Incidence of hip problems in developmental central hypotonia: A scoping review.

AIM: To describe what is known about hip problems in individuals with developmental central hypotonia. METHOD: Searches were conducted in five databases to October 2023. Down syndrome was excluded from this analysis of less well-known genetic diagnoses. At least two reviewers independently screened titles, abstracts, read full-text articles, and extracted data. RESULTS: Of 89 full-text articles, 79 met inclusion criteria. Studies included 544 individuals aged 1 month to 63 years with Kabuki, 49, XXXXY, Prader-Willi, PURA, Koolen de Vries, Emanuel, TRPM3, Wolf-Hirschhorn, and other rare syndromes. Most diagnoses may be associated with a combination of differences in hip structure or stability that are evident at birth, or develop in early infancy, with increasing hip dysplasia and subluxation over time. Joint or ligamentous laxity was most reported along with hypotonia and hypermobility as risk factors. Limited data were identified about conservative or surgical intervention and outcomes in these populations. INTERPRETATION: Children with significant hypotonia, with or without a confirmed genetic diagnosis, are at increased risk of hip problems that may be missed with standard neonatal screening. Ultrasound is recommended between 6 weeks and 6 months, and annual orthopaedic review with regular radiographs for older children and adults with significant and persistent hypotonia.

Reliability of a Photo-Based Modified Foot Posture Index (MFPI) in Quantifying Severity of Foot Deformity in Children With Cerebral Palsy.

INTRODUCTION: Children with cerebral palsy (CP) have high rates of foot deformity. Accurate assessment of foot morphology is crucial for therapeutic planning and outcome evaluation. This study aims to evaluate the reliability of a novel photo-based Modified Foot Posture Index (MFPI) in the evaluation of foot deformity in children with CP. METHODS: Thirteen orthopaedic surgeons with neuromuscular clinical focus from 12 institutions evaluated standardized standing foot photographs of 20 children with CP, scoring foot morphology using the MFPI. Raters scored the standardized photographs based on five standard parameters. Two parameters assessed the hindfoot: curvature above and below the malleoli and calcaneal inversion/eversion. Three parameters assessed the midfoot and forefoot: talonavicular congruence, medial arch height, and forefoot abduction/adduction. Summary MFPI scores range from -10 to +10, where positive numbers connote planovalgus, whereas negative numbers connote a tendency toward cavovarus. Intra- and interrater reliability were calculated using a 2-way mixed model of the intraclass correlation coefficient (ICC) set to absolute agreement. RESULTS: Feet spanned the spectrum of potential pathology assessable by the MFPI, including no deformity, mild, moderate, and severe planovalgus or cavovarus deformities. All scored variables showed high intrarater reliability with ICCs from 0.891 to 1. ICCs for interrater reliability ranged from 0.965 to 0.984. Hindfoot total score had an ICC of 0.979, with a 95% CI, 0.968-0.988 (P<0.001). The forefoot total score had an ICC of 0.984 (95% CI, 0.976-0.991, P<0.001). Mean total score by the MFPI was 3.67 with an ICC of 0.982 (95% CI, 0.972-0.990, P<0.001). CONCLUSIONS: The photo-based MFPI demonstrates high intra- and interrater reliability in assessing foot deformities in children with CP. Its noninvasive nature and ease of use make it a promising tool for both clinical and research settings. MFPI should be considered as part of standard outcomes scores in studies regarding the treatment of CP-associated foot deformities. LEVEL OF EVIDENCE: Level V.

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.

Development and validation of a stakeholder-driven, self-contained electronic informed consent platform for trio-based genomic research studies.

Increasingly long and complex informed consents have yielded studies demonstrating comparatively low participant comprehension and satisfaction with traditional face-to-face approaches. In parallel, interest in electronic consents for clinical and research genomics has steadily increased, yet limited data are available for trio-based genomic discovery studies. We describe the design, development, implementation, and validation of an electronic iConsent application for trio-based genomic research deployed to support genomic studies of cerebral palsy. iConsent development incorporated stakeholder perspectives including researchers, patient advocates, institutional review board members, and genomic data-sharing considerations. The iConsent platform integrated principles derived from prior electronic consenting research and elements of multimedia learning theory. Participant comprehension was assessed in an interactive teachback format. The iConsent application achieved nine of ten proposed desiderata for effective patient-focused electronic consenting for genomic research. Overall, participants demonstrated high comprehension and retention of key human subjects' considerations. Enrollees reported high levels of satisfaction with the iConsent, and we found that participant comprehension, iConsent clarity, privacy protections, and study goal explanations were associated with overall satisfaction. Although opportunities exist to optimize iConsent, we show that such an approach is feasible, can satisfy multiple stakeholder requirements, and can realize high participant satisfaction and comprehension while increasing study reach.

Scoliosis Development in Spinal Muscular Atrophy: The Influences of Genetic Severity, Functional Level, and Disease-Modifying Treatments.

BACKGROUND: Spinal muscular atrophy (SMA) is caused by abnormalities of the survival motor neuron (SMN) 1 gene, leading to deficiency in SMN protein and loss of spinal cord alpha motor neurons. Newer disease-modifying agents (DMA) targeting the involved genes, including nusinersen and gene replacement therapies, have improved gross motor and respiratory function, but their impact on scoliosis development has not been established. This study aimed to determine risk factors for scoliosis development in SMA, specifically genetic severity and DMA use. METHODS: In this retrospective cohort study, children with SMA and minimum 2-year follow-up were included. The primary outcome was the prevalence of clinically relevant scoliosis. Secondary outcomes included SMA type, SMN2 copy number, Hammersmith Functional Motor Scale (HFMS), ambulatory status [functional mobility scale at 50m (FMS 50 )], DMA use, and hip displacement as risk factors. Univariate/multivariate logistic regression analyses were performed to identify dependent/independent risk factors. RESULTS: One hundred sixty-five patients (51% female) with SMA types I-III met the inclusion criteria, with total follow-up of 9.8 years. The prevalence of scoliosis was 79%; age of onset 7.9 years. The major curve angle for the entire cohort at first assessment and final follow-up was 37 degrees (SD: 27 degrees) and 62 degrees (SD: 31 degrees) ( P <0.0001), respectively. Significant risk factors for scoliosis by univariate analysis were SMA type (I/II, P =0.02), HFMS (>23, P <0.001), nonambulatory status (FMS 50 =1, P <0.0001), DMA treatment ( P =0.02), and hip displacement ( P <0.0001). Multivariate analysis revealed that HFMS >23 ( P =0.02) and DMA ( P =0.05) treatment were independent (protective) risk factors. CONCLUSIONS: The development of scoliosis in SMA is high, with risk factors associated with proxy measures of disease severity, including SMA type, nonambulatory status, hip displacement, and most notably, gross motor function (by HFMS). DMA use and HFMS >23 were associated with a decreased risk of scoliosis development. Identified risk factors can be used in the development of surveillance programs for early detection of scoliosis in SMA. LEVEL OF EVIDENCE: Level III.

Prevalence and Treatment of Surgical Complications Following Proximal Femoral Osteotomies in Children with Cerebral Palsy: An Analysis of 1085 Hips.

Purpose: Proximal femoral osteotomy (PFO) is a reconstructive surgical option used to improve hip containment or correct internal hip rotation gait in children with cerebral palsy (CP). A few reports describe the risk of surgical complications after PFO. The purpose of this study was to determine the risk factors associated with adverse postoperative surgical outcomes in pediatric patients with CP following PFO and to report the treatment of complications. Methods: Following institutional review board approval, 1085 (1003 primary and 82 secondary) PFO procedures were retrospectively reviewed in 563 children with CP with at least 1 year of follow-up after final surgery over an 18-year follow-up period. Demographic characteristics, motor type, gross motor function classification system (GMFCS) level, medical comorbidities, feeding tube status, seizure history, intervention type, and prevalence of PFO-related surgical complications and associated treatments were evaluated. Multivariate regression analysis was performed to determine risk factors for all surgical complications. Results: During a 5.8-year (± 3.8 years) follow-up, at least 1 surgical complication was identified in 143 (13.1%) hips in 121 (21.5%) patients after PFO in children with CP. Of these complications, the most common was heterotopic ossification (65 [6%] of hips); most of which were asymptomatic and required no treatment. Other complications included 25 (2%) nonunions, 21 (2%) deep or superficial infections, 13 (1%) delayed unions, 12 (1%) peri-implant fractures, and 7 early implant failures (0.64). The rate of revision surgery due to these complications was 13.1% (6.8% of hips), of which 41% (30 revision surgeries) were for the treatment of nonunion. Regarding the development of delayed union or nonunion, dystonia, GMFCS level IV/V, and seizure history were identified as risk factors by multivariate analysis. Conclusions: The prevalence of surgical complications after PFO was 13.1% with 6.8% of hips requiring revision surgery. Dystonia, seizure history, and nonambulatory status were the strongest predictors for the need for revision surgery after PFO. These data can be used to help counsel patients and families regarding the risks associated with PFO for children with CP.Level of proof: IV; retrospective study.

The impact of hamstring lengthening on stance knee flexion at skeletal maturity in ambulatory cerebral palsy.

This study reports the long-term outcomes of hamstring lengthening to treat flexed knee gait in children with ambulatory cerebral palsy (CP) after skeletal maturity. This retrospective longitudinal observational study used instrumented gait analysis (GA) <8 and >15 years old in children with bilateral CP. The primary variable was knee flexion in stance phase. Eighty children (160 limbs) were included; 49% were male, 51% female. Mean age at first GA was 6.0 (SD: 1.2) years and 19.6 (SD: 4.5) years at final GA. Mean follow-up was 13.7 (SD: 4.7) years. Children were classified as Gross Motor Function Classification System I-8, II-46 and III-26. Average Gross Motor Function Measure Dimension D was 72% (SD: 20%). Hamstring lengthenings occurred once in 82, twice in 54 and three times in 10 limbs. From initial to final GA, average knee flexion in stance was unchanged, 27.8° (SD: 14.8°) to final 27.0° (SD: 11.2°; P = 0.54). Knee flexion at foot contact was 39.6° (SD: 13.0°), improving to final GA of 30.7° (SD: 10.6°; P < 0.001). Initial gait deviation index was 65.8 (SD: 31.9), improving to final 78.9 (SD: 28.2; P < 0.001). Older age, males and concomitant plantar flexor lengthening predicted change toward more flexed knee gait. Hamstring lengthening did not lead to back-kneeing gait at maturity while maintaining childhood stance phase knee flexion. A subgroup still developed significant flexed knee gait posture and may have benefited from more aggressive treatment options. This outcome may also be impacted by diverse functional levels, etiologies and treatments of flexed knee gait.

Changes in foot posture evaluated with dynamic pedobarography over the course of childhood in ambulatory youth with cerebral palsy.

Purpose: Foot deformities are prevalent in children with cerebral palsy, but there is limited research on the progression of foot posture during growth. Our study aimed to evaluate the change in dynamic foot posture in children with cerebral palsy. Methods: Children with cerebral palsy, aged 17-40 months, were recruited to participate in this Institutional Review Board-approved prospective longitudinal study by having serial foot posture evaluations. The coronal plane index and foot segmental impulses were measured with dynamic pedobarography. Data were compared between children stratified by Gross Motor Function Classification System level and typically developing children using serial Welch's t-tests across time with Holm correction for multiple comparisons. Results: In total, 33 children (54 limbs) were included in the analysis (21 bilateral and 12 unilateral; Gross Motor Function Classification System: I-13, II-14, III-4, IV-2. Children completed 16.9 (± 4.4) evaluations (initial age 2.9 (± 0.7) and final age 18.6 (± 1.7) years)). Early valgus foot posture normalizes in children at Gross Motor Function Classification System levels I/II and persists in children at levels III/IV who do not have foot surgery. For most young children, foot posture development is variable. Conclusion: Foot posture in young children with cerebral palsy begins in valgus and tends to normalize in youth who walk without an assistive device. Conservative management of foot deformity is recommended in early childhood. Level of evidence: Level II, prognostic study.

Hip Displacement in Spinal Muscular Atrophy: The Influences of Genetic Severity, Functional Level, and Disease-modifying Treatments.

PURPOSE: Hip displacement (HD) is common in spinal muscular atrophy (SMA), but neither genetic severity nor gross motor function level have been investigated as risk factors. Although disease-modifying agents (DMA) have improved function and overall health, their effects on the prevention of HD are unknown. The purpose of this study was to determine risk factors for HD development in SMA. METHODS: Retrospective cohort. Children with SMA presenting between January 2005 and August 2021, at least 1 hip radiograph, and a minimum 2-year follow-up were included. The primary outcome measure was the prevalence of HD (migration percentage ≥40%). Secondary outcomes included SMA type (I/II/III), survival motor neuron 2 copy number, Hammersmith Functional Motor Scale (HFMS, out of 66), ambulatory status (Functional Mobility Scale at 50 m), clinically relevant scoliosis (>40 degrees and/or surgery), and DMA treatment (>1-year duration, nusinersen/risdiplam/onasemnogene abeparvovec) as risk factors. Univariate and multivariate logistic regression analyses were performed. RESULTS: Eighty-two patients (52% female) with SMA type I (n=32, 39%), II (n=36, 44%), and III (n=14, 17%) met the inclusion criteria, with a final follow-up of 4.5 (SD: 2.7) years. Age at first hip radiograph was 3.4 (SD: 2.9) years. The prevalence of HD was 75.6%, with a mean age of onset of 4.6 (SD: 2.7) years. When stratified by SMA type, the prevalence/age of onset (mean, years) was 84%/3.1 (SD: 1.7), 80%/5.8 (SD: 2.3), and 36%/9.0 (SD: 4.3), respectively. HFMS score >23 was protective against HD by receiver operating characteristic analysis ( P =0.008). Significant risk factors by univariate analysis were SMA type I ( P =0.002) and II ( P =0.002), HFMS ≤23 ( P =0.01), nonambulatory status (Functional Mobility Scale at 50 m = 1, P =0.001), clinically relevant scoliosis ( P =0.01), and DMA treatment ( P =0.01). By multivariate analysis, only SMA type II ( P =0.04) and scoliosis ( P =0.04) were independent risk factors. CONCLUSIONS: The prevalence of HD in SMA is highly linked to disease severity. Identified risk factors can be used in the development of surveillance programs for early detection of HD in SMA, allowing for timely management. LEVEL OF EVIDENCE: Level III.

Self-reported Health-related Quality of Life in Adolescents With Cerebral Palsy.

BACKGROUND: Happiness, comfort, and motor function contribute to satisfaction with life for individuals with cerebral palsy (CP). Evidence-based medical care can improve motor function and physical health of youth with CP. Less is known about medical care and its relationship to health-related quality of life (HRQOL) in adolescents and young adults with CP. This study aimed to describe HRQOL among adolescents with CP to examine differences between adolescent (self) and parent (proxy) reports of HRQOL and to explore associations of pain, age, and gross motor function with HRQOL. METHODS: This is a retrospective study including adolescents with CP classified as Gross Motor Function Classification System levels I to V, ages 11 to 20 years, reading ≥ a fourth-grade level, and who completed the self-reported Pediatric Outcomes Data Collection Instrument (PODCI). Parents completed the PODCI concurrently or within 12 months and scores were compared. In addition, self-reported scores were compared between age bands, across Gross Motor Function Classification System levels, with typically developing youth (TDY), and between youth with/without pain. RESULTS: PODCI scores from 102 adolescents [59 males; 15.0 (SD: 2.6) years old] were examined. Scores from 50 adolescents and parents were matched. Mean self-reported scores were significantly higher than mean parent-reported scores in 4 domains: upper extremity and physical function ( P =0.018), sports and physical function ( P =0.005), happiness ( P =0.023), and global functioning ( P =0.018). All domains, except Happiness, were significantly < TDY ( P <0.01). The presence of pain was associated with lower scores in all domains ( P <0.05). CONCLUSION: Examining HRQOL with the PODCI revealed significant limitations in physical function and higher pain in adolescents with CP compared with TDY. Self- and parent-reported PODCI results should be considered separately. Adolescents report higher HRQOL compared with parent proxy. Recognizing and validating the perspectives of youth and their parents presents an opportunity for providers to discuss different points of view with families. Such engagement can help promote self-efficacy in youth with CP as they transition to the responsibility of guiding their own care in adulthood. LEVEL OF EVIDENCE: III, Retrospective comparative study.

Bone alterations of pamidronate therapy in children with cerebral palsy complicating orthopedic management.

Cerebral palsy (CP) is a heterogeneous group of disorders with different clinical types and underlying genetic variants. Children with CP are at risk for fragility fractures secondary to low bone mineral density, and although bisphosphonates are prescribed for the treatment of children with bone fragility, there is limited information on long-term bone impact and safety. Children with CP usually present overtubulated bones, and the thickening of cortical bone by pamidronate treatment can potentially further narrow the medullary canal. Our purpose was to report bone alterations attributable to pamidronate therapy that impact orthopedic care in children with CP. The study consisted of 41 children with CP treated with pamidronate for low bone mineral density from 2006 to 2020. Six children presented unique bone deformities and unusual radiologic features attributed to pamidronate treatment, which affected their orthopedic care. The cases included narrowing of the medullary canal and sclerotic bone, atypical femoral fracture, and heterotopic ossification. Treatment with bisphosphonate reduced the number of fractures from 101 in the pretreatment period to seven in the post-treatment period (P < 0.001). In conclusion, children with CP treated with bisphosphonate have a reduction in low-energy fractures; however, some fractures still happen, and pamidronate treatment can lead to bone alterations including medullary canal narrowing with sclerotic bone and atypical femoral fractures. In very young children, failure to remodel may lead to thin, large femoral shafts with cystic medullary canals. More widespread use of bisphosphonates in children with CP may make these bone alterations more frequent. Level of evidence: Level IV: Case series with post-test outcomes.

The Impact of COVID-19 on Multidisciplinary Care Delivery to Children with Cerebral Palsy and Other Neuromuscular Complex Chronic Conditions.

The COVID-19 pandemic has caused unprecedented challenges in the care of children with cerebral palsy (CP) and other neuromuscular complex chronic conditions (NCCCs). The purpose of this study is to explore the direct impact of the COVID-19 pandemic on healthcare delivery. From May to August 2020, medical professionals caring for CP and NCCC patients across multiple countries and disciplines completed a self-administered cross-sectional survey comparing practices before and during the COVID-19 pandemic. Of the 79 healthcare workers from eight countries who participated-predominantly pediatric orthopedic surgeons (32%), pediatricians (30%), and pediatric physiatrists (23%)-most of them felt that caring for NCCC patients during the pandemic presented unique difficulties, and they reported a significant decrease in the in-person NCCC clinic volume (p < 0.001), multidisciplinary appointments (p < 0.001), surgical cases (p = 0.008), and botulinum toxin/phenol injections. Most providers affirmed that institutional guidelines for perioperative emergent/urgent and elective procedures, workplace settings, and technology were modified to accommodate the ongoing public health crisis. The usage of telemedicine significantly increased for NCCC patient visits (p < 0.001). During the COVID-19 pandemic, many children with NCCCs lost access to routine, multidisciplinary care. Telemedicine became an integral part of communication and management. In the setting of the COVID-19 pandemic and with the threat of future healthcare disruptions, these data lay the foundation for trending the evolution of healthcare delivery and accelerating best practice guidelines for children with CP and NCCCs.

Transcranial electric motor evoked potential monitoring during scoliosis surgery in children with cerebral palsy and active seizure disorder: is it feasible and safe?

PURPOSE: Use of spinal cord monitoring in children with cerebral palsy (CP) and neuromuscular scoliosis is challenging. The previous reports suggest low success rates in the setting of CP, and it is unclear if transcranial electric motor evoked potentials (TcMEP) monitoring is contraindicated in patients with an active seizure disorder. The purpose of this study was to determine (1) are patients with CP able to be appropriately monitored with TcMEP? and (2) does TcMEP cause an increase in seizure activity? METHODS: This was an institutional review board-approved retrospective cohort study observing 304 patients from 2011 to 2020. Inclusion criteria included all patients with CP undergoing posterior spinal fusion during this time. Intraoperative data were examined for the ability to obtain monitoring and any intraoperative events. Patients were followed for 3 months postoperatively to determine any increase in seizure activity that could have been attributed to the TcMEP monitoring. RESULTS: Of the 304 patients who were observed, 21% (20.8%) were unable to be monitored due to lacking baseline signals from the extremities. Seventy-seven percent (77.5%) were successfully monitored with TcMEP. For these patients, no increased seizure activity was documented either intra- or postoperatively. CONCLUSION: A high percentage of children (77.5%) with CP were able to be successfully monitored with TcMEP during posterior spinal fusion. Furthermore, the concerns about increased seizure activity after TcMEP were not supported by the data from this cohort. Technical details of successful neuromonitoring in these patients are important and included increased stimulation voltage requirements and latency times. LEVEL OF EVIDENCE: III retrospective comparative study.

Identification of risk factors for reconstructive hip surgery after intrathecal baclofen therapy in children with cerebral palsy.

OBJECTIVE: This study aimed to determine the risk factors for reconstructive hip surgery after intrathecal baclofen pump application in children with cerebral palsy. METHODS: Inclusion criteria were children with hypertonic (spastic or mixed spastic/dystonic motor type) cerebral palsy, intrathecal baclofen implantation <8 years of age, no reconstructive osteotomies prior to or concomitant with intrathecal baclofen implantation and at least a 5-year follow-up. Exclusion criteria included reconstructive osteotomies prior to or concurrent with intrathecal baclofen implantation, lack of at least 1 hip surveillance radiograph before intrathecal baclofen, lack of a 5-year follow-up, or having selective dorsal rhizotomy. In addition, patients with bony surgery plus last follow-up migration percentage ≥50% were labeled as required reconstruction hips. RESULTS: We identified 34 patients (68 hips). The mean follow-up was 9.2 ± 2.8 years. The mean age for intrathecal baclofen application was 6.4 ± 1.2 years. Seven patients were Gross Motor Function Classification System IV, and 27 were V. Eighteen patients (52.9%) with 31 hips (45.6%) were requiring reconstruction at the final follow-up. In multivariate analysis, male sex (odds ratio 12.8, P=.012), pre-intrathecal baclofen migration percentage (odds ratio 1.1, P=0.003), age at intrathecal baclofen implantation (odds ratio 0.24, P=.002), and delta migration percentage (odds ratio 1.1, P=.002) were significant risk factors for requiring reconstruction. Patients with intrathecal baclofen <6.2 years of age had a significantly higher rate of requiring reconstruction. A pre-intrathecal baclofen migration percentage >31% had a greater risk of progression to requiring reconstruction (P=.001). Delta migration percentage higher than 15% was significantly associated with progression to requiring reconstruction (P=.043). CONCLUSION: The risk of requiring reconstruction osteotomies after intrathecal baclofen was significantly increased in males, those younger (±migration percentage >31%) at the time of intrathecal baclofen implantation and those with an increased rate of migration percentage progression after intrathecal baclofen implantation. LEVEL OF EVIDENCE: Level IV, Prognostic Study.

Collagenase treatment decreases muscle stiffness in cerebral palsy: A preclinical ex vivo biomechanical analysis of hip adductor muscle fiber bundles.

AIM: To determine the dose-response relationship of collagenase Clostridium histolyticum (CCH) on collagen content and the change in muscle fiber bundle stiffness after ex vivo treatment of adductor longus biopsies with CCH in children with cerebral palsy (CP). METHOD: Biopsy samples of adductor longus from children with CP (classified in Gross Motor Function Classification System levels IV and V) were treated with 0 U/mL, 200 U/mL, 350 U/mL, or 500 U/mL CCH; percentage collagen reduction was measured to determine the dose-response. Peak and steady-state stresses were determined at 1%, 2.5%, 5%, and 7.5% strain increments; Young's modulus was calculated. RESULTS: Eleven patients were enrolled (nine males, two females, mean age at surgery 6 years 5 months; range: 2-16 years). A linear CCH dose-response relationship was determined. Peak and steady-state stress generation increased linearly at 5.9/2.3mN/mm2 , 12.4/5.3mN/mm2 , 22.2/9.7mN/mm2 , and 33.3/15.5mN/mm2 at each percentage strain increment respectively. After CCH treatment, peak and steady-state stress generation decreased to 3.2/1.2mN/mm2 , 6.5/2.9mN/mm2 , 12.2/5.7mN/mm2 , and 15.4/7.7mN/mm2 respectively (p < 0.004). Young's modulus decreased from 205 kPa to 100 kPa after CCH (p = 0.003). INTERPRETATION: This preclinical ex vivo study provides proof of concept for the use of collagenase to decrease muscle stiffness in individuals with CP.

Preoperative Biopsychosocial Assessment and Length of Stay in Orthopaedic Surgery Admissions of Youth with Cerebral Palsy.

Caregivers of children with cerebral palsy (CP) experience stress surrounding orthopaedic surgery related to their child's pain and recovery needs. Social determinants of health can affect the severity of this stress and hinder health care delivery. A preoperative biopsychosocial assessment (BPSA) can identify risk factors and assist in alleviating psychosocial risk. This study examined the relationship between the completion of a BPSA, hospital length of stay (LOS), and 30-day readmission rates for children with CP who underwent hip reconstruction (HR) or posterior spinal fusion (PSF). Outcomes were compared with a matched group who did not have a preoperative BPSA. The BPSA involved meeting with a social worker to discuss support systems, financial needs, transportation, equipment, housing, and other services. A total of 92 children (28 HR pairs, 18 PSF pairs) were identified. Wilcoxon analysis was statistically significant (p = 0.000228) for shorter LOS in children who underwent PSF with preoperative BPSA (median = 7.0 days) vs. without (median = 12.5 days). Multivariate analysis showed that a BPSA, a lower Gross Motor Function Classification System level, and fewer comorbidities were associated with a shorter LOS after both PSF and HR (p < 0.05). Identifying and addressing the psychosocial needs of patients and caregivers prior to surgery can lead to more timely discharge postoperatively.