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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.)

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.

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.

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: 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.

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.

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.

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.

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.

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.

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.

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: 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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]