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.

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.

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.

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.

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.

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.

Symptomatic cervical spinal stenosis in spastic cerebral palsy.

AIM: To describe the prevalence of symptomatic cervical spinal stenosis (CSS) in spastic cerebral palsy (CP) and associated characteristics. METHOD: This cross-sectional study of adults (>18y) with CP (2006-2016) at a single institution compared the patient characteristics (demographics, comorbidities, surgical history, medications, Gross Motor Function Classification System [GMFCS] level, and CP type) of patients with and without CSS. RESULTS: Of 424 patients (mean age 33y 4mo, SD 13y 6mo, range 18-78y; 225 females, 199 males), 32 patients (7.5%) had symptomatic CSS. GMFCS levels in the study cohort were distributed as follows: level I, 25%; level II, 25%; level III, 22%; level IV, 19%; level V, 9%. Twenty-five out of 32 (78.1%) patients had spastic CP, two (6.3%) had dystonic CP, and one (3.1%) had mixed characteristics. Individuals with CSS were older (mean age 54y 6mo, SD 10y 5mo vs mean age 31y 7mo, SD 12y 1mo, p<0.05) and had a higher body mass index (26.1, SD 4.8 vs 23.4, SD 6.2, p<0.05) than those without CSS. Presentations included upper-extremity symptoms (73%), ambulation decline (70%), neck pain (53%), and incontinence (30%). Common stenosis levels were C5-C6 (59%), C4-C5 (56%), and C6-C7 (53%). INTERPRETATION: Symptomatic CSS was identified in 7.5% of this adult cohort during the 2006 to 2016 period. Diagnosis in CP is difficult due to impaired communication and pre-existing gait abnormalities and spasticity. Given the high prevalence of symptomatic CSS in adults, we propose developing screening guidelines. Physicians must maintain a high level of suspicion for CSS if patients present with changes in gait or spasticity.

Establishing Consensus on the Best Practice Guidelines for Use of Halo Gravity Traction for Pediatric Spinal Deformity.

BACKGROUND: Although halo gravity traction (HGT) has been used to treat children with severe spinal deformity for decades, there is a distinct lack of high-quality evidence to speak to its merits or to dictate ideal manner of implementation. In addition, no guidelines exist to drive research or assist surgeons in their practice. The aim of this study was to establish best practice guidelines (BPG) using formal techniques of consensus building among a group of experienced pediatric spinal deformity surgeons to determine ideal indications and implementation of HGT for pediatric spinal deformity. METHODS: The Delphi process and nominal group technique were used to formally derive consensus among leaders in pediatric spine surgery. Initial work identified significant areas of variability in practice for which we sought to garner consensus. After review of the literature, 3 iterative surveys were administered from February through April 2018 to nationwide experts in pediatric spinal deformity. Surveys assessed anonymous opinions on ideal practices for indications, preoperative evaluation, protocols, and complications, with agreement of 80% or higher considered consensus. Final determination of consensus items and equipoise were established using the Nominal group technique in a facilitated meeting. RESULTS: Of the 42 surgeons invited, responses were received from 32, 40, and 31 surgeons for each survey, respectively. The final meeting included 14 experts with an average 10.5 years in practice and average 88 annual spinal deformity cases. Experts reached consensus on 67 items [indications (17), goals (1), preoperative evaluations (5), protocols (36), complications (8)]; these were consolidated to create final BPG in all categories, including statements to help dictate practice such as using at least 6 to 8 pins under 4 to 8 lbs of torque, with a small, tolerable starting weight and reaching goal weight of 50% TBW in ∼2 weeks. Nine items remained items of equipoise for the purposes of guiding future research. CONCLUSIONS: We developed consensus-based BPG for the use and implementation of HGT for pediatric spinal deformity. This can serve as a measure to help drive future research as well as give new surgeons a place to begin their practice of HGT. LEVEL OF EVIDENCE: Level V-expert opinion.

Gross Motor Function Classification System Specific Growth Charts-Utility as a Risk Stratification Tool for Surgical Site Infection Following Spine Surgery.

BACKGROUND: There is currently minimal evidence that preoperative malnutrition increases surgical site infection (SSI) risk in children with cerebral palsy (CP) undergoing spinal deformity surgery. Growth charts specifically for patients with CP have been created to aid in the clinical interpretation of body mass index (BMI) as a marker of nutritional status, but to our knowledge these charts have never been used to risk stratify patients before orthopaedic surgery. We hypothesize that patients with CP who have BMI-for-age below the 10th percentile (BMI≤10) on CP-specific growth charts are at increased risk of surgical site infection following spinal deformity surgery compared with patients with BMI-for-age above the 10th percentile (BMI>10). METHODS: Single-center, retrospective review comparing the rate of SSI in patients with CP stratified by BMI-for-age percentiles on CP-specific growth charts who underwent spinal deformity surgery. Odds ratios with 95% confidence intervals and Pearson χ tests were used to analyze the association of the measured nutritional indicators with SSI. RESULTS: In total, 65 patients, who underwent 74 procedures, had complete follow-up data and were included in this analysis. Ten patients (15.4%) were GMFCS I-III and 55 (84.6%) were GMFCS IV-V; 39 (60%) were orally fed and 26 (40%) were tube-fed. The rate of SSI in this patient population was 13.5% with 10 SSIs reported within 90 days of surgery. There was a significant association between patients with a BMI below the 10th percentile on GMFCS-stratified growth charts and the development of SSI (OR, 13.6; 95% CI, 2.4-75.4; P=0.005). All SSIs occurred in patients that were GMFCS IV-V. There was no association between height, weight, feeding method, or pelvic instrumentation and development of SSI. CONCLUSIONS: CP-specific growth charts are useful tools for identifying patients at increased risk for SSI following spinal instrumentation procedures, whereas standard CDC growth charts are much less sensitive. There is a strong association between preoperative BMI percentile on GMFCS-stratified growth charts and SSI following spinal deformity surgery. LEVEL OF EVIDENCE: Level III-Retrospective Study.

The Association Between the Classification of Early-onset Scoliosis and Smith Complications After Initiation of Growth-friendly Spine Surgery: A Preliminary Study.

BACKGROUND: Early-onset scoliosis (EOS) is a complex, heterogeneous condition involving multiple etiologies, genetic associations, and treatment plans. In 2014, Williams and colleagues proposed a classification system of EOS (C-EOS) that categorizes patients by etiology, Cobb angle, and kyphosis. Shortly after, Smith and colleagues validated a classification system to report complications of growth-friendly spine instrumentation. Severity refers to the level of care and urgency required to treat the complication, and can be classified as device-related or disease-related complications. The purpose of this study was to investigate if C-EOS can be used as a reliable predictor of Smith complications to better risk stratify these young, surgical patients. METHODS: This study queried retrospective data from a large multicenter registry with regard to growth-friendly spine instrumentation in the EOS population. One-hundred sixteen patients were included in final data analysis to investigate the outcomes of their growth-friendly procedures according to the Smith complication classification system. RESULTS: There were 245 Smith complications distributed among 116 patients included in this study (2.1 complications per patient). The majority of the complications were device related requiring at least one unplanned trip to the operating room (Smith Device Complication IIA or IIB; 111 complications). There were no complications that caused abandonment of growth-friendly instrumentation (Smith Complication III) and no mortalities (Smith Complication IV). The most severely affected EOS group was the hyperkyphotic syndromic group with a major curve angle >50 degrees (S3+), with 3.4 complications per patient. The least affect EOS group was the hyperkyphotic congenital group with a major curve angle between 20 and 50 degrees, with 0.3 complications per patient. CONCLUSIONS: The C-EOS simplifies a complex pathology and the Smith complication classification scheme creates a language to discuss treatment of known complications of growth-friendly spine surgery. Although there is an association between more advanced C-EOS and Smith complications, there does not appear to be a correlation that can satisfy a risk stratification at this time. LEVEL OF EVIDENCE: Level II.

Post-Operative Pain Assessment and Management in Cerebral Palsy (CP): A Two-Pronged Comparative Study on the Experience of Surgical Patients.

INTRODUCTION: This study compares the current practice patterns of pain assessment and management between children with and without CP following either posterior spinal instrumentation and fusion (PSIF) or hip osteotomy (HO). METHODS: Two cohorts of CP patients were retrospectively identified and matched with non-CP patients based on age, surgical procedure, and approach to post-operative pain management. Sixteen CP patients undergoing PSIF and twenty-two undergoing HO were respectively matched with the same numbers of non-CP patients receiving the same procedures. The frequency of assessments conducted, highest pain scores recorded on each post-operative day (POD), and the amount of adjuvant analgesics administered were collected for POD 0-4. RESULTS: Patients with CP were significantly more frequently evaluated for pain post-operatively, tended to have lower pain scores as measured by current scales, and received slightly fewer analgesics. Patients with CP differed from their non-CP counterparts in both frequency and method of post-operative pain assessment. CONCLUSIONS: The purpose of this study is to elucidate the current state of post-operative pain assessment and management in children with CP undergoing major orthopaedic surgeries, to improve CP patient/caregiver understanding and expectation of the post-operative experience regarding pain, and to provide recommendations for improving the post-operative care for these patients.

The Final 24-Item Early Onset Scoliosis Questionnaires (EOSQ-24): Validity, Reliability and Responsiveness.

INTRODUCTION: The goal of early-onset scoliosis (EOS) treatment is to improve health-related quality of life (HRQoL) for patients and to reduce the burden on their parents or caregivers. The purpose of this study is to develop and finalize the 24-item Early-Onset Scoliosis Questionnaire (EOSQ-24), and examine the validity, reliability, and responsiveness of the EOSQ-24 in measuring patients' HRQoL, the burden on their caregivers, and the burden on their caregiver's finances. The study also established aged-matched normative values for the EOSQ-24. METHODS: The EOSQ-24 was administered to caregivers of male and female patients aged 0 to 18 years with EOS. Patients with EOS are diagnosed before 10 years of age. Criterion validity was investigated by measuring agreement between its scores and pulmonary function testing. Construct validity was established by comparing values across different etiology groups using the known-group method, and measuring internal consistency reliability. Content validity was confirmed by reviewing caregiver and health provider ratings for the relativity and clarity of the EOSQ-24 questions. Test-retest reliability was examined through intraclass correlation coefficients. Responsiveness of the EOSQ-24 before and after surgical interventions was also investigated. Age-matched, healthy patients, without spinal deformity, were enrolled to establish normative EOSQ-24 values. RESULTS: The pulmonary function subdomain score in the EOSQ-24 was positively correlated with pulmonary function testing values, establishing criterion validity. The EOSQ-24 scores for neuromuscular patients were significantly decreased compared with idiopathic or congenital/structural patients, demonstrating known-group validity. Internal consistency reliability of patients' HRQoL was excellent (0.92), but Family Burden was questionable (0.64) indicating that Parental Burden and Financial Burden should be in separate domains. All 24 EOSQ items were rated as essential and clear, confirming content validity. All EOSQ-24 domains demonstrated good to excellent agreement (0.68 to 0.98) between test and retest scores. Neuromuscular patients improved their HRQoL postoperatively, whereas idiopathic patients worsened their HRQoL postoperatively, indicating the ability of the instrument to respond to different trajectories of HRQoL according to etiology in patients with EOS. DISCUSSION: The developed and finalized EOSQ-24 is a valid, reliable, and responsive instrument that is able to serve as a patient-reported outcome measure evaluating health status for patients with EOS and burden of their caregivers. This instrument will be able to serve as an outcome measure for future research including clinical trials evaluating the effectiveness of various treatments. In addition, the EOSQ-24 allows assessment of patients' HRQoL, and the burden on their caregivers relative to age-matched healthy peers. LEVEL OF EVIDENCE: Level II-diagnostic study with consecutive patients enrolled in national registries.

The Classification for Early-onset Scoliosis (C-EOS) Correlates With the Speed of Vertical Expandable Prosthetic Titanium Rib (VEPTR) Proximal Anchor Failure.

BACKGROUND: The Classification for Early-onset Scoliosis (C-EOS) was developed by a consortium of early-onset scoliosis (EOS) surgeons. This study aims to examine if the C-EOS classification correlates with the speed (failure/unit time) of proximal anchor failure in EOS surgery patients. METHODS: A total of 106 EOS patients were retrospectively queried from an EOS database. All patients were treated with vertical expandable prosthetic titanium rib and experienced proximal anchor failure. Patients were classified by the C-EOS, which includes a term for etiology [C: Congenital (54.2%), M: Neuromuscular (32.3%), S: Syndromic (8.3%), I: Idiopathic (5.2%)], major curve angle [1: ≤20 degrees (0%), 2: 21 to 50 degrees (15.6%), 3: 51 to 90 degrees (66.7%), 4: >90 degrees (17.7%)], and kyphosis ["-": ≤20 (13.5%), "N": 21 to 50 (42.7%), "+": >50 (43.8%)]. Outcome was measured by time and number of lengthenings to failure. RESULTS: Analyzing C-EOS classes with >3 subjects, survival analysis demonstrates that the C-EOS discriminates low, medium, and high speed of failure. The low speed of failure group consisted of congenital/51-90/hypokyphosis (C3-) class. The medium-speed group consisted of congenital/51-90/normal and hyperkyphosis (C3N, C3+), and neuromuscular/51-90/hyperkyphosis (M3+) classes. The high-speed group consisted of neuromuscular/51-90/normal kyphosis (M3N), and neuromuscular/>90/normal and hyperkyphosis (M4N, M4+) classes. Significant differences were found in time (P<0.05) and number of expansions (P<0.05) before failure between congenital and neuromuscular classes.As isolated variables, neuromuscular etiology experienced a significantly faster time to failure compared with patients with idiopathic (P<0.001) and congenital (P=0.026) etiology. Patients with a major curve angle >90 degrees demonstrated significantly faster speed of failure compared with patients with major curve angle 21 to 50 degrees (P=0.011). CONCLUSIONS: The ability of the C-EOS to discriminate the speeds of failure of the various classification subgroups supports its validity and demonstrates its potential use in guiding decision making. Further experience with the C-EOS may allow more tailored treatment, and perhaps better outcomes of patients with EOS. LEVEL OF EVIDENCE: Level III.

Reliability analysis of Cobb angle measurements of congenital scoliosis using X-ray and 3D-CT images.

PURPOSE: Therapeutic decisions for congenital scoliosis rely on Cobb angle measurements on consecutive radiographs. There have been no studies documenting the variability of measuring the Cobb angle using 3D-CT images in children with congenital scoliosis. The purpose of this study was to compare the reliability and measurement errors using X-ray images and those utilizing 3D-CT images. MATERIALS AND METHODS: The X-ray and 3D-CT images of 20 patients diagnosed with congenital scoliosis were used to assess the reliability of the digital 3D-CT images for the measurement of the Cobb angle. Thirteen observers performed the measurements, and each image was analyzed by each observer twice with a minimum interval of 1 week between measurements. The analysis of intraobserver variation was expressed as the mean absolute difference (MAD) and standard deviation (SD) between measurements and the intraclass correlation coefficient (IaCC) of the measurements. In addition, the interobserver variation was expressed as the MAD and interclass correlation coefficient (IeCC). RESULTS: The average MAD and SD was 4.5° and 3.2° by the X-ray method and 3.7° and 2.6° by the 3D-CT method. The intraobserver and interobserver intraclass ICCs were excellent in both methods (X-ray: IaCC 0.835-0.994 IeCC 0.847, 3D-CT: IaCC 0.819-0.996 IeCC 0.893). There was no significant MAD difference between X-ray and 3D-CT images in measuring each type of congenital scoliosis by each observer. CONCLUSIONS: Results of Cobb angle measurements in patients with congenital scoliosis using X-ray images in the frontal plane could be reproduced with almost the same measurement variance (3°-4° measurement error) using 3D-CT images. This suggests that X-ray images are clinically useful for assessing any type of congenital scoliosis about measuring the Cobb angle alone. However, since 3D-CT can provide more detailed images of the anterior and posterior components of malformed vertebrae, the volume of information that can be obtained by evaluating them has contributed greatly to the development of strategies for the surgical treatment of congenital scoliosis.

Psychosocial effects of repetitive surgeries in children with early-onset scoliosis: are we putting them at risk?

BACKGROUND: The use of growing instrumentation in children with early-onset scoliosis (EOS) has created interest in determining if these repetitive procedures are prompting the development of lasting psychosocial problems. Given the increasing role of this treatment modality in the management of EOS, this study aimed to assess the psychological status of this patient population and to determine factors associated with worse scores in various psychosocial domains. METHODS: A cross-sectional assessment of 34 EOS patients was performed utilizing 2 well-established, caregiver-completed psychiatric instruments: the Child Behavior Checklist (CBCL) and the Strength and Difficulties Questionnaire. Scores were calculated for 15 CBCL and 6 Strength and Difficulties Questionnaire domains and subdomains and grouped as "Normal" or "Abnormal" according to published normative values. The prevalence of abnormal scores was within each instrument subdomain and was compared with the national norms. Domain scores were also correlated with age at first scoliosis surgery, total number of operative procedures, and total number of growing instrumentation surgeries. RESULTS: A higher prevalence of Abnormal scores were found in multiple psychosocial domains in our cohort as compared with national normative data. Children with Abnormal CBCL "Total Problems" domain scores were younger at the time of first scoliosis surgery (2.50 vs. 5.52 y). Normal and Abnormal scoring groups showed significant differences in the number of (1) total surgeries; (2) total scoliosis surgeries; and (3) growing instrumentation surgeries in multiple domains on both instruments. Aggression, Rule-breaking, and Conduct were positively correlated with total number of surgeries. CONCLUSIONS: Our findings showed a higher prevalence of Abnormal psychosocial scores in multiple domains in multioperated EOS patients as compared with national norms. Our findings suggest that EOS patients with abnormal psychosocial scores were younger at the time of their initial scoliosis surgery. The number of repetitive surgeries also correlated positively with 3 behavioral problem scores. Although healthier scores were seen in 1 positive behavioral domain in more operated children suggesting the potential for "posttraumatic growth," the other findings of this study are concerning given the increasing use of this treatment modality and indicate a need for ongoing screening and mental health care in this high-risk population. LEVEL OF EVIDENCE: Level III--case-control.

The effect of rod diameter on correction of adolescent idiopathic scoliosis at two years follow-up.

STUDY DESIGN: The review of multicenter national pediatric scoliosis database. OBJECTIVE: The purpose of this study was to compare the radiographic outcomes of patients who underwent scoliosis surgery utilizing different rod diameter constructs by the posterior approach. BACKGROUND: Little attention has specifically been focused on the effect of rod diameter on correction of spinal deformity after posterior spinal instrumentation and fusion in children with adolescent idiopathic scoliosis (AIS). METHODS: The review of national database comprised of 1125 patients, of which 352 patients had a minimum follow-up of 2 years. Of these, 163 patients received 5.5 mm and 189 patients received 6.35 mm diameter rods for posterior spinal instrumentation. RESULTS: The 6.35 mm rods were used more often for patients who were male, taller, heavier, with larger coronal curves, and more flexible curves. Larger diameter rods were also more likely to be stainless steel, implanted with an increased number of implants per level, and an increased number of pedicle screws used on the concavity of the curve. Univariate analysis of coronal curve showed a significant difference between 5.5 and 6.35 mm rods in correction (67.0% vs. 57.3%) at 2 years. Multivariate analysis revealed that the most significant factors affecting coronal curve correction at 2 years were rod diameter, the patient's preoperative coronal major curve and flexibility, and the implant density. In the sagittal plane, preoperative sagittal curve and rod diameter are the predictors of sagittal correction at 2 years. CONCLUSIONS: The study did not support our hypothesis that larger rods would be associated with a greater correction of frontal and sagittal plane in patients with AIS. In addition to rod diameter, implant density and the inherent flexibility and deformity of the patient were found to be influential factors contributing for the correction and maintenance of coronal and sagittal curves in AIS.

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.

More experienced surgeons less likely to fuse: a focus group review of 315 hypothetical EOS cases.

INTRODUCTION: Previous work has identified significant variability in decision making and multiple areas of clinical equipoise in the treatment of early-onset scoliosis (EOS). In an attempt to better understand possible determinants of this variability, we examined the relationship between socioclinical attributes of 11 participating surgeons and decision making regarding the treatment of EOS. METHODS: Eleven experienced EOS surgeons were surveyed. The first part of the survey consisted of questions regarding surgeon and practice demographics. Next, surgeons were queried regarding their preferred management of 315 hypothetical EOS cases. Cases varied considerably in etiology [idiopathic, and low-tone and high-tone neuromuscular (HTNM)], age, and curve severity and progression. Treatment options were analyzed both individually and grouped as conservative (observation, bracing, or casting) versus surgical (spine-based or rib-based distraction, growth guidance, growth modulation, or definitive fusion). An "outlier" variable was created to determine the extent of a surgeon's deviation from the group in management decisions. A univariate and multivariate regression analysis to identify statistical associations between physician characteristics and their management decisions in the presented hypothetical cases was performed. RESULTS: The cohort's mean years in practice was 20.7±7.36 years. Fifty-six percent of the cohort held Chest Wall and Spine Deformity Study Group (CWSDSG) membership and 56% were members of the Growing Spine Study Group. Multivariate regression demonstrated more years of practice predicted a lower preference for fusion (P<0.05). This effect was greater among HTNM cases (P<0.05). Overall, there was equal interest among groups regarding the choice between rib-based and spine-based distraction methods; however, for the subset of patients with HTNM scoliosis, membership in the CWSDSG (P<0.05) and the percentage of practice spent treating spinal deformity (P<0.05) predicted more rib-based distraction use. CONCLUSIONS: EOS surgeons with more experience were less likely to opt for definitive fusion. Use of rib-based distraction methods was common across surgeons in both study groups and within various cohorts of patients. LEVEL OF EVIDENCE: Level V (survey of experts).

What's the evidence? Systematic literature review of risk factors and preventive strategies for surgical site infection following pediatric spine surgery.

BACKGROUND: Despite relatively high rates of surgical site infections (SSIs) after pediatric spine surgery, practice guidelines are absent. We performed a systematic review of the literature, determining the level of evidence for risk factors for SSIs and prevention practices to reduce SSIs following pediatric spine surgery. METHODS: The search utilized the root search words "spine," "scoliosis," and "infection" resulting in 9594 abstracts. Following removal of duplicate abstracts, those that assessed only SSI rates, SSI treatment, nonoperative spine infections, or adult populations, 57 relevant studies were rated for level of evidence and graded using previously validated scales. RESULTS: Very few studies lead to grade A (good evidence) or grade B (fair evidence) recommendations. Ceramic bone substitute did not increase the risk of SSIs when compared with autograft (grade A). Comorbid medical conditions, particularly cerebral palsy or myelodysplasia; urinary or bowel incontinence; nonadherence to antibiotic prophylaxis protocols; and increased implant prominence increase the risk of SSIs (grade B). SSIs caused by gram-negative bacilli were more frequent in neuromuscular populations and first-generation stainless steel implants increased the risk of delayed infection compared to newer generation titanium implants (grade B). Evaluations of other risk factors for SSIs yielded conflicting or poor-quality evidence (grade C); these included malnutrition or obesity; number of levels fused or fusion extended to the sacrum/pelvis; blood loss; and use of allograft. Insufficient evidence (0 to 1 published studies) was available to recommend numerous practices shown to reduce SSI risk in other populations such as chlorhexidine skin wash the night before surgery, preoperative nasal swabs for Staphylococcus aureus, chlorhexidine skin disinfection, perioperative prophylaxis with intravenous vancomycin, vancomycin, or gentamicin powder in the surgical site or graft. CONCLUSIONS: Few studies have evaluated risk factors and preventive strategies for SSIs following pediatric spine surgery. This systematic review documents the relative lack of evidence supporting SSI prevention practices and highlights priorities for research. LEVEL OF EVIDENCE: Level III therapeutic study.

Building consensus: development of a Best Practice Guideline (BPG) for surgical site infection (SSI) prevention in high-risk pediatric spine surgery.

BACKGROUND: Perioperative surgical site infection (SSI) after pediatric spine fusion is a recognized complication with rates between 0.5% and 1.6% in adolescent idiopathic scoliosis and up to 22% in "high risk" patients. Significant variation in the approach to infection prophylaxis has been well documented. The purpose of this initiative is to develop a consensus-based "Best Practice" Guideline (BPG), informed by both the available evidence in the literature and expert opinion, for high-risk pediatric patients undergoing spine fusion. For the purpose of this effort, high risk was defined as anything other than a primary fusion in a patient with idiopathic scoliosis without significant comorbidities. The ultimate goal of this initiative is to decrease the wide variability in SSI prevention strategies in this area, ultimately leading to improved patient outcomes and reduced health care costs. METHODS: An expert panel composed of 20 pediatric spine surgeons and 3 infectious disease specialists from North America, selected for their extensive experience in the field of pediatric spine surgery, was developed. Using the Delphi process and iterative rounds using a nominal group technique, participants in this panel were as follows: (1) surveyed for current practices; (2) presented with a detailed systematic review of the relevant literature; (3) given the opportunity to voice opinion collectively; and (4) asked to vote regarding preferences privately. Round 1 was conducted using an electronic survey. Initial results were compiled and discussed face-to-face. Round 2 was conducted using the Audience Response System, allowing participants to vote for (strongly support or support) or against inclusion of each intervention. Agreement >80% was considered consensus. Interventions without consensus were discussed and revised, if feasible. Repeat voting for consensus was performed. RESULTS: Consensus was reached to support 14 SSI prevention strategies and all participants agreed to implement the BPG in their practices. All agreed to participate in further studies assessing implementation and effectiveness of the BPG. The final consensus driven BPG for high-risk pediatric spine surgery patients includes: (1) patients should have a chlorhexidine skin wash the night before surgery; (2) patients should have preoperative urine cultures obtained; (3) patients should receive a preoperative Patient Education Sheet; (4) patients should have a preoperative nutritional assessment; (5) if removing hair, clipping is preferred to shaving; (6) patients should receive perioperative intravenous cefazolin; (7) patients should receive perioperative intravenous prophylaxis for gram-negative bacilli; (8) adherence to perioperative antimicrobial regimens should be monitored; (9) operating room access should be limited during scoliosis surgery (whenever practical); (10) UV lights need NOT be used in the operating room; (11) patients should have intraoperative wound irrigation; (12) vancomycin powder should be used in the bone graft and/or the surgical site; (13) impervious dressings are preferred postoperatively; (14) postoperative dressing changes should be minimized before discharge to the extent possible. CONCLUSIONS: In conclusion, we present a consensus-based BPG consisting of 14 recommendations for the prevention of SSIs after spine surgery in high-risk pediatric patients. This can serve as a tool to reduce the variability in practice in this area and help guide research priorities in the future. Pending such data, it is the unsubstantiated opinion of the authors of the current paper that adherence to recommendations in the BPG will not only decrease variability in practice but also result in fewer SSI in high-risk children undergoing spinal fusion. LEVEL OF EVIDENCE: Not applicable.