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

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

AIM To determine which patients with cerebral palsy (CP) should undergo genetic testing, we compared the rate of likely causative genetic variants from whole‐exome sequencing in individuals with and without environmental risk factors. METHOD Patients were part of a convenience and physician‐referred cohort recruited from a single medical center, and research whole‐exome sequencing was completed. Participants were evaluated for the following risk factors: extreme preterm birth, brain bleed or stroke, birth asphyxia, brain malformations, and intrauterine infection. RESULTS A total of 151 unrelated individuals with CP (81 females, 70 males; mean age 25y 7mo [SD 17y 5mo], range 3wks–72y) participated. Causative genetic variants were identified in 14 participants (9.3%). There was no significant difference in diagnostic rate between individuals with risk factors (10 out of 123; 8.1%) and those without (4 out of 28; 14.3%) (Fisher's exact p=0.3). INTERPRETATION While the rate of genetic diagnoses among individuals without risk factors was higher than those with risk factors, the difference was not statistically significant at this sample size. The identification of genetic diagnoses in over 8% of cases with risk factors suggests that these might confer susceptibility to environmental factors, and that further research should include individuals with risk factors. What this paper addsThere is no significant difference in diagnostic rate between individuals with and without risk factors.Genetic variants may confer susceptibility to environmental risk factors.Six causative variants were identified in genes not previously associated with cerebral palsy.Global developmental delay/intellectual disability is positively associated with a genetic etiology.Extreme preterm birth, stroke/brain hemorrhage, and older age are negatively associated with a genetic etiology. What this paper adds: There is no significant difference in diagnostic rate between individuals with and without risk factors.Genetic variants may confer susceptibility to environmental risk factors.Six causative variants were identified in genes not previously associated with cerebral palsy.Global developmental delay/intellectual disability is positively associated with a genetic etiology.Extreme preterm birth, stroke/brain hemorrhage, and older age are negatively associated with a genetic etiology. This original article is commented by MacLennan on page 1369 of this issue. [ABSTRACT FROM AUTHOR]

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

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

Abstract: 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. [Copyright &y& Elsevier]

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. [ABSTRACT FROM AUTHOR]

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. [ABSTRACT FROM AUTHOR]

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. [Copyright &y& Elsevier]