We determined the frequency, rate and extent of development of scoliosis (coronal plane deformity) in wheelchair-dependent patients with Duchenne muscular dystrophy (DMD) who were not receiving steroid treatment. We also assessed kyphosis and lordosis (sagittal plane deformity). The extent of scoliosis was assessed on sitting anteroposterior (AP) spinal radiographs in 88 consecutive non-ambulatory patients with DMD. Radiographs were studied from the time the patients became wheelchair-dependent until the time of spinal fusion, or the latest assessment if surgery was not undertaken. Progression was estimated using a longitudinal mixed-model regression analysis to handle repeated measurements. Scoliosis ≥ 10° occurred in 85 of 88 patients (97%), ≥ 20° in 78 of 88 (89%) and ≥ 30° in 66 of 88 patients (75%). The fitted longitudinal model revealed that time in a wheelchair was a highly significant predictor of the magnitude of the curve, independent of the age of the patient (p <  0.001). Scoliosis developed in virtually all DMD patients not receiving steroids once they became wheelchair-dependent, and the degree of deformity deteriorated over time. In general, scoliosis increased at a constant rate, beginning at the time of wheelchair-dependency (p < 0.001). In some there was no scoliosis for as long as three years after dependency, but scoliosis then developed and increased at a constant rate. Some patients showed a rapid increase in the rate of progression of the curve after a few years – the clinical phenomenon of a rapidly collapsing curve over a few months. A sagittal plane kyphotic deformity was seen in 37 of 60 patients (62%) with appropriate radiographs, with 23 (38%) showing lumbar lordosis (16 (27%) abnormal and seven (11%) normal). This study provides a baseline to assess the effects of steroids and other forms of treatment on the natural history of scoliosis in patients with DMD, and an approach to assessing spinal deformity in the coronal and sagittal planes in wheelchair-dependent patients with other neuromuscular disorders. Cite this article: Bone Joint J 2014;96-B:100–5

A consecutive series of 85 patients with Duchenne’s muscular dystrophy who underwent spinal fusion over a period of 16 years was followed up with regard to the progression of the scoliosis and pelvic obliquity. Of 74 patients with adequate radiographic follow-up, 55 were instrumented with the Luque single-unit rod system and 19 with the Isola pedicle screw system; seven were instrumented to L3/4, 42 to L5, 15 to S1 and 10 to the pelvis with intrailiac rods. The mean period of follow-up was 49 months (SD 22) before and 47 months (SD 24) after operation. There was one peri-operative death and three cases of failure of hardware. The mean improvement in the Cobb angle was 26° and in pelvic obliquity, 9.2°. Fusion to L3/4 achieved a poorer correction of both curves while intrapelvic rods, achieved and maintained the best correction of pelvic obliquity. Fusion to S1 did not provide any benefit over more proximal fusion excluding the sacrum, with regard to correction and maintenance of both angles. The Isola system appeared to provide and maintain a slightly better correction of the Cobb angle

Aims. The prevalence of scoliosis is not known in patients with idiopathic short stature, and the impact of treatment with recombinant human growth hormone on those with scoliosis remains controversial. We investigated the prevalence of scoliosis radiologically in children with idiopathic short stature, and the impact of treatment with growth hormone in a cross-sectional and retrospective cohort study. Methods. A total of 2,053 children with idiopathic short stature and 4,106 age- and sex-matched (1:2) children without short stature with available whole-spine radiographs were enrolled in the cross-sectional study. Among them, 1,056 with idiopathic short stature and 790 controls who had radiographs more than twice were recruited to assess the development and progression of scoliosis, and the need for bracing and surgery. Results. In the cross-sectional study, there was an unexpectedly higher prevalence of scoliosis (33.1% (681/2,053) vs 8.52% (350/4,106)) in children with idiopathic short stature compared with controls (odds ratio 3.722; p < 0.001), although most cases were mild. In the longitudinal study, children with idiopathic short stature had a higher risk of the development and progression of scoliosis than the controls. Among children with idiopathic short stature without scoliosis at baseline, treatment with growth hormone significantly increased the risk of developing scoliosis (p = 0.015) and the need for bracing (p < 0.001). Among those with idiopathic short stature and scoliosis at baseline, treatment with growth hormone did not increase the risk of progression of the scoliosis, the need for bracing, or surgery. Conclusion. The impact of treatment with growth hormone on scoliosis in children with idiopathic short stature was considered controllable. However, physicians should pay close attention to the assessment of spinal curves in these children. Cite this article: Bone Joint J 2023;105-B(4):439–448

Aims. The aim of this study was to assess the ability of morphological spinal parameters to predict the outcome of bracing in patients with adolescent idiopathic scoliosis (AIS) and to establish a novel supine correction index (SCI) for guiding bracing treatment. Methods. Patients with AIS to be treated by bracing were prospectively recruited between December 2016 and 2018, and were followed until brace removal. In all, 207 patients with a mean age at recruitment of 12.8 years (SD 1.2) were enrolled. Cobb angles, supine flexibility, and the rate of in-brace correction were measured and used to predict curve progression at the end of follow-up. The SCI was defined as the ratio between correction rate and flexibility. Receiver operating characteristic (ROC) curve analysis was carried out to assess the optimal thresholds for flexibility, correction rate, and SCI in predicting a higher risk of progression, defined by a change in Cobb angle of ≥ 5° or the need for surgery. Results. The baseline Cobb angles were similar (p = 0.374) in patients whose curves progressed (32.7° (SD 10.7)) and in those whose curves remained stable (31.4° (SD 6.1)). High supine flexibility (odds ratio (OR) 0.947 (95% CI 0.910 to 0.984); p = 0.006) and correction rate (OR 0.926 (95% CI 0.890 to 0.964); p < 0.001) predicted a lower incidence of progression after adjusting for Cobb angle, Risser sign, curve type, menarche status, distal radius and ulna grading, and brace compliance. ROC curve analysis identified a cut-off of 18.1% for flexibility (sensitivity 0.682, specificity 0.704) and a cut-off of 28.8% for correction rate (sensitivity 0.773, specificity 0.691) in predicting a lower risk of curve progression. A SCI of greater than 1.21 predicted a lower risk of progression (OR 0.4 (95% CI 0.251 to 0.955); sensitivity 0.583, specificity 0.591; p = 0.036). Conclusion. A higher supine flexibility (18.1%) and correction rate (28.8%), and a SCI of greater than 1.21 predicted a lower risk of progression. These novel parameters can be used as a guide to optimize the outcome of bracing. Cite this article: Bone Joint J 2022;104-B(4):495–503

Aims. The aim of this study was to investigate whether including the stages of ulnar physeal closure in Sanders stage 7 aids in a more accurate assessment for brace weaning in patients with adolescent idiopathic scoliosis (AIS). Methods. This was a retrospective analysis of patients who were weaned from their brace and reviewed between June 2016 and December 2018. Patients who weaned from their brace at Risser stage ≥ 4, had static standing height and arm span for at least six months, and were ≥ two years post-menarche were included. Skeletal maturity at weaning was assessed using Sanders staging with stage 7 subclassified into 7a, in which all phalangeal physes are fused and only the distal radial physis is open, with narrowing of the medial physeal plate of the distal ulna, and 7b, in which fusion of > 50% of the medial growth plate of distal ulna exists, as well as the distal radius and ulna (DRU) classification, an established skeletal maturity index which assesses skeletal maturation using finer stages of the distal radial and ulnar physes, from open to complete fusion. The grade of maturity at the time of weaning and any progression of the curve were analyzed using Fisher’s exact test, with Cramer’s V, and Goodman and Kruskal’s tau. Results. We studied a total of 179 patients with AIS, of whom 149 (83.2%) were female. Their mean age was 14.8 years (SD 1.1) and the mean Cobb angle was 34.6° (SD 7.7°) at the time of weaning. The mean follow-up was 3.4 years (SD 1.8). At six months after weaning, the rates of progression of the curve for patients weaning at Sanders stage 7a and 7b were 11.4% and 0%, respectively for those with curves of < 40°. Similarly, the rates of progression of the curve for those being weaned at ulnar grade 7 and 8 using the DRU classification were 13.5% and 0%, respectively. The use of Sanders stages 6, 7a, 7b, and 8 for the assessment of maturity at the time of weaning were strongly and significantly associated (Cramer’s V 0.326; p = 0.016) with whether the curve progressed at six months after weaning. Weaning at Sanders stage 7 with subclassification allowed 10.6% reduction of error in predicting the progression of the curve. Conclusion. The use of Sanders stages 7a and 7b allows the accurate assessment of skeletal maturity for guiding brace weaning in patients with AIS. Weaning at Sanders stage 7b, or at ulnar grade 8 with the DRU classification, is more appropriate as the curve did not progress in any patient with a curve of < 40° immediately post-weaning. Thus, reaching full fusion in both distal radial and ulnar physes (as at Sanders stage 8) is not necessary and this allows weaning from a brace to be initiated about nine months earlier. Cite this article: Bone Joint J 2021;103-B(1):141–147

Aims. The aim of the present study was to answer the question whether curve morphology and location have an influence on rigid conservative treatment in patients with adolescent idiopathic scoliosis (AIS). Methods. We retrospectively analyzed AIS in 127 patients with single and double curves who had been treated with a Chêneau brace and physiotherapeutic specific exercises (B-PSE). The inclusion criteria were the presence of structural major curves ≥ 20° and < 50° (Risser stage 0 to 2) at the time when B-PSE was initiated. The patients were divided into two groups according to the outcome of treatment: failure (curve progression to ≥ 45° or surgery) and success (curve progression < 45° and no surgery). The main curve type (MCT), curve magnitude, and length (overall, above and below the apex), apical rotation, initial curve correction, flexibility, and derotation by the brace were compared between the two groups. Results. In univariate analysis treatment failure depended significantly on: 1) MCT (p = 0.008); 2) the apical rotation of the major curve before (p = 0.007) and during brace treatment (p < 0.001); 3) the initial and in-brace Cobb angles of the major (p = 0.001 and p < 0.001, respectively) and minor curves (p = 0.015 and p = 0.002); 4) major curve flexibility (p = 0.005) and the in-brace curve correction rates (major p = 0.008, minor p = 0.034); and 5) the length of the major curve (LoC) above (p < 0.001) and below (p = 0.002) the apex. Furthermore, MCT (p = 0.043, p = 0.129, and p = 0.017 in MCT comparisons), LoC (upper length p = 0.003, lower length p = 0.005), and in-brace Cobb angles (major p = 0.002, minor p = 0.027) were significant in binary logistic regression analysis. Conclusion. Curve size, location, and morphology were found to influence the outcome of rigid conservative treatment of AIS. These findings may improve future brace design and patient selection for conservative treatment. Cite this article: Bone Joint J 2021;103-B(2):373–381

Aims

To systematically evaluate whether bracing can effectively achieve curve regression in patients with adolescent idiopathic scoliosis (AIS), and to identify any predictors of curve regression after bracing.

Aims. Magnetically controlled growing rod (MCGR) systems use non-invasive spinal lengthening for the surgical treatment of early-onset scoliosis (EOS). The primary aim of this study was to evaluate the performance of these devices in the prevention of progression of the deformity. A secondary aim was to record the rate of complications. Patients and Methods. An observational study of 31 consecutive children with EOS, of whom 15 were male, who were treated between December 2011 and October 2017 was undertaken. Their mean age was 7.7 years (2 to 14). The mean follow-up was 47 months (24 to 69). Distractions were completed using the tailgating technique. The primary outcome measure was correction of the radiographic deformity. Secondary outcomes were growth, functional outcomes and complication rates. Results. The mean Cobb angle was 54° (14° to 91°) preoperatively and 37° (11° to 69°) at the latest follow-up (p < 0.001). The mean thoracic kyphosis (TK) was 45° (10° to 89°) preoperatively and 42° (9° to 84°) at the latest follow-up. The mean T1–S1 height increased from 287 mm (209 to 378) to 338 mm (240 to 427) (p < 0.001) and the mean sagittal balance reduced from 68 mm (-76 to 1470) preoperatively to 18 mm (-32 to 166) at the latest follow-up. The mean coronal balance was 3 mm (-336 to 64) preoperatively and 8 mm (-144 to 64) at the latest follow-up. The mean increase in weight and sitting and standing height at the latest follow-up was 45%, 10% and 15%, respectively. The mean Activity Scale for Kids (ASKp) scores increased in all domains, with only personal care and standing skills being significant at the latest follow-up (p = 0.02, p = 0.03). The improvements in Cobb angle, TK and T1-S1 heights were not related to gender, the aetiology of the EOS, or whether the procedure was primary or conversion from a conventional growing rod system. A total of 21 children developed 23 complications at a rate of 0.23 per patient per year. Seven developed MCGR-specific complications. Complications developed at a mean of 38 months (3 to 67) after the initial surgery and required 22 further procedures. Children who developed a complication were more likely to be younger, have syndromic EOS, and have a single-rod construct (6.9 versus 9.3 years, p = 0.034). Conclusion. The progression of EOS can be controlled using MCGRs allowing growth and improved function. Younger and syndromic children are more likely to develop complications following surgery. Cite this article: Bone Joint J 2018;100-B:1187–1200

Aims

Only a few studies have investigated the long-term health-related quality of life (HRQoL) in patients with an idiopathic scoliosis. The aim of this study was to investigate the overall HRQoL and employment status of patients with an idiopathic scoliosis 40 years after diagnosis, to compare it with that of the normal population, and to identify possible predictors for a better long-term HRQoL.

Aims

The aim of this study was to report the long-term prognosis of patients with multiple Langerhans cell histiocytosis (LCH) involving the spine, and to analyze the risk factors for progression-free survival (PFS).

Aims

The aim of this study was to compare outcomes after growth-friendly treatment for early-onset scoliosis (EOS) between patients with skeletal dysplasias versus those with other syndromes.

Aims

Historically, patients undergoing surgery for adolescent idiopathic scoliosis (AIS) have been nursed postoperatively in a critical care (CC) setting because of the challenges posed by prone positioning, extensive exposures, prolonged operating times, significant blood loss, major intraoperative fluid shifts, cardiopulmonary complications, and difficulty in postoperative pain management. The primary aim of this paper was to determine whether a scoring system, which uses Cobb angle, forced vital capacity (FVC), forced expiratory volume in one second (FEV1), and number of levels to be fused, is a valid method of predicting the need for postoperative critical care in AIS patients who are to undergo scoliosis correction with posterior spinal fusion (PSF).

Aims

The aim of this study was to investigate the incidence and characteristics of instrumentation failure (IF) after total en bloc spondylectomy (TES), and to analyze risk factors for IF.

The progression of post-tubercular kyphosis in 61 children who received ambulatory chemotherapy was studied prospectively. The angles of deformity and kyphosis were measured for each patient at diagnosis, 3, 6, 9, 12 and 18 months later and every year thereafter for 15 years. During the course of the disease signs of instability appeared on the radiographs of some of the children. These were dislocation of the facets, posterior retropulsion of the diseased fragments, lateral translation of the vertebrae in the anteroposterior view and toppling of the superior vertebra. Each sign was allocated one point to create a spinal instability score. The influence on the progression of the deformity of the level of the lesion, the vertebral body loss, the number of segments involved, the angle of deformity before treatment and the spinal instability score was analysed. The mean angle of deformity at the start of treatment was 35°. This increased to 41° at 15 years. Progression occurred during the active phase of the disease and again after cure when variations in progression were observed. Type-I progression showed an increase in deformity until growth had ceased. This could occur either continuously (type Ia) or after a lag period of three to five years (type Ib). Type-II progression showed decrease in deformity with growth. This could occur immediately after the active phase (type IIa) or after a lag period of three to five years (type IIb). Type-III progression showed minimal change during either the active or healed phases and was seen only in those with limited disease. Multiple regression analysis showed that a spinal instability score of more than 2 was a reliable predictor of patients with an increase of more than 30° in deformity and a final deformity of over 60°. Since signs of radiological instability appear early in the disease, they can be reliably used to identify children whose spine is at risk for late progressive collapse. Surgery is advised in these cases

Aims. The primary aim of this study was to evaluate the performance and safety of magnetically controlled growth rods in the treatment of early onset scoliosis. Secondary aims were to evaluate the clinical outcome, the rate of further surgery, the rate of complications, and the durability of correction. Patients and Methods. We undertook an observational prospective cohort study of children with early onset scoliosis, who were recruited over a one-year period and followed up for a minimum of two years. Magnetically controlled rods were introduced in a standardized manner with distractions performed three-monthly thereafter. Adverse events which were both related and unrelated to the device were recorded. Ten children, for whom relevant key data points (such as demographic information, growth parameters, Cobb angles, and functional outcomes) were available, were recruited and followed up over the period of the study. There were five boys and five girls. Their mean age was 6.2 years (2.5 to 10). Results. The mean coronal Cobb angle improved from 57.6° (40° to 81°) preoperatively, 32.8° (28° to 46°) postoperatively, and 41° (19° to 57°) at two years. Five children had an adverse event, with four requiring return to theatre, but none were related to the device. There were no neurological complications or infections. No devices failed. One child developed a proximal junctional kyphosis. The mean gain in spinal column height from T1 to S1 was 45.4 mm (24 to 81) over the period of the study. Conclusion. Magnetically controlled growth rods provide an alternative solution to traditional growing rods in the surgical management of children with early onset scoliosis, supporting growth of the spine while controlling curve progression. Their use has clear psychosocial and economic benefits, with the reduction of the need for repeat surgery as required with traditional growing rods. Cite this article: Bone Joint J 2018;100-B:507–15

Aims

To determine the value of scoliosis surgery, it is necessary to evaluate outcomes in domains that matter to patients. Since randomized trials on adolescent idiopathic scoliosis (AIS) are scarce, prospective cohort studies with comparable outcome measures are important. To enhance comparison, a core set of patient-related outcome measures is available. The aim of this study was to evaluate the outcomes of AIS fusion surgery at two-year follow-up using the core outcomes set.

Aims

The aim of this study was to compare the clinical and radiological outcomes of patients with early-onset scoliosis (EOS), who had undergone spinal fusion after distraction-based spinal growth modulation using either traditional growing rods (TGRs) or magnetically controlled growing rods (MCGRs).

Randomised controlled trials (RCTs) that assessed the efficacy of bracing for adolescent idiopathic scoliosis have suffered from small sample sizes, low compliance and lack of willingness to participate. The aim of this study was to assess the feasibility of a comprehensive cohort study for evaluating both the efficacy and the effectiveness of bracing in patients with adolescent idiopathic scoliosis. Patients with curves at greater risk of progression were invited to join a randomised controlled trial. Those who declined were given the option to remain in the study and to choose whether they wished to be braced or observed. Of 87 eligible patients (5 boys and 63 girls) identified over one year, 68 (78%) with mean age of 12.5 years (10 to 15) consented to participate, with a mean follow-up of 168 weeks (0 to 290). Of these, 19 (28%) accepted randomisation. Of those who declined randomisation, 18 (37%) chose a brace. Patients who were more satisfied with their image were more likely to choose bracing (Odds Ratio 4.1; 95% confidence interval 1.1 to 15.0; p = 0.035). This comprehensive cohort study design facilitates the assessment of both efficacy and effectiveness of bracing in patients with adolescent idiopathic scoliosis, which is not feasible in a conventional randomised controlled trial. Cite this article: Bone Joint J 2015; 97-B:973–81

Aims. Clinical and radiological data were reviewed for all patients with mucopolysaccharidoses (MPS) with thoracolumbar kyphosis managed non-operatively or operatively in our institution. Methods. In all 16 patients were included (eight female: eight male; 50% male), of whom nine had Hurler, five Morquio and two Hunter syndrome. Six patients were treated non-operatively (mean age at presentation of 6.3 years; 0.4 to 12.9); mean kyphotic progression +1.5. o. /year; mean follow-up of 3.1 years (1 to 5.1) and ten patients operatively (mean age at presentation of 4.7 years; 0.9 to 14.4); mean kyphotic progression 10.8. o. /year; mean follow-up of 8.2 years; 4.8 to 11.8) by circumferential arthrodesis with posterior instrumentation in patients with flexible deformities (n = 6). Results. In the surgical group (mean age at surgery of 6.6 years; 2.4 to 16.8); mean post-operative follow-up of 6.3 years (3.5 to 10.3), mean pre-operative thoracolumbar kyphosis of 74.3. o. (42. o. to 110. o. ) was corrected to mean of 28.6. o. (0. o. to 65. o. ) post-operatively, relating to a mean deformity correction of 66.9% (31% to 100%). Surgical complications included a deep wound infection treated by early debridement, apical non-union treated by posterior re-grafting, and stable adjacent segment spondylolisthesis managed non-operatively. Thoracolumbar kyphosis > +38. o. at initial presentation was identified as predicting progressively severe deformity with 90% sensitivity and 83% specificity. Discussion. This study demonstrates that severe thoracolumbar kyphosis in patients with MPS can be effectively treated by circumferential arthrodesis. Severity of kyphosis at initial presentation may predict progression of thoracolumbar deformity. Patients with MPS may be particularly susceptible to post-operative complications due to the underlying connective tissue disorder and inherent immunological compromise. Take home message: Clinical and radiological data were reviewed for all patients with mucopolysaccharidoses with thoracolumbar kyphosis managed non-operatively or operatively in our institution. Cite this article: Bone Joint J 2016;98-B:229–37

We analysed the cases of lumbar kyphosis in 151 (21%) of a series of 719 patients with myelomeningocele. Three different types were distinguished: paralytic, sharp-angled and congenital. In a cross-sectional and partly longitudinal study the size and magnitude of the kyphosis, the apex of the curve and the level of paralysis of each group were recorded and statistically analysed. Paralytic kyphosis (less than 90° at birth) occurred in 44.4% and increased linearly during further development. Sharp-angled kyphosis (90° or more at birth) was present in 38.4% and also showed a linear progression. In both types, progression seemed to depend also on the level of paralysis. Congenital kyphosis occurred in 13.9% and we could find no significant factor which correlated with progression