Are Generative Pretrained Transformer 4 Responses to Developmental Dysplasia of the Hip Clinical Scenarios Universal? An International Review.

OBJECTIVE: There is increasing interest in applying artificial intelligence chatbots like generative pretrained transformer 4 (GPT-4) in the medical field. This study aimed to explore the universality of GPT-4 responses to simulated clinical scenarios of developmental dysplasia of the hip (DDH) across diverse global settings. METHODS: Seventeen international experts with more than 15 years of experience in pediatric orthopaedics were selected for the evaluation panel. Eight simulated DDH clinical scenarios were created, covering 4 key areas: (1) initial evaluation and diagnosis, (2) initial examination and treatment, (3) nursing care and follow-up, and (4) prognosis and rehabilitation planning. Each scenario was completed independently in a new GPT-4 session. Interrater reliability was assessed using Fleiss kappa, and the quality, relevance, and applicability of GPT-4 responses were analyzed using median scores and interquartile ranges. Following scoring, experts met in ZOOM sessions to generate Regional Consensus Assessment Scores, which were intended to represent a consistent regional assessment of the use of the GPT-4 in pediatric orthopaedic care. RESULTS: GPT-4's responses to the 8 clinical DDH scenarios received performance scores ranging from 44.3% to 98.9% of the 88-point maximum. The Fleiss kappa statistic of 0.113 ( P = 0.001) indicated low agreement among experts in their ratings. When assessing the responses' quality, relevance, and applicability, the median scores were 3, with interquartile ranges of 3 to 4, 3 to 4, and 2 to 3, respectively. Significant differences were noted in the prognosis and rehabilitation domain scores ( P < 0.05 for all). Regional consensus scores were 75 for Africa, 74 for Asia, 73 for India, 80 for Europe, and 65 for North America, with the Kruskal-Wallis test highlighting significant disparities between these regions ( P = 0.034). CONCLUSIONS: This study demonstrates the promise of GPT-4 in pediatric orthopaedic care, particularly in supporting preliminary DDH assessments and guiding treatment strategies for specialist care. However, effective integration of GPT-4 into clinical practice will require adaptation to specific regional health care contexts, highlighting the importance of a nuanced approach to health technology adaptation. LEVEL OF EVIDENCE: Level IV.

Improvement in a post-stroke pediatric patient with hemiplegia: Use of a hand-arm bimanual intensive therapy with hybrid assistive limb.

BACKGROUND: Pediatric stroke is a rare medical condition that often leads to long-lasting motor and cognitive impairments. Although therapies for adults after a stroke are well described, treatments for motor deficits following a pediatric stroke are yet to be investigated. We report a case of pediatric stroke in the chronic phase, in which a combination of novel treatments resulted in a significant improvement in physical function. CASE REPORT: A seven-year-old girl with a left hemispheric cerebral infarction lost almost all right upper extremity motor function. Following onabotulinumtoxinA treatment, she underwent hand-arm bimanual intensive therapy augmented with a hybrid assistive limb for 90 h over 15 days. Evaluation after the training revealed significant improvements in physical function, daily activities, and occupational performance. CONCLUSIONS: This report highlights the importance of innovative combinations of techniques in the treatment of pediatric stroke.

Re-evaluating consensus and uncertainty among treatment options for early onset scoliosis: a 10-year update.

PURPOSE: Consensus and uncertainty in early onset scoliosis (EOS) treatment were evaluated in 2010. It is currently unknown how treatment preferences have evolved over the past decade. The purpose of this study was to re-evaluate consensus and uncertainty among treatment options for EOS patients to understand how they compare to 10 years ago. METHODS: 11 pediatric spinal surgeons (similar participants as in 2010) were invited to complete a survey of 315 idiopathic and neuromuscular EOS cases (same cases as in 2010). Treatment options included the following: conservative management, distraction-based methods, growth guidance/modulation, and arthrodesis. Consensus was defined as ≥ 70% agreement, and uncertainty was < 70%. Associations between case characteristics and consensus for treatments were assessed via chi-squared and multiple regression analyses. Case characteristics associated with uncertainty were described. RESULTS: Eleven surgeons [31.7 ± 7.8 years of experience] in the original 2010 cohort completed the survey. Consensus for conservative management was found in idiopathic patients aged ≤ 3, whereas in 2010, some of these cases were selected for surgery. There is currently consensus for casting idiopathic patients aged 1 or 2 with moderate curves, whereas in 2010, there was uncertainty between casting and bracing. Among neuromuscular cases with consensus for surgery, arthrodesis was chosen for patients aged 9 with larger curves. CONCLUSION: Presently, preferences for conservative management have increased in comparison to 2010, and casting appears to be preferred over bracing in select infantile cases. Future research efforts with higher levels-of-evidence should be devoted to elucidate the areas of uncertainty to improve care in the EOS population. LEVEL OF EVIDENCE: Level V.

Strategies reducing risk of surgical-site infection following pediatric spinal deformity surgery.

BACKGROUND: Identifying beneficial preventive strategies for surgical-site infection (SSI) in individual patients with different clinical and surgical characteristics is challenging. The purpose of this study was to investigate the association between preventive strategies and patient risk of SSI taking into consideration baseline risks and estimating the reduction of SSI probability in individual patients attributed to these strategies. METHODS: Pediatric patients who underwent primary, revision, or final fusion for their spinal deformity at 7 institutions between 2004 and 2018 were included. Preventive strategies included the use of topical vancomycin, bone graft, povidone-iodine (PI) irrigations, multilayered closure, impermeable dressing, enrollment in quality improvement (QI) programs, and adherence to antibiotic prophylaxis. The CDC definition of SSI as occurring within 90 days postoperatively was used. Multiple regression modeling was performed following multiple imputation and multicollinearity testing to investigate the effect of preventive strategies on SSI in individual patients adjusted for patient and surgical characteristics. RESULTS: Univariable regressions demonstrated that enrollment in QI programs and PI irrigation were significantly associated, and topical vancomycin, multilayered closure, and correct intraoperative dosing of antibiotics trended toward association with reduction of SSI. In the final prediction model using multiple regression, enrollment in QI programs remained significant and PI irrigation had an effect in decreasing risks of SSI by average of 49% and 18%, respectively, at the individual patient level. CONCLUSION: Considering baseline patient characteristics and predetermined surgical and hospital factors, enrollment in QI programs and PI irrigation reduce the risk of SSI in individual patients. Multidisciplinary efforts should be made to implement these practices to increase patient safety. LEVEL OF EVIDENCE: Prognostic level III study.

Development and Validation of a Health-Related Quality-of-Life Measure in Older Children and Adolescents with Early-Onset Scoliosis: Early-Onset Scoliosis Self-Report Questionnaire (EOSQ-SELF).

BACKGROUND: The 24-question Early-Onset Scoliosis Questionnaire (EOSQ-24) is a proxy measure assessing health-related quality of life (HRQoL) among patients with early-onset scoliosis (EOS). There exists an increasing need to assess HRQoL through a child's own perspective, particularly for older children and adolescents with EOS. The purpose of this study was to develop and validate a self-reported questionnaire, the Early-Onset Scoliosis Self-Report Questionnaire (EOSQ-SELF), to assess HRQoL in older children and adolescents with EOS. METHODS: A literature review, an expert focus group, and patient interviews were used to generate a preliminary survey of appropriate domains and question items. This survey was provided to English-speaking patients with EOS who were 8 to 18 years of age and capable of answering survey questions. Content validity was assessed for clarity and relevance of questions. Confirmatory factors analysis was performed to reduce the number of items and determine domains that fit items. Reliability was evaluated by measuring the internal consistency of items and test-retest reliability. Construct validity was evaluated by convergent, discriminant, and known-group validity. RESULTS: The literature review, expert focus group, and patient interviews identified 59 questions in 14 domains. Psychometric analysis reduced these to 30 questions across 12 domains: General Health, Pain/Discomfort, Pulmonary Function, Transfer, Physical Function/Daily Living, Participation, Fatigue/Energy Level, Sleep, Appearance, Relationships, Emotion, and Satisfaction. The final questionnaire was found to have good content and construct validity and adequate reliability. CONCLUSIONS: The EOSQ-SELF is a valid and reliable instrument for measuring self-reported HRQoL among older children and adolescents with EOS (ages 8 to 18 years). This will serve as an important research outcome measure and enhance clinical care by providing a better understanding of HRQoL for these patients. LEVEL OF EVIDENCE: Diagnostic Level II . See Instructions for Authors for a complete description of levels of evidence.

Uncorrected Pelvic Obliquity Is Associated With Worse Health-related Quality of Life (HRQoL) in Children and Their Caregivers at the End of Surgical Treatment for Early Onset Scoliosis (EOS).

BACKGROUND: While largely of concern in nonambulatory patients, pelvic obliquity (PO) can be found in many patients with early onset scoliosis (EOS) and may remain following surgery. However, its association with health-related quality of life (HRQoL) in children and their caregivers at the end of treatment is not well understood. The purpose of this study was to investigate the association between residual PO and HRQoL in children and their caregivers at the end of surgical treatment in ambulatory patients with EOS. METHODS: In this retrospective cohort study, a multicenter EOS registry was queried to identify ambulatory patients who underwent definitive posterior spinal fusion (PSF) from 2012 to 2019. Patients with fusions extending to the pelvis were excluded. PO was measured at least 1 year following PSF. HRQoL, Parental Burden, Financial Burden, and Satisfaction were assessed through the 24-Item Early Onset Scoliosis Questionnaire (EOSQ-24) also at a minimum of 1 year following PSF. RESULTS: A total of 155 patients (12.5±2.1 y, 73.5% female) were included. Etiology distribution was 30.3% congenital, 12.9% neuromuscular, 21.3% syndromic, and 35.5% idiopathic. In congenital patients, those with residual PO >8 degrees had worse Satisfaction by 23.2 points compared with those with PO ≤8 degrees. In neuromuscular patients, those with residual PO >7 degrees had worse HRQoL by 16.1 points and Parental Burden by 22.3 points compared with their counterparts. In syndromic patients, those with residual PO >8 degrees had worse HRQoL by 14.8 points, Parental Burden by 16.4 points, and Satisfaction by 21.2 points compared with their counterparts. In idiopathic patients, those with >9 degrees of residual PO had worse HRQoL by 15.0 points and Financial Burden by 26.8 points compared with their counterparts. CONCLUSIONS: Remaining PO at the end of surgical treatment is associated with worse HRQoL in ambulatory children and their caregivers. These results suggest that correction of PO should remain a primary goal of treatment in patients with EOS undergoing surgery. LEVEL OF EVIDENCE: Level II-multicenter retrospective cohort study investigating prognosis.

A Clinical Risk Model for Surgical Site Infection Following Pediatric Spine Deformity Surgery.

BACKGROUND: Despite tremendous efforts, the incidence of surgical site infection (SSI) following the surgical treatment of pediatric spinal deformity remains a concern. Although previous studies have reported some risk factors for SSI, these studies have been limited by not being able to investigate multiple risk factors at the same time. The aim of the present study was to evaluate a wide range of preoperative and intraoperative factors in predicting SSI and to develop and validate a prediction model that quantifies the risk of SSI for individual pediatric spinal deformity patients. METHODS: Pediatric patients with spinal deformity who underwent primary, revision, or definitive spinal fusion at 1 of 7 institutions were included. Candidate predictors were known preoperatively and were not modifiable in most cases; these included 31 patient, 12 surgical, and 4 hospital factors. The Centers for Disease Control and Prevention definition of SSI within 90 days of surgery was utilized. Following multiple imputation and multicollinearity testing, predictor selection was conducted with use of logistic regression to develop multiple models. The data set was randomly split into training and testing sets, and fivefold cross-validation was performed to compare discrimination, calibration, and overfitting of each model and to determine the final model. A risk probability calculator and a mobile device application were developed from the model in order to calculate the probability of SSI in individual patients. RESULTS: A total of 3,092 spinal deformity surgeries were included, in which there were 132 cases of SSI (4.3%). The final model achieved adequate discrimination (area under the receiver operating characteristic curve: 0.76), as well as calibration and no overfitting. Predictors included in the model were nonambulatory status, neuromuscular etiology, pelvic instrumentation, procedure time ≥7 hours, American Society of Anesthesiologists grade >2, revision procedure, hospital spine surgical cases <100/year, abnormal hemoglobin level, and overweight or obese body mass index. CONCLUSIONS: The risk probability calculator encompassing patient, surgical, and hospital factors developed in the present study predicts the probability of 90-day SSI in pediatric spinal deformity surgery. This validated calculator can be utilized to improve informed consent and shared decision-making and may allow the deployment of additional resources and strategies selectively in high-risk patients. LEVEL OF EVIDENCE: Prognostic Level III. See Instructions for Authors for a complete description of levels of evidence.

Outcomes and Complications in Management of Congenital Myopathy Early-Onset Scoliosis.

BACKGROUND: Congenital myopathies (CMs) are complex conditions often associated with early-onset scoliosis (EOS). The purpose of this study was to investigate radiographic outcomes in CM patients undergoing EOS instrumentation as well as complications. Secondarily, we sought to compare these patients to a population with higher prevalence, cerebral palsy (CP) EOS patients. METHODS: This is a retrospective study of a prospectively collected multicenter registry. The registry was queried for EOS patients with growth-sparing instrumentation (vertical expandable prosthetic titanium ribs, magnetically controlled growing rods, traditional growing rod, or Shilla) and a CM or CP diagnosis with minimum 2 years follow-up. Outcomes included major curve magnitude, T1-S1 height, kyphosis, and complications. RESULTS: Sixteen patients with CM were included. Six (37.5%) children with CM experienced 11 complications by 2 years. Mean major curve magnitude for CM patients was improved postoperatively and maintained at 2 years (P<0.01), with no significant increase in T1-S1 height or maximum kyphosis(P>0.05). Ninety-seven patients with CP EOS were included as a comparative cohort. Fewer CP patients required baseline respiratory support compared with CM patients (20.0% vs. 92.9%, P<0.01). Fifty-four (55.7%) CP patients experienced a total of 105 complications at 2 years. There was no evidence that the risk of complication or radiographic outcomes differs between cohorts at 2 years, though CP EOS patients experienced significant improvement in all measurements at 2 years. CONCLUSIONS: EOS CM children face a high risk of complication after growing instrumentation, with similar curve correction and risk of complication to CP patients. LEVEL OF EVIDENCE: Level III.

Genetic testing in individuals with cerebral palsy.

AIM To determine which patients with cerebral palsy (CP) should undergo genetic testing, we compared the rate of likely causative genetic variants from whole-exome sequencing in individuals with and without environmental risk factors. METHOD Patients were part of a convenience and physician-referred cohort recruited from a single medical center, and research whole-exome sequencing was completed. Participants were evaluated for the following risk factors: extreme preterm birth, brain bleed or stroke, birth asphyxia, brain malformations, and intrauterine infection. RESULTS A total of 151 unrelated individuals with CP (81 females, 70 males; mean age 25y 7mo [SD 17y 5mo], range 3wks-72y) participated. Causative genetic variants were identified in 14 participants (9.3%). There was no significant difference in diagnostic rate between individuals with risk factors (10 out of 123; 8.1%) and those without (4 out of 28; 14.3%) (Fisher's exact p=0.3). INTERPRETATION While the rate of genetic diagnoses among individuals without risk factors was higher than those with risk factors, the difference was not statistically significant at this sample size. The identification of genetic diagnoses in over 8% of cases with risk factors suggests that these might confer susceptibility to environmental factors, and that further research should include individuals with risk factors. What this paper adds There is no significant difference in diagnostic rate between individuals with and without risk factors. Genetic variants may confer susceptibility to environmental risk factors. Six causative variants were identified in genes not previously associated with cerebral palsy. Global developmental delay/intellectual disability is positively associated with a genetic etiology. Extreme preterm birth, stroke/brain hemorrhage, and older age are negatively associated with a genetic etiology.

Complications in ambulatory pediatric patients with nonidiopathic spinal deformity undergoing fusion to the pelvis using the sacral-alar-iliac technique within 2 years of surgery.

OBJECTIVE: Significant investigation in the adult population has generated a body of research regarding proximal junctional kyphosis (PJK) and proximal junctional failure (PJF) following long fusions to the sacrum and pelvis. However, much less is known regarding early complications, including PJK and PJF, in the ambulatory pediatric patient. As such, the objective of this study was to address the minimal literature on early complications after ambulatory pediatric patients underwent fusion to the sacrum with instrumentation to the pelvis in the era of sacral-alar-iliac (S2AI) instrumentation. METHODS: The authors performed a retrospective review of pediatric patients with nonidiopathic spinal deformity < 18 years of age with ambulatory capacity who underwent fusion to the pelvis at a multisurgeon pediatric academic spine practice from 2016 to 2018. All surgeries were posterior-only approaches with S2AI screws as the primary technique for sacropelvic fixation. Descriptive, outcome, and radiographic data were obtained. The definition of PJF included symptomatic PJK presenting as fracture, screw pullout, or disruption of the posterior osseoligamentous complex. RESULTS: Twenty-five patients were included in this study. Nine patients (36.0%) had 15 complications for an overall complication rate of 60.0%. Unplanned return to the operating room occurred 8 times in 6 patients (24.0%). Four patients (16.0%) had wound issues (3 with deep wound infection and 1 with wound breakdown) requiring reoperation. Three patients (12.0%) had PJF, all requiring reoperation. A 16-year-old female patient with syndromic scoliosis underwent extension of fusion due to posterior tension band failure at 6 months. A 17-year-old male patient with neuromuscular scoliosis underwent extension of fusion due to proximal screw pullout at 5 months. A 10-year-old female patient with congenital scoliosis underwent extension for PJF at 5 months following posterior tension band failure. One patient had pseudarthrosis requiring reoperation 20 months postoperatively. CONCLUSIONS: Fixation to the pelvis enables significant deformity correction, but with rather high rates of complications and unexpected returns to the operating room. Considerations of sagittal plane dynamics for PJK and PJF should be strongly analyzed when performing fixation to the pelvis in ambulatory pediatric patients.

Growth-friendly Spinal Instrumentation in Marfan Syndrome Achieves Sustained Gains in Thoracic Height Amidst High Rates of Implant Failure.

BACKGROUND: There are few reports on the surgical management of early-onset scoliosis (EOS) associated with Marfan syndrome (MFS). Affected patients tend to have more rapid curve progression than those with idiopathic EOS, and their course is further complicated by medical comorbidities. As surgical techniques and implants for growing spines become more widely applied, this study seeks to better delineate the safety and efficacy of growth-friendly spinal instrumentation in treating this population. METHODS: A prospective registry of children treated for EOS was queried for MFS patients treated between 1996 and 2016. Forty-two patients underwent rib-based or spine-based growing instrumentation and were assessed on preoperative, surgical, and postoperative clinical and radiographic parameters including complications and reoperations. Subgroup analysis was performed based on spine-based versus rib-based fixation. RESULTS: Patients underwent their index surgery at a mean age of 5.5 years, when the major coronal curve and kyphosis measured 77 and 50 degrees, respectively. Over half were treated with traditional growing rods. Patients underwent 7.2 total surgical procedures-4.7 lengthening and 1.9 revision surgeries not including conversion to fusion-over a follow-up of 6.5 (±4.1) years. Radiographic correction was greatest at index surgery but maintained over time, with a final thoracic height measuring 23.8 cm. Patients experienced a mean of 2.6 complications over the course of the study period; however, a small group of 6 patients experienced ≥6 complications while over half of patients experienced 0 or 1. Implant failures represented 42% of all complications with infection and pulmonary complications following. CONCLUSIONS: This is the largest report on patients with EOS and MFS. All subtypes of growth-friendly constructs reduced curve progression in this cohort, but complications and reoperations were nearly universal; patients were particularly plagued by implant failure and migration. Further collaborations are needed to enhance understanding of optimal timing and fixation constructs for those with MFS and other connective tissue diseases.

Completion of a formal physiotherapeutic scoliosis-specific exercise training program for adolescent idiopathic scoliosis increases patient compliance to home exercise programs.

INTRODUCTION: Physiotherapeutic Scoliosis-Specific Exercise (PSSE) is a conservative approach for management of adolescent idiopathic scoliosis (AIS). Although there is Level I evidence for the efficacy of PSSE, compliance in the teenage population remains in question. The purpose of this study is to investigate the association between completion of formal PSSE training and compliance to prescribed home exercise programs (HEP). METHODS: Patients with AIS evaluated at our institution between 2013 and 2015 with a minimum of one PSSE session were enrolled. A chart review and questionnaire completed by caregivers was utilized to assess HEP compliance following the final PSSE session at 1 week, 3 months, 1 year, and 2 years. Patients were divided into two groups, those completing formal training (10 or more PSSE sessions), and those who did not complete formal training (fewer than 10 sessions). RESULTS: 81 patients were identified (mean age of 13.1 years; major curve of 31.3°). Patients who completed training demonstrated 50% compliance at 1 week (vs. 25.6% for non-completers, Odds Ratio (OR): 2.9, p = 0.027), 41.2% at 3 months (vs. 18.9% for non-completers, OR: 3.0, p = 0.044), 23.5% at 1 year (vs. 13.5% for non-completers, OR: 2.0, p = 0.281), and 25.7% at 2 years (vs. 13.5% for non-completers, OR: 2.2, p = 0.197). The mean HEP duration (minutes) in patients who completed training was higher at 1 week (80.8 vs. 48, p = 0.010), 3 months (64.6 vs. 23.7, p ≤ 0.001), 1 year (35.3 vs. 22.7, p = 0.270), and 2 years (34.3 vs. 18.9, p = 0.140). CONCLUSION: Patients who completed PSSE training maintained higher HEP compliance.

Complications in the treatment of EOS: Is there a difference between rib vs. spine-based proximal anchors?

INTRODUCTION: Currently, there is significant equipoise regarding the selection and placement of growing spinal instrumentation when treating patients with early-onset scoliosis (EOS). The primary purpose of this study was to compare complications following surgery in patients receiving rib-based versus spine-based proximal anchors as a part of posterior growing instrumentation in the management of EOS. METHODS: Retrospective cohort study. Inclusion criteria required: age 3-10 years old, diagnosis of EOS, treatment with a growing construct that utilized rib- or spine-based proximal anchors, and a major coronal curve larger than 40 degrees. The primary outcome analyzed was postoperative complications. Secondary outcomes included coronal major curve correction and patient reported outcomes measured by the Early-Onset Scoliosis 24-item Questionnaire (EOSQ-24). Subjects were categorized into rib- or spine-based proximal fixation groups for comparison. RESULTS: Of 104 patients included in the study, 76 (73.1%) were treated with rib-based constructs and 28 (26.9%) were treated with spine-based constructs. 24 (31.6%) patients with rib-based constructs and 9 (32.1%) patients with spine-based constructs experienced at least one implant related complication (p = 0.956). Rod fracture was observed more often in spine-based groups than rib-based groups for both patients with congenital/idiopathic EOS (rib: 0 (0%) vs. spine: 3 (13.6%), p = 0.009) and neuromuscular/syndromic EOS (rib: 0 (0%) vs. spine: 2 (33.3%), p = 0.002). Furthermore, surgical site infection was found to be more frequent in rib-based than spine-based groups for neuromuscular/syndromic patients (rib: (13) 27.15 vs. spine: (1) 4.5%, p = 0.029). The most commonly reported complication was device migration. In patients with rib-based constructs, 2 (12.5%) patients with ≥ 5 anchors and 13 (21.7%) patients with < 5 anchors experienced device migration (p = 0.413). In patients with spine-based constructs, 1 (11.1%) patient with ≥ 5 anchors and 4 (21.1%) patients with < 5 anchors experienced device migration (p = 0.064). Spine-based anchors had significantly higher% correction (42.0%) compared to rib-based anchors (20.6%) (p = 0.003) at the most recent follow-up. There were no significant differences in the change of patient reported outcomes as measured by the EOSQ-24 between patients who received rib or spine-based anchors. DISCUSSION: The number of patients with at least one implant related complication was similar between the rib- and spine-based groups. Having 5 or more proximal anchors appeared protective against proximal device migration; however, this result was not statistically significant. Spine-based anchors had better overall correction than rib-based anchors. There were no differences in the change in patient reported outcomes between spine- and rib-based cohorts.

Surgical site infections in pediatric spinal surgery after implementation of a quality assurance program.

STUDY DESIGN: Retrospective cohort study. OBJECTIVE: To assess the effectiveness of two infection-reducing programs in mitigating the incidence of post-operative surgical site infections (SSI) in pediatric patients after spinal deformity surgery at our institution. Infections following spinal deformity surgery are associated with higher morbidity as well as significantly increased healthcare costs. SSI in patients with neuromuscular etiologies is especially high, exceeding 8 percent for myelodysplasia patients and 6 percent for cerebral palsy patients. METHODS: Manual chart review was conducted for 1934 pediatric spine procedures in 1200 patients at our institution between 2008 and 2018. Patients between the ages of 0 and 21 having any spinal surgical procedure including lengthening of growing rods were included. RESULTS: Institution of two separate infection-reducing programs reduced risk of SSI in this population by 65.4%, when adjusted for age and number of instrumentation levels (risk ratio [RR] = 0.3, 95% confidence interval [CI] = 0.2; 0.6, p = 0.001). Patients undergoing Initial Instrumentation demonstrated 68.8% less risk of SSI compared to those who had other types of surgical procedures, after adjusting for age and the number of level instrumented (RR = 0.3, 95% CI 0.2; .6, p = 0.002). It was observed that the effect of each of these infection-reducing programs diminished with time. This effect was also observed with prior programs implemented at our institution. CONCLUSION: The incidence of SSI decreased following the implementation of two infection-reducing programs especially in patients undergoing Initial Instrumentation procedures. However, time-series analysis suggests these programs may have maximal effect immediately following institution that diminishes with time. LEVEL OF EVIDENCE: Level III.

Association between health-related quality of life outcomes and pulmonary function testing.

INTRODUCTION: Investigations in associations between subjective health-related quality of life (HRQoL) measures and objective clinical assessments in patients with early-onset scoliosis (EOS) are limited. The purpose of this study is to investigate the association between pulmonary function rated by parents and pulmonary function testing (PFT) in patients with EOS. MATERIALS/METHODS: In this cross-sectional study, patients with EOS at any stage of treatment from 2011 to 2018 were identified in 2 registries including 33 centers. Parents' perception of pulmonary function was evaluated using pulmonary function (PF) domain in the Early-Onset Scoliosis 24 item Questionnaire (EOSQ-24). PFT measures included FVC% predicted, FEV1/FVC, and TLC% predicted. All PFT predicted values utilized arm span. PFT and EOSQ-24 questionnaire were completed within 180 days of each other with an average day difference of 26 days. RESULTS: 176 patients (mean age: 10.4 years old, female: 56%) were identified. 33% of patients were of congenital/structural etiology, 27% neuromuscular, 26% syndromic, and 14% idiopathic. Wide variance and lower scores of PF domain were reported by parents at lower FVC% predicted values (< 50%). As FVC% predicted values increased, PFD scores increased with simultaneous decreases in variance with few exceptions. CONCLUSION: More variability and frequent lower pulmonary function values are reported by parents when percent forced vital capacity (FVC%) is < 50%. This likely reflects the degree to which children adapt to restrictive lung disease and the limits on adaptation that occur increasingly as lung function falls below 50% predicted. As a direct linear association with high correlation was expected, more research into the character of what the PF domain is measuring is necessary. LEVEL OF EVIDENCE: IV.

Ambulatory capacity following fusion to the sacrum with pelvic fixations pediatric spinal deformity patients.

OBJECTIVE: To investigate outcomes of ambulatory pediatric patients fused to the sacrum with pelvic fixation. STUDY DESIGN: Retrospective cohort. BACKGROUND: Historically in the pediatric spinal deformity literature, long fusions to the sacrum with pelvic fixation have been limited to non-ambulatory patients with pelvic obliquity. This analysis assesses the impact of how long fusions that include pelvic fixation in ambulatory pediatric patients. METHODS: Consecutive pediatric patients undergoing fusion to the sacrum with pelvic fixation with any ambulatory capacity at a tertiary academic pediatric spine program from 2016 to 2018 were included in this review. Patient demographics, surgical details, and postoperative results were collected and analyzed to identify the impact on postoperative gait and ambulation function. Outcomes were determined from PROMIS, Parent Proxy Mobility questionnaires, as well as an institution specific pediatric gait questionnaire. RESULTS: 25 patients met inclusion criteria. 96.0% of patients had non-idiopathic scoliosis. 48% (12/25) of patients had previous spinal surgery. Median fusion levels were 17.0. 40% (10/25) had preoperative motor and gait deficits and only one patient with a documented decline at postoperative clinical examination. All 25 patients retained ambulatory capacity following their spinal fusion to the sacrum with pelvic fixation. 64% (16/25) of patients or family members completed the postoperative gait questionnaire. The mean PROMIS T-score was 43.4 (95% CI 36.5-50.0) for intact patients. The postoperative gait questionnaires revealed that 75% (6/8) of neurologically intact patients stated they would have the surgery again. 100% (8/8) of these patients noted an improved posture and 25% (2/8) reported a subjective decrease in ambulatory capacity status/post fusion to the sacrum. 100.0% (8/8) of limited ambulatory patients stated they would have the surgery again and noted improved posture with only 25% (2/8) noting a subjective decrease in ambulation postoperatively. CONCLUSION: In appropriately selected pediatric spinal deformity patients fusion to the sacrum using pelvic fixation can maintain ambulation with high overall surgical satisfaction. LEVEL OF EVIDENCE: III.

Improvement of Pulmonary Function Measured by Patient-reported Outcomes in Patients With Spinal Muscular Atrophy After Growth-friendly Instrumentation.

BACKGROUND: Children with spinal muscular atrophy (SMA) sustain a progressive reduction in pulmonary function (PF) related to both muscular weakness and the concomitant effects of spinal deformity on the thorax. Growth-friendly instrumentation is commonly utilized for younger patients with scoliosis and SMA to halt the progression of spinal curvature, but its effect on PF in these patients has not previously been investigated. Using the change in Early Onset Scoliosis 24-Item Questionnaire (EOSQ-24) PF subdomain scores, the authors will investigate whether PF improves in patients with SMA after a growth-friendly intervention. METHODS: This was a multicenter retrospective cohort study from 2 international registries of patients with SMA undergoing spinal deformity surgery from 2005 to 2015. Data collected were age, sex, degree of major coronal curve, type of growth-friendly construct, forced vital capacity (FVC), and EOSQ-24 scores at the patient's preoperative, 1-year postoperative, and 2-year postoperative visits. Differences in EOSQ-24 PF scores and FVC between baseline and postoperative assessment were examined by paired tests. RESULTS: A total of 74 patients were identified (mean age, 7.6±2.3 y, major curve 68.1±22.4 degrees, 51.4% female individuals). The mean EOSQ-24 PF scores improved significantly from 70.6 preoperatively to 83.6 at 1 year (P=0.092) and 86.5 at 2 years postoperatively (P=0.020). The scores in patients with rib-based constructs showed steeper increases at 1-year assessments than those in patients with spine-based constructs. The mean paired FVC value decreased from 63.9% predicted preoperatively, to 57.6% predicted at 1 year postoperatively (P=0.035), and 61.9% predicted preoperatively, to 56.3% predicted at 2 years postoperatively (P=0.178). CONCLUSIONS: Patients with SMA who received growth-friendly instrumentation did experience improvements in PF as measured by EOSQ-24 assessing the caregivers' perception. Given the uncertain reliability of PFTs in this young population, EOSQ-24 is an important tool for measuring improvements in health-related quality of life. LEVEL OF EVIDENCE: Level III-retrospective study.

Evaluation of assessment of caregiver experience with neuromuscular disease: reliability and responsiveness of a new caregiver-reported outcome measure in patients with cerebral palsy.

BACKGROUND: Cerebral palsy (CP) is the most common cause of chronic childhood disability. Caregivers often provide prolonged care over patients' life span, thus measuring the impact of the disease and its treatments on caregivers has become a recent focus in research. The current study aims to present an evaluation of the reliability and responsiveness of assessment of caregiver experience with neuromuscular disease (ACEND) following botulinum toxin injection to relieve spasticity in children with CP. METHODS: Patients with baseline ACEND scores and at least one assessment following botulinum toxin injection were enrolled. Data on their gender, age, diagnoses, and functional levels (according to The Gross Motor Function Classification System, GMFCS), and ACEND scores were analyzed. Statistical analyses performed included paired t-test and linear regression. RESULTS: Baseline ACEND scores (117.7±47.7) were strongly correlated with follow-up scores (120.4±49.5) with a coefficient of 0.929 (P<0.001), suggesting the high reliability of the questionnaire. Paired-sample t-test revealed an insignificant average improvement in ACEND of 2.7 (P=0.352). The ICD-10 code and the GMFCS level were found to be significant predictors for baseline (P=0.043, P<0.001) and follow-up ACEND scores (P=0.025, P<0.001). Male gender was a significant predictor for improvement in ACEND scores. CONCLUSIONS: We demonstrated the reliability of ACEND through strong correlations of scores before and after botulinum toxin injection. In terms of responsiveness, while the burden of care is largely determined by ICD-10 diagnosis and the GMFCS level, changes in care burden are only related to the gender of the patient and the follow-up time interval.