs is the gold standard to assess the severity of hip displacement in children with cerebral palsy (CP). Repeated exposure of these children to ionizing radiation under a hip surveillance program is undesirable. Recently, a semi-automatic approach to measure MPUS on ultrasound (US) images was validated in a phantom study. This pilot in vivo study applied the previous phantom method and aimed to determine the reliability and accuracy of the MPUS. METHODS: Thirty-four children (23 boys and 11 girls) aged 8.9 ± 3.1 y old and diagnosed with CP were recruited. A total of 59 hips were scanned once, while 43 of these were scanned twice to evaluate the test-retest reliability. Two raters (R1 and R2) manually measured MPUS; procedures included selecting images of interest, cropping a region of interest and removing soft tissues on hip US images. Custom software was developed to measure MP automatically after the manual pre-image processing. RESULTS: The intra-class correlation coefficients (ICC2,1) for the test-retest (R1), intra-rater (R1) and inter-rater (R1 vs R2) reliabilities were 0.90, 0.94 and 0.82, respectively. The standard error of measurement of MPUS for all three evaluations was ≤3.0%. The mean absolute difference between MPUS and MPX-ray and the percentage of MPUS within clinical acceptance error of 10% for R1 and R2 were (R1: 6.2% ± 4.9%, 84.7%) and (R2: 7.6% ± 6.1%, 73.7%), respectively. CONCLUSION: This study demonstrated that US scans were repeatable and MPUS could be measured reliably and accurately.

Hip Displacement After Triradiate Closure in Ambulatory Cerebral Palsy: Who Needs Continued Surveillance?

BACKGROUND: Hip surveillance in cerebral palsy (CP) is an accepted practice with evidence-based guidelines implemented. For the skeletally immature with open triradiate cartilage (TRC), recommendations for radiographic surveillance stemmed from population-based studies. For nonambulatory CP, progression of hip displacement after skeletal maturity has been reported; less is known for ambulatory CP. We aimed to determine the prevalence and risk factors associated with progressive hip displacement after TRC closure, a proxy for skeletal maturity, for ambulatory CP. METHODS: This is a retrospective cohort study of patients with ambulatory CP (Gross Motor Function Classification System I-III), with unilateral or bilateral involvement, hypertonic motor type, regular hip surveillance (≥3 radiographs after age 10 yr, 1 before TRC closure, ≥1 after age 16 yr), and 2-year follow-up post-TRC closure. The primary outcome was migration percentage (MP). Other variables included previous preventative/reconstructive surgery, topographic pattern, sex, scoliosis, epilepsy, and ventriculoperitoneal shunt. An "unsuccessful hip" was defined by MP ≥30%, MP progression ≥10%, and/or requiring reconstructive surgery after TRC closure. Statistical analyses included chi-square and multivariate Cox regression. Kaplan-Meier survivorship curves were also determined. Receiver operating characteristic analysis was used to determine the MP threshold for progression to an "unsuccessful hip" after TRC closure. RESULTS: Seventy-six patients (39.5% female) met the inclusion criteria, mean follow-up 4.7±2.1 years after TRC closure. Sixteen (21.1%) patients had an unsuccessful hip outcome. By chi-square analysis, diplegia ( P =0.002) and epilepsy ( P =0.04) were risk factors for an unsuccessful hip. By multivariate analysis, only first MP after TRC closure ( P <0.001) was a significant risk factor for progression to an unsuccessful hip; MP ≥28% being the determined threshold (receiver operating characteristic curve analysis, area under curve: 0.845, P <0.02). CONCLUSIONS: The risk of MP progression after skeletal maturity is relatively high (21%), similar to nonambulatory CP. Annual hip surveillance radiographs after TRC closure should continue for Gross Motor Function Classification System I-III with an MP ≥28% after TRC closure, especially for bilateral CP and epilepsy. LEVEL OF EVIDENCE: III.

Efficacy of a Multimodal Surgical Site Injection for Postoperative Pain Control in Pediatric Patients With Cerebral Palsy Undergoing Hip Reconstruction: A Randomized Controlled Trial.

BACKGROUND: One in 4 children with cerebral palsy (CP) will undergo orthopaedic surgery during their childhood. Despite its ubiquity, postoperative pain control has been poorly studied in this patient population. Moreover, poor pain management has been associated with adverse surgical outcomes. Multimodal analgesic injections have been well studied in the adult population, demonstrating safety and efficacy in reducing postoperative pain and narcotic consumption, but this modality has not been studied in pediatric patients undergoing similarly complex procedures. The objective of this study was to evaluate the efficacy of a multimodal surgical site injection for postoperative pain control following operative management of hip dysplasia in patients with CP. METHODS: After obtaining IRB approval, a multicenter, randomized double-blind placebo control trial was completed. Patients below 18 years old with a diagnosis of CP who were scheduled for varus derotation osteotomy (VDRO) of the proximal femur were randomized to receive a surgical-site injection with either a combination of ropivacaine (3 mg/kg), epinephrine (0.5 mg), and ketorolac (0.5 mg/kg) (experimental group) or normal saline (control). All included patients had identical postoperative care, including immobilization, physical therapy, and standardized, multimodal postoperative pain control. Pain scores and narcotic consumption were recorded at regular intervals and compared between groups utilizing two-tailed t test or a nonparametric Mann-Whitney test for quantitative variables and a Fischer exact test for categorical variables. RESULTS: Thirty-four patients were included, evenly divided between study arms. There were no significant differences in demographic variables, gross motor function classification system (GMFCS), comorbidities, preoperative radiographic parameters, or concomitant surgeries between groups. Patients in the experimental group required significantly lower narcotic medications at all postoperative time points from PACU until hospital discharge compared with controls (0.41 ± 0.42 vs. 1.87 ± 2.05 total morphine mEQ/kg, P =0.01). Similarly, patients in the experimental group were found to have significantly lower pain scores throughout their hospital stays compared with controls (1.0 ± 0.6 vs. 2.4 ± 1.1 mean pain score, P <0.001). There were no significant differences in operative time, OR time, blood transfusion requirements or hospital length of stay between groups. There were no adverse medication reactions or injection site complications in either group. CONCLUSIONS: In patients with CP undergoing hip reconstruction, surgical-site injection with a multimodal analgesic combination improves pain control and reduces narcotic consumption in the early postoperative period with no observed adverse effects. SIGNIFICANCE: Local multimodal analgesic injections should be adopted as part of standard multimodal pain control in this patient population for all osseous surgeries. LEVEL OF EVIDENCE: Level I-therapeutic.

'I Didn't Know What Was Going to Happen': Children's and Young People's Experiences and Their Involvement Before and After Selective Dorsal Rhizotomy.

AIMS: To explore experiences, expectations, and involvement of children and young people (CYP) in decision-making for selective dorsal rhizotomy (SDR) surgery, post-operative physiotherapy treatment and outcomes. METHODS: A qualitative study design using one to one interviews. Five CYP (2 girls and 3 boys) participated, and interviews lasted between 45 min and 2 h. Data were analyzed using thematic analysis. RESULTS: Children and young people are reliant on their parents to make decisions and inform them of the SDR process. Experiences of living with cerebral palsy and its management are centered on their routine social, psychological, and physiological challenges. Individual characteristics and attributes of CYP have an impact on how they cope with the rehabilitation burden and adjust to their changing levels of function and participation. CONCLUSIONS: Although CYP reported that SDR offers them a greater 'freedom to choose' in how they participate in daily life, further consideration is required to meet their psychosocial needs, particularly in preparing for SDR and adjusting afterwards.

Ultrasonic neuromodulation as a new therapy for spasticity in an animal model of spastic cerebral palsy.

PURPOSE: This study aimed to evaluate a new therapeutic option for the spasticity using ultrasound neuromodulation in an animal model of spastic cerebral palsy. METHODS: Thirty-two adult male Wistar rats were randomly distributed in: negative control (NC); positive control (PC); untreated model (UTM); and treated model (TM). Rats in the control groups received sham surgery, and rats in the model groups received the spastic cerebral palsy model surgery. The rats' motor functions were evaluated by the Rotarod and CatWalk tests before and after surgery. PC and TM groups underwent ultrasonic neuromodulation by a physiotherapeutic ultrasound (intensity 0.1 W/cm2, at 1 MHz) continuous mode for 5 seconds, for seven days. RESULTS: Twelve rats showed a spastic pattern (UTM = 6 and TM = 6), motor limitations (UTM = 6 and TM = 6), and ten had difficulty feeding (UTM = 5 and TM = 5). One UTM group rat could not recover its preoperative latency time, while the other rats in the model groups did. The speed at which the limbs swung reduced after surgery and increased in subsequent assessments, demonstrating greater instability and a deficit in locomotion balance. CONCLUSIONS: Results were not yet sufficient to assert ultrasound neuromodulation as a possible therapy for spasticity in spastic cerebral palsy in the parameters used, and more studies are necessary.

Do Adductor Tenotomies Prevent Progressive Migration in Children with Cerebral Palsy?: A Systematic Review.

BACKGROUND: Up to one-third of children with cerebral palsy (CP) develop migration of the hip, and the risk increases with a higher Gross Motor Function Classification System (GMFCS). In progressive hip migration in young children, adductor tenotomy is an accepted treatment option to delay or prevent progressive hip migration. However, there is quite a large variability in reported results. This systematic review aims to determine the effectiveness of a soft-tissue release in the prevention of progressive hip migration in children with CP. METHODS: This systematic review was performed in accordance with the guidelines of the Cochrane Handbook for Systematic Reviews and the Preferred Reporting Items for Systematic Review and Meta-Analysis Protocols statements. Our inclusion criteria were studies describing pediatric, skeletally immature patients with CP and a "hip at risk" of progressive hip migration. Exclusion criteria were simultaneous bony reconstructions, case reports, technical notes, published abstracts, or studies with a follow-up under 1 year postoperatively. The primary outcomes were defined as failure rate (progressive hip migration and/or need for bony surgery, as defined by each paper) and change in migration percentage (MP) at final follow-up. As secondary analyses, we evaluated the outcome after specific subtypes of surgeries and assessed whether performing lengthening of iliopsoas, neurectomy of the anterior branch of the obturator nerve, age at the time of surgery, GMFCS level, and postoperative management impact the outcome. RESULTS: Our literature search identified 380 titles. Eighty-four articles underwent full-text review, of which 27 met our inclusion/exclusion criteria and were subsequently selected for quantitative analysis. A prevalence meta-analysis was performed including 17 studies (2,213 hips). Mean follow-up ranged from 12 to 148.8 months. The mean preoperative MP was 33.4% (2,740 hips) and 29.9% at follow-up. The overall reported failure rate was 39% (95% confidence interval, 26%-52%). Performing a release of only adductor longus had a failure rate of 87%, whereas more extensive soft-tissue releases showed significantly better results with failure rates ranging from 0 to 44% (p < 0.001). Lengthening of the iliopsoas had no significant impact on failure rate (p = 0.48), nor did performing an obturator neurectomy (p = 0.92). CONCLUSION: The failure rate of adductor tenotomies to prevent progressive hip migration appears to be as high as 39% in studies with a varying follow-up. The failure rates are significantly higher when isolated release of the adductor longus is performed. This systematic review supports clinical decision making in children with CP and early hip migration. LEVEL OF EVIDENCE: Level IIA. See Instructions for Authors for a complete description of levels of evidence.

Total Knee Arthroplasty in Patients with Cerebral Palsy: A Large Database Analysis.

Cerebral palsy (CP) is a neurodevelopmental condition that can result in altered gait biomechanics, joint dysfunction, and imbalance. The complications associated with total knee arthroplasty (TKA) in patients with CP have not yet been well described. Therefore, our analysis sought to compare the 90-day and 2-year complications following TKA in patients with and without CP. The PearlDiver Mariner database was utilized to identify patients with CP undergoing primary TKA between 2010 and 2020. This cohort was matched 1:4 to a control cohort without neurodegenerative disorders based on age, sex, Elixhauser Comorbidity Index (ECI), tobacco use, obesity, and diabetes. A total of 3,257 patients (657 CP patients 2,600 controls) were included in our final analysis. A multivariable logistic regression analysis was utilized to determine the risk of CP on medical and surgical complications at 90 days and all-cause revision rates at 2 years. Patients with CP had an increased risk of acute kidney injury (odds ratio [OR]: 1.66; 95% confidence interval [CI]: 1.07-2.5; p = 0.019), pneumonia (OR: 5.63; 95% CI: 3.69-8.67; p < 0.001), urinary tract infection (OR: 5.01; 95% CI: 3.85-6.52; p < 0.001), and transfusion (OR: 2.21; 95% CI: 1.50-3.23; p < 0.001). CP patients additionally had a higher incidence of emergency department (ED) visits (OR: 5.24; 95% CI: 3.76-7.32; p < 0.001) and readmissions (OR: 5.24; 95% CI: 2.57-4.96; p < 0.001). There were no differences in rates of periprosthetic joint infection (PJI; OR: 1.23; 95% CI: 0.69-2.10; p = 0.463), surgical site infection (SSI; OR: 0.51; 95% CI: 0.12-1.46; p = 0.463), and reoperation (OR: 1.35; 95% CI: 0.71-2.43; p = 0.339) at 90 days postoperatively. The all-cause revision rates at 2 years were comparable (OR: 1.02; 95% CI: 0.67-1.51; p = 0.927). In this database review, we found that CP patients have a higher risk of medical complications in the acute postoperative period following TKA. The 90-day surgical complication and 2-year revision rates in CP patients were comparable to matched controls.

Preoperative gastric volume assessment using ultrasound in cerebral palsy pediatric patients: a prospective observational study.

BACKGROUND: Although cerebral palsy is a risk factor for aspiration, there is insufficient research on residual gastric volume after preoperative fasting in children with cerebral palsy. We evaluated the incidence of a full stomach by ultrasound assessment of the gastric volume in children with cerebral palsy who underwent orthopedic surgery after preoperative fasting. METHODS: The patients fasted for 8 h for solid foods and 2 h for clear liquids. We obtained the gastric antral cross-sectional area using ultrasound in the semi-recumbent and right lateral decubitus positions. A calculated stomach volume > 1.5 mL.kg-1 was considered as full, which poses a high aspiration risk. The primary outcome was the incidence of full stomach, and the secondary outcomes were the qualitative gastric volume, correlation of disease severity categorized according to the Gross Motor Function Classification System with the residual gastric volume, gastric volume per body weight, and qualitative gastric volume. RESULTS: Thirty-seven pediatric patients with cerebral palsy, scheduled for elective orthopedic surgery, were included for analysis. Full-stomach status was observed in none, and the gastric volume per body weight was 0.5 (0.4-0.7) mL.kg-1. No significant differences were observed in the residual gastric volume (p = 0.114), gastric volume per body weight (p = 0.117), or qualitative grade of gastric volume (p = 0.642) in relation to disease severities. CONCLUSION: Children with cerebral palsy who fasted preoperatively had empty or nearly empty stomachs. Further studies are required to determine the optimal fasting duration for such children.

Collagen architecture and biomechanics of gracilis and adductor longus muscles from children with cerebral palsy.

Cerebral palsy (CP) describes some upper motoneuron disorders due to non-progressive disturbances occurring in the developing brain that cause progressive changes to muscle. While longer sarcomeres increase muscle stiffness in patients with CP compared to typically developing (TD) patients, changes in extracellular matrix (ECM) architecture can increase stiffness. Our goal was to investigate how changes in muscle and ECM architecture impact muscle stiffness, gait and joint function in CP. Gracilis and adductor longus biopsies were collected from children with CP undergoing tendon lengthening surgery for hamstring and hip adduction contractures, respectively. Gracilis biopsies were collected from TD patients undergoing anterior cruciate ligament reconstruction surgery with hamstring autograft. Muscle mechanical testing, two-photon imaging and hydroxyproline assay were performed on biopsies. Corresponding data were compared to radiographic hip displacement in CP adductors (CPA), gait kinematics in CP hamstrings (CPH), and joint range of motion in CPA and CPH. We found at matched sarcomere lengths muscle stiffness and collagen architecture were similar between TD and CP hamstrings. However, CPH stiffness (R2 = 0.1973), collagen content (R2 = 0.5099) and cross-linking (R2 = 0.3233) were correlated to decreased knee range of motion. Additionally, we observed collagen fibres within the muscle ECM increase alignment during muscular stretching. These data demonstrate that while ECM architecture is similar between TD and CP hamstrings, collagen fibres biomechanics are sensitive to muscle strain and may be altered at longer in vivo sarcomere lengths in CP muscle. Future studies could evaluate the impact of ECM architecture on TD and CP muscle stiffness across in vivo operating ranges. KEY POINTS: At matched sarcomere lengths, gracilis muscle mechanics and collagen architecture are similar in TD patients and patients with CP. In both TD and CP muscles, collagen fibres dynamically increase their alignment during muscle stretching. Aspects of muscle mechanics and collagen architecture are predictive of in vivo knee joint motion and radiographic hip displacement in patients with CP. Longer sarcomere lengths in CP muscle in vivo may alter collagen architecture and biomechanics to drive deficits in joint mobility and gait function.

Methods of muscle spasticity assessment in children with cerebral palsy: a scoping review.

BACKGROUND: Evaluating muscle spasticity in children with cerebral palsy (CP) is essential for determining the most effective treatment strategies. This scoping review assesses the current methods used to evaluate muscle spasticity, highlighting both traditional and innovative technologies, and their respective advantages and limitations. METHODS: A search (to April 2024) used keywords such as muscle spasticity, cerebral palsy, and assessment methods. Selection criteria included articles involving CP children, assessing spasticity objectively/subjectively, comparing methods, or evaluating method effectiveness. RESULTS: From an initial pool of 1971 articles, 30 met our inclusion criteria. These studies collectively appraised a variety of techniques ranging from well-established clinical scales like the modified Ashworth Scale and Tardieu Scale, to cutting-edge technologies such as real-time sonoelastography and inertial sensors. Notably, innovative methods such as the dynamic evaluation of range of motion scale and the stiffness tool were highlighted for their potential to provide more nuanced and precise assessments of spasticity. The review unveiled a critical insight: while traditional methods are convenient and widely used, they often fall short in reliability and objectivity. CONCLUSION: The review discussed the strengths and limitations of each method and concluded that more reliable methods are needed to measure the level of muscle spasticity more accurately.

In-hospital post-operative fever after posterior spinal fusion for neuromuscular scoliosis in non-ambulatory cerebral palsy patients: is there value for clinical workup for an isolated fever?

PURPOSE: Children with neuromuscular scoliosis (NMS) resultant to cerebral palsy (CP) are at a heightened risk for complications following surgical treatment. These children have a reported 22-64% rate of post-operative fever development, and additional fever workup has been shown to have limited clinical utility. However, this has yet to be investigated in the setting of an accelerated discharge (AD) pathway. METHODS: A retrospective review of children with non-ambulatory CP treated at 2 centers with posterior spinal fusion (PSF) for NMS was performed. One institution uses a standardized AD post-operative pathway for NMS patients, whereas the second institution had no standard pathway. A post-operative fever was defined as temperature > 38.5 °C. Target outcome variables included the development of a fever as well as re-admission within 90 days of surgery. RESULTS: A total of 122 non-ambulatory children were identified (82% GMFCS V, mean 14.3 ± 3.4 years at surgery). A post-operative fever was documented in 75.4% of patients (N = 92) and all additional culture studies reported negative results. Children admitted to the PICU were more likely to undergo a fever workup (P < 0.001) and more likely to receive additional or extended antibiotic therapy (P < 0.001). Children treated at the AD pathway had a significantly lower rate of PICU admission (P < 0.001). Post-operative PICU admission was associated with a post-operative fever (49.5% vs 25%, P = 0.03). CONCLUSION: Non-ambulatory CP children with NMS undergoing PSF have a 75.4% rate of developing early post-operative fevers. Reflexive fever work-ups provided limited clinical utility while increasing the hospital length of stay and potentially exposing patients to antibiotic-related complications.

Impact of Spinal Deformity and Surgery on Health-Related Quality of Life in Cerebral Palsy: A Multicenter Prospective Controlled Trial.

BACKGROUND: Spinal fusion for scoliosis associated with cerebral palsy (CP) is challenging to study because specialized outcome measures are needed. Therefore, evidence in favor of the benefits of surgery has not been firmly established. This study aimed to determine if corrective spinal fusion improves health-related quality of life (HRQoL) in children with CP scoliosis at 2 years. METHODS: Children with CP and scoliosis who met the criteria for posterior spinal fusion were offered enrollment at 16 US and Canada centers. Participants' families selected either operative intervention (OP) or nonoperative treatment (NON) in discussion with their surgeon with no influence by the decision to participate in the research study. Demographic, clinical data (function level, magnitude of deformity, comorbidities), and HRQoL (CPCHILD Questionnaire) were collected at baseline and 2 years. Change (from baseline) in total CPCHIL scores was the primary outcome. RESULTS: Three hundred one OP and 34 NON subjects had complete baseline and 2-year data. At baseline, both groups were comparable in function level, comorbid status, and CPCHILD scores (52.1 ±15.3 vs. 53.4 ±14.5; P =0.66). The OP group had a larger spinal deformity magnitude (84.5˚ ± 21.8˚ vs. 66.3˚ ± 18.1˚) ( P =0.001). The total CPCHILD score improved in the OP group by 6.6 points ( P <0.001). NON scores were unchanged (+1.2; P =0.65) during follow-up. There were also significant score increases in the OP group for 5 of 6 CPCHILD domains. The change in CPCHILD scores from enrollment to 2 years was more significant in the OP group ( P =0.05). CONCLUSION: For children with CP who undergo spinal fusion, HRQoL improved over preoperative levels and an unchanged nonoperative control group. LEVEL OF EVIDENCE: Level II.