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Background: 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.Methods: 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.Results: 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.Conclusions: 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.Level Of Evidence: Prognostic Level II. See Instructions for Authors for a complete description of levels of evidence. [ABSTRACT FROM AUTHOR]

Background: Arthrogryposis multiplex congenita (AMC) is a condition that describes neonates born with ≥2 distinct congenital contractures. Despite spinal deformity in 3% to 69% of patients, inadequate data exist on growth-friendly instrumentation (GFI) in AMC. Our study objectives were to describe current GFI trends in children with AMC and early-onset scoliosis (EOS) and to compare long-term outcomes with a matched idiopathic EOS (IEOS) cohort to determine whether spinal rigidity or extremity contractures influenced outcomes.Methods: Children with AMC and spinal deformity of ≥30° who were treated with GFI for ≥24 months were identified from a multicenter EOS database (1993 to 2017). Propensity scoring matched 35 patients with AMC to 112 patients with IEOS with regard to age, sex, construct, and curve. Multivariable linear mixed modeling compared changes in spinal deformity and the 24-item Early Onset Scoliosis Questionnaire (EOSQ-24) across cohorts. Cohort complications and reoperations were analyzed using multivariable Poisson regression.Results: Preoperatively, groups did not differ with regard to age (p = 0.87), sex (p = 0.96), construct (p = 0.62), rate of nonoperative treatment (p = 0.54), and major coronal curve magnitude (p = 0.96). After the index GFI, patients with AMC had reduced percentage of coronal correction (35% compared with 44%; p = 0.01), larger residual coronal curves (49° compared with 42°; p = 0.03), and comparable percentage of kyphosis correction (17% compared with 21%; p = 0.52). In GFI graduates (n = 81), final coronal curve magnitude (55° compared with 43°; p = 0.22) and final sagittal curve magnitude (47° compared with 47°; p = 0.45) were not significantly different at the latest follow-up after definitive surgery. The patients with AMC had reduced T1-S1 length (p < 0.001), comparable T1-S1 growth velocity (0.66 compared with 0.85 mm/month; p = 0.05), and poorer EOSQ-24 scores at the time of the latest follow-up (64 compared with 83 points; p < 0.001). After adjusting for ambulatory status and GFI duration, patients with AMC developed 51% more complications (incidence rate ratio, 1.51 [95% confidence interval (CI), 1.11 to 2.04]; p = 0.009) and 0.2 more complications/year (95% CI, 0.02 to 0.33 more; p = 0.03) compared with patients with IEOS.Conclusions: Patients with AMC and EOS experienced less initial deformity correction after the index surgical procedure, but final GFI curve magnitudes and total T1-S1 growth during active treatment were statistically and clinically comparable with IEOS. Nonambulatory patients with AMC with longer GFI treatment durations developed the most complications. Multidisciplinary perioperative management is necessary to optimize GFI and to improve quality of life in this complex population.Level Of Evidence: Therapeutic Level III. See Instructions for Authors for a complete description of levels of evidence. [ABSTRACT FROM AUTHOR]

Background: Although there is a high rate of reoperation after final fusion following the treatment of early-onset scoliosis with use of traditional growing rods, the risk factors for reoperation are unknown. The purpose of the present study was to identify risk factors associated with the need for reoperation after final fusion for the treatment of early-onset scoliosis.Methods: A multicenter database for patients with early-onset scoliosis was retrospectively analyzed. Patients managed with traditional growing rods and final fusion were identified (n = 248). The inclusion criteria were ≥1 lengthening procedure with traditional growing rods and ≥2 years of follow-up after final fusion or revision surgery within 2 years after final fusion (167 patients; 67%). Patients requiring reoperation following final fusion were compared with patients who did not require reoperation. The data that were analyzed included demographic characteristics, comorbidities, spinal deformity characteristics, radiographic measurements, perioperative details, and complications during all stages of treatment. A multivariate regression model was used to identify independent risk factors.Results: The mean duration of follow-up from the initial visit to the latest visit was 10.7 ± 4.1 years, and the mean duration of follow-up after final fusion was 4.9 ± 3.1 years. Thirty-two (19%) of the 167 patients required reoperation following final fusion. Curve progression requiring revision surgery during lengthening with traditional growing rods (adjusted odds ratio [aOR], 21.137 per event; p = 0.028), the number of levels spanned with traditional growing rods (aOR, 1.378 per level; p = 0.007), and the duration of treatment with traditional growing rods (aOR, 1.220 per year; p = 0.035) were independently associated with revision surgery after final fusion.Conclusions: Independent risk factors for curve progression requiring reoperation during lengthening with traditional growing rods that require operative intervention include increasing number of levels spanned with traditional growing rods and longer duration of treatment with traditional growing rods. These findings may help with patient counseling and potentially guide surgeon decision-making.Level Of Evidence: Prognostic Level IV. See Instructions for Authors for a complete description of levels of evidence. [ABSTRACT FROM AUTHOR]

Purpose: Using patient-reported outcome measures (PROMs), this study was undertaken to determine how well patients with early onset scoliosis (EOS) fare in adulthood., Methods: Among eight healthcare centers, 272 patients (≥ 18 years) surgically managed for EOS (≥ 5 years) completed the Scoliosis Research Society (SRS)-22r, Functional Assessment of Chronic Illness Therapy-10 (FACIT-Dyspnea-10), and Short Form (SF)-12. Functional and demographic data were collected., Results: The response rate was 40% (108/272). EOS etiologies were congenital (45%), neuromuscular (20%), idiopathic (20%) syndromic (11%), and unknown (4%). All patients scored within normal limits on the FACIT-Dyspnea-10 pulmonary (no breathing aids, 78%; no oxygen, 92%). SF-12 physical health scores and most SRS-22r domains were significantly decreased (p < 0.05 and p < 0.001, respectively) compared with normative values. SF-12 and SRS-22r mental health scores (MHS) were lower than normative values (p < 0.05 and p < 0.02, respectively). Physical health PROMs varied between etiologies. Treatment varied by etiology. Patients with congenital EOS were half as likely to undergo definitive fusion. There was no difference between EOS etiologies in SF-12 MHS, with t scores being slightly lower than normative peers., Conclusion: Good long-term physical and social function and patient-reported quality of life were noted in surgically managed patients. Patients with idiopathic EOS physically outperformed those with other etiologies in objective and PROM categories but had similar MHS PROMs. Compared to normative values, EOS patients demonstrated decreased long-term physical capacity, slightly lower MHS, and preserved cardiopulmonary function., Level of Evidence: Level IV Case Series., (© 2024. The Author(s).)

Purpose: Scoliosis can be treated with vertebral body tethering (VBT) as a motion-sparing procedure. However, the knowledge of how growth is affected by a tether spanning multiple levels is unclear in the literature. Three-dimensional true spine length (3D-TSL) is a validated assessment technique that accounts for the shape of the spine in both the coronal and sagittal planes. This study aimed to assess if 3D-TSL increases over a five-year period after VBT implantation in thoracic curves for idiopathic scoliosis., Methods: Prospectively collected radiographic data from an international pediatric spine registry was analyzed. Complete radiographic data over three visits (post-operative, 2 years, and 5 years) was available for 53 patients who underwent VBT., Results: The mean age at instrumentation of this cohort was 12.2 (9-15) years. The average number of vertebrae instrumented was 7.3 (SD 0.7). Maximum Cobb angles were 50° pre-op, which improved to 26° post-op (p < 0.001) and was maintained at 5 years (30°; p = 0.543). Instrumented Cobb angle was 22° at 5 years (p < 0.001 vs 5-year maximum Cobb angle). An accentuation was seen in global kyphosis from 29° pre-operative to 41° at 5 years (p < 0.05). The global spine length (T1-S1 3D-TSL) started at 40.6 cm; measured 42.8 cm at 2 years; and 44.0 cm at the final visit (all p < 0.05). At 5 years, patients reached an average T1-S1 length that is comparable to a normal population at maturity. Immediate mean post-operative instrumented 3D-TSL (top of UIV-top of LIV) was 13.8 cm two-year length was 14.3 cm; and five-year length was 14.6 cm (all p < 0.05). The mean growth of 0.09 cm per instrumented level at 2 years was approximately 50% of normal thoracic growth. Patients who grew more than 0.5 cm at 2 years had a significantly lower BMI (17.0 vs 19.0, p < 0.05) and smaller pre-operative scoliosis (48° vs 53°, p < 0.05). Other subgroup analyses were not significant for age, skeletal maturity, Cobb angles or number of spanned vertebras as contributing factors., Conclusions: This series demonstrates that 3D-TSL increased significantly over the thoracic instrumented levels after VBT surgery for idiopathic scoliosis. This represented approximately 50% of expected normal thoracic growth over 2 years., (© 2024. The Author(s), under exclusive licence to Scoliosis Research Society.)

Purpose: Large registries are increasingly at the forefront of modern pediatric spine research, with manual, centralized, trained radiographic measurement serving as the gold standard for spine research. However, there is limited data regarding the reliability of registry measurements which may be subject to differences in radiographic calibration. We undertook this study to evaluate reliability of T1-T12 height, L1-S1 height, and coronal balance measurements for a large registry of early onset scoliosis patients., Methods: Three trained technicians from the Pediatric Spine Study Group measured 43 radiographs for T1-T12, L1-S1, and coronal balance using 3 different calibration techniques. All radiographs were AP views of patients with magnetically controlled growing rods with known diameters. The calibration techniques used a pre-export manually drawn line, a digital automatically generated computerized marker, and the diameter of a magnetically controlled growing rod. The intraclass correlation coefficient (ICC) was calculated to determine reliability., Results: 1161 measurements were performed. For each of the three raters, coronal balance, T1-T12 height and L1-S1 height had excellent agreement regardless of the calibration technique (alpha 0.93-1.0). Among the parameters, coronal balance had the worst inter-rater reliability, whereas there was excellent interrater reliability regarding T1-T12 height and L1-S1 height (alpha 0.91-0.99)., Conclusion: There was excellent agreement among reviewers and between the 3 different calibration techniques. While calibration using rod diameter served as the gold standard, this data shows that other standard calibration methods were adequate and achieved excellent reliability for registry radiographs., (© 2023. The Author(s), under exclusive licence to Scoliosis Research Society.)

Purpose: Vertebral body tethering (VBT) has been described for patients with idiopathic scoliosis. Results of the technique for non-idiopathic scoliosis have not yet been reported., Methods: An international multicenter registry was retrospectively queried for non-idiopathic scoliosis patients who underwent VBT with minimum 2-year follow-up. Success at 2 years was defined as Cobb angle < 35 degrees and no fusion surgery., Results: Of the 251 patients treated with VBT, 20 had non-idiopathic scoliosis and minimum 2-year follow-up. Mean age at surgery was 12.4 years (range 10 to 17 years). Mean major Cobb angle at enrollment was 56 degrees. Of those, 18 patients had a major thoracic curve and two had a major lumbar curve. Of the 20 patients, nine met criteria for success (45%). Eight of the 20 patients had poor outcomes (four fusions, four with curve > 50 degrees). Success was associated with smaller preoperative Cobb angle (50 vs. 62 degrees, p = 0.01) and smaller Cobb angle on initial postop imaging (28 degrees vs. 46 degrees, p = 0.0007). All patients with Cobb angle < 35 degrees on 1st postop imaging had a successful result, with the exception of one patient who overcorrected and required fusion. Syndromic vs. neuromuscular patients had a higher likelihood of success (5 of 7, 71%, 2 of 10, 20%, p = 0.03)., Conclusion: Selected non-idiopathic scoliosis can be successfully treated with VBT, but failure rates are high and were associated with large curves, inadequate intraoperative correction and neuromuscular diagnosis. Achieving a Cobb angle less than 35 degrees on 1st standing radiograph was associated with a successful outcome which was achieved in 45% of patients., Level of Evidence: Level IV (retrospective review study)., (© 2022. The Author(s), under exclusive licence to Scoliosis Research Society.)

Purpose: To investigate the risk and predictive factors of junctional issues after conversion from Traditional growing rod (TGR) to definitive spinal fusion in Early-onset scoliosis (EOS)., Methods: Retrospective review of a multicenter EOS database. TGR patients who received final fusion with at least two-year follow-up were included. Proximal (PJA) and Distal junctional angles (DJA) on pre-final fusion, post-final fusion (within one year of surgery), and at latest follow-up were measured on lateral upright spinal radiographs. Differences in values among designated time points and predictive factors of junctional issues were evaluated statistically., Results: Forty-six of 251 patients (28 females, 18 males and mean age at final fusion: 12 ± 2 [9-17] years) met the inclusion criteria. Mean follow-up between first postoperative measurement and latest follow-up was 49 ± 22 (24-112) months. No statistical differences in PJA and DJA values were available at pre-fusion, first post-fusion, or latest follow-up (p = 0.827, p = 0.076). Fifty percent of patients had extension of TGR instrumentation at fusion, either proximal or distal. No factor including sex and etiology, lumbar lordosis, thoracic kyphosis, major curve magnitude, PJA, and DJA at pre-fusion was found to be a predictive issue for extension of index TGR instrumentation, except the history of at least one implant-related complication during the period from index surgery to the definitive fusion., Conclusion: PJA and DJA remained stable when transitioning from TGR to final posterior spinal fusion. But 50% of patients had extension of construction at fusion, either proximal or distal., (© 2021. The Author(s), under exclusive licence to Springer-Verlag GmbH Germany, part of Springer Nature.)

Purpose: Karol et al. introduced the concept that 18 cm thoracic height is the critical point where a patient with early onset scoliosis (EOS) can maintain adequate pulmonary function. Our purpose was to determine if distraction-based surgeries will increase thoracic spine height to at least 18 cm in patients with EOS., Methods: Patients with EOS treated with distraction-based systems (minimum 5 years follow up, minimum five lengthenings). Radiographic analysis of thoracic spine height (T1-T12) at the last lengthening procedure., Results: One hundred and fifty-three patients (67 congenital, 21 neuromuscular, 38 syndromic, 27 idiopathic) with pre-operative mean age 4.6 years, scoliosis 75°, kyphosis 47° were evaluated. Their mean age at final lengthening procedure was 11 years (6-16), average number of lengthening procedures was 10.5 (4-21), mean final scoliosis was 53°, and mean final kyphosis was 58°. Final thoracic height was > 18 cm in 65% and was > 22 cm in 31% of patients. Based on etiology, only 48% of the congenital patients reached 18 cm compared to 81% neuromuscular, 84% syndromic and 67% idiopathic. This height gain was closely related to the percentage of scoliosis correction achieved for each etiology. Comparing congenital etiology to other etiologies, there was a lower percentage of patients in the congenital group that passed the 18 cm threshold (48% vs. 78%) (p < 0.05)., Conclusion: At minimum 5 years follow up, distraction-based surgeries increased thoracic height for patients with EOS to greater than 18 cm in 65% of patients; however, only 48% of congenital patients reached this thoracic height threshold., Design: Retrospective review of prospectively collected registry data. LOI III.

Study Design: Retrospective, comparative, multicenter., Objectives: To determine if the choice of proximal anchor affects thoracic sagittal spine length (SSL) for children with idiopathic early-onset scoliosis (EOS). Debate exists as to whether spine growth is maintained during treatment for EOS. As rib- (RB) and spine-based (SB) distraction procedures may be kyphogenic, the traditional measurement of spine growth on coronal radiographs may not identify out-of-plane increase in spine length. A measure of SSL, along the spine's sagittal arc of curvature, has been validated to reliably assess the length of the thoracic spine., Methods: Patients with idiopathic EOS treated with distraction-based systems (minimum 5-year follow-up, five lengthening surgeries) with radiographic analysis preoperatively, postimplant (L1), and during lengthening periods (L2-L5, L6-L10) were evaluated with primary outcome of T1-T12 SSL., Results: We identified 34 patients (14 RB, 20 SB) with preoperative age 4.9 years (4.2 RB vs. 5.4 SB), scoliosis 72° (60° RB vs. 77° SB; p < 0.05), kyphosis 39° (50° RB vs. 34° SB; p < 0.05), and SSL 17.8 cm (15.5 RB vs. 18.5 SB; p < 0.05). After initial scoliosis correction from implantation, scoliosis remained constant over time. RB patients had greater kyphosis than SB patients: L1, 46° RB vs. 19° SB (p < 0.05); L2-L5, 50° RB vs. 27° SB (p < 0.05); L6-L10, 56° RB vs. 26° SB (p < 0.05). SSL increased for both groups from preoperative to the tenth lengthening (p < 0.05). As compared with RB patients, SB patients had higher SSL preoperatively and maintained this difference to the tenth lengthening (p < 0.05). After ten lengthening surgeries, when normalized to preoperative SSL, relative thoracic growth was greater for RB (27%) than for SB patients (19%) (p < 0.05)., Conclusion: Regardless of proximal anchor choice, thoracic length continued to increase during the distraction phase of treatment for idiopathic EOS., Level of Evidence: Level III.

Aims: The aim of this study was to compare the surgical and quality-of-life outcomes of children with skeletal dysplasia to those in children with idiopathic early-onset scoliosis (EOS) undergoing growth-friendly management., Patients and Methods: A retrospective review of two prospective multicentre EOS databases identified 33 children with skeletal dysplasia and EOS (major curve ≥ 30°) who were treated with growth-friendly instrumentation at younger than ten years of age, had a minimum two years of postoperative follow-up, and had undergone three or more lengthening procedures. From the same registries, 33 matched controls with idiopathic EOS were identified. A total of 20 children in both groups were treated with growing rods and 13 children were treated with vertical expandable prosthetic titanium rib (VEPTR) instrumentation., Results: Mean preoperative major curves were 76° (34° to 115°) in the skeletal dysplasia group and 75° (51° to 113°) in the idiopathic group (p = 0.55), which were corrected at final follow-up to 49° (13° to 113°) and 46° (12° to 112°; p = 0.68), respectively. T1-S1 height increased by a mean of 36 mm (0 to 105) in the skeletal dysplasia group and 38 mm (7 to 104) in the idiopathic group at the index surgery (p = 0.40), and by 21 mm (1 to 68) and 46 mm (7 to 157), respectively, during the distraction period (p = 0.0085). The skeletal dysplasia group had significantly worse scores in the physical function, daily living, financial impact, and parent satisfaction preoperatively, as well as on financial impact and child satisfaction at final follow-up, than the idiopathic group (all p < 0.05). The domains of the 24-Item Early-Onset Scoliosis Questionnaire (EOSQ24) remained at the same level from preoperative to final follow-up in the skeletal dysplasia group (all p > 0.10)., Conclusion: Children with skeletal dysplasia gained significantly less spinal growth during growth-friendly management of their EOS and their health-related quality of life was significantly lower both preoperatively and at final follow-up than in children with idiopathic EOS. Cite this article: Bone Joint J 2019;101-B:1563-1569.