Idiopathic Early-onset Scoliosis Research Articles

PREVALENCE OF PJK AFTER ARTHRODESIS IN PATIENTS WITH NEUROMUSCULAR SCOLIOSIS IN THE SECOND POSTOPERATIVE YEAR

ABSTRACT Objective The primary objective of this study was to analyze the prevalence of PJK in patients with neuromuscular scoliosis submitted to posterior spinal arthrodesis with instrumentation. Proximal junctional kyphosis (PJK) is a frequent phenomenon that, due to its importance, began to be studied by several authors, who laid the foundations for the radiographic definition and possible risk factors for its occurrence after long spinal arthrodesis with instrumentation. Despite the large number of PJK studies, most were related to the occurrence of adolescent idiopathic scoliosis, adult deformity and early-onset scoliosis, with few being targeted to patients with congenital and neuromuscular scoliosis. Methods In this study, data from electronic medical records of patients with neuromuscular scoliosis who underwent posterior arthrodesis with instrumentation between the years 2014 and 2016 were analyzed. Information on age, gender, pathology and radiographic measurements were extracted from this sample at the 2nd and 24th postoperative months. Results A total of 39 patients with neuromuscular scoliosis were analyzed. The sample was predominantly male (58.87%) and the mean age was 14.05 years. PJK occurred in 18 patients during the two years following surgery, with a prevalence of 46.15%. The incidence of PJK in the 2nd and 24th postoperative months was 23.1% and 30%, respectively. Conclusions A prevalence of PJK of 46.15% was found in patients with neuromuscular scoliosis treated surgically with posterior instrumentation after two years of follow-up, as compared to previous results . Level of Evidence III; Cross-sectional observational study.

Diagnostic yield and clinical impact of exome sequencing in early-onset scoliosis (EOS)

BackgroundEarly-onset scoliosis (EOS), defined by an onset age of scoliosis less than 10 years, conveys significant health risk to affected children. Identification of the molecular aetiology underlying patients with EOS...

Growing-rod Graduates With Idiopathic Early-onset Scoliosis Have Comparable Exercise Tolerance to Patients With Surgically Treated Adolescent Idiopathic Scoliosis.

Growing-rod (GR) treatment is the current standard for progressive idiopathic early-onset scoliosis (I-EOS) in young children. Despite good radiographic outcomes, the impact of scoliosis treatment on pulmonary functions is not well-defined in this patient population. The aim of this study was to evaluate pulmonary functions and exercise tolerance in I-EOS patients graduated from GR treatment and to compare them with age-matched, surgically treated adolescent idiopathic scoliosis (AIS) patients and healthy controls. Eight GR graduates with I-EOS with pulmonary function tests and complete radiographic results were compared with a group of 9 thoracic AIS patients at least 2 years out from posterior fusion. Both groups were also compared with a set of 10 healthy individuals. All subjects underwent cardiopulmonary exercise testing and spirometry to evaluate pulmonary function. Age, sex, height, arm span, weight, residual deformity, and level of instrumentation in GR and AIS patients were similar. In the GR group, forced vital capacity % and forced expiratory volume in 1 second % values were reduced compared with the healthy controls and AIS group (P<0.001, <0.001 and 0.036, 0.046, respectively). Breathing reserve index at lactate threshold (BRILT) was higher in GR and AIS patients (P=0.001 and 0.002, respectively), and was similar between GR and AIS patients (P=0.916). Heart rate at lactate threshold was higher in GR and AIS groups compared with controls (P<0.001 and 0.001, respectively). AIS and GR patients demonstrated reduced pulmonary reserve and exercise tolerance compared with their peers with no spinal deformity. However, exercise tolerance of I-EOS patients treated with the GR method was similar to that of operated AIS patients. These results suggest a positive impact of GR treatment in children with I-EOS. Level III-cross-sectional comparative study.

What Is the Cost of a "Cast Holiday" in Treating Children With Early Onset Scoliosis (EOS) With Elongation Derotation Flexion (EDF, "Mehta") Casting?

Serial casting of children with early onset scoliosis (EOS) is an established treatment option. A break from cast treatment often called a "cast holiday," (CH) is often allowed by some centers, particularly over the summer months. The impact of CHs on treatment duration or outcome has not been examined. Institution review board approved retrospective review of children treated for EOS with elongation derotation flexion ("Mehta") casting at a children's hospital between 2001 and 2016 with a minimum of 2 years' follow-up. A CH was defined as a minimum of 4 weeks out of the cast, braced, or unbraced.The analysis was performed to determine the impact of a CH within the first 18 months of treatment. Separate analyses were performed for the entire cohort of children castedduring the study period, and then separately looking at idiopathic EOS in isolation. The impact of a CH was assessed in terms of the likelihood of achieving scoliosis <15 degrees at the final follow-up ("success"). Odds ratios were used to assess group differences between "success" ratios, and Student t tests assessed group differences for parametric data. Ninety children met inclusion and exclusion criteria, 31 of whom took a CH during the first 18 months of treatment (34%). This included 59 patients with idiopathic EOS (66%), 18 with syndromic EOS, 5 congenital, and 1 neuromuscular. There were no statistically significant differences between CH and no CH groups.Forty-four percent of the no CH group achieved scoliosis <15 degrees at final follow-up, as opposed to 13% of the CH group, an odds ratio of 5.3 for success without a CH. When limited to children with idiopathic EOS, 56% achieved success in the no CH, versus only 22% in the group that took a CH, an odds ratio of 4.4 for success with no CH. This study demonstrates that children treated for EOS with serial casting who take a CH within the first 18 months of treatment are less likely to achieve scoliosis <15 degrees than those who persist with treatment. Level III.

Thoracic mobility and its relation to pulmonary function and rib-cage deformity in patients with early onset idiopathic scoliosis: a long-term follow-up

Study designA group of adult patients with idiopathic scoliosis, diagnosed before the age of ten, at a mean of 26.5 years after treatment with either brace or surgery during childhood and adolescence attended a clinical follow-up.ObjectivesTo evaluate the relation between thoracic mobility, rib-cage deformity, and pulmonary function.Summary of background dataLong-term studies of pulmonary function in relation to thoracic mobility after treatment in this patient group have not been published.MethodsA total of 106 patients, 57 braced and 49 operated patients, attended the follow-up. We examined thoracic mobility (range of motion of the thoracic spine, thorax expansion, and breathing movements) and rib-cage deformity (curve size and trunk deformity) as well as pulmonary function, especially total lung capacity (TLC). Respiratory muscle strength was evaluated in a subgroup.ResultsThoracic range of motion was significantly less among the surgically treated patients compared with both the brace-treated and comparison group. Thorax expansion and breathing movements during maximal breathing were significantly reduced in the scoliotic patients compared with the reference values, with no significant differences between the treatment groups. The brace-treated group had better pulmonary function than the operated group, as measured by the TLC, forced vital capacity (FVC), and forced expiratory volume in one second (FEV1) (percentage of predicted values). The respiratory muscle strength was significantly lower only in the surgically treated patients when compared with reference values. The results of a multivariate analysis revealed that the strongest factors explaining TLC percentage of predicted were gender, brace model, and smoking habits.ConclusionsThoracic mobility was significantly reduced at mean 26.5 years after completed treatment in both brace-treated and surgically treated patients with early onset scoliosis, compared with the reference values, which did not influence TLC as strongly as gender, brace model, and smoking habits.Levels of evidenceLevel III.

Distraction-based surgeries increase thoracic sagittal spine length after ten lengthening surgeries for patients with idiopathic early-onset scoliosis

Retrospective, comparative, multicenter. 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. 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. We identified 34 patients (14 RB, 20 SB) with preoperative age 4.9years (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 17.8cm (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). Regardless of proximal anchor choice, thoracic length continued to increase during the distraction phase of treatment for idiopathic EOS. Level III.

CHD7 gene polymorphisms in female patients with idiopathic scoliosis

BackgroundThe CHD7 (chromosome domain helicase DNA binding protein 7) gene has been associated with familial idiopathic scoliosis (IS) in families of European descent. The CHD7 single-nucleotide polymorphisms have never been studied in Polish Caucasian IS patients.MethodsThe aim of this study was to investigate the relationship of CHD7 gene polymorphisms with susceptibility to or progression of IS in Polish Caucasian females. The study group comprised 211 females who underwent clinical, radiological and genetic examination. The study group was analyzed in three subgroups according to: (1) Cobb angle (Cobb angle ≤30° vs. Cobb angle ≥35°), (2) age of diagnosis (adolescent IS vs. early-onset IS) and (3) rate of progression (non-progressive vs. slowly progressive vs. rapidly progressive IS). The control group comprised 83 females with no scoliosis and with a negative family history who underwent clinical and genetic examination. In total six CHD7 gene polymorphisms were examined. Three polymorphisms (rs1017861, rs13248429, and rs4738813) were examined by RFLP (restriction fragment length polymorphism) analysis, and three were quantified by Sanger sequencing (rs78874766, rs4738824, and rs74797613).ResultsIn rs13248429, rs78874766, and rs74797613 polymorphisms only the wild allele was present. The rs1017861 polymorphism demonstrated an association with IS susceptibility (p < 0.01). Two polymorphisms, rs1017861 and rs4738813, were associated with curve severity and progression rate (p < 0.05). None of the evaluated polymorphisms in CHD7 gene showed any association with the age of IS onset.ConclusionsThe polymorphism rs1017861 in CHD7 gene showed an association with IS susceptibility. Two polymorphisms (rs1017861 and rs4738813) were associated with curve severity and progression rate. None of the evaluated polymorphisms in CHD7 gene showed any association with the age of IS onset. Further evaluation of CHD7 gene should be considered as IS modifying factor.

Results of growth-friendly management of early-onset scoliosis in children with and without skeletal dysplasias: a matched comparison.

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. 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. 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). 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.

Serial casting in early onset scoliosis: syndromic scoliosis is no contraindication

BackgroundSerial casting is a treatment for early onset scoliosis (EOS) in young children to achieve curve correction before bracing or to postpone initial surgical treatment until the patient is older. Good results have been reported for patients with idiopathic early onset scoliosis (IS). However, there are few reports of results in non-idiopathic cases, and the benefits of non-surgical methods in the syndromic-associated early onset scoliosis subgroup are unknown.MethodsRetrospective single-institution study of patient charts and X-rays of all cases of sustained serial casting for EOS.Staged correction was obtained by applying three consecutive casts under general anaesthesia. These were changed every 4 weeks, followed by the implementation of a custom-made full-time Chêneau brace. Correction was measured by Cobb angle (CA) and rib-vertebra angle difference (RVAD) on whole spine anterior-posterior radiographs. Statistical analysis was performed via ANOVA.ResultsThe study group consisted of 6 patiens with IS and 10 with non-idiopathic scoliosis (NIS) – exclusively syndromic-associated. The mean age at onset of treatment was 35 months (±15). The mean follow up was 21 months (±15). In IS patients average CA/RVAD before treatment was 46°(±8)/20°(±12). In NIS patients average CA/RVAD before treatment was 55°(±15)/24°(±14). After application of the third cast, the CA/RVAD was reduced to 20°(±11)/11°(±10) in IS patients. Whereas in NIS patients average CA/RVAD after the thrid cast was 28°(±12)/18°(±13). At latest follow-up the CA/RVAD was 16°(±7)/9°(±8) in IS patients and 31°(±11)/17° (±15) in NIS patients.ConclusionSyndromic etiology is not a contraindication for serial casting in EOS. Our results show a curve correction, measured in CA, of 65% in IS patients and 44% in NIS patients. Significant reduction in the morphologic deformity, measured in RVAD, was achieved in the IS cohort, but not in the NIS cohort. In all cases surgical treatment could be delayed.

Trunk Growth in Early-Onset Idiopathic Scoliosis Measured With Biplanar Radiography

Study DesignCross-sectional and longitudinal retrospective study. ObjectivesTo measure thoracic dimensions and volume during growth in early-onset idiopathic scoliosis (EOIS) patients and to compare them to a population of asymptomatic adults and to the previous literature. Summary of Background DataData on trunk growth for scoliotic children between 6 and 14 years of age is sparse in the literature. MethodsThirty-six patients (29 girls and 7 boys, between 3 and 14 years old, average Cobb angle 33°±15°) were included, all with a minimum two-year follow-up. Sixty-one asymptomatic girls and 54 asymptomatic adults were included as control groups. All subjects underwent biplanar radiography and 3D reconstruction of the spine, pelvis, and rib cage. EOIS patients repeated their radiologic examination every six months. Cobb angle, rib cage volume, anteroposterior and transverse diameters, thoracic index, thoracic perimeter, pelvic incidence, and T1–T12 and T1–S1 distance were calculated. Reproducibility of measurement was assessed. ResultsMeasurement reliability in such young patients was comparable to previous studies in adolescents and adults. Geometrical parameters of EOIS patients increased linearly with age. For instance, rib cage volume in girls with EOIS increased from 2200 cm3 at six to seven years of age to 4100 cm3 at 13–14 years (65% of adult values, 294 cm3/y). Comparison with asymptomatic girls showed that EOIS could affect growth spurt. Longitudinal analysis on a cohort of six girls who had a follow-up of six years confirmed the cross-sectional data. ConclusionsIn this longitudinal and cross-sectional study, trunk growth between 3 and 14 years of age was characterized, for the first time, with biplanar radiography and 3D reconstruction. The results can be useful to estimate patient growth and thus have potential application in the surgical planning of EOIS patients. Level of EvidenceLevel II, retrospective study.

Similar Deformity Correction, but Limited Spinal Growth and Lower Health Related Quality of Life in Children with Skeletal Dysplasia Undergoing Growth-Friendly Management for Early-Onset Scoliosis. A Matched Comparison with Idiopathic Early-Onset Scoliosis

Similar Deformity Correction, but Limited Spinal Growth and Lower Health Related Quality of Life in Children with Skeletal Dysplasia Undergoing Growth-Friendly Management for Early-Onset Scoliosis. A Matched Comparison with Idiopathic Early-Onset Scoliosis

Age Stratified Outcomes of Mehta Casting in a Large Multi-Center Cohort of Idiopathic Early-Onset Scoliosis Patients

Age Stratified Outcomes of Mehta Casting in a Large Multi-Center Cohort of Idiopathic Early-Onset Scoliosis Patients

Diagnostic yield and clinical effects of exome sequencing analysis in patients with early-onset scoliosis

BackgroundScoliosis is clinically defined as a spine deformity with lateral curvature greater than 10° and the cause of the disease is elusive. Early onset scoliosis, defined by an onset age before 10 years, conveys a substantial health risk to affected children. Although exome sequencing has emerged as a first-line diagnostic method for rare diseases, genetic testing is not routinely done for children with early onset scoliosis. We aimed to explore the diagnostic yield of exome sequencing in patients with early onset scoliosis. MethodsWe recruited a Chinese cohort of patients who were clinically diagnosed with early onset scoliosis through the Deciphering disorders Involving Scoliosis and COmorbidities (DISCO). We did exome sequencing on these individuals and available family members. Exome sequencing data were interpreted according to the American College of Medical Genetics and Genomics guidelines. Patients were followed up for a minimum of 2 years. We also recruited an American cohort of 13 patients with clinically diagnosed idiopathic early onset scoliosis, who underwent exome sequencing. FindingsWe recruited 447 patients in the Chinese cohort and 323 (72%) of 447 patients with early onset scoliosis were followed up for a minimum of 2 years. In the Chinese cohort, we detected diagnostic variants in 92 (21%) of 447 patients with early onset scoliosis, encompassing 33 disease-causing genes and five genomic regions. The age at presentation, the number of organ systems involved, and the Cobb angle were the three features that were most predictive of a molecular diagnosis. Notably, 14 (41%) of 34 patients with non-TBX6-associated congenital scoliosis (TACS) molecular diagnoses had a higher post-surgery complication rate than did the 24 (9·2%) of 260 patients who were molecularly undiagnosed (p=0·000008). In the American cohort, one (8%) of 13 patients had a molecular diagnosis of Sotos syndrome, making the total number of patients in whom we found diagnostic variants 93 (20·2%) of 460. InterpretationExome sequencing could identify the genetic cause in patients with early onset scoliosis. Specific clinical features and feature pairs provided indications for genetic testing in these patients. The prevention of post-surgery complications could potentially be implemented for patients with a non-TACS molecular diagnosis. FundingNational Natural Science Foundation of China, Beijing Natural Science Foundation, 2016 Milstein Medical Asian American Partnership Foundation Fellowship Award in Translational Medicine, CAMS Initiative Fund for Medical Sciences, the Central Level Public Interest Program for Scientific Research Institute, the National Key Research and Development Program of China, the National Undergraduates Innovation and Training Program of Peking Union Medical College, US National Institutes of Health, National Institute of Neurological Disorders and Stroke, National Human Genome Research Institute and National Heart, Lung, and Blood Institute, the National Human Genome Research Institute, TX Scottish Rite Hospital Research Fund, Foundation Cotrel, and P01 HD084387.

203. Cobb angle measurement reliability using low-dose radiographs in early-onset idiopathic scoliosis

BACKGROUND CONTEXT Early-onset idiopathic scoliosis (EOIS) management needs a long follow-up during the entire patient growth. The very young patient's age at the diagnosis can expose them to high cumulated radiation doses. Low-dose radiographs allow high-quality view of the spine in standing position. Accuracy and reproducibility of digital measurements of Cobb angle have already been assessed in adults and adolescents. Nevertheless, reliability of Cobb angle measurement has not been evaluated in EOIS follow-up PURPOSE Low dose stereoradiography could be a minimally invasive way to evaluate early-onset scoliosis before six years of age. STUDY DESIGN/SETTING Prospective data collection. PATIENT SAMPLE Forty-one patients younger than 6 years with thoracic or thoracolumbar scoliosis were evaluated. Patients with spine congenital abnormalities were excluded. OUTCOME MEASURES Intra- and interoperator reliability of 2D Cobb angle measurement in EOIS low-dose radiographs. METHODS Patients underwent low-dose standing radiographs. Three operators independently measured Cobb angles on two separate occasions. A fourth trained operator had previously labeled the superior and inferior limits vertebrae to avoid a potential source of uncertainty unrelated to low-dose radiography. Interoperator reproducibility, intraoperator repeatability and intraclass correlation coefficients (ICC) were calculated. Bland-Altmann plot was used to present the limits of agreement. RESULTS The mean age was 4.4 years (SD 0.8 years, range 2.2-5.8). Average Cobb angle was 22° (SD 9°, range 11-80°). ICCs were higher than 0.98 for all three operators, showing an excellent agreement. Reproducibility and repeatability were respectively of 1.2° (coefficient of variation: 4.5 %) and 0.8° (coefficient of variation 3.2%). Measurements were easily feasible in all cases. There was no effect of age or curve severity on uncertainty. CONCLUSIONS EOIS management includes bracing or surgical growth-friendly techniques. These require multiple radiological assessments responsible for a significant cumulative radiation exposure. Cobb angle measurements on low-dose radiographs showed an excellent reproducibility despite bone immaturity. Clinical follow-up could be done with this low-dose technique. FDA DEVICE/DRUG STATUS This abstract does not discuss or include any applicable devices or drugs.

Growth-Friendly Spine Surgery in Escobar Syndrome.

The aims of this study were to characterize the spinal deformity of patients with Escobar syndrome, describe results of growth-friendly treatments, and compare these results with those of an idiopathic early-onset scoliosis (EOS) cohort to determine whether the axial stiffness in Escobar syndrome limited correction. We used 2 multicenter databases to review the records of 8 patients with EOS associated with Escobar syndrome who had at least 2-year follow-up after initiation of growth-friendly treatment from 1990 to 2016. An idiopathic EOS cohort of 16 patients matched for age at surgery (±1 y), postoperative follow-up (±1 y), and initial curve magnitude (±10 degrees) was identified. A randomized 1:2 matching algorithm was applied (α=0.05). In the Escobar group, spinal deformity involved 7 to 13 vertebrae and ranged from no vertebral anomalies in 3 patients to multiple segmentation defects in 6 patients. Mean age at first surgery was 5 years (range, 1.4 to 7.8 y) with a mean follow-up of 7.5 years (range, 4.0 to 10 y). Mean major curve improved from 76 degrees at initial presentation, to 43 degrees at first instrumentation, to 37 degrees at final follow-up (both P<0.001). Mean pelvic obliquity improved from 16 degrees (range, 5 to 31 degrees) preoperatively to 4 degrees (range, 0 to 8 degrees) at final follow-up (P=0.005). There were no differences in the mean percentage of major curve correction between the idiopathic EOS and Escobar groups at the immediate postoperative visit (P=0.743) or final follow-up (P=0.511). There were no differences between the cohorts in T1-S1 height at initial presentation (P=0.129) or in growth per month (P=0.211). Multiple congenital fusions and spinal curve deformity are common in Escobar syndrome. Despite large areas of congenital fusion, growth-friendly constructs facilitate spinal growth and improve curve correction. These results are comparable to those in idiopathic EOS. Level III-case-control study.

Health-related Quality of Life and Body Image Disturbance of Adolescents With Severe Untreated Idiopathic Early-onset Scoliosis in a Developing Country.

A cross-sectional study. This study aimed to investigate the health-related quality of life (HRQOL) and body image disturbance of adolescents with untreated severe idiopathic early-onset scoliosis (IEOS) in a developing country. IEOS has the potential to progress rapidly and may lead to significant disability if left untreated. Moreover, it can lead to significant body image changes that may lead to social and psychological stresses on developing adolescents. In developing countries, untreated severe IEOS among adolescents is common. The Scoliosis Research Society 22 revision (SRS-22r) Arabic version questionnaire and Body Image Disturbance Questionnaire-Scoliosis version (BIDQ-S) were used to investigate the HRQOL and body image disturbance of these patients. A total of 137 patients with untreated severe IEOS (mean age, 15.5 years; range, 12-21 years) were included: group 1 (76 patients) with major curve angles of 90° to 120°, group 2 (61 patients) with major curve angles above 120°, and group 3 comprising 50 healthy controls were used for comparison. No significant differences were found regarding age, sex, and body mass index among the groups. Group 1 had significantly lower scores than group 3 in all SRS-22r domains (P < 0.001). Group 2 had significantly lower scores than group 1 in all SRS-22r domains, except for satisfaction (P < 0.005); the satisfaction domain had 100% worst answers in groups 1 and 2. Group 2 had worse scores than group 1 in all BIDQ-S questions (P < 0.001). No significant differences were found between males and females in all SRS-22r domains and BIDQ-S questions (P > 0.05). Further, 13% to 100% and 25% to 71% of the patients provided the worst answers to the SRS-22r and BIDQ-S questions, respectively. The HRQOL and body image of the adolescents with untreated severe IEOS in a developing country were severely impaired. 3.

Idiopathic Early-onset Scoliosis: Growing Rods Versus Vertically Expandable Prosthetic Titanium Ribs at 5-year Follow-up.

Distraction-based techniques allow spinal growth until skeletal maturity while preventing curve progression. Two multicenter early-onset scoliosis databases were used to identify patients with idiopathic spine abnormalities treated with traditional growing rods (TGR) or vertically expandable titanium ribs (VEPTR). Patients underwent at least 4 lengthenings and had at least 5-year follow-up. Significance was set at P<0.05. In total, 50 patients treated with TGR and 22 treated with VEPTR were included. Mean (±SD) age at surgery was 5.5 (±2.0) years for the TGR group versus 4.3 (±1.9) years for the VEPTR group (P=0.044); other demographic parameters were similar. VEPTR patients had more procedures (mean 15±4.2) than TGR patients (mean 10±4.0) (P=0.001). Unilateral constructs were present in 18% (4 of 22) of VEPTR and 16% (8 of 50) of TGR patients. Bilateral constructs spanned a mean 2.1 additional surgical levels and exposed patients to 1.6 fewer procedures than unilateral constructs. Curve correction was similar between bilateral and unilateral constructs. TGR patients experienced greater curve correction (50%) than VEPTR patients (27%) (P<0.001) and achieved a greater percentage of thoracic height gain (24%) than VEPTR patients (12%) (P=0.024). At latest follow-up, TGR patients had better maintenance of curve correction, less kyphosis, and 15% greater absolute gain in thoracic height versus VEPTR patients. TGR patients had a lower rate of wound complications (14%) than VEPTR patients (41%) (P=0.011). In patients with idiopathic early-onset scoliosis, TGRs produced greater initial curve correction, greater thoracic height gains, less kyphosis, and lower incidence of wound complications compared with VEPTR. Level III.

Surgical treatment of early-onset idiopathic scoliosis in the United States: a trend analysis of 15 years (1997–2012)

Background ContextEarly-onset scoliosis is a challenging problem that is defined as a curvature of the spine of more than 10 degrees identified in a child less than 10 years. Early-onset idiopathic scoliosis (EOIS) can cause substantial morbidity and may require surgical intervention. PurposeThe aim of the present study was to identify the trends of EOIS type of surgeries, length of hospital stay, in-hospital complications, and total inpatient admission charges over a 15-year study period in the United States from 1997 to 2012. Study Design/SettingThis retrospective study used the ICD-9-CM (International Classification of Diseases, Ninth Revision, Clinical Modification) codes from the Healthcare Cost and Utilization Project (HCUP) Kids Inpatient's Database (KID) for a 15-year period (1997–2012). Patient SampleWe identified a total of 897 patients with EOIS over the 15-year study period. Outcome MeasuresThe present study determines the current trends for EOIS surgeries. MethodsThe present study had no funding sources or any potential conflicts of interest associated biases. Idiopathic scoliosis patients with ages between 0 and <10 years were identified from the Kids' Inpatient Database with ICD-9-CM code 737.30. Posterior, anterior, and combined spinal surgeries were identified in EOIS through the procedure codes. Patients' gender, discharge diagnosis (comorbidities), hospital length of stay (LOS), mortality rates, hospital charges, and in-hospital complication rate data were collected between 1997 and 2012. The primary grouping variable of the study was the type of surgery (posterior, anterior, and combined). The trends of each variable (female gender, mortality rates, in-hospital complications rates, discharge diagnosis, LOS, and total hospital charges) were assessed for each surgical group separately. Cost inflation of hospital charges was adjusted for the year 2012. An analysis of variance test was used to analyze continuous variables and a chi-square test was used for categorical variables. A linear regression test was used to assess the trend of changes. p≤.05 was considered statistically significant. ResultsThe study identified 897 patients, with 546 (61%) of them requiring surgery. Spine deformity surgery rates significantly decreased in patients with EOIS over time from 75% in 1997 to 47% in 2012, p=.019. In the surgery cohort, the male to female distribution was 37% and 63%, respectively. The overall mortality rate was 0.1%. The average length of hospital stay was 8 days and the average number of discharge diagnosis was 5.3. Aggregated complications were seen in 6% of the patients. The total mean hospital charge (per 2012 US dollars) was $119,613, which increased significantly for all types of surgeries. Over the 15-year study period, 62% (n=342) of the patients had posterior surgeries, 13% (n=71) of the patients had anterior surgeries, and 24% (n=133) of the patients had combined (anterior and posterior) surgeries. Posterior surgeries increased significantly from 33% in 1997 to 91% in 2012 (p<.004). Combined surgeries saw a significant decline from 50% to 4.3% (0<0.001). Anterior surgeries also decreased from 17% to 4.3% (p<.126), but this did not reach statistical significance. ConclusionsFrom 1997 to 2012 (15 years) study period of patients with EOIS, posterior-based surgeries significantly increased. The overall surgery rate has significantly decreased for these patients. A significant increase in hospital charges were noticed in posterior, anterior, and combined surgeries.

Management of Spinal Deformities and Evidence of Treatment Effectiveness.

Introduction:The review evaluates the up-to-date evidence for the treatment of spinal deformities, including scoliosis and hyperkyphosis in adolescents and adults.Material and Methods:The PubMed database was searched for review articles, prospective controlled trials and randomized controlled trials related to the treatment of spinal deformities. Articles on syndromic scoliosis were excluded and so were the articles on hyperkyphosis of the spine with causes other than Scheuermann’s disease and osteoporosis. Articles on conservative and surgical treatments of idiopathic scoliosis, adult scoliosis and hyperkyphosis were also included. For retrospective papers, only studies with a follow up period exceeding 10 years were included.Results:The review showed that early-onset idiopathic scoliosis has a worse outcome than late-onset idiopathic scoliosis, which is rather benign. Patients with AIS function well as adults; they have no more health problems when compared to patients without scoliosis, other than a slight increase in back pain and aesthetic concern. Conservative treatment of adolescent idiopathic scoliosis (AIS) using physiotherapeutic scoliosis-specific exercises (PSSE), specifically PSSR and rigid bracing was supported by level I evidence. Yet to date, there is no high quality evidence (RCT`s) demonstrating that surgical treatment is superior to conservative treatment for the management of AIS. For adult scoliosis, there are only a few studies on the effectiveness of PSSEs and a conclusion cannot as yet be drawn.For hyperkyphosis, there is no high-quality evidence for physiotherapy, bracing or surgery for the treatment of adolescents and adults. However, bracing has been found to reduce thoracic hyperkyphosis, ranging from 55 to 80° in adolescents. In patients over the age of 60, bracing improves the balance score, and reduces spinal deformity and pain. Surgery is indicated in adolescents and adults in the presence of progression of kyphosis, refractory pain and loss of balance.Discussion:The available evidence reviewed has suggested that different approaches are needed towards the management of different spinal deformities. Specific exercises should be prescribed in children and adolescents with a Cobb angle in excess of 15°. In progressive curves, they should be used in conjunction with bracing. Clarity regarding differences and similarities is given as to what makes PSSE and PSSR specific exercises. As AIS is relatively benign in nature, conservative treatment should be tried when the curve is at a surgical threshold, before surgery is considered. Similarly, bracing and exercises should be prescribed for patients with hyperkyphosis, particularly when the lumbar spine is afflicted. Surgery should be considered only when the symptoms cannot be managed conservatively.Conclusion:There is at present high quality evidence in support of the conservative treatment of AIS. The current evidence supports the use of PSSE, especially those using PSSR, together with bracing in the treatment of AIS. In view of the lack of medical consequences in adults with AIS, conservative treatment should be considered for curves exceeding the formerly assumed range of conservative indications.There is, however a lack of evidence in support of any treatment of choice for hyperkyphosis in adolescents and spinal deformities in adults. Yet, conservative treatment should be considered first. Yet to date, there is no high quality evidence (RCT`s) demonstrating that surgical treatment is superior to conservative treatment for the management of AIS and hyperkyphosis. Additionally, surgery needs to be considered with caution, as it is associated with a number of long-term complications.

Intraspinal anomalies in early-onset idiopathic scoliosis.

In the United Kingdom, lower incidences of intraspinal abnormalities in patients with early onset idiopathic scoliosis have been observed than in studies in other countries. We aimed to determine the rates of these abnormalities in United Kingdom patients diagnosed with idiopathic scoliosis before the age of 11 years. This retrospective study of patients attending an urban scoliosis clinic identified 71 patients satisfying a criteria of: clinical diagnosis of idiopathic scoliosis; age of onset ten years and 11 months or less; MRI screening for intraspinal abnormalities. United Kingdom census data combined with patient referral data was used to calculate incidence. Mean age at diagnosis was six years with 39 right-sided and 32 left-sided curves. Four patients (5.6%) were found to have intraspinal abnormalities on MRI. These consisted of: two combined Arnold-Chiari type 1 malformations with syrinx; one syrinx with a low lying conus; and one isolated syrinx. Overall annual incidence of early onset idiopathic scoliosis was one out of 182 000 (0.0006%). This study reports the lowest rates to date of intraspinal anomalies in patients with early onset idiopathic scoliosis, adding to knowledge regarding current incidences of these abnormalities as well as any geographical variation in the nature of the disease. Cite this article: Bone Joint J 2017;99-B:829-33.