Classification Of Early-Onset Scoliosis Research Articles

Comparison of two types of casting in early-onset scoliosis.

Early-onset scoliosis (EOS) can be a progressive and debilitating condition if left untreated. Different casting techniques have fallen in and out of favor over the years for conservative management. Two types of casting, elongation-derotation-flexion (EDF) and body casting (BC) are employed at our institution. Here we compare the radiographic outcomes between these two types of casting in a cohort of patients diagnosed with EOS. Sixteen children with EOS were treated by EDF serial casting while seventeen children with the same diagnosis were treated by BC. Radiographic measurements included Cobb angle, rib-vertebral-angle difference (RVAD) and vertebral rotation (VR) by Nash-Moe method in casting (IC) or out of casting (OOC), thoracic height (TH) and width (TW). All of the patients had x-ray measurements at pre-casting OOC, 1st IC and final post-casting OOC. Casts were changed every 2-4 months. Independent two sample t-test, Wilcoxon rank-sum test, and Chi-square test were performed. There were no significant differences at the initial treatment for age, classification of EOS, OOC, RVAD, VR, kyphosis, TH, and TW between EDF and BC casting. There were no significant differences of changes for OOC, RVAD, VR, kyphosis, TH and TW from pre-casting to the final post-casting status between two casting techniques (P>0.05). However, children with EDF tended to receive 3 to 4 more castings than those with BC (7.5 vs.4 casts) (P=0.007) and achieved better outcomes in success (25% vs.20%) and improvement (50% vs.10%) (P=0.03). EDF has better outcomes with EOS improvement when there is treatment of longer duration.

Effect of Etiology, Radiographic Severity, and Comorbidities on Baseline Parent-Reported Health Measures for Children with Early-Onset Scoliosis.

The Classification of Early-Onset Scoliosis (C-EOS) allows providers to differentiate patients, for clinical and research purposes, on the basis of the etiology of their disease as well as radiographic parameters. The Early Onset Scoliosis Questionnaire (EOSQ) is the first disease-specific, parent-reported HRQOL (health-related quality-of-life) outcome measure for this condition. We sought to determine the influence of the C-EOS etiology designation, radiographic parameters, and medical comorbidities on EOSQ scores to differentiate quality of life in this heterogeneous patient population. We hypothesized that baseline EOSQ scores for patients with EOS would be strongly affected by the C-EOS etiology designation. The analysis included prospectively enrolled patients with EOSQ scores recorded in a multicenter EOS database prior to intervention for the EOS. EOSQ scores were compared across C-EOS etiologies, severity of disease based on radiographic measurements, and patient comorbidities prior to scoliosis intervention. Six hundred and ten patients with EOS were available for analysis; 119 had congenital, 201 had idiopathic, 156 had neuromuscular, and 134 had syndromic EOS. In multivariate analysis, neuromuscular and syndromic etiologies were associated with lower scores than congenital and idiopathic etiologies in many EOSQ domains including general health, transfer, daily living, fatigue/energy level, and emotion. Patients with neuromuscular EOS had the lowest EOSQ scores in general. Congenital and idiopathic EOS did not differ from each other in any EOSQ domain. Coronal Cobb and kyphosis angles had significant inverse but generally weak correlations with EOSQ domains. Individual medical comorbidities had a minor effect on certain domains while American Society of Anesthesiologists (ASA) class and total number of comorbidities had inverse correlations with most domains. The underlying etiology of EOS appears to have a significant influence on the parent-reported HRQOL outcomes of the disease. Specifically, syndromic and neuromuscular C-EOS diagnoses are associated with lower EOSQ scores before treatment compared with congenital and idiopathic diagnoses. Radiographic measurements of severity have a relatively small influence on EOSQ scores. These baseline differences in C-EOS-designated etiology should be accounted for in studies comparing outcomes of treatment for this heterogeneous patient population. Prognostic Level II. See Instructions for Authors for a complete description of levels of evidence.

Correlation between surgical site infection and classification of early onset scoliosis (C-EOS) in patients managed by rib-based distraction instrumentation.

The aim of this study is to determine risk factors for infection among EOS patients treated by rib-based distraction instrumentation, and to further assess the incidence of infection among C-EOS categories and sub-types. Despite the heterogonous nature of early onset scoliosis, the classification of early onset scoliosis (C-EOS) has proven to have excellent reliability across its major categories. C-EOS's reliability has been verified; however, little data exist on the utility of this categorization in clinical decision-making and risk assessment. After institutional review board approval, data for EOS patients treated by rib-based distraction instrumentation were collected between 2013 and 2017 in a single institution. Data collection included: major categories of early onset scoliosis classification (etiology, major curve and kyphosis), BMI, height, weight, procedure type, site of procedure, presence of tracheostomy, and bowel/urinary incontinence. 156 EOS patients underwent 843 rib-based distraction instrumentation procedures. 22.4% of patients (35/156 patients, 42 procedures) developed infections, 30/35 requiring irrigation and debridement. Type of procedure was significantly associated with infection rate, with rib-based distraction instrumentation insertion corresponding with the highest incidence of infection, as compared to instrumentation revisions or expansions (p = 0.006). Infection rates were also more common in shorter and lighter weight children (p = 0.001 and 0.03; respectively). Patients with a neuromuscular etiology had the highest rate of infection in comparison to congenital, syndromic, and idiopathic (5.7% vs, 4.9%, 4.7%, and 2.6%; respectively). Notably, high infection rates occurred neuromuscular hyper-kyphotic subjects (M+), occurring in all major curve C-EOS subgroups and at a rate of 8.3% for all procedures. Neuromuscular, larger magnitude major curve, and larger magnitude kyphotic angle C-EOS categories appear to be at a higher risk of infection. Such information potentiates the usefulness of C-EOS in surgical decision-making and in the informed consent process. Level III therapeutic.

Reproducibility of the classification of early onset scoliosis (C-EOS).

Reproducibility study. Assess the agreement and reliability of the classification of early onset scoliosis (C-EOS). C-EOS is a promising tool for patients with early onset scoliosis (EOS). However, the reliability has only been examined without measuring radiographs and not including the annual progression rate (APR) modifier. We included a single-center consecutive cohort of patients diagnosed with EOS seen in our outpatient clinic. Patients had no previous spine surgery. Four raters rated 60 cases. Two anterior-posterior full-spine radiographs, taken minimum 6months apart, and one sagittal radiograph were measured twice by all raters in a blinded test-retest setup. Results were assessed using crude frequency of overall agreement (OA), intra- and inter-rater Fleiss kappa (κ) statistics, and intraclass correlation coefficient (ICC). We calculated the 95% limits of agreement (LOA) for major curve angle (MCA), kyphosis, and APR using a linear mixed-effects model. Inter- and intra-rater LOA were analyzed for each etiology separately. Mean age was 8.7 ± 3.4years and the etiology were congenital/structural (n = 20), idiopathic (n = 19), neuromuscular (n = 13), or syndromic (n = 8). For etiology, OA was 75.8% and κ = 0.80. For major curve angle, OA was 84.2%, κ = 0.86, ICC = 0.97, and LOA = 12.8°. For kyphosis, OA was 55.8%, κ = 0.52, ICC = 0.87, and LOA = 20.6°. For APR, OA was 76.7%, κ = 0.61, ICC = 0.77, and LOA = 17.4°/year. Inter- and intra-rater LOA were generally largest for neuromuscular and smallest for idiopathic patients. We found substantial agreement for etiology, however, with disagreement in certain cases. The reliability of MCA was excellent; however, somewhat lower for kyphosis and APR with less accuracy. The measurement errors of MCA, kyphosis, and APR depended largely on the etiology. Regarding APR, LOA exceeded the 10°/year increments proposed in the C-EOS, suggesting a revision of this optional modifier. Diagnostic study level 1.

Validation of the Early Onset Scoliosis Questionnaire (EOSQ) as applied to the Classification of Early Onset Scoliosis (C-EOS) Etiology Designation Before Scoliosis Treatment

Validation of the Early Onset Scoliosis Questionnaire (EOSQ) as applied to the Classification of Early Onset Scoliosis (C-EOS) Etiology Designation Before Scoliosis Treatment

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

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

Magnetically Controlled Growing Rods in Treatment of Early-Onset Scoliosis: A Single Center Study With a Minimum of 2-Year-Follow up and Preliminary Results After Converting Surgery.

Case series. To evaluate complications and radiographic parameters after magnetically controlled growing rod (MCGR) index surgery (IS), during lengthening and following converting surgery (CS) with a minimum of 2-year follow up (FU). MCGR are maintaining skeletal growth in treatment of early onset scoliosis (EOS). There is no data regarding correction potential after CS available. Twenty-four cases were included. Two patients with rib and pelvic hook fixation instead of pedicle screws and three patients with previous spinal surgery were excluded from radiographic analysis. Twenty-one patients received grade 3 or 4 in Classification of Early-Onset-Scoliosis (C-EOS) for main curve severity. The kyphotic modifiers (-) were given to seven and (+) to seven patients. Mean age at IS was 10.5 ± 2.4 years with a mean FU time of 42.3 ± 11.3 months. Deformity correction was only achieved during IS (46%) and CS (36%). During MCGR treatment a 5° loss of correction seen, while 25 mm of T1-S1 length was gained during the lengthening period. An overall average lengthening of 1.6 mm per lengthening procedure was achieved. Possibility to gain length during distractions decreases over time. No major failure of the distraction mechanism was observed, only 16 lengthening procedures failed within a total of 264 lengthening procedures. A total of 19 revision surgeries in 10 patients were observed. Four patients received more than one revision surgery. Applying MCGR results in a revision rate of 0.23 per patient and per one FU year, while making further lengthening procedures obsolete compared with conventional growing rod techniques. Correction of major curve is possible during IS und CS. The law of diminishing returns applies during the period of lengthening. 4.

COMPLICATIONS IN SURGERY OF EARLY-ONSET SCOLIOSIS WITH VEPTR INSTRUMENTATION: PRELIMINARY REPORT

Objective. To analyze complications developing during multistage correction of early severe spinal deformities of various etiologies using VEPTR instrumentation. Materials and Methods. The study included prospective group of 76 patients treated for early-onset scoliosis (EOS) with the VEPTR and VEPTR II instrumentation. Patients were classified using a randomized Classification of Early-Onset Scoliosis (C-EOS) system. In accordance with scoliosis etiology the cases were distributed as follows: 28 idiopathic, 26 congenital, 20 syndromic, and 2 neuromuscular scoliosis cases; and ratio of boys to girls was 32 : 44. The average age at the beginning of treatment was 5.4 ± 2.1 (1.6 to 9.8) years. The average follow-up period since the first stage was 2.2 (2 to 2.5) years. Results. Twenty three complications were revealed in 15 (19.7 %) patients. According to the C-EOS system, the patients with complications were referred to normo- and hyperkyphotic groups. The most common complication was migration of the cranial point of implant fixation. Most complications were eliminated during the planned stage of deformity correction. In two cases, the complication resulted in cessation of the VEPTR technique using. Conclusions. The use of VEPTR instrumentation for the treatment of EOS is associated with a rather high risk of complications. This is most likely in patients with normokyphotic and hyperkyphotic deformities. Most complications are eliminated during stage correction. The need to abandon the further use of the VEPTR instrumentation is rare.

Spine and Thoracic Height Measurements Have Excellent Interrater and Intrarater Reliability in Patients With Early Onset Scoliosis.

Reproducibility of measurements. This study investigates the reliability and standard error of measurement of spine and thoracic height radiographic measurements in patients with early onset scoliosis (EOS). Spine and thoracic height radiographic measurements are often used as a surrogate for pulmonary development in patients with EOS. There is limited literature validating the reliability of spine and thoracic height measurements in the EOS population. Using pilot data, we determined measuring 49 unique radiographs would provide 80% power to obtain a 95% confidence interval (CI) width of 0.05 for the interclass correlation coefficients (ICCs). A random sampling strategy, stratified by underlying diagnosis from the Classification of Early Onset Scoliosis (C-EOS), was used to distribute the diagnoses in the study sample. Two attending pediatric spine surgeons, two pediatric orthopedic fellows, and two research assistants measured coronal spine (T1-S1) and thoracic (T1-T12) height on digital radiographs using imaging software (Surgimap; Nemaris, Inc, New York) on two separate occasions at least 3 weeks apart. Order of images was randomized for the second iteration. Linear mixed model regression analyses were used to estimate interrater and intrarater reliability. The study sample included subjects (N = 48) with idiopathic (N = 17, 35%), congenital (N = 16, 33%, 1 patient excluded), neuromuscular (N = 11, 23%), and syndromic (N = 4, 8%) scoliosis. Overall interrater reliability estimates for spine height (ICC: 0.894, 95% CI: 0.847-0.932) and thoracic height (ICC: 0.890, 95% CI: 0.844-0.929) were excellent. Intrarater reliability estimates for spine height (ICC: 0.906, 95% CI: 0.830-0.943) and thoracic height (ICC: 0.898, 95% CI: 0.817-0.938) were also excellent. There is excellent interrater and intrarater reliability for radiographic measurements of spine and thoracic height in the EOS population at our institution. 2.

Quantifying Anesthesia Exposure in Growing Rod Treatment for Early Onset Scoliosis.

Growing rod (GR) treatment for early-onset scoliosis requires repeated anesthesia exposure (AE). At a minimum, GR treatment requires AE for diagnostic imaging, index GR surgery, periodic lengthenings, and final fusion. Adjunct procedures and complication-related procedures also increase AE. To our knowledge, this is the first study to quantify AE in GR treatment and to establish preoperative expectations. A single-center retrospective review of 16 patients who completed GR treatment and underwent final fusion. Duration of all AE related to GR treatment for "standard" care procedures (ie, advanced imaging, index surgery, lengthenings, final fusion) and "associated" care procedures (ie, revisions, adjunctive surgical procedures, wound-related complications) were reviewed. Etiologies were classified per the classification of early-onset scoliosis. Mean total anesthesia time (TAT) was tallied and analyzed for standard care and associated care procedures. There were 5 syndromic, 8 neuromuscular, and 3 idiopathic patients. The mean age at the first AE event related to GR treatment was 7.4 years (range, 3.8 to 11 y). Mean age at the index GR surgery and final fusion was 8.1 years (range, 3.9 to 14.4 y) and 12.8 years (range, 9.7 to 19 y), respectively. The percentage of TAT for each procedural category was 7% for advanced imaging, 14% for index GR, 14% for lengthenings, 21% for final fusion, 27% for revisions, 9% for adjunct surgery, and 9% for wound complications. Standard care procedures accounted for 55% of TAT, whereas associated care procedures accounted for 45%. This study quantified expected duration of AE in GR treatment. Revisions and final fusion contributed most to TAT. Given the recent controversy of repeated AE in young children, efficiency measures should be implemented to reduce AE and avoid duplication without compromising the goals of surgical treatment. Associated care procedures accounted for 45% of the total AE. Level IV.

Classification of Early Onset Scoliosis (C-EOS) and Pulmonary Function Outcomes

Etiology of early onset scoliosis as described by C-EOS does not predict pulmonary function. Further study is required to provide granularity with regard to specific C-EOS classifications.

Classification of Early Onset Scoliosis has Excellent Interobserver and Intraobserver Reliability.

Classification systems can be useful tools for clinical care and research but must be proven as reliable. The purpose of this study was to evaluate the interobserver and intraobserver reliability of the Classification of Early Onset Scoliosis (C-EOS) scheme. After IRB approval, 50 cases were drawn from a prospectively collected database of patients with EOS. Cases were selected using a stratified randomization scheme based on etiology. These cases were used to create an internet survey that was sent to pediatric orthopaedic faculty, research coordinators, and fellows involved in EOS care and research. Participants were asked to classify each case and were provided with a written C-EOS scheme that could be referenced while they completed the survey. Surveys were sent to participants twice, 3 weeks apart, to assess both intraobserver and interobserver reliability. Fleiss κ and Cohen κ were used to assess interobserver and intraobserver reliability, respectively. There were 36 total participants, 29 who completed the survey twice (21 faculty, 13 research coordinators, and 2 fellows). Overall Fleiss κ coefficient for interobserver reliability was excellent across the major categories of etiology (0.84), major curve (0.93), and kyphosis (0.96). Overall intraobserver reliability was excellent with Cohen κ values for etiology (0.92), major curve (0.96), and kyphosis (0.98). Faculty members had excellent agreement for etiology (0.90), major curve (0.91), and kyphosis (0.96). Research coordinators had high levels of agreement for etiology (0.78), and excellent for major curve (0.95) and kyphosis (0.96). Intraobserver reliability was excellent across all major categories for all groups: faculty, research coordinators, and fellows. The study shows high levels of interobserver and intraobserver agreement of the C-EOS scheme. The C-EOS scheme can be used as a reliable tool for classifying EOS patients for clinical communication and research. Level II-diagnostic study.

Growing rods in early-onset scoliosis

We present a case of a currently 11-year-old patient with early-onset scoliosis. (Fig. 1) At the age of 6 years, the diagnosis neurofibromatosis was confirmed via skin biopsy. Because of progressive deformity at the age of 8, the patient underwent surgery in 2012 with implantation of two magnetically controlled growing rods (MAGEC, Magnetic Expansion Control, Ellipse, Ellipse Technologies Inc, Aliso Viejo, CA, USA), a spinal bracing and distraction system. This system was first published in 2012 [1]. In the past 3 years, the patient returned nine times to our outpatient clinic for distraction of the implant (2.0 cm on the left side and 2.2 cm on the right side) (Fig. 2). Following the Classification of Early-Onset Scoliosis [2], he was 8M3N on his first visit and 11M2NP on his last visit. His thoracic height (T1–T12) increased from 21.15 cm to 24.30 cm, the Cobb

Variability of Surgical Site Infection With VEPTR at Eight Centers: A Retrospective Cohort Analysis

Study DesignRetrospective review. ObjectivesTo describe clinical characteristics and infection rates in modern vertical expandable prosthetic titanium rib (VEPTR) surgery. Summary of Background DataPrior studies have demonstrated infection rates from 10% to 30% with VEPTR surgery. MethodsA retrospective query was done on an institutional review board–approved, multicenter prospectively collected database for patients implanted with VEPTR from 2007 to 2013 at eight sites. This identified 213 patients with appropriate data for analysis. Average follow-up was 4.1 years (range 1.7–6.3). Data collected included a Classification of Early-Onset Scoliosis (C-EOS) diagnosis, American Society of Anesthesiologists Physical Status (ASA-PS), major Cobb angle, construct type, clinical symptoms, and microbiology. The distribution of infection rates across all the study sites was compared. The exact p value was estimated by Monte Carlo simulation. ResultsOverall, 18% (38/213) of patients implanted with VEPTR developed infection requiring operative debridement. There were significantly different infection rates among the sites, ranging from 2.9% to 42.9% (p = .029). The average time to infection was 70 days (range 8–236) after the infecting procedure. The majority of infections were due to gram-positive bacteria (80%, 44/55), the most prevalent being methicillin-sensitive Staphylococcus aureus (45%, 25/55). There were 20 patients (53%, 20/38) with either partial or complete implant removal to resolve infection; however, only 3 of 38 (8%) of these resulted in abandonment of VEPTR treatment. There was no difference in infection rate across the primary C-EOS diagnosis categories (p = .21) or based on ASA score (p = .53). After controlling for study site, the odds ratio of an infection following an implant procedure versus an expansion was 2.8 (p = .002). There was no difference in the odds ratio of an infection between the other procedure types (implant, expansion, exchange/revision). ConclusionsThere were significant differences in infection rates between sites. The variability in infection rate indicates a need for guided efforts to standardize best practices for infection control in VEPTR surgery. Level of EvidenceIII, therapeutic study.

Paper #6 Classification of Early Onset Scoliosis Predicts Complications after Initiation of Growth Friendly Spine Surgery

Paper #6 Classification of Early Onset Scoliosis Predicts Complications after Initiation of Growth Friendly Spine Surgery