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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., (© 2022. The Author(s), under exclusive licence to Scoliosis Research Society.)

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., Competing Interests: Disclosure: The Disclosure of Potential Conflicts of Interest forms are provided with the online version of the article ( http://links.lww.com/JBJS/H106 )., (Copyright © 2022 by The Journal of Bone and Joint Surgery, Incorporated.)

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., Competing Interests: The authors declare no conflicts of interest., (Copyright © 2022 Wolters Kluwer Health, Inc. All rights reserved.)

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.

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.

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.

Purpose: Surgical planning for Adolescent Idiopathic Scoliosis (AIS) relies on the coronal and sagittal plane to determine the lowest instrumented vertebra (LIV). Failure to include the stable sagittal vertebra (SSV) within the construct can increase the incidence of postoperative distal junctional kyphosis (DJK). The purpose of this study is to assess the variability of SSV within patients and to identify positional parameters that may lead to its change., Methods: This is a case-control study of AIS patients with changes in SSV throughout serial radiographs. Radiographic sagittal parameters and hand positioning for the patients with changes in SSV were compared to patients with stable SSV. Additionally, a subgroup analysis was conducted to compare the positional parameters of only the patients with changes in SSV., Results: 46 patients with a mean age of 15 ± 1.8 years old at the time of surgery were included in this study. 33/76 (43.4%) image pairs were found to have a change in SSV. Positional parameters associated with the more distally measured SSV were found to have a more negative sagittal vertebral axis (p = 0.001), more positive pelvic shift (p = 0.023), and more negative Global Sagittal Axis (p = 0.001) when compared to the more proximally measured SSV., Conclusion: Significant variability exists in the determination of SSV in AIS patients undergoing serial radiographs. Positional parameters associated with the proximal and distally measured SSV also have variability which indicates that posture has a significant impact on this measure. Surgeons need to be aware of SSV variability during preoperative planning and must consider multiple parameters for the determination of LIV., Level of Evidence: 3.

Early onset scoliosis is a complex manifestation of a heterogenous group of disorders, often necessitating multiple medical and surgical interventions to address the spinal deformity and its accompanying comorbidities. Current literature documents decreases in the health-related quality of life of these patients; however, there is a distinct lack of published data examining the burden on their caregivers. The purpose of this study is to compare burden on caregivers of children with early onset scoliosis and those on caregivers of age-matched healthy peers. A multicenter retrospective cohort study was conducted by querying a national registry for patients with early onset scoliosis diagnosed before 10 years old whose caregivers completed the caregiver burden (CB) and financial burden (FB) domains of the Early Onset Scoliosis Questionnaire (EOSQ-24) before their treatment. Scores were compared by etiology and planned treatment. The study identified 503 patients categorized by etiology and eventual treatment type. Overall, FB and CB scores for patients with early onset scoliosis were ≥10% worse than those of their age-matched peers, greater than the minimal clinically important difference for the EOSQ-24 (P < 0.001). Non-idiopathic patients' scores were ≥16% worse than age-matched peers regardless of future treatment (P < 0.001), while scores for idiopathic patients were varied. Idiopathic patients who went on to be observed had similar scores to national norms, but those who were managed either non-operatively (14% worse FB, 7% worse CB; P < 0.001) or operatively (25% worse FB, 27% worse CB; P > 0.05) had caregivers who reported greater burdens compared to those of healthy peers. This study suggests burdens on caregivers of patients with early onset scoliosis of nearly all etiologies are greater than those imposed on caregivers of healthy children, even before the additional stress of treatment is imposed. Level of evidence: II.

Study Design: Retrospective cohort study., Objectives: The purpose of this study was to explore the association between pre-brace and in-brace sagittal parameters and curve progression. To date, there has been no published research focused on spinopelvic sagittal parameters and bracing outcomes in AIS. We hypothesize that sagittal spinopelvic parameters are associated with curve progression at 2 years., Methods: This study included AIS patients with a pre-brace (PB) major curve between 20° and 45°. The outcome was defined as > 10° curve progression or surgery within 2 years of brace initiation. Spinopelvic parameters included C7-Central Sacral Vertebral Line shift (C7-CSVL), thoracic trunk shift, lumbar lordosis (LL), pelvic incidence (PI), T2-T12 thoracic kyphosis (TK) pelvic incidence-lumbar lordosis (PI-LL) mismatch, sagittal vertical axis (SVA), and pelvic tilt (PT)., Results: Of 50 patients included in this review, [70% Rigo (RCSO) and 30% Boston (BSO)], 16 (32%) patients demonstrated progression (23% of patients with RCSO vs 53% with BSO; p = 0.034). In patients with more than 30% major coronal curve correction (CCC), 23% had progression. 45% of patients progressed when they achieved ≤ 30% correction (p = 0.108). Among PB sagittal parameters and adjusting for coronal curve, patients with an abnormal PB SVA had 3.1 times increased risk of treatment failure compared with patients who had a normal PB SVA. Patients with PB hypo-LL had a 2.8 times increased risk of treatment failure compared with patients who had normal or hyper-LL. Among IB sagittal parameters, patients who had a normal PB PI-LL had a 3.9 times increased risk of treatment failure when they became mismatched in-brace (IB). Patients who had normal pre-brace kyphosis who became hypo-kyphotic IB had an 8.4 times increased risk of treatment failure compared with patients who maintained normal TK or became hyper-kyphotic., Conclusion: These data suggest that we should pay careful attention to sagittal parameters prior to and during brace treatment as braces can control these parameters., Level of Evidence: Level III.

Study Design: Expert consensus building using combined Delphi method and Nominal group technique., Objectives: To identify the current state of equipoise surrounding the use of magnetically controlled growing rods (MCGRs) and to determine consensus for planning a randomized controlled trial (RCT) with MCGRs., Background: The use of MCGRs for the treatment of early-onset scoliosis (EOS) is a new technology. Optimal use has not been thoroughly investigated and much uncertainty exists. Areas of uncertainty include construct architecture, timing of lengthenings, and amount of distraction per lengthening. Expert discussion and consensus is useful at this early juncture and necessary when designing an RCT., Methods: Two rounds of surveys were administered to a group of experienced pediatric spine surgeons, followed by a 2-hour, face-to-face meeting in November 2015 and a 1-hour, face-to-face meeting in February 2016. The first survey used example cases to establish agreement around the proper use of MCGRs and identified areas of equipoise and disagreement. The second survey again used example cases-this time selected for their equipoise status-to solicit trial arms for a potential RCT of MCGRs and identified important open questions in the use of MCGRs. Lastly, the face-to-face meetings employed iterative voting to preliminarily plan an RCT of MCGRs., Results: Following the Delphi survey rounds and the two Nominal face-to-face meetings, the group of experts decided on an MCGR RCT design that standardized all patients to bidirectional constructs, and randomized to a lengthening interval of 6 versus 16 weeks with a standardized equation for calculating the total yearly lengthening that approximates normal spine growth., Conclusion: This endeavor indicates expert support for the use of MCGR in children older than 6 years, with curves greater than 60°. The uncertainty surrounding frequency of lengthening justifies an RCT of MCGRs., Level of Evidence: Level V., (Copyright © 2017 Scoliosis Research Society. Published by Elsevier Inc. All rights reserved.)

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.

Background: Autologous blood transfusion, commonly referred to as cell saver, is frequently used in spinal fusion to salvage red blood cells because of the risk of significant intraoperative blood loss. This case report describes a case of acute kidney injury (AKI) secondary to cell saver use. Our objective is to increase the knowledge about the process of red blood cell salvage and this exceedingly rare complication., Methods: Chart and renal biopsy results for a single case were reviewed and reported in this retrospective study., Results: A healthy 18-year-old male patient underwent posterior spinal instrumentation and fusion for adolescent idiopathic scoliosis with utilization of intraoperative autologous blood transfusion. The patient subsequently developed hematuria and AKI with a peak creatinine of 13.9 mg/dL. An extensive clinical workup, including autoimmune serology, excluded any identifying causes. A renal biopsy showed pigment-induced acute tubular necrosis., Conclusions: This case, to our knowledge, is the first and only case report of AKI secondary to cell saver demonstrated by renal biopsy. The literature has shown both the benefit of cell saver by decreasing the need for allogeneic transfusion and the risk of transient hematuria. However, this case demonstrates the importance of monitoring patients for potential complications., (Copyright © 2017 Scoliosis Research Society. Published by Elsevier Inc. All rights reserved.)

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.

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.

Study Design: This study determines the rate of change in the scoliosis surgery plan in cases presented in preoperative indications conference., Objectives: To determine the effect of preoperative indications conference on the plan of surgery and to identify characteristics that increased the likelihood of change., Summary of Background Data: Preoperative indications conferences are used as a teaching and planning tool. Levels of fusion, construct options, and necessity for osteotomies are often debated in the planning of scoliosis surgery., Methods: Scoliosis surgeries were presented at preoperative indications conference with four attending pediatric orthopedic surgeons present. The operative surgeon committed to a surgical plan before conference. A consensus-based plan was made without knowledge of the operative surgeon's preconference plan. Changes of plan were classified as major, minor, or no change., Results: Of the 107 surgical plans, 50 were index surgeries, 13 were revisions, and 44 were scheduled growing rod lengthenings. There were two major changes, including a change to a growing construct from planned fusion, and a change in fusion levels in an adolescent idiopathic scoliosis (AIS) patient. There were 13 minor changes, which included changes in fusion levels (1 to 3; mean = 1.23) and the addition of an osteotomy. The rate of change was 28% for index surgeries and 7.69% for revisions. Of the 14 changes in the 50 index surgeries, there were 8 AIS, 3 cerebral palsy, 1 congenital scoliosis, 1 Ehlers-Danlos, and 1 patient with an undetermined neuromuscular condition. There was 1 change in 13 revision surgeries. There were no changes for growing rod lengthenings and no cancellations as a result of indications conference., Conclusions: Although revision scoliosis surgery is complex, index AIS/JIS surgery was most subject to the influence of indications conference. This likely reflects controversy around choosing levels of fusion., Level of Evidence: IV., (Copyright © 2016 Scoliosis Research Society. Published by Elsevier Inc. All rights reserved.)

Background: ScoliScore is a DNA-based prognostic test, designed and used to help to predict the risk of curve progression in patients with adolescent idiopathic scoliosis. The role of this test in clinical practice remains unclear as the published results of the ScoliScore have not been validated independently. The purpose of this study was to determine if the ScoliScore effectively predicted the risk of curve progression in patients with mild and moderate adolescent idiopathic scoliosis in two urban academic medical centers., Methods: One hundred and twenty-six patients with adolescent idiopathic scoliosis who met inclusion criteria at two centers were administered the ScoliScore test. Two groups were created: a progression group (those who had a Cobb angle of >40° or those who had undergone surgical fusion) and a non-progression group (those who had skeletal maturity without curve progression to 40°). ScoliScore values and risk levels were compared between the two groups. The negative predictive value was calculated for low-risk scores and the positive predictive value was calculated for high-risk scores., Results: There was no significant difference (p = 0.706) in the mean ScoliScore (and standard deviation) between patients with curve progression (107 ± 55 points) and those without curve progression (102 ± 62 points). There was also no significant difference (p = 0.399) in curve progression between patients with high-risk scores (26.7%) and those with low-risk scores (12.9%). The positive predictive value of the test was 0.27 (95% confidence interval, 0.09 to 0.55), and the negative predictive value was 0.87 (95% confidence interval, 0.69 to 0.96). ScoliScores and rates of progression were not affected by brace-wear., Conclusions: ScoliScores did not differ between patients with and without curve progression, and the negative and positive predictive values were lower in our study than in the previously published validation study by the developers of the test. This may be due to differences in our test population, genetic variability, or failure of patients in the non-progression group to follow up., (Copyright © 2015 by The Journal of Bone and Joint Surgery, Incorporated.)

Background: Early-onset scoliosis is a heterogeneous condition, with highly variable manifestations and natural history. No standardized classification system exists to describe and group patients, to guide optimal care, or to prognosticate outcomes within this population. A classification system for early-onset scoliosis is thus a necessary prerequisite to the timely evolution of care of these patients., Methods: Fifteen experienced surgeons participated in a nominal group technique designed to achieve a consensus-based classification system for early-onset scoliosis. A comprehensive list of factors important in managing early-onset scoliosis was generated using a standardized literature review, semi-structured interviews, and open forum discussion. Three group meetings and two rounds of surveying guided the selection of classification components, subgroupings, and cut-points. Initial validation of the system was conducted using an interobserver reliability assessment based on the classification of a series of thirty cases., Results: Nominal group technique was used to identify three core variables (major curve angle, etiology, and kyphosis) with high group content validity scores. Age and curve progression ranked slightly lower. Participants evaluated the cases of thirty patients with early-onset scoliosis for reliability testing. The mean kappa value for etiology (0.64) was substantial, while the mean kappa values for major curve angle (0.95) and kyphosis (0.93) indicated almost perfect agreement. The final classification consisted of a continuous age prefix, etiology (congenital or structural, neuromuscular, syndromic, and idiopathic), major curve angle (1, 2, 3, or 4), and kyphosis (-, N, or +) variables, and an optional progression modifier (P0, P1, or P2)., Conclusions: Utilizing formal consensus-building methods in a large group of surgeons experienced in treating early-onset scoliosis, a novel classification system for early-onset scoliosis was developed with all core components demonstrating substantial to excellent interobserver reliability. This classification system will serve as a foundation to guide ongoing research efforts and standardize communication in the clinical setting., (Copyright © 2014 by The Journal of Bone and Joint Surgery, Incorporated.)

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.

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.

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.

Background: Literature guiding the management of early-onset scoliosis consists primarily of studies with a low level of evidence. Evaluation of clinical equipoise (i.e., when there is no known superiority among treatment modalities) allows for prioritization of research efforts. The objective of this study was to evaluate areas of clinical uncertainty among pediatric spine surgeons regarding the treatment of early-onset scoliosis., Methods: Fourteen experienced pediatric spine surgeons participated in semistructured interviews to identify clinical variables that influence decision making in the treatment of early-onset scoliosis. A series of case scenarios of 315 patients with idiopathic and neuromuscular early-onset scoliosis was then developed to be representative of those encountered in clinical practice. Using an online survey, eleven surgeons selected their choice of eight treatment options for each case scenario. Associations between case characteristics and treatment choices were assessed with chi-square and logistic regression analysis. Participants then reviewed the areas of treatment uncertainty identified in the survey, nominated additional research questions of interest, and ranked their interest to further explore the identified research questions., Results: Collective equipoise was identified in numerous scenarios in the survey spanning a range of ages and magnitudes of scoliosis, and additional questions were identified during the nominal group technique. Areas that had the greatest clinical uncertainty included the management of patients who have finished treatment with a growing-rod, timing of rod-lengthening intervals, and indications for spine-based and rib-based proximal instrumentation anchors. The use of rib anchors compared with spine-based anchors was ranked highly for consideration in future clinical trials., Conclusions: Variability in decision making with regard to the optimum treatment of certain subsets of patients with early-onset scoliosis reflects gaps in the available evidence. Structured consensus methods identified priorities for higher levels of research in this area of scoliosis. Higher-level studies, including randomized trials, should focus on answering the questions highlighted in this report.

Background: Surgical site infection following correction of pediatric scoliosis is well described. However, we are aware of no recent multicenter study describing the rates of surgical site infection, and associated pathogens, among patients with different etiologies for scoliosis., Methods: A multicenter, retrospective review of surgical site infections among pediatric patients undergoing spinal instrumentation to correct scoliosis was performed at three children's hospitals in the United States. Study subjects included all patients undergoing posterior spinal instrumentation from January 2006 to December 2008. Surgical site infections were defined according to the Centers for Disease Control and Prevention's National Healthcare Safety Network case definition, with infections occurring within one year after surgery., Results: Following the analysis of 1347 procedures performed in 946 patients, surgical site infection rates varied among procedures performed in patients with different scoliosis etiologies. Procedures performed in patients with neuromuscular scoliosis had the highest surgical site infection rates (9.2%), followed by those performed in patients with syndromic scoliosis (8.8%), those performed in patients with other scoliosis (8.4%), those performed in patients with congenital scoliosis (3.9%), and those performed in patients with idiopathic scoliosis (2.6%). Surgical site infection rates varied among procedures in patients undergoing primary spinal arthrodesis based on etiology, ranging from 1.2% (95% confidence interval, 0.1% to 1.3%) in patients with idiopathic scoliosis to 13.1% (95% confidence interval, 8.4% to 17.8%) in patients with neuromuscular scoliosis. Surgical site infection rates following primary and revision procedures were similar among patients with different etiologies. In distraction-based growing constructs, rates were significantly lower for lengthening procedures than for revision procedures (p = 0.012). Multivariate analysis demonstrated that non-idiopathic scoliosis and extension of instrumentation to the pelvis were risk factors for surgical site infections. The three most common pathogens were Staphylococcus aureus (25.0% [95% confidence interval, 17.8% to 32.2%]), coagulase-negative staphylococci (17.1% [95% confidence interval, 10.9% to 23.3%]), and Pseudomonas aeruginosa (10.7% [95% confidence interval, 5.6% to 15.8%]). Overall, 46.5% (95% confidence interval, 35.5% to 57.5%) of surgical site infections contained at least one gram-negative organism; 97.0% (95% confidence interval, 90.8% to 100.0%) of these infections were in patients with non-idiopathic scoliosis., Conclusions: Surgical site infection rates were significantly higher following procedures in patients with non-idiopathic scoliosis (p < 0.001). Lengthening procedures had the lowest rate of surgical site infection among patients with early onset scoliosis who had undergone instrumentation with growing constructs. Gram-negative pathogens were common and were most common following procedures in patients with non-idiopathic scoliosis. These findings suggest a role for targeted perioperative antibiotic prophylaxis to prevent surgical site infection following pediatric scoliosis instrumentation procedures.

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).

Study Design: Retrospective study comparing ScoliScore and clinical risk estimates., Objective: The purpose of this study was to compare risk stratification between ScoliScore and traditional clinical estimates to determine whether ScoliScore provides unique information., Summary of Background Data: ScoliScore is a genetic prognostic test designed to evaluate the risk of curve progression in skeletally immature patients with adolescent idiopathic scoliosis with Cobb angles of 10° to 25°. Clinicians are currently trying to better understand the role this test may play in guiding clinical decision making because current standards of curve progression are largely based on radiographical markers, such as curve magnitude and bone age., Methods: Ninety-one patients who received ScoliScore testing at our center and met study inclusion criteria were identified. Patients were given a "clinical risk" level using their Risser sign and Cobb angle. Assigned clinical risk levels were compared with the ScoliScore risk levels reported by the manufacturer's scoring algorithm., Results: ScoliScore risk distribution in our population was 36% low risk, 55% intermediate risk, and 9% high risk. This compares with 2%, 51%, and 47%, respectively, for comparable clinical risk groupings. Only 25% of patients were in the same risk category for both systems. There were no significant correlations between ScoliScore and age, race, menarcheal status, Risser sign, or sex. There was a positive correlation between the Cobb angle and the ScoliScore (r = 0.581, P < 0.001). Cobb angle remained significant in the multivariate regression model (P < 0.001), and Cobb angle was found to account for 33.3% of ScoliScore's variance., Conclusion: Only Cobb angle showed significant correlation with ScoliScore among the socioclinical variables studied. The risk distribution of the 2 risk estimation systems examined differed markedly: ScoliScore predicted nearly 16 times more low-risk patients and more than 5 times fewer high-risk patients. This demonstrates that ScoliScore provides unique information to traditional predictors of curve progression, advancing our understanding of the role of ScoliScore in the clinical setting.

Early diagnosis is critical in patients with congenital scoliosis to identify vertebral defects that have a high risk for progression. A review of the medical records and chest radiographs for 562 patients who underwent corrective surgery for tetralogy of Fallot (TOF) at our institution between 1992 and 2007 was conducted. Of the 364 patients examined in the study, 12 patients (3.3%), six girls and six boys, had a positive diagnosis of congenital scoliosis confirmed by examination of radiographs. This incidence is significantly higher than both the incidence reported in prior studies (0-1.4%) and in the general population (0.05-0.10%). Given that patients with TOF routinely undergo chest radiograph, physicians examining chest radiographs of TOF patients should be aware of the potential for congenital scoliosis to provide early diagnosis and referral for orthopaedic evaluation and treatment.

Study Design: Randomized controlled trial., Objective: To assess whether monitoring increases brace-wearing compliance in patients with adolescent idiopathic scoliosis (AIS)., Summary of Background Data: Noncompliance is a barrier to brace treatment of AIS. Studies have demonstrated that monitoring improves medication compliance; however, this has not been investigated in spinal braces., Methods: Twenty-one patients (mean age = 12.4 ± 2.0 years) with AIS were prescribed treatment with a custom-made Thoraco-Lumbo-Sacral-Orthosis for 18 hours a day using a standardized script. Before beginning treatment, 10 patients were randomized to be informed that their compliance was monitored, whereas 11 patients were unaware. Compliance was measured via a temperature probe embedded within the Thoraco-Lumbo-Sacral-Orthosis hidden from view., Results: Patients who were notified that they had a monitor in their brace demonstrated significantly increased compliance during the first 14 weeks of treatment compared with those who were uninformed (85.7% vs. 56.5%, P = 0.029), corresponding to a mean difference of 5.24 hours of daily brace wear., Conclusion: Electronic monitoring can improve compliance with orthoses in patients with spinal deformity during a short observation period.

The natural history of early onset scoliosis is dismal and associated with poor pulmonary function and increased mortality. Although limited in situ fusion may be appropriate for certain types of congenital scoliosis deformities, spinal deformity that affects young children often requires a "growth friendly" surgical approach that allows for curve control while maintaining growth of the spine and thorax. Growth-friendly surgical management of early onset scoliosis can follow a distraction-based (ie, growth rods, vertical expandable prosthetic titanium rib [Synthes, West Chester, PA]), guided-growth (ie, Luque trolley technique, Shilla technique), or compression-based (ie, tethers, staples) strategy.

Background: In contrast with treatment recommendations for adolescent idiopathic scoliosis, there are no clear algorithms for treating patients with early-onset scoliosis. There has been rapid expansion of treatment options for children with early-onset scoliosis, including casting, growth rods, the vertical expandable prosthetic titanium rib, and anterior vertebral stapling., Questions/purposes: Given the range of treatment options, we assessed variability in decision making regarding treatment of patients with early-onset scoliosis., Methods: We presented 12 clinical and radiographic vignettes about patients with early-onset scoliosis to 13 experienced spine surgeons who are members of the Chest Wall and Spine Deformity Study Group. The reviewers were asked to choose type of treatment, type of construct, construct location, and whether a thoracotomy should be performed., Results: All 13 surgeons agreed regarding the need for surgery in eight of the 12 cases. When the reviewers chose surgery, 76% (40%-100%) selected the vertical expandable prosthetic titanium rib; of those selecting that approach, 61% (0%-100%) coincided on using it bilaterally. Agreement was 20% (0%-60%) for growing rods and 4% (0%-25%) for fusions. Among all cases, agreement regarding whether instrumentation should extend to the pelvis was 71% (50%-100%). In all but two cases, at least 85% of surgeons recommended against a thoracotomy., Conclusions: Although most surgeons agreed about the indication for surgery, we found wide variability in choice of construct type, number of constructs, and level of instrumentation.

Background: Treatment of children with early onset scoliosis (EOS) seeks to improve natural history and health related quality of life (QOL). QOL measurement presents a significant challenge given the young age, comorbidities, and heterogeneity of this population. The purpose of this study is to develop a disease specific measure reflecting issues of importance to EOS patients and caretakers that is responsive to clinical and treatment changes., Methods: Review of the literature and relevant measures informed development of semistructured qualitative interviews. Interviews of caregivers of EOS patients were interpreted through the framework technique. A masterlist of 75 items was created and responses were scaled on a 5-point Likert scale. Psychometric analysis was carried out to group items into domains, to maintain score distribution, to create a responsive instrument, and to identify question redundancy., Results: Content validation showed 8 items with low content validity indices (<0.70). Item distribution analysis showed 19 items with skewed distribution. Item reliability showed redundant items within domains (r≥0.5). Construct validity showed unique domains measuring intended issues of interest. The resulting Early Onset Scoliosis Questionnaire (EOSQ) includes 33 items in 13 domains: General Health, Pain, Physical Function, Pulmonary Function, Daily Living, Fatigue, Emotion, Surgical Concerns, Satisfaction, Transfer, Financial Burden, Parental Burden, and Treatment Outcomes., Conclusions: The EOSQ reflects QOL and caregiver burden in the EOS population. The EOSQ will expand options for outcome assessment in this unique population.

Introduction: Treatment of children with early onset scoliosis (EOS) seeks to improve natural history and health related quality of life (QOL). QOL measurement presents a significant challenge given the young age, comorbidities, and heterogeneity of this population., Objective: To develop a disease specific measure reflecting issues of importance to EOS patients and caretakers., Materials and Methods: Review of the literature and relevant measures informed development of semi-structured qualitative interviews. Interviews of caregivers of EOS patients were interpreted through the framework technique. A master-list of 75-items was created and responses were scaled on a 5-point Likert-scale. Psychometric analysis was performed to group items into domains, to maintain score distribution, to create a responsive instrument, and to identify question redundancy., Results: Content validation revealed 8-items with low content validity indices. Item distribution analysis demonstrated 19-items with skewed distribution. Item reliability demonstrated redundant items within domains (r> or =0.5). Construct validity demonstrated unique domains measuring intended issues of interest. The resulting Early Onset Scoliosis Questionnaire (EOSQ) includes 33-items in 13-domains: General Health, Pain, Physical Function, Pulmonary Function, Daily Living, Fatigue, Emotion, Surgical Concerns, Satisfaction, Transfer, Financial Burden, Parental Burden, and Treatment Outcomes., Conclusion: The EOSQ reflects QOL and caregiver burden in the EOS population. The EOSQ will expand options for outcome assessment in this unique population.

Study Design: Retrospective cohort study., Objective: The purpose of this study is to evaluate pulmonary function and quality of life (QOL) in children treated with early spinal fusion for progressive congenital scoliosis., Summary of Background Data: The relationships between radiographic measures, pulmonary function tests (PFT), and QOL were examined. METHODS.: Twenty-one patients with congenital scoliosis treated with early spinal fusion were evaluated using radiographs, PFT, and the Child Health Questionnaire (CHQ) at 12.6 +/- 3.5 years. They were 6.9 +/- 2.3 years postdefinitive fusion, which occurred at 4.9 +/- 3.1 year of age. The cohort was also divided in two groups, "thoracic fusion" (apex above the thoracolumbar T-L junction) and "nonthoracic fusion" (below T-L junction)., Results: Forced vital capacity, forced expiratory volume (FEV1), vital capacity (P < 0.0001), and total lung capacity (P = 0.017) were significantly lower compared with healthy children. CHQ scores were significantly lower than in healthy children in physical function (P = 0.036), general health (P = 0.007), physical summary (P = 0.026), and parental impact/emotional (P = 0.01). Correlation analysis showed that the degrees of thoracic curves were negatively correlated with FEV1 (P < 0.05), family activities, role/social physical (P < 0.05), and physical summary (P < 0.01). The degree of kyphosis was negatively correlated with Self-Esteem (P < 0.01). Patients who had thoracic fusions had shorter spinal height (P = 0.049), lower forced vital capacity (P = 0.004), FEV (P = 0.012), vital capacity (P = 0.031), and reported more pain (P = 0.033) than nonthoracic fused., Conclusion: Compared with healthy peers, congenital scoliosis patients treated with early spinal fusion have worse PFT and QOL scores at 6.9 years follow up. Patients with thoracic fusions had shorter spines, worse pulmonary function, and more pain than nonthoracic fused. The results may support alternatives to early spinal fusion such as growing rods, epiphysiodesis, and distraction thoracoplasty. Current efforts are underway to compare outcomes of this study to those of other treatments.

Study Design: This is a retrospective cohort study examining 61 patients with neurogenic scoliosis who underwent anterior and/or posterior spinal instrumentation at the age of 18 and younger., Objective: The purpose of this study is to investigate this finding further by analyzing the effect of recombinant human erythropoietin (rhEPO) on hematocrit, transfusion and complication rates, and the length of intensive care unit (ICU) days in patients with neurogenic scoliosis., Summary of Background Data: The preoperative use of rhEPO has been shown to decrease perioperative transfusion requirements in many adult and pediatric patients. A recent study at our institution demonstrated the efficacy of rhEPO in pediatric idiopathic scoliosis patients, but suggested the possibility of an "erythropoietin resistance" in the pediatric neurogenic scoliosis population., Methods: The patients' age at the time of surgery, gender, Cobb angle, erythropoietin administration and dosage, hematocrit levels, type of surgery, intraoperative blood loss, duration of surgery, number of vertebrae fused, comorbidities, complications, transfusion status, and the length of ICU days were collected., Results: Thirty-five (57.3%) children received preoperative rhEPO, whereas 26 patients (42.7%) did not receive rhEPO. The mean preoperative and discharge hematocrit levels in the patients treated with rhEPO were significantly higher than the non-rhEPO group (P = 0.05). There were no significant difference in likelihood of transfusion, complications, and the length of ICU days between the rhEPO and the non-rhEPO groups. A multivariate analysis demonstrated that the number of fused vertebral levels maintained its significance (P = 0.044) and surgical time had a trend toward significance (P = 0.051) in predicting likelihood of transfusion., Conclusion: The use of rhEPO effectively stimulated erythropoiesis in these patients and yet demonstrated no significant clinical benefit in reducing the likelihood of transfusion in neurogenic patients in this study. More research is necessary to design a transfusion risk reduction protocols that will minimize the exposure of neurogenic scoliosis patients to allogeneic blood products.

Background Context: Spine surgery for scoliosis is associated with significant blood loss and may require blood transfusion. The risks inherent in blood transfusion have inspired interventions, including human recombinant erythropoietin (rHuEPO), which has emerged as a safe and effective adjunct in minimizing exposure to allogenic blood in children undergoing scoliosis surgery. However, there is little information in the literature on their economic impact on the health-care system., Purpose: The purpose of this study was to evaluate the cost-effectiveness of rHuEPO in adolescent idiopathic scoliosis., Study Design/setting: We designed a decision analytic model capturing costs and potential long-term outcomes of transfusion-related complications to compare the cost-effectiveness of preoperative rHuEPO with a control group and a common alternative, preoperative autologous donation (PAD)., Patient Sample: This is a decision analytic model. Decision modeling relies on a summation of the probability of different clinical studies; no patients were specifically studied., Outcome Measures: The standard cost-effectiveness ratio (C/E) of $50,000 per quality-adjusted life year (QALY) was used as the threshold value for determining the cost-effectiveness of these two preoperative intervention strategies., Methods: From the model we calculated the probability that a patient would experience transfusion-related complications based on the mean number of allogenic units transferred as a result of scoliosis surgery. The standard C/E of $50,000 per QALY was used as the threshold value for determining the cost-effectiveness. Some aspects of the model were derived from retrospective data from the literature. Sensitivity analyses were also conducted to discover which variables, when changed within the accepted range, caused the final result of the model to change significantly., Results: Results indicated a cost per year-of-life-saved exceeding $1 million for the PAD arm and over $1.5 million for the rHuEPO arm. The low rates of transfusion in adolescent idiopathic scoliosis surgery and the relative safety of the blood supply create a scenario where even inexpensive interventions are not cost-effective. However, rHuEPO would become cost-effective if decreased transfusion rates or higher postoperative hematocrit resulted in decreased length of stay., Conclusion: The use of rHuEPO preoperatively and the use of PAD are not cost-effective although both techniques remain clinically effective tools.

Study Design: This was a retrospective cohort study using a previously matched convenience sample of 34 patients., Objective: This study sought to determine the relative corrective benefits of these 2 types of constructs in the correction of coronal and sagittal curves in patients with adolescent idiopathic scoliosis (AIS). In addition, the 2 constructs were compared for coronal and sagittal balance., Summary of Background Information: Recent clinical research suggests that thoracic pedicle screw constructs (all-screw constructs) are more effective than hybrid lumbar screw thoracic hook constructs (hybrid constructs) in correcting spine deformity., Methods: The sample consisted of patients with AIS who underwent isolated posterior spinal fusion and instrumentation. Seventeen patients underwent fusion using all-screw constructs, and 17 underwent fusion with hybrid constructs; preoperative and postoperative radiographs and measurements were compared., Results: There was no significant difference observed when comparing the 2 groups, although there was a trend toward better correction of the main thoracic curve in the all-screw construct group (P = 0.089). In the all-screw group, mean thoracic kyphosis decreased from 29.6 degrees to 19.4 degrees (P = 0.012). Sagittal balance changed in the hybrid group from -21.2 mm to 8.2 mm, and in the all-screw group changed from -28.8 mm to 1.5 mm. The major curve in the hybrid group improved from 54.06 degrees to 20.25 degrees and improved from 54.88 degrees to 15.06 degrees in the all-screw group., Conclusions: There was no statistically significant difference comparing the 2 groups, although a trend was observed toward better correction of the main thoracic curve in the all-screw construct group. The all-screw group demonstrated a significant decrease in kyphosis, which was not seen in the hybrid group. Hybrid constructs were comparable to all-screw constructs in the correction of coronal plane deformity and sagittal balance.

Background: Controversy exists regarding the optimal fellowship training experience for surgeons who perform scoliosis surgery in pediatric patients. While many studies have demonstrated that higher surgical volumes are associated with superior outcomes, the volume of scoliosis procedures performed by pediatric orthopaedic-trained surgeons as opposed to spine surgery-trained surgeons has not been reported., Methods: Validated, statewide hospital discharge databases from the states of New York and California were utilized to examine the volume of spinal fusion procedures performed for the treatment of scoliosis in patients who were eighteen years of age or less. Fellowship training of surgeons in New York who had performed more than fifty procedures from 1992 to 2001 (that is, more than five procedures per year) was determined, and the operative volumes of surgeons who had received pediatric orthopaedic as opposed to spine fellowship training were compared. Hospitals in California with either type of fellowship program were identified, and the operative volumes of hospitals and fellows with pediatric orthopaedic or spine fellowship training from 1995 to 1999 were compared., Results: Among the 228 surgeons in New York who had performed one or more spinal fusion procedures in patients eighteen years of age or less from 1992 to 2001, only 13% (thirty) had performed more than five procedures per year. However, these thirty surgeons accounted for 75% (3858) of all 5136 procedures in this age-group. Surgeons who had completed a pediatric orthopaedic fellowship had performed a mean of 14.5 procedures per physician per year, whereas those who had completed a spine fellowship had performed a mean of 10.5 procedures per physician per year. Surgeons who had not completed either type of fellowship had performed a mean of 14.4 procedures per physician per year. In California, the mean annual volume of scoliosis procedures from 1995 to 1999 was 59.0 procedures per year at hospitals with pediatric orthopaedic fellowship programs and 15.7 procedures per year at those with spine surgery programs. The mean number of procedures per fellow at hospitals with pediatric orthopaedic fellowship programs was 31.6 procedures per fellow per year, and the mean number at hospitals with spine surgery programs was 12.7 procedures per fellow per year. Over time, there was a significant increase in the number of procedures per year at hospitals with both types of fellowship programs, but the percentage increase was greater for hospitals with pediatric orthopaedic fellowship programs than for hospitals with spine surgery fellowship programs (45.2% compared with 13.5%)., Conclusions: These data indicate that, on the average, a large number of surgeons in New York performed five scoliosis procedures per year or fewer. Among higher-volume surgeons in New York, those with pediatric orthopaedic fellowship training performed more scoliosis procedures on children and adolescents than those with orthopaedic spine training did. In California, the volume of scoliosis procedures at hospitals with pediatric orthopaedic fellowship programs was nearly four times greater than that at hospitals with spine fellowship programs and the volume of procedures per fellow was more than two times greater, and this disparity is widening over time. These data are an important element in establishing what type of fellowship best prepares surgeons for scoliosis surgery.

This retrospective study was undertaken to determine the effectiveness and cost of thoracic pedicle screws versus laminar and pedicle hooks in patients undergoing surgical correction of adolescent idiopathic scoliosis (AIS). Immediate preoperative and 6-week postoperative radiographs were examined in 25 consecutive cases of children with AIS who were divided into two groups, those with thoracic pedicle screw constructs and those with thoracic hook constructs. Endpoints collected included radiographic measures, complications, surgical time, implant cost, and quality-of-life measures. Ten children underwent spinal fusion using thoracic pedicle screw fixation and 15 underwent thoracic constructs composed of hooks. Similar sex and age distribution were noted in both groups, and among the 20 girls and 5 boys the average age was 14.5. The mean preoperative Cobb angle was 53.5 degrees for the screw group and 52.5 degrees for the hook group. Correction averaged 70.2% for the screw group and 68.1% for the hook group. There were no significant differences between the two patient groups in terms of percentage of or absolute curve change after surgery. The apical vertebral translation, end vertebral tilt angle, and coronal balance did not differ significantly between the two patient groups. Comparison of operative time and quality of life revealed no significant differences. Screw constructs were significantly more expensive than hook constructs. The correction obtained from thoracic pedicle screw fixation is comparable to traditional hook constructs in AIS. Surgery using either construct effectively corrects AIS.

While volume/outcomes relationships have been shown for several areas of orthopaedics, previous studies have not examined this relationship in the area of scoliosis surgery. The Office of Statewide Planning and Development (OSHPD) California inpatient discharge database was used for a retrospective review of all patients 25 years of age or younger with a diagnosis of scoliosis and a spinal fusion procedure from 1995 to 1999 (n = 3,606). Univariate and multivariate analyses were conducted to determine the effect of various factors on in-hospital mortality, surgical complications, reoperations, and length of stay (LOS). Univariate analyses revealed significant effects of age, sex, illness severity, neuromuscular disease, surgical approach, Medicaid status, and annual hospital volume on outcomes (P < 0.05). After controlling for these factors using multivariate regression, patients insured by Medicaid were found to have a significantly greater odds for complications (P = 0.017) and a significantly increased LOS (P < 0.001) compared with patients with all other sources of payment. Additionally, multivariate regression revealed an inverse relationship between annual hospital volume and likelihood of reoperation, as patients treated at hospitals with annual volumes of 5.1 to 25.0, 25.1 to 50.0, and greater than 50.0 spinal fusions all had approximately half the odds of reoperation (P = 0.042, P = 0.004, and P = 0.028 respectively) as patients treated at hospitals with an annual volume of 5.0 or fewer spinal fusions per year. The current data suggest that being insured with Medicaid in the state of California is associated with poorer outcomes after scoliosis surgery. Additionally, this study documents a volume/outcomes relationship in scoliosis surgery.

Background Context: There is considerable controversy regarding the effectiveness of bracing for patients with adolescent idiopathic scoliosis (AIS). Furthermore, little work has focused on the effect of formal bracing on the health-related quality of life (QOL) of affected adolescents., Purpose: This study is an attempt to assess the QOL of adolescents with AIS who were treated with a brace in comparison to those who were not., Study Design/setting: This is a questionnaire-based study of patients presenting to our institution for evaluation and treatment of AIS. The study was conducted at Columbia University and the New York Presbyterian Medical Center, New York, NY., Patient Sample: A total of 136 patients with AIS who were being observed and 78 patients who were braced were included in this study., Outcome Measures: The Child Health Questionnaire (CHQ) and the American Academy of Orthopaedic Surgeons Pediatric Outcomes Data Collection Instrument (PODCI) were administered to 214 parents of adolescents undergoing evaluation for AIS., Methods: Parents of AIS patients were administered the CHQ and PODCI to assess their child's QOL. Independent samples t tests were conducted to compare the QOL among braced versus observed patients. Univariate and multivariate analyses were conducted to determine the effect of gender, age, curvature and treatment type on QOL., Results: There were few differences in QOL between braced versus observed patients, but boys tended to outscore the girls across most domains. Surprisingly, the QOL of adolescents undergoing bracing in this study was not significantly lower than age-adjusted norms., Conclusions: Brace wearing did not decrease the QOL of adolescents, compared with their observed counterparts, in our study population.

Unlabelled: A 34-mo-old boy with neurofibromatosis, scoliosis, and pectus excavatum developed severe hypotension when positioned prone. A magnetic resonance image study revealed neurofibromas encircling the great vessels. During the next anesthetic the patient was placed in the prone position on transverse bolsters and hypotension ensued again. A transesophageal echocardiogram (TEE) revealed compression of the right ventricle by the sternum. When the child was turned supine, the blood pressure returned to baseline. The patient was returned to the prone position, this time with bolsters placed longitudinally, without problem. This case supports a cardiac evaluation, possible intraoperative TEE, and avoidance of sternal pressure in patients with chest wall deformities requiring prone positioning., Implications: A child with neurofibromatosis, scoliosis, and a chest wall deformity presenting for spinal fusion developed severe hypotension while prone. This was due to compression of the heart by the sternum, not compression of the great vessels by neurofibromas. Sternal pressure in prone patients with chest wall deformities should be avoided. Unique management included the use of transesophageal echocardiography to determine the cause of the hypotension.

Background Context: Ketorolac Tromethamine (ketorolac) is a nonsteroidal anti-inflammatory drug (NSAID) with proven efficacy in decreasing postoperative pain in various surgical settings, including the treatment of spine deformities. However, some studies have raised questions regarding the potential side effects of this agent, such as increased bleeding and inhibition of bony fusion., Purpose: This study was conducted to determine whether there is any association between the use of ketorolac and postoperative complications in a group of children who underwent scoliosis surgery., Study Design/setting: This is a retrospective review of a group of children who underwent spinal fusion between 1989 to 1999 at our institution., Patient Sample: Data on a total of 208 children were analyzed in this study. Sixty received ketorolac and 148 did not., Outcome Measures: Postoperative transfusion and reoperation rates were the two main outcome measures of interest., Methods: A retrospective review of 208 children who underwent scoliosis surgery was conducted, with a focus on ketorolac use. Univariate analysis and logistic regression were used to quantify the determinants of postoperative complications., Results: Our analyses detected no significant differences in a broad range of socioclinical variables between the two patient groups, including age at surgery, gender, type of scoliosis, surgical approach, use of erythropoietin, levels of curvature and degree of curvature. Analysis of complication rates focusing on postoperative transfusion and revision surgery showed that there were no significant differences between the two groups., Conclusions: In this retrospective study of 208 children undergoing spine surgery, postoperative use of ketorolac did not significantly increase complications, including transfusion and reoperation.

Background Context: The risks and costs of transfusion are a great concern in the area of pediatric spine surgery, because it is a blood-intensive procedure with a high risk for transfusion. Therefore, determining the predictors of transfusion in this patient population is an important first step and has the potential to improve upon the current approaches to reducing transfusion rates. In this study, we reveal several predictors of transfusion in a pediatric patient population undergoing spine surgery. In turn, we present a general rule of thumb ("rule of two's") for gauging transfusion risk, thus enhancing the surgeon's approach to avoiding transfusion in certain clinical scenarios., Purpose: This study was conducted to determine the main factors of transfusion in a population of pediatric patients undergoing scoliosis surgery. The goal was to present an algorithm for quantifying the true risk of transfusion for various patient groups that would highlight patients "at high risk" for transfusion. This is especially important in light of the various risks associated with undergoing a transfusion, as well as the costs involved in maintaining and disposing of exogenous blood materials., Study Design/setting: This is a retrospective review of a group of children who underwent scoliosis surgery between 1988 and 1995 at an academic institution., Patient Sample: A total of 290 patients were analyzed in this study, of which 63 were transfused and 227 were not., Outcome Measures: No outcomes measures were used in this study., Methods: A retrospective review of 290 patients presenting to our institution for scoliosis surgery was conducted, with a focus on socioclinical data related to transfusion risk. Univariate analysis and logistic regression were used to quantify the determinants of transfusion risk., Results: Univariate analysis identified many factors that were associated with the risk of transfusion. However, it is clear that several of these factors are dependent on each other, obscuring the true issues driving transfusion need. We used multivariate analysis to control for the various univariate predictors of transfusion. Our logistic regression model suggested that the type of scoliosis (odds ratio [OR], 2.02; 95% confidence interval [CI], 1.07 to 3.82), degree of curvature (OR, 1.012/degree curve; 95% CI, 1.01 to 1.03), and use of erythropoietin (OR, 0.29; 95% CI, 0.14 to 0.62) were the main determinants of transfusion risk for our population., Conclusions: The main risk factors of transfusion were used to formulate a simple algorithm, which can be used to quantify transfusion risk and to guide efforts to avoid transfusion in children undergoing spinal surgery. Given a 10% baseline risk for transfusion, our "rule of two's" indicates that each risk factor approximately doubles the chance of transfusion, whereas the administration of recombinant human erythropoietin roughly halves the risk of transfusion.