Adolescent idiopathic scoliosis (AIS), defined by an age at presentation of 11 to 18 years, has a prevalence of 0.47% and accounts for approximately 90% of all cases of idiopathic scoliosis. Despite decades of research, the exact aetiology of AIS remains unknown. It is becoming evident that it is the result of a complex interplay of genetic, internal, and environmental factors. It has been hypothesized that genetic variants act as the initial trigger that allow epigenetic factors to propagate AIS, which could also explain the wide phenotypic variation in the presentation of the disorder. A better understanding of the underlying aetiological mechanisms could help to establish the diagnosis earlier and allow a more accurate prediction of deformity progression. This, in turn, would prompt imaging and therapeutic intervention at the appropriate time, thereby achieving the best clinical outcome for this group of patients. Cite this article: Bone Joint J 2022;104-B(8):915–921

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. Methods. Two independent reviewers performed a comprehensive literature search in PubMed, Ovid, Web of Science, Scopus, and Cochrane Library to obtain all published information about the effectiveness of bracing in achieving curve regression in AIS patients. Search terms included “brace treatment” or “bracing,” “idiopathic scoliosis,” and “curve regression” or “curve reduction.” Inclusion criteria were studies recruiting patients with AIS undergoing brace treatment and one of the study outcomes must be curve regression or reduction, defined as > 5° reduction in coronal Cobb angle of a major curve upon bracing completion. Exclusion criteria were studies including non-AIS patients, studies not reporting p-value or confidence interval, animal studies, case reports, case series, and systematic reviews. The GRADE approach to assessing quality of evidence was used to evaluate each publication. Results. After abstract and full-text screening, 205 out of 216 articles were excluded. The 11 included studies all reported occurrence of curve regression among AIS patients who were braced. Regression rate ranged from 16.7% to 100%. We found evidence that bracing is effective in achieving curve regression among compliant AIS patients eligible for bracing, i.e. curves of 25° to 40°. A similar effect was also found in patients with major curve sizes ranging from 40° to 60° when combined with scoliosis-specific exercises. There was also evidence showing that a low apical vertebral body height ratio, in-brace correction, smaller pre-brace Cobb angle, and daily pattern of brace-wear compliance predict curve regression after bracing. Conclusion. Bracing provides a corrective effect on scoliotic curves of AIS patients to achieve curve regression, given there is high compliance rate and the incorporation of exercises. Cite this article: Bone Joint J 2024;106-B(3):286–292

Aims. The aim of this study was to evaluate whether, after correction of an adolescent idiopathic scoliosis (AIS), leaving out the subfascial drain gives results that are no worse than using a drain in terms of total blood loss, drop in haemoglobin level, and opioid consumption. Methods. Adolescents (aged between 10 and 21 years) with an idiopathic scoliosis (major curve ≥ 45°) were eligible for inclusion in this randomized controlled noninferiority trial (n = 125). A total of 90 adolescents who had undergone segmental pedicle screw instrumentation were randomized into no-drain or drain groups at the time of wound closure using the sealed envelope technique (1:1). The primary outcome was a drop in the haemoglobin level during first three postoperative days. Secondary outcomes were 48-hour postoperative oxycodone consumption and surgical complications. Results. All 90 patients were included in the primary outcome analysis (no drain = 43; drain = 47). The mean total postoperative blood loss (intraoperative and drain output) was significantly higher in the group with a subfascial drain than in the no-drain group (1,008 ml (SD 520) vs 631 ml (SD 518); p < 0.001). The drop in haemoglobin level did not differ between the study groups over the postoperative timepoints (p = 0.290). The 48-hour opioid consumption was significantly higher in the no-drain group (2.0 mg/kg (SD 0.9) vs 1.4 (SD 0.6); p = 0.005). Two patients in the no-drain and one patient in the drain group developed a surgical site infection. Conclusion. Leaving the subfascial drain out after pedicle screw instrumentation for AIS is not associated with higher postoperative haemoglobin levels. Patients treated without a subfascial drain needed 30% more opioids during the first 48 hours than those who had a drain. Cite this article: Bone Joint J 2022;104-B(9):1067–1072

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. Methods. We reviewed the full medical records and radiological reports of patients referred to our hospital with a scoliosis of childhood between April 1972 and April 1982. Of 129 eligible patients with a juvenile or adolescent idiopathic scoliosis, 91 took part in the study (71%). They were evaluated with full-spine radiographs and HRQoL questionnaires and compared with normative data. We compared the HRQoL between observation (n = 27), bracing (n = 46), and surgical treatment (n = 18), and between thoracic and thoracolumbar/lumbar (TL/L) curves. Results. The mean time to follow-up was 40.8 years (SD 2.6) and the mean age of patients was 54.0 years (SD 2.7). Of the 91 patients, 86 were female (95%) and 51 had a main thoracic curve (53%). We found a significantly lower HRQoL measured on all the Scoliosis Research Society 22r instrument (SRS-22r) subdomains (p < 0.001) with the exception of mental health, than in an age-matched normal population. Incapacity to work was more prevalent in scoliosis patients (21%) than in the normal population (11%). The median SRS-22r subscore was 4.0 (interquartile range (IQR) 3.3 to 4.4) for TL/L curves and 4.1 (IQR 3.8 to 4.4) for thoracic curves (p = 0.300). We found a significantly lower self-image score for braced (median 3.6 (IQR 3.0 to 4.0)) and surgically treated patients (median 3.6 (IQR 3.2 to 4.3)) than for those treated by observation (median 4.0 (IQR 4.1 to 4.8); p = 0.010), but no statistically significant differences were found for the remaining subdomains. Conclusion. In this long-term follow-up study, we found a significantly decreased HRQoL and capacity to work in patients with an idiopathic scoliosis 40 years after diagnosis. Cite this article: Bone Joint J 2023;105-B(2):166–171

Aims. Idiopathic scoliosis is the most common spinal deformity in adolescents and children. The aetiology of the disease remains unknown. Previous studies have shown a lower bone mineral density in individuals with idiopathic scoliosis, which may contribute to the causation. The aim of the present study was to compare bone health in adolescents with idiopathic scoliosis with controls. Methods. We included 78 adolescents with idiopathic scoliosis (57 female patients) at a mean age of 13.7 years (8.5 to 19.6) and 52 age- and sex-matched healthy controls (39 female patients) at a mean age of 13.8 years (9.1 to 17.6). Mean skeletal age, estimated according to the Tanner-Whitehouse 3 system (TW3), was 13.4 years (7.4 to 17.8) for those with idiopathic scoliosis, and 13.1 years (7.4 to 16.5) for the controls. Mean Cobb angle for those with idiopathic scoliosis was 29° (SD 11°). All individuals were scanned with dual energy x-ray absorptiometry (DXA) and peripheral quantitative CT (pQCT) of the left radius and tibia to assess bone density. Statistical analyses were performed with independent-samples t-test, the Mann-Whitney U test, and the chi-squared test. Results. Compared with controls, adolescents with idiopathic scoliosis had mean lower DXA values in the left femoral neck (0.94 g/cm. 2. (SD 0.14) vs 1.00 g/cm. 2. (SD 0.15)), left total hip (0.94 g/cm. 2. (SD 0.14) vs 1.01 g/cm. 2. (SD 0.17)), L1 to L4 (0.99 g/cm. 2. (SD 0.15) vs 1.06 g/cm. 2. (SD 0.17)) and distal radius (0.35 g/cm. 2. (SD 0.07) vs 0.39 g/cm. 2. (SD 0.08; all p ≤ 0.024), but not in the mid-radius (0.72 g/cm. 2. vs 0.74 g/cm. 2. ; p = 0.198, independent t-test) and total body less head (1,559 g (SD 380) vs 1,649 g (SD 492; p = 0.0.247, independent t-test). Compared with controls, adolescents with idiopathic scoliosis had lower trabecular volume bone mineral density (BMD) on pQCT in the distal radius (184.7 mg/cm. 3. (SD 40.0) vs 201.7 mg/cm. 3. (SD 46.8); p = 0.029), but not in other parts of the radius or the tibia (p ≥ 0.062, Mann-Whitney U test). Conclusion. In the present study, idiopathic scoliosis patients seemed to have lower BMD at central skeletal sites and less evident differences at peripheral skeletal sites when compared with controls. Cite this article: Bone Joint J 2020;102-B(2):268–272

Aims. Spinal fusion remains the gold standard in the treatment of idiopathic scoliosis. However, anterior vertebral body tethering (AVBT) is gaining widespread interest, despite the limited data on its efficacy. The aim of our study was to determine the clinical efficacy of AVBT in skeletally immature patients with idiopathic scoliosis. Methods. All consecutive skeletally immature patients with idiopathic scoliosis treated with AVBT enrolled in a longitudinal, multicentre, prospective database between 2013 and 2016 were analyzed. All patients were treated by one of two surgeons working at two independent centres. Data were collected prospectively in a multicentre database and supplemented retrospectively where necessary. Patients with a minimum follow-up of two years were included in the analysis. Clinical success was set a priori as a major coronal Cobb angle of < 35° at the most recent follow-up. Results. A total of 57 patients were included in the study. Their mean age was 12.7 years (SD 1.5; 8.2 to 16.7), with 95% being female. The mean preoperative Sanders score and Risser grade was 3.3 (SD 1.2), and 0.05 (0 to 3), respectively. The majority were thoracic tethers (96.5%) and the mean follow-up was 40.4 months (SD 9.3). The mean preoperative major curve of 51° (SD 10.9°; 31° to 81°) was significantly improved to a mean of 24.6° (SD 11.8°; 0° to 57°) at the first postoperative visit (45.6% (SD 17.6%; 7% to 107%); p < 0.001)) with further significant correction to a mean of 16.3° (SD 12.8°; -12 to 55; p < 0.001) at one year and a significant correction to a mean of 23° (SD 15.4°; -18° to 57°) at the final follow-up (42.9% (-16% to 147%); p < 0.001). Clinical success was achieved in 44 patients (77%). Most patients reached skeletal maturity, with a mean Risser score of 4.3 (SD 1.02), at final follow-up. The complication rate was 28.1% with a 15.8% rate of unplanned revision procedures. Conclusion. AVBT is associated with satisfactory correction of deformity and an acceptable complication rate when used in skeletally immature patients with idiopathic scoliosis. Improved patient selection and better implant technology may improve the 15.8% rate of revision surgery in these patients. Further scrutiny of the true effectiveness and long-term risks of this technique remains critical. Cite this article: Bone Joint J 2020;102-B(12):1703–1708

Aims. 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. Patients and Methods. 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. Results. 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%). Conclusion. 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

Aims. The health-related quality of life (HRQoL) of paediatric patients with orthopaedic conditions and spinal deformity is important, but existing generic tools have their shortcomings. We aim to evaluate the use of Paediatric Quality of Life Inventory (PedsQL) 4.0 generic core scales in the paediatric population with specific comparisons between those with spinal and limb pathologies, and to explore the feasibility of using PedsQL for studying scoliosis patients’ HRQoL. Methods. Paediatric patients attending a speciality outpatient clinic were recruited through consecutive sampling. Two groups of patients were included: idiopathic scoliosis, and paediatric orthopaedic upper and lower limb condition without scoliosis. Patients were asked to complete PedsQL 4.0 generic core scales, Youth version of 5-level EuroQol-5-dimension questionnaire, and Refined Scoliosis Research Society 22-item (SRS-22r) questionnaire. Statistical analyses included scores comparison between scoliosis and limb pathology patients using independent-samples t-test, and correlation tests of PedsQL and SRS-22r. Results. A total of 566 paediatric patients were recruited: 357 (63.0%) having idiopathic scoliosis, and 209 (37.0%) with limb conditions. Patients with limb pathology had lower functioning scale, summary, and total scores of PedsQL than scoliosis patients (p < 0.05 to p < 0.001). No floor or ceiling effects (< 15%) were detected for PedsQL Psychosocial Health Summary and total scores in both groups. PedsQL was sensitive in differentiating patients with/without problems in their daily lives (p < 0.05 to p < 0.01). PedsQL summary and total scores correlated with SRS-22r total score. Conclusion. PedsQL is an effective HRQoL measure for both paediatric orthopaedic groups with minimal ceiling and floor effects, and is capable of detecting worse HRQoL in patients with limb pathology. The multidimensional PedsQL is sensitive in differentiating among those with daily life problems, especially for scoliosis patients. Cite this article: Bone Joint J 2020;102-B(7):890–898

Aims. The aim of this study was to review the current evidence surrounding curve type and morphology on curve progression risk in adolescent idiopathic scoliosis (AIS). Methods. A comprehensive search was conducted by two independent reviewers on PubMed, Embase, Medline, and Web of Science to obtain all published information on morphological predictors of AIS progression. Search items included ‘adolescent idiopathic scoliosis’, ‘progression’, and ‘imaging’. The inclusion and exclusion criteria were carefully defined. Risk of bias of studies was assessed with the Quality in Prognostic Studies tool, and level of evidence for each predictor was rated with the Grading of Recommendations, Assessment, Development and Evaluations (GRADE) approach. In all, 6,286 publications were identified with 3,598 being subjected to secondary scrutiny. Ultimately, 26 publications (25 datasets) were included in this review. Results. For unbraced patients, high and moderate evidence was found for Cobb angle and curve type as predictors, respectively. Initial Cobb angle > 25° and thoracic curves were predictive of curve progression. For braced patients, flexibility < 28% and limited in-brace correction were factors predictive of progression with high and moderate evidence, respectively. Thoracic curves, high apical vertebral rotation, large rib vertebra angle difference, small rib vertebra angle on the convex side, and low pelvic tilt had weak evidence as predictors of curve progression. Conclusion. For curve progression, strong and consistent evidence is found for Cobb angle, curve type, flexibility, and correction rate. Cobb angle > 25° and flexibility < 28% are found to be important thresholds to guide clinical prognostication. Despite the low evidence, apical vertebral rotation, rib morphology, and pelvic tilt may be promising factors. Cite this article: Bone Joint J 2022;104-B(4):424–432

Aims. This study addressed two questions: first, does surgical correction of an idiopathic scoliosis increase the volume of the rib cage, and second, is it possible to evaluate the change in lung function after corrective surgery for adolescent idiopathic scoliosis (AIS) using biplanar radiographs of the ribcage with 3D reconstruction?. Methods. A total of 45 patients with a thoracic AIS which needed surgical correction and fusion were included in a prospective study. All patients underwent pulmonary function testing (PFT) and low-dose biplanar radiographs both preoperatively and one year after surgery. The following measurements were recorded: forced vital capacity (FVC), slow vital capacity (SVC), and total lung capacity (TLC). Rib cage volume (RCV), maximum rib hump, main thoracic curve Cobb angle (MCCA), medial-lateral and anteroposterior diameter, and T4-T12 kyphosis were calculated from 3D reconstructions of the biplanar radiographs. Results. All spinal and thoracic measurements improved significantly after surgery (p < 0.001). RCV increased from 4.9 l (SD 1) preoperatively to 5.3 l (SD 0.9) (p < 0.001) while TLC increased from 4.1 l (SD 0.9) preoperatively to 4.3 l (SD 0.8) (p < 0.001). RCV was correlated with all functional indexes before and after correction of the deformity. Improvement in RCV was weakly correlated with correction of the mean thoracic Cobb angle (p = 0.006). The difference in TLC was significantly correlated with changes in RCV (p = 0.041). It was possible to predict postoperative TLC from the postoperative RCV. Conclusion. 3D rib cage assessment from biplanar radiographs could be a minimally invasive method of estimating pulmonary function before and after spinal fusion in patients with an AIS. The 3D RCV reflects virtual chest capacity and hence pulmonary function in this group of patients. Cite this article: Bone Joint J 2022;104-B(1):112–119

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). Methods. We retrospectively reviewed all AIS patients who had undergone PSF between January 2018 and January 2020 in a specialist tertiary spinal referral centre. All patients were assessed preoperatively in an anaesthetic clinic. Postoperative care was defined as ward-based (WB) or critical care (CC), based on the preoperative FEV1, FVC, major curve Cobb angle, and the planned number of instrumented levels. Results. Overall, 105 patients were enrolled. Their mean age was 15.5 years (11 to 25) with a mean weight of 55 kg (35 to 103). The mean Cobb angle was 68° (38° to 122°). Of these, 38 patients were preoperatively scored to receive postoperative CC. However, only 19% of the cohort (20/105) actually needed CC-level support. Based on these figures, and an average paediatric intensive care unit stay of one day before stepdown to ward-based care, the potential cost-saving on the first postoperative night for this cohort was over £20,000. There was no statistically significant difference between the Total Pathway Score (TPS), the numerical representation of the four factors being assessed, and the actual level of care received (p = 0.052) or the American Society of Anesthesiologists grade (p = 0.187). Binary logistic regression analysis of the TPS variables showed that the preoperative Cobb angle was the only variable which significantly predicted the need for critical care. Conclusion. Most patients undergoing posterior fusion surgery for AIS do not need critical care. Of the readily available preoperative measures, the Cobb angle is the only predictor of the need for higher levels of care, and has a threshold value of 74.5°. Cite this article: Bone Joint J 2024;106-B(7):713–719

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. Methods. AIS patients were systematically enrolled in an institutional registry. In all, 144 AIS patients aged ≤ 25 years undergoing primary surgery (median age 15 years (interquartile range 14 to 17) were included. Patient-reported (condition-specific and health-related quality of life (QoL); functional status; back and leg pain intensity) and clinician-reported outcomes (complications, revision surgery) were recorded. Changes in patient-reported outcome measures (PROMs) were analyzed using Friedman’s analysis of variance. Clinical relevancy was determined using minimally important changes (Scoliosis Research Society (SRS)-22r), cut-off values for relevant effect on functioning (pain scores) and a patient-acceptable symptom state (PASS; Oswestry Disability Index). Results. At baseline, 65 out of 144 patients (45%) reported numerical rating scale (NRS) back pain scores > 5. All PROMs significantly improved at two-year follow-up. Mean improvements in SRS-22r function (+ 1.2 (SD 0.6)), pain (+ 0.6 (SD 0.8)), and self-image (+ 1.1 (SD 0.7)) domain scores, and the SRS-22r total score (+ 0.5 (SD 0.5)), were clinically relevant. At two-year follow-up, 14 out of 144 patients (10%) reported NRS back pain > 5. Surgical site infections did not occur. Only one patient (0.7%) underwent revision surgery. Conclusion. Relevant improvement in functioning, condition-specific and health-related QoL, self-image, and a relevant decrease in pain is shown at two-year follow-up after fusion surgery for AIS, with few adverse events. Contrary to the general perception that AIS is a largely asymptomatic condition, nearly half of patients report significant preoperative back pain, which reduced to 10% at two-year follow-up. Cite this article: Bone Joint J 2022;104-B(2):265–273

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

The scoliosis observed in chickens after pinealectomy resembles that seen in humans with an adolescent idiopathic scoliosis, suggesting that melatonin deficiency may be responsible. However, to date there have been no studies of pineal gland glucose metabolism in patients with adolescent idiopathic scoliosis that might support this hypothesis. We examined the excretion of urinary 6-sulfatoxyl-melatonin as well as the glucose metabolism of the pineal gland in 14 patients with an adolescent idiopathic scoliosis and compared them with those of 13 gender-matched healthy controls using F-18 fluorodeoxyglucose brain positron emission tomography. There was no significant difference in the level of urinary 6-sulfatoxyl-melatonin or pineal gland metabolism between the study and the control group. We conclude that permanent melatonin deficiency is not a causative factor in the aetiology of adolescent idiopathic scoliosis

Aims. There is little information about the optimum number of implants to be used in the surgical treatment of idiopathic scoliosis. Retrospective analysis of prospectively collected data from the Swedish spine register was undertaken to discover whether more implants per operated vertebra (implant density) leads to a better outcome in the treatment of idiopathic scoliosis. The hypothesis was that implant density is not associated with patient-reported outcomes, the correction of the curve or the rate of reoperation. Patients and Methods. A total of 328 patients with idiopathic scoliosis, aged between ten and 20 years at the time of surgery, were identified in the Swedish spine register (Swespine) and had patient reported outcomes including the Scoliosis Research Society 22r instrument (SRS-22r) score, EuroQol 5 dimensions quality of life, 3 level (EQ-5D-3L) score and a Viual Analogue Score (VAS) for back pain, at a mean follow-up of 3.1 years and reoperation data at a mean follow-up of 5.5 years. Implant data and the correction of the curve were assessed from radiographs, preoperatively and a mean of 1.9 years postoperatively. The patients were divided into tertiles based on implant density. Data were analyzed with analysis of variance, logistic regression or log-rank test. Some analyses were adjusted for gender, age at the time of surgery, the flexibility of the major curve and follow-up. Results. The mean number of implants per operated vertebra in the low, medium and high-density groups were 1.36 (1.00 to 1.54), 1.65 (1.55 to 1.75) and 1.91 (1.77 to 2.00), respectively. There were no statistically significant differences in the correction of the curve, the SRS-22r total score, EQ-5D-3L index or number of reoperations between the groups (all p > 0.34). In the SRS-22r domains, self-image was marginally higher in the medium implant density group (p = 0.029) and satisfaction marginally higher in the high implant density group (p = 0.034). Conclusion. These findings suggest that there is no clear advantage in using a high number of implants per operated vertebra in the surgical treatment of patients with idiopathic scoliosis. Cite this article: Bone Joint J 2018;100-B:1080–6

Aims. To compare the rates of sagittal and coronal correction for all-pedicle screw instrumentation and hybrid instrumentation using sublaminar bands in the treatment of thoracic adolescent idiopathic scoliosis (AIS). Methods. We retrospectively reviewed the medical records of 124 patients who had undergone surgery in two centres for the correction of Lenke 1 or 2 AIS. Radiological evaluation was carried out preoperatively, in the early postoperative phase, and at two-year follow-up. Parameters measured included coronal Cobb angles and thoracic kyphosis. Postoperative alignment was compared after matching the cohorts by preoperative coronal Cobb angle, thoracic kyphosis, lumbar lordosis, and pelvic incidence. Results. A total of 179 patients were available for analysis. After matching, 124 patients remained (62 in each cohort). Restoration of thoracic kyphosis was significantly better in the sublaminar band group than in the pedicle screw group (from 23.7° to 27.5° to 34.0° versus 23.9° to 18.7° to 21.5°; all p < 0.001). When the preoperative thoracic kyphosis was less than 20°, sublaminar bands achieved a normal postoperative thoracic kyphosis, whereas pedicle screws did not. In the coronal plane, pedicle screws resulted in a significantly better correction than sublaminar bands at final follow-up (73.0% versus 59.7%; p < 0.001). Conclusion. This is the first study to compare sublaminar bands and pedicle screws for the correction of a thoracic AIS. We have shown that pedicle screws give a good coronal correction which is maintained at two-year follow-up. Conversely, sublaminar bands restore the thoracic kyphosis better while pedicle screws are associated with a flattening of the thoracic spine. In patients with preoperative hypokyphosis, sublaminar bands should be used to restore a proper sagittal profile. Cite this article: Bone Joint J 2020;102-B(3):376–382

Aims. The aim of this retrospective study was to compare the correction achieved using a convex pedicle screw technique and a low implant density achieved using periapical concave-sided screws and a high implant density. We hypothesized that there would be no difference in outcome between the two techniques. Methods. We retrospectively analyzed a series of 51 patients with a thoracic adolescent idiopathic scoliosis. There were 26 patients in the convex pedicle screw group who had screws implanted periapically (Group 2) and a control group of 25 patients with bilateral pedicle screws (Group 1). The patients’ charts were reviewed and pre- and postoperative radiographs evaluated. Postoperative patient-reported outcome measures (PROMs) were recorded. Results. The number of implants (14.5 vs 17.1) and the implant density (1.5 vs 1.9) were significantly lower in Group 2 (p < 0.001). Operating time was 27 minutes shorter in Group 2 than in Group 1, with a mean of 217 minutes (SD 50.5; 120 to 346). The duration of surgery per instrumented vertebra was reduced by 19% in Group 2 (p = 0.011). No statistical difference was found in the postoperative Cobb angle, vertebral rotation, the relative correction achieved, or postoperative PROMs. Conclusion. Despite a lower implant density and achieving correction through a convex rod, surgical correction of the Cobb angle and vertebral body rotation was similar in both groups. Periapical pedicle screws and primary correction on the concave side do not seem to be mandatory in order to achieve good surgical results in idiopathic thoracic scoliosis. The operating time was shorter in the group with lower implant density. In conclusion, the technique provided good results and has the potential to reduce complications and costs. Cite this article: Bone Joint J 2021;103-B(3):536–541

Aims. The purpose of this study was to evaluate the incidence and analyze the trends of surgeon-reported complications following surgery for adolescent idiopathic scoliosis (AIS) over a 13-year period from the Scoliosis Research Society (SRS) Morbidity and Mortality database. Methods. All patients with AIS between ten and 18 years of age, entered into the SRS Morbidity and Mortality database between 2004 and 2016, were analyzed. All perioperative complications were evaluated for correlations with associated factors. Complication trends were analyzed by comparing the cohorts between 2004 to 2007 and 2013 to 2016. Results. Between 2004 and 2016, a total of 84,320 patients were entered into the database. There were 1,268 patients associated with complications, giving an overall complication rate of 1.5%. Death occurred in 12 patients (0.014%). The three most commonly reported complications were surgical site infection (SSI) (441 patients; 0.52%), new neurological deficit (293; 0.35%), and implant-related complications (172; 0.20%). There was a statistically significant but weak correlation between the occurrence of a SSI and the magnitude of the primary curve (r = 0.227; p < 0.001), and blood loss in surgery (r = 0.111; p = 0.038), while the occurrence of a new neurological deficit was correlated statistically significantly but weakly with age at surgery (r = 0.147; p = 0.004) and magnitude of the primary curve (r = 0.258; p < 0.001). The overall complication rate decreased from 4.95% during 2004 to 2007 to 0.98% during 2013 to 2016 (p = 0.023). Conclusion. An overall complication rate of 1.5% was found in our series after surgery for AIS, with a reduction of complication rates found in the second period of the analysis. Cite this article: Bone Joint J 2020;102-B(4):519–523

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. Closed suction subfascial drainage is widely used after instrumented posterior spinal fusion in patients with a spinal deformity. The aim of this study was to determine the effect of this wound drainage on the outcomes in patients with adolescent idiopathic scoliosis (AIS). This was a further analysis of a randomized, multicentre clinical trial reporting on patients after posterior spinal fusion using segmental pedicle screw instrumentation. In this study the incidence of deep surgical site infection (SSI) and chronic postoperative pain at two years’ follow-up are reported. Methods. We conducted a randomized, multicentre clinical trial on adolescents undergoing posterior spinal fusion for AIS using segmental pedicle screw instrumentation. A total of 90 consecutive patients were randomized into a ‘drain’ or ‘no drain’ group at the time of wound closure, using the sealed envelope technique (1:1). The primary outcomes in the initial study were the change in the level of haemoglobin in the blood postoperatively and total blood loss. A secondary outcome was the opioid consumption immediately after surgery. The aim of this further study was to report the rate of deep SSI and persistent postoperative pain, at two years' follow-up. Results. As previously reported, the mean 48-hour opioid consumption was significantly higher in the no drain group (2.0 mg/kg (SD 0.8) vs 1.4 mg/kg (SD 0.7); p = 0.005). There were no delayed deep SSIs. At two years’ follow-up, the mean Scoliosis Research Society 24-item questionnaire (SRS-24) total score did not differ between the groups (4.11 (SD 0.47) vs 4.16 (SD 0.33); p = 0.910). The mean SRS-24 pain score was 4.28 (SD 0.48) in the no drain group compared with 4.33 (SD 0.66) in the drain group (p = 0.245). Seven patients (19%) in the no drain group and six in the drain group (14%) reported moderate to severe pain (numerical rating scale ≥ 4) at two years’ follow-up (p = 0.484). The general self-image domain score was significantly higher in the no drain group compared with the drain group (4.02 (SD 0.74) vs 4.59 (SD 0.54); p < 0.001). Conclusion. The main finding in this study was that more severe pain immediately after surgery did not result in a higher incidence of chronic pain in the no drain group compared with the drain group. Back pain and health-related quality of life at two years’ follow-up did not differ between the groups. The patient-reported self-image domain was significantly better in the no drain group compared with the drain group. Cite this article: Bone Joint J 2024;106-B(10):1176–1181

We studied 70 consecutive patients with adolescent idiopathic scoliosis who underwent corrective surgery. They were divided into two groups. In the study group of 38 patients one or more modern blood-conservation measures was used peri-operatively. The 32 patients in the control group did not have these measures. Both groups were similar in regard to age, body-weight, the number of levels fused and the type of surgery. Only two patients in the study group were transfused with homologous blood and these transfusions were ‘off-protocol’. Wastage of autologous pre-donated units was minimal (6 of 83 units). By contrast, all patients in the control group were transfused with homologous blood. In the study group there was a significant decrease (p = 0.005) in the estimated blood loss when all the blood-conservation methods were used. The use of blood-conservation measures, the lowering of the haemoglobin trigger for transfusion and the education of the entire team involved in the care of the patient can prevent the need for homologous blood transfusion in patients undergoing surgery for adolescent idiopathic scoliosis

The changing incidence of idiopathic scoliosis in 672 patients who attended the Edinburgh Scoliosis Clinic between 1968 and 1982 inclusive is reported. Of these patients, 144 had infantile, 51 juvenile and 477 adolescent idiopathic scoliosis. Thirty-seven of the infantile curves progressed and 107 resolved. The relative frequency of both progressive and resolving infantile idiopathic scoliosis (given as a percentage of the total number of patients with idiopathic scoliosis) declined from 41.75 per cent in the four years from 1968 to 1971, to four per cent in the three years from 1980 to 1982. It is suggested that infantile idiopathic scoliosis is a preventable deformity and that the position in which the infant is laid may be a causative factor

Aims. In a multicentre, randomised study of adolescents undergoing posterior spinal fusion for idiopathic scoliosis, we investigated the effect of adding gelatine matrix with human thrombin to the standard surgical methods of controlling blood loss. Patients and Methods. Patients in the intervention group (n = 30) were randomised to receive a minimum of two and a maximum of four units of gelatine matrix with thrombin in addition to conventional surgical methods of achieving haemostasis. Only conventional surgical methods were used in the control group (n = 30). We measured the intra-operative and total blood loss (intra-operative blood loss plus post-operative drain output). Results. Each additional hour of operating time increased the intra-operative blood loss by 356.9 ml (p < 0.001) and the total blood loss by 430.5 ml (p < 0.001). Multiple linear regression analysis showed that the intervention significantly decreased the intra-operative (-171 ml, p = 0.025) and total blood loss (-177 ml, p = 0.027). The decrease in haemoglobin concentration from the day before the operation to the second post-operative day was significantly smaller in the intervention group (-6 g/l, p = 0.013) than in the control group. . Conclusion. The addition of gelatine matrix with human thrombin to conventional methods of achieving haemostasis reduces both the intra-operative blood loss and the decrease in haemoglobin concentration post-operatively in adolescents undergoing posterior spinal fusion for idiopathic scoliosis. Take home message: A randomised clinical trial showed that gelatine matrix with human thrombin decreases intra-operative blood loss by 30% when added to traditional surgical haemostatic methods in adolescents undergoing posterior spinal fusion for idiopathic scoliosis. Cite this article: Bone Joint J 2016;98-B:395–401

Scoliosis seen in the chicken after pinealectomy resembles adolescent idiopathic scoliosis in man. It has been suggested that in both species, deficiency of the pineal hormone, melatonin, is responsible for this phenomenon. In nine patients with adolescent idiopathic scoliosis and in ten age- and gender-matched controls, the circadian levels of serum melatonin and the excretion of urinary 6-hydroxy-melatonin-sulphate, the principal metabolite of melatonin, were determined. There were no statistically significant differences in the secretion of serum melatonin or the excretion of urinary 6-hydroxy-melatonin-sulphate between the patients and the control group. The hypothesis of melatonin deficiency as a causative factor in the aetiology of adolescent idiopathic scoliosis cannot be supported by our data

Aims

This study aimed to evaluate rasterstereography of the spine as a diagnostic test for adolescent idiopathic soliosis (AIS), and to compare its results with those obtained using a scoliometer.

Recent clinical studies have suggested that a neurological lesion may be a cause of adolescent idiopathic scoliosis and animal experiments have implicated the posterior column pathway. We have tried to determine if differences in neurological response could be detected and measured clinically, and have compared the threshold of detection of vibratory sensation in 20 girls with adolescent idiopathic scoliosis with that in 20 clinically normal age-matched controls. A highly significant reduction of the threshold of detection of vibration was seen in the scoliotic group compared to the controls (p less than 0.001). Curve magnitude did not correlate with this threshold for either the upper (r = 0.172) or lower extremity (r = 0.126). Significant asymmetry between right- and left-sided thresholds to vibration was demonstrated in the scoliotic group. Our study supports the concept that an aberration in the function of the posterior column pathway of the cord may be of primary importance in the aetiology of idiopathic scoliosis. A clinically practical test to measure this function is presented

We investigated 21 pairs of twins for zygosity and idiopathic scoliosis. DNA fingerprinting confirmed that 13 pairs were monozygotic and eight were dizygotic. There was concordance for idiopathic scoliosis in 92.3% of monozygotic and 62.5% of dizygotic twins. Of the 12 pairs of monozygotic twins concordant for idiopathic scoliosis, six showed discordant curve patterns but eight had differences in Cobb angle of less than 10°. Seven of the ten pairs of monozygotic twins had similar back shapes. Our findings suggest that there is a genetic factor in the aetiology of idiopathic scoliosis; they also indicate that there is a genetic factor in both the severity of the curve and the general shape of the back

Aims. The aim of this study was to determine the influence of pelvic parameters on the tendency of patients with adolescent idiopathic scoliosis (AIS) to develop flatback deformity (thoracic hypokyphosis and lumbar hypolordosis) and its effect on quality-of-life outcomes. Patients and Methods. This was a radiological study of 265 patients recruited for Boston bracing between December 2008 and December 2013. Posteroanterior and lateral radiographs were obtained before, immediately after, and two-years after completion of bracing. Measurements of coronal and sagittal Cobb angles, coronal balance, sagittal vertical axis, and pelvic parameters were made. The refined 22-item Scoliosis Research Society (SRS-22r) questionnaire was recorded. Association between independent factors and outcomes of postbracing ≥ 6° kyphotic changes in the thoracic spine and ≥ 6° lordotic changes in the lumbar spine were tested using likelihood ratio chi-squared test and univariable logistic regression. Multivariable logistic regression models were then generated for both outcomes with odds ratios (ORs), and with SRS-22r scores. Results. Reduced T5-12 kyphosis (mean -4.3° (. sd. 8.2); p < 0.001), maximum thoracic kyphosis (mean -4.3° (. sd. 9.3); p < 0.001), and lumbar lordosis (mean -5.6° (. sd. 12.0); p < 0.001) were observed after bracing treatment. Increasing prebrace maximum kyphosis (OR 1.133) and lumbar lordosis (OR 0.92) was associated with postbracing hypokyphotic change. Prebrace sagittal vertical axis (OR 0.975), prebrace sacral slope (OR 1.127), prebrace pelvic tilt (OR 0.940), and change in maximum thoracic kyphosis (OR 0.878) were predictors for lumbar hypolordotic changes. There were no relationships between coronal deformity, thoracic kyphosis, or lumbar lordosis with SRS-22r scores. Conclusion. Brace treatment leads to flatback deformity with thoracic hypokyphosis and lumbar hypolordosis. Changes in the thoracic spine are associated with similar changes in the lumbar spine. Increased sacral slope, reduced pelvic tilt, and pelvic incidence are associated with reduced lordosis in the lumbar spine after bracing. Nevertheless, these sagittal parameter changes do not appear to be associated with worse quality of life. Cite this article: Bone Joint J 2019;101-B:1370–1378

Adolescent idiopathic scoliosis (AIS) is a complex 3D deformity of the spine. Its prevalence is between 2% and 3% in the general population, with almost 10% of patients requiring some form of treatment and up to 0.1% undergoing surgery. The cosmetic aspect of the deformity is the biggest concern to the patient and is often accompanied by psychosocial distress. In addition, severe curves can cause cardiopulmonary distress. With proven benefits from surgery, the aims of treatment are to improve the cosmetic and functional outcomes. Obtaining correction in the coronal plane is not the only important endpoint anymore. With better understanding of spinal biomechanics and the long-term effects of multiplanar imbalance, we now know that sagittal balance is equally, if not more, important. Better correction of deformities has also been facilitated by an improvement in the design of implants and a better understanding of metallurgy. Understanding the unique character of each deformity is important. In addition, using the most appropriate implant and applying all the principles of correction in a bespoke manner is important to achieve optimum correction. In this article, we review the current concepts in AIS surgery. Cite this article: Bone Joint J 2018;100-B:415–24

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 this study was to report a retrospective, consecutive series of patients with adolescent idiopathic scoliosis (AIS) who were treated with posterior minimally invasive surgery (MIS) with a mean follow-up of two years (. sd. 1.4; 0.9 to 0 3.7). Our objectives were to measure the correction of the deformity and record the peri-operative morbidity. Special attention was paid to the operating time (ORT), estimated blood loss (EBL), length of stay (LOS) and further complications. Patients and Methods. We prospectively collected the data of 70 consecutive patients with AIS treated with MIS using three incisions and a muscle-splitting approach by a single surgeon between June 2013 and February 2016 and these were retrospectively reviewed. There were eight male and 62 female patients with a mean age of 15 years (. sd. 4.5 ) with a mean body mass index of 19.8 kg/m. 2. (. sd. 5.4). The curves were classified according to Lenke; 40 curves were type 1, 15 were type 2, three were type 3, two were type 4, eight were type 5 and two were type 6. Results. The mean primary Cobb angle was corrected from 58.9° (. sd. 12.6°) pre-operatively to 17.7° (. sd.  10.2°) post-operatively with a mean correction of 69% (. sd. 20%, p < 0.001). The mean kyphosis at T5 to T12 increased from 24.2° (. sd. 12.2°) pre-operatively to 30.1° (. sd. 9.6°, p < 0.001) post-operatively. Peri-operative (30 days) complications occurred in three patients(4.2%): one subcutaneous haematoma, one deep venous thrombosis and one pulmonary complication. Five additional complications occurred in five patients (7.1%): one superficial wound infection, one suture granuloma and three delayed deep surgical site infections. The mean ORT was 337.1 mins (. sd. 121.3); the mean EBL was 345.7 ml (. sd. 175.1) and the mean LOS was 4.6 days (. sd. 0.8). Conclusion. The use of MIS for patients with AIS results in a significant correction of spinal deformity in both the frontal and sagittal planes, with a low EBL and a short LOS. The rate of peri-operative complications compares well with that following a routine open technique. The longer term safety and benefit of MIS in these patients needs to be evaluated with further follow-up of a larger cohort of patients. Cite this article: Bone Joint J 2017;99-B:1651–7

A clinical, cadaveric, biomechanical and radiological investigation of the pathogenesis of idiopathic scoliosis indicates that biplanar asymmetry is the essential lesion. Many normal children have coronal plane asymmetry (an inconsequential lateral curvature of the spine), and certainly all have vertebral body asymmetry in the transverse plane, but when median plane asymmetry (flattening or more usually reversal of the normal thoracic kyphosis at the apex of the scoliosis) is superimposed during growth, a progressive idiopathic scoliosis occurs. Idiopathic kyphoscoliosis cannot and does not exist, from the mildest cases in the community to the most severe cases in pathology museums. Median plane asymmetry is crucial for progression and the lateral profile of the spine must be carefully scrutinised. Increased anterior vertebral height at the apex of the curve with posterior end-plate irregularity characterises the median plane asymmetry and suggests that idiopathic scoliosis is the reverse of Scheuermann's disease

Aims. The direct posterior approach with subperiosteal dissection of the paraspinal muscles from the vertebrae is considered to be the standard approach for the surgical treatment of adolescent idiopathic scoliosis (AIS). We investigated whether or not a minimally-invasive surgery (MIS) technique could offer improved results. Methods. Consecutive AIS patients treated with an MIS technique at two tertiary centres from June 2013 to March 2016 were retrospectively included. Preoperative patient deformity characteristics, perioperative parameters, power of deformity correction, and complications were studied. A total of 93 patients were included. The outcome of the first 25 patients and the latter 68 were compared as part of our safety analysis to examine the effect of the learning curve. Results. In the first 25 cases, with a mean follow-up of 5.6 years (standard deviation (SD) 0.4), the mean preoperative major Cobb angle was 57.6° (SD 9.8°) and significantly corrected to mean 15.4° (SD 5.6°, 73% curve correction). The mean preoperative T5-T12 was 26.2 (SD 12.8) and significantly increased to mean 32.9 (SD 8.3). Both frontal and sagittal plane correction was conserved two years after surgery. The rate of perioperative complications was 12% and three further complications occurred (three deep delayed infection). In the latter cases, 68 patients were included with a mean follow-up time of three years (SD 0.6). The mean preoperative major Cobb angle was 58.4° (SD 9.2°) and significantly corrected to mean 20.4° (SD 7.3°).The mean preoperative T5-T12 kyphosis was 26.6° (SD 12.8°) and was significantly increased to mean 31.4° (SD 8.3°). Both frontal and sagittal correction was conserved two years after surgery. The perioperative (30 day) complication rate was 1.4%. Two (2.9%) additional complications occurred in two patients. Conclusion. MIS for AIS is associated with a significant correction of spine deformity in the frontal and sagittal planes, together with low estimated blood loss and short length of stay. The perioperative complication rate seems to be lower compared with the standard open technique based on the literature data. The longer-term safety of MIS for AIS needs to be documented with a larger cohort and compared with the standard posterior approach. Cite this article: Bone Joint J 2020;102-B(4):506–512

Aims. The aim of this study was to assess whether supine flexibility predicts the likelihood of curve progression in patients with adolescent idiopathic scoliosis (AIS) undergoing brace treatment. Methods. This was a retrospective analysis of patients with AIS prescribed with an underarm brace between September 2008 to April 2013 and followed up until 18 years of age or required surgery. Patients with structural proximal curves that preclude underarm bracing, those who were lost to follow-up, and those who had poor compliance to bracing (<16 hours a day) were excluded. The major curve Cobb angle, curve type, and location were measured on the pre-brace standing posteroanterior (PA) radiograph, supine whole spine radiograph, initial in-brace standing PA radiograph, and the post-brace weaning standing PA radiograph. Validation of the previous in-brace Cobb angle regression model was performed. The outcome of curve progression post-bracing was tested using a logistic regression model. The supine flexibility cut-off for curve progression was analyzed with receiver operating characteristic curve. Results. A total of 586 patients with mean age of 12.6 years (SD 1.2) remained for analysis after exclusion. The baseline Cobb angle was similar for thoracic major curves (31.6° (SD 3.8°)) and lumbar major curves (30.3° (SD 3.7°)). Curve progression was more common in the thoracic curves than lumbar curves with mean final Cobb angles of 40.5° (SD 12.5°) and 31.8° (SD 9.8°) respectively. This dataset matched the prediction model for in-brace Cobb angle with less mean absolute error in thoracic curves (0.61) as compared to lumbar curves (1.04). Reduced age and Risser stage, thoracic curves, increased pre-brace Cobb angle, and reduced correction and flexibility rates predicted increased likelihood of curve progression. Flexibility rate of more than 28% has likelihood of preventing curve progression with bracing. Conclusion. Supine radiographs provide satisfactory prediction for in-brace correction and post-bracing curve magnitude. The flexibility of the curve is a guide to determine the likelihood for brace success. Cite this article: Bone Joint J 2020;102-B(2):254–260

Adolescent idiopathic scoliosis affects about 3% of children. Non-operative measures are aimed at altering the natural history to maintain the size of the curve below 40° at skeletal maturity. The application of braces to treat spinal deformity pre-dates the era of evidence-based medicine, and there is a paucity of irrefutable prospective evidence in the literature to support their use and their effectiveness has been questioned. This review considers this evidence. The weight of the evidence is in favour of bracing over observation. The most recent literature has moved away from addressing this question, and instead focuses on developments in the design of braces and ways to improve compliance. Cite this article: Bone Joint J 2013;95-B:1308–16

We undertook a radiographic analysis with pre-operative computed tomographic myelography in 78 patients with idiopathic scoliosis in order to analyse rotation of the spinal cord and to investigate its clinical significance. The angle of rotation of the cord had a statistically significant relationship to both that of the apical vertebra and the size of the primary curve. The relationship between the rotation of the cord and that of the apical vertebra was divided into three types. The cord rotated in the same direction as the apical vertebra in 55 patients and rotated in the opposite direction in the remaining 23 patients. In the first group, the angle of rotation of the cord was more than that of the vertebra in six patients, but less than it in 49 patients. These results suggest that the neuraxis in idiopathic scoliosis may be under tension in the axial dimension

Ultrasound can be used to outline the spinous processes and the laminae, and thus to measure axial rotation. Using our own technique, we measured vertebral rotation in 47 patients with idiopathic scoliosis. There was a strong linear relationship between the Cobb angle and the rotation of the apical vertebra in untreated patients, but this relationship was lost in patients who had had brace treatment. Vertebral rotation can easily be measured by ultrasound. This is a harmless and fairly rapid investigation which can be used at routine follow-up examination of patients with idiopathic scoliosis

Of 42 patients with resolvingscoliosis, 34 were followed up for more than 25 infantile idiopathicyears. Twenty had been primarily treated in a plaster bed and 14 by physiotherapy. The mean angle of the curve at presentation was 17° and at follow-up it was 5°. No patient had significant progression of the scoliosis during the growth spurt. When adults few had back pain or an increased disability score and there was no interference with work or social activities. The rib-vertebra angle difference proved to be an essential radiological sign when distinguishing resolving from progressive infantile idiopathic scoliosis. There was no advantage of plaster over physiotherapy with regard to either the time to resolution or the functional outcome. Treatment of resolving infantile idiopathic scoliosis in a plaster bed is therefore now outdated

Aims. The aim of this study was to investigate the impact of maturity status at the time of surgery on final spinal height in patients with an adolescent idiopathic scoliosis (AIS) using the spine-pelvic index (SPI). The SPI is a self-control ratio that is independent of age and maturity status. Patients and Methods. The study recruited 152 female patients with a Lenke 1 AIS. The additional inclusion criteria were a thoracic Cobb angle between 45° and 70°, Risser 0 to 1 or 3 to 4 at the time of surgery, and follow-up until 18 years of age or Risser stage 5. The patients were stratified into four groups: Risser 0 to 1 and selective fusion surgery (Group 1), Risser 0 to 1 and non-selective fusion (Group 2), Risser 3 to 4 and selective fusion surgery (Group 3), and Risser 3 to 4 and non-selective fusion (Group 4). The height of spine at follow-up (HOS. f. ) and height of pelvis at follow-up (HOP. f. ) were measured and the predicted HOS (pHOS) was calculated as 2.22 (SPI) × HOP. f. One-way analysis of variance (ANOVA) was performed for statistical analysis. Results. Of the 152 patients, there were 32 patients in Group 1, 27 patients in Group 2, 48 patients in Group 3, and 45 patients in Group 4. Significantly greater HOS. f. was observed in Group 3 compared with Group 1 (p = 0.03) and in Group 4 compared with Group 2 (p = 0.02), with similar HOP. f. (p = 0.75 and p = 0.83, respectively), suggesting that patients who undergo surgery at Risser grade of 0 to 1 have a shorter spinal height at follow-up than those who have surgery at Risser 4 to 5. HOS. f. was similar to pHOS in both Group 1 and Group 2 (p = 0.62 and p = 0.45, respectively), indicating that undergoing surgery at Risser 0 to 1 does not necessarily affect final spinal height. Conclusion. This study shows that fusion surgery at Risser 0 may result in growth restriction unlike fusion surgery at Risser 3 to 4. Despite such growth restriction, AIS patients could reach their predicted or ‘normal’ spinal height after surgery regardless of baseline maturity status due to the longer baseline spinal length in AIS patients and the remaining growth potential at the non-fusion levels. Cite this article: Bone Joint J 2018;100-B:1372–6

Aims

The aim of this study was to determine whether there is an increased prevalence of scoliosis in patients who have suffered from a haematopoietic malignancy in childhood.

Low bone mass and osteopenia have been described in the axial and peripheral skeleton of patients with adolescent idiopathic scoliosis (AIS). Recently, many studies have shown that gene polymorphism is related to osteoporosis. However, no studies have linked the association between IL6 gene polymorphism and bone mass in AIS. This study examined the association between bone mass and IL6 gene polymorphism in 198 girls with AIS. The polymorphisms of IL6-597 G→A, IL6-572 G→C and IL6-174 G→A and the bone mineral density in the lumbar spine and femoral neck were analysed and compared with their levels in healthy controls. The mean bone mineral density at both sites in patients with AIS was decreased compared with controls (p = 0.0022 and p = 0.0013, respectively). Comparison of genotype frequencies between AIS and healthy controls revealed a statistically significant difference in IL6-572 G→C polymorphism (p = 0.0305). There was a significant association between the IL6-572 G→C polymorphism and bone mineral density in the lumbar spine, with the CC genotype significantly higher with the GC (p = 0.0124) or GG (p = 0.0066) genotypes. These results suggest that the IL6-572 G→C polymorphism is associated with bone mineral density in the lumbar spine in Korean girls with AIS

A series of 98 patients with juvenile idiopathic scoliosis have been analysed. This showed that between the ages of four and six there was a higher incidence in boys whereas between seven and nine years of age, the proportion of girls was higher. Regardless of sex and age the majority of the curves were convex to the right and the single thoracic curve was the commonest pattern. Spontaneous resolution occurred in seven patients: in four the curves resolved within two years; in the three others the curves resolved in three, four and five years respectively. Forty-four per cent of all patients were managed conservatively and in 56 per cent spinal fusion was carried out

A review was performed of 86 cases of infantile idiopathic scoliosis treated between 1962 and 1979. The single primary curves were classified as resolving, stable, progressive with a low rib--vertebra angle difference (RVAD) and progressive with a high RVAD. Two single primary curves subsequently developed a second curve and 17 were double when first diagnosed. Prognosis was difficult to establish before the age of five years. Only 18 per cent of curves showing progression beyond 50 degrees reached that point before the age of four. Conversely, if a scoliosis of 50 degrees or more was present before the age of four it always progressed. A more favourable outcome was indicated by male sex, a left-sided curve, a low initial curve measurement, an RVAD of less than 20 degrees in the initial radiograph, and the onset of scoliosis in the first year of life

Stature and its components were examined in 143 girls aged 11 to 15 years with adolescent idiopathic scoliosis. Correction was made for loss of height due to the lateral spinal curvature, and the findings were compared with those from 202 healthy girls of similar age. Using three components of stature (suprapelvic, pelvic and subischial heights) we were able to show that the relatively greater stature of girls with adolescent idiopathic scoliosis was due to changes in the pelvis and lower limbs but not significantly in the spine. Suprapelvic height was reduced relative to subischial height; this probably represents the growth pattern of predominantly ectomorphic individuals, reflecting the physique of many of these girls. Pelvic height was disproportionately increased, and this is considered to be a true rather than an apparent difference. Cephalocaudal disproportion involving two segments suggests a common mechanism of causation which is unlikely to be secondary to the scoliosis. These physical features may in some way be associated with a predisposition to progression of the scoliosis

Analysis was made of the results of two methods of treatment of progressive infantile idiopathic scoliosis: a group of forty-eight children treated by rib resection, localiser jackets and Milwaukee brace were compared with a 'control' group of nineteen children treated by localiser jackets and Milwaukee brace alone. This failed to show any significant difference between the two groups. Carefully controlled splintage was effective in slowing the rate of progression of most curves which was not significantly affected by the addition of rib resection

A study of 130 scoliotic children with curves measuring 10 degrees or more has been performed in order to elucidate the importance of stature, growth and development. Girls with adolescent idiopathic curves measuring 15 degrees or more were taller than girls with smaller idiopathic curves and taller than those whose scoliosis was secondary to leg-length inequality (pelvic tilt scoliosis). No differences were observed as regards growth velocity or development. The increased standing height may be genetic but the uncoiling effect of the normal kyphosis to give a flat lateral profile is a more likely cause. The familial trend in idiopathic scoliosis may therefore be explained by the genetically determined shape of the spine in the median (sagittal) plane

Twenty-seven children with progressive infantile idiopathic scoliosis have been reviewed after long-term management. Twenty-two children had single thoracic curves which were diagnosed at an early age and treated in a modified Milwaukee brace until the age of ten years when the spine was corrected and fused. The mean correction after operation was 40 per cent of the initial degree of curvature seen in early childhood before treatment. Solid spinal fusion led to a further moderate loss in correction due to bending of the fusion mass before the spine became stable several years later. Five children had double structural curves and were treated only in a brace. This provided less satisfactory control of these curves but because of the minimal cosmetic deformity, extensive spinal fusion was avoided

The incidence of congenital dislocation of the hip in 156 children with infantile idiopathic scoliosis was 6.4 per cent, approximately 10 times its frequency in the general population. In both of these deformities there was a predominance of girls (eight girls: two boys). In unilateral dislocation of the hip the convexity of the thoracic scoliosis was on the same side as the dislocation. Eight out of the 10 children with both deformities also had plagiocephaly

One hundred and sixty-seven patients with adolescent idiopathic scoliosis were allocated prospectively to one of three different groups for correction before undergoing posterior spinal fusion and Harrington instrumentation, In group 1 single curves were corrected by a Risser turnbuckle plaster jacket and double curves by halo-pelvic traction. In Group 2 patients performed Cotrel dynamic traction for three weeks and this was followed by correction in a plaster cast. In Group 3 patients were given Cotrel dynamic traction for one week only and the operation was performed without a plaster cast. There was no significant difference in the overall correction achieved among the patients in the three groups except that double curves corrected slightly better in Group 2. The correction achieved by Cotrel dynamic traction after three weeks was not significantly different from that obtained at 48 hours. An anteroposterior radiograph of the spine taken during Cotrel dynamic traction was a valuable guide to the mobility of the curve and is preferable to radiographs of the patients bending laterally, particularly with respect to curves over 70 degrees. The paper concludes that correction before operation is not required routinely in adolescent idiopathic curves unless the deformity is a severe and rigid one in which case a radiograph during Cotrel traction is a useful assessment

Twenty-eight patients with adolescent idiopathic scoliosis treated by anterior spinal fusion with Dwyer instrumentation were reviewed. The average length of follow-up was 6.9 years. This technique produced better correction of lateral curvature and rotation than Harrington instrumentation, particularly in the thoracolumbar and lumbar region. The length of spine requiring fusion was also shorter. There is, however, a tendency for Dwyer instrumentation to lead to kyphosis. Morbidity was significant and included one case of paraplegia, four cases of deep infection and one case of instrument failure. All of these complications, except one case of deep infection, occurred in patients with curves with an apex above the seventh thoracic vertebra

We reviewed 77 unfused and 91 fused patients with idiopathic scoliosis who first attended between 1949 and 1965. Both groups were re-examined at least 10 years after reaching skeletal maturity, with attention to progression of the Cobb angle, increased in vertebral rotation, back pain and psychosocial problems. We found that spinal fusion protects the scoliotic spine from further deterioration during adult life except for those with severe curves and marked rotation. Fusion also significantly reduced the incidence of severe pain and allowed patients to carry out heavy physical work, but did not confer complete immunity from backache. Surgery improved the appearance, but patients were not always completely satisfied with the cosmetic result

We have investigated the effect of multisegmental spinal fusion on the long-term functional and radiological outcome in patients with scoliosis. We compared these patients both with those whose spine had not been fused, and with a control group. We studied 68 patients with idiopathic scoliosis (34 operative and 34 non-operative) who had been followed up for a minimum of five years after treatment. They were matched for age (mean 44 years) and Cobb angle (mean 54°) at follow-up. An age- and gender-matched control group of 34 subjects was also recruited. All participants completed a questionnaire to assess spinal function and to grade the severity of back pain using a numerical rating scale. Radiographs of the spine were taken in the patients with scoliosis and lumbar degenerative changes were recorded. The spinal function scores for the patients with scoliosis who had had a fusion were similar to those who had not. Both scoliosis groups, however, had lower scores than the control group (p < 0.001). The frequency and severity of back pain were lower for patients with scoliosis and fusion than for those without, but higher for both scoliosis groups compared with the control group. Radiographs showed similar degenerative changes in both scoliosis groups

We present a method of visualising spinal deformities in three dimensions using conventional radiographs and computer graphics. The shape of the spinal column can be determined from the anteroposterior and lateral radiographs and displayed in any projection. In patients with adolescent idiopathic scoliosis, the fundamental lesion, an abnormal lordosis, can be demonstrated without the need for additional views. The method is applicable to other spinal deformities and may help to elucidate their three-dimensional shape

Routine examination for spinal deformity as part of a school health screening programme was introduced in Singapore in 1981. The three different ethnic groups included in the study provided figures for the prevalence of idiopathic scoliosis in an Asian population. A three-tier system of examination was used and a total of 110744 children in three age groups were studied. In those aged 6 to 7 years the prevalence was 0.12%. The prevalence in those aged 11 to 12 years was 1.7% for girls and 0.4% for boys, a ratio of 3.2 to 1. In girls aged 16 to 17 years the prevalence was 3.1%. In the latter two age groups there was a significantly higher prevalence in Chinese girls as compared with Malay and Indian girls. The optimal age for school screening seemed to be 11 to 12 years, but repeated examinations may be worthwhile

We have developed a simple technique for demonstrating the sagittal profile of each rotated level of a scoliotic spine and used it to determine the patterns of lordosis and kyphosis in each of six clinical types of idiopathic scoliosis. The currently accepted classification of scoliosis is inaccurate and a modification is proposed. The three main types of scoliosis were shown to have sagittal profiles distinctly different from each other and from normal. Single structural curves had short lordotic sections at their apices, limited above and below by kyphosis. Double curves showed longer lordotic sections limited only by one area of kyphosis. Lordosis throughout the thoracic and lumbar spine was associated with triple curve patterns. The biomechanical effects of the abnormal sagittal profiles provide a simple explanation for the genesis and progression of the different types of scoliosis, and the recognition of the pattern of the sagittal abnormalities permits treatment to be designed on a sound anatomical basis for individual cases

Fifty-nine children with juvenile idiopathic scoliosis have been reviewed. In six of eighteen who had no treatment the curvatures did not increase over eighteen months or more, while in twelve they increased at a median annual rate of six degrees. The pattern and magnitude of the curvature and the age at diagnosis could not be used accurately to predict progression. In forty-two patients progression occurred and Milwaukee brace treatment was begun, thirty being managed with periods of part-time wear. The median correction was 13 per cent. Serial measurements of the rib-vertebra angle difference (RVAD) were useful to predict a tendency to progress. Part-time wear of the brace was successful in cases where the RVAD values fell towards zero or became negative with treatment. Sixteen patients required surgical treatment; all had high positive RVAD values. However, with the plan of brace treatment outlined, part-time wear controlled many of the curvatures

This paper describes a simple method for the recording of rib deformity in idiopathic scoliosis. The relationships have been recorded between the measured rib hump and rib depression deformities and 1) the rotation of the vertebral bodies (as measured by the method of Nash and Moe on the standing radiograph); 2) the degree of lateral curvature (as measured by the method of Cobb on the standing radiograph); and 3) the rib-vertebra angles and their differences (as described by Mehta). No clear linear relationships were found. Many examples of irregular relationship were recorded, for example, marked spinal rotation with minimal rib hump. The response of the rib deformities to treatment by Milwaukee brace in fifty-two patients is described; the hump is little changed but the depression on the opposite side may be considerably reduced. Harrington instrumentation may have a similar effect

We have investigated whether patients with adolescent-onset idiopathic scoliosis (AIS) are more likely to have a low body-weight. Measurements of weight, height and body mass index (BMI) were made in 44 young women with AIS and compared with age- and gender-adjusted normative data. The body mass criteria of the International Classification of Diseases for eating disorders was used to determine how many patients were within the range considered to be ‘eating disordered’. Compared with the normative data, the AIS group did not differ significantly in terms of height, (p = 0.646), but they were significantly lighter (p < 0.001) and had significantly lower BMI scores (p < 0.001); 25% of the series had BMI scores which were within the range considered to be anorexic. The relationship between a diagnosis of AIS and low body-weight may indicate disordered eating and is thus a cause for concern, particularly in the light of the well-established relationship between eating psychopathology and osteoporosis. Aspects of organic health may need to be considered in addition to the cosmetic deformity

Aims. The aims of our study were to provide long-term information on the behaviour of the thoracolumbar/lumbar (TL/L) curve after thoracic anterior correction and fusion (ASF) and to determine the impact of ASF on pulmonary function. Patients and Methods. A total of 41 patients (four males, 37 females) with main thoracic (MT) adolescent idiopathic scoliosis (AIS) treated with ASF were included. Mean age at surgery was 15.2 years (11 to 27). Mean follow-up period was 13.5 years (10 to 18). Results. For the TL/L curve, the mean curve flexibility evaluated with supine pre-operative bending radiographs was 78.6% (standard deviation 16.5%), with no significant loss of correction observed. On comparing patients with an increase of the TL/L curve increase (> 4º, n = 9, 22%) to those without, significant differences were observed in the correction rate of the MT curve at the final follow-up (p = 0.011), correction loss of the MT curve (p = 0.003) and the proportion of patients who had semi-rigid instrumentation (p = 0.003). Pre-operative percentage predicted forced vital capacity (%FVC) was 80%, dropping to 72% at final follow-up (p < 0.001). The Scoliosis Research Society questionnaire score was not significantly different between patients with and without a TL/L curve increase (p = 0.606). Spontaneous lumbar curve correction (SLCC) was maintained up to 18 years following selective ASF in most patients and demonstrated significant correlation with maintenance of MT curve correction. Conclusion. Maintenance of MT curve correction using rigid instrumentation provided stable SLCC over time. An observed 8% decrease in %FVC indicates that ASF should be reserved for patients with no or only mild pulmonary impairment. Cite this article: Bone Joint J 2016;98-B:997–1002

Twenty-four children with infantile or juvenile idiopathic scoliosis had their spines corrected and solidly fused posteriorly before the age of eleven years. The growth of the fusion area was then accurately measured for a mean of 4.5 years during the adolescent growth spurt. During this period all longitudinal growth in the posterior elements ceased. The vertebral bodies continued to grow anteriorly, but the thick posterior fusion prevented the development of a lordosis. Initially the anterior growth was accommodated by narrowing of the intervertebral disc spaces, but eventually the vertebral bodies bulged laterally towards the convexity and pivoted on the posterior fusion, giving rise to loss of correction, increasing vertebral rotation and recurrence of the rib hump

Our aim in this prospective radiological study was to determine whether the flexibility rate calculated from radiographs obtained during forced traction under general anaesthesia, was better than that of fulcrum-bending radiographs before corrective surgery in predicting the extent of the available correction in patients with idiopathic scoliosis. We evaluated 33 patients with a Cobb angle > 60° on a standing posteroanterior radiograph, who had been treated by posterior correction. Pre-operative standing fulcrum-bending radiographs and those with forced-traction under general anaesthesia were obtained. Post-operative standing radiographs were taken after surgical correction. The mean forced-traction flexibility rate was 55% (. sd. 11.3) which was significantly higher than the mean fulcrum-bending flexibility rate of 32% (. sd. 16.1) (p < 0.001). We found no correlation between either the forced-traction or fulcrum-bending flexibility rates and the correction rate post-operatively (p = 0.24 and p = 0.44, respectively). Radiographs obtained during forced traction under general anaesthesia were better at predicting the flexibility of the curve than fulcrum-bending radiographs in curves with a Cobb angle > 60° in the standing position and may identify those patients for whom supplementary anterior surgery can be avoided

Adolescent idiopathic scoliosis in 152 patients was treated by Luque L-rod instrumentation and early mobilisation without external support. This series was compared with a matched group of 156 patients treated by Harrington instrumentation and immobilised in an underarm jacket for nine months. All the operations in both groups were performed by one surgeon and the patients were followed prospectively for more than two years. Correction of the scoliosis in the frontal plane was similar in both groups. However, the normal sagittal contour was better maintained with Luque rods, especially in the thoracolumbar and lumbar regions, and provided less loss of correction than with Harrington rods. Neither method significantly derotated the scoliosis. All the patients with Luque instrumentation developed a solid fusion despite breakage of the sublaminar wires at one or two levels in 4.9%. There were no major neurological complications with either type of instrumentation

A prospective study to investigate changes in the rib hump or rib deformity after correction of the lateral curvature in adolescent idiopathic scoliosis is reported. The operative treatment for 47 patients was by a Harrington distraction rod and posterior fusion. Before operation and at follow-up, measurements of the Cobb angle, of vertebral rotation, and of the rib deformity were taken. Despite operative correction of the lateral curve, there was a progression of the rib deformity in 64% of the cases after four years. Correction of the lateral curve may thus have no effect on vertebral rotation and cannot be guaranteed to effect a permanent reduction of the rib hump

Aims

To clarify the asymmetrical ossification of the epiphyseal ring between the convex and concave sides in patients with adolescent idiopathic scoliosis (AIS).

We studied 29 girls and one boy with adolescent idiopathic scoliosis who were at Risser grade 0 at the time of posterior spinal fusion and were followed until maturity (mean 7.8 years). We used serial radiographs to measure the ratio of disc to vertebral height in the fused segments and to detect differential anterior spinal growth and assess its effect on scoliosis, vertebral rotation, kyphosis, and rib-vertebral-angle difference (RVAD). From one year after surgery to the latest review, the percentage anterior disc height decreased by nearly one-half and the percentage posterior disc height by nearly one-third in the fused segments (p < 0.001). There was a 4 degree increase in mean Cobb angle (p < 0.001), 11 patients (37%) having an increase of between 6 degrees and 10 degrees. There was a significant increase in mean apical rotation by 2 degrees (p = 0.003), and four patients (13%) had an increase of between 6 degrees and 16 degrees. There was little change in kyphosis. There was an increase in mean RVAD by 4 degrees (p = 0.003), seven patients (23%) showing a reduction by 1 degree to 7 degrees, and 11 (37%) increases of between 6 degrees and 16 degrees. Spinal growth occurs after posterior fusion in adolescents who are skeletally immature, as a result of continued anterior vertebral growth. There is some progression of scoliosis, vertebral rotation, and RVAD, but little change in kyphosis. The increase in deformity is not enough to warrant the use of combined anterior and posterior fusion. The findings are relevant to the management of progressive curves, the timing and extent of surgery, and the prognosis for progression of deformity in this group of patients

Aims

A total of 30 patients with thoracolumbar/lumbar adolescent idiopathic scoliosis (AIS) treated between 1989 and 2000 with anterior correction and fusion surgery using dual-rod instrumentation were reviewed.

We undertook a comparative study of magnetic resonance imaging (MRI) vertebral morphometry of thoracic vertebrae of girls with adolescent idiopathic thoracic scoliosis (AIS) and age and gender-matched normal subjects, in order to investigate abnormal differential growth of the anterior and posterior elements of the thoracic vertebrae in patients with scoliosis. Previous studies have suggested that disproportionate growth of the anterior and posterior columns may contribute to the development of AIS. Whole spine MRI was undertaken on 83 girls with AIS between the age of 12 and 14 years, and Cobb’s angles of between 20° and 90°, and 22 age-matched controls. Multiple measurements of each thoracic vertebra were obtained from the best sagittal and axial MRI cuts. Compared with the controls, the scoliotic spines had longer vertebral bodies between T1 and T12 in the anterior column and shorter pedicles with a larger interpedicular distance in the posterior column. The differential growth between the anterior and the posterior elements of each thoracic vertebra in the patients with AIS was significantly different from that in the controls (p < 0.01). There was also a significant positive correlation between the scoliosis severity score and the ratio of differential growth between the anterior and posterior columns for each thoracic vertebra (p < 0.01). Compared with age-matched controls, the longitudinal growth of the vertebral bodies in patients with AIS is disproportionate and faster and mainly occurs by endochondral ossification. In contrast, the circumferential growth by membranous ossification is slower in both the vertebral bodies and pedicles.

We have studied 34 consecutive patients receiving Cotrel- Dubousset instrumentation for a single and flexible thoracic scoliotic curve, evaluating the rib hump deformity from a single CT scan through the apical vertebra of the curve. Using two measures of rotation we found a mean improvement of 25% in the rotation of the vertebra after operation. Any, usually minor, deterioration occurred in the first six months postoperatively, and there was no significant further deterioration in 19 patients assessed over two years after surgery. Cotrel-Dubousset instrumentation can produce a significant correction of vertebral rotation and of the associated rib hump deformity.

Eleven articulated scoliotic spines were examined radiographically and morphometrically. Measurement of the curve on anteroposterior radiographs of the specimens gave a mean Cobb angle of 70 degrees, though true anteroposterior radiographs of the deformity revealed a mean Cobb angle of 99 degrees (41% greater). Lateral radiographs gave the erroneous impression that there was a mean kyphosis of 41 degrees while true lateral projections revealed a mean apical lordosis of 14 degrees. Morphometric measurements confirmed the presence of a lordosis at bony level, the apical vertebral bodies being significantly taller anteriorly (P less than 0.02). There were significant correlations (P less than 0.01) between the true size of the lateral scoliosis, the amount of axial rotation and the size of the apical lordosis. This study illustrates the three-dimensional nature of the deformity in scoliosis and its property of changing in character and magnitude according to the plane of radiographic projection.

Recent surveys have shown that idiopathic structural scoliosis of mild degree is generally not progressive. We will propose a mechanism which may be responsible for deterioration in the few. It has been observed that the spinal cord, although displaced towards the concavity, does not rotate in company with the vertebrae, thus exposing the emerging nerve roots to the effects of traction and possibly of entrapment. We suggest that progression occurs when the neuraxis is unable to adjust to the change in the anatomy of vertebral column. Our proposition is based upon our findings in a complete spinal column obtained from a baby with structural scoliosis. Support is provided by intercostal angiography, and by observations upon normal anatomy, the pathological anatomy of mature scoliotic spines and the anatomy of contrived scoliosis in normal spines. Although our histological and electrophysiological investigations are incomplete we can demonstrate a significant increase in degenerate cells in the dorsal root ganglia at the apex on the convex side. Lack of suitable necropsy material prevents us from confirming our observations so that our report is inevitably preliminary. We enter a plea that careful examination of the neuraxis be undertaken whenever a specimen of a scoliotic spine becomes available.

Costoplasty can reduce the important cosmetic deformity of rib prominence in scoliosis but there are few objective reports of correction. We recorded the results of three objective methods of assessing back shape before and after short-segment costoplasty in 55 patients. We showed that significant improvement was maintained over a two-year follow-up period. Primary costoplasty at the time of scoliosis surgery (n = 35) achieved greater proportional correction than secondary costoplasty performed after fusion of the spine (n = 20). The rib segments removed at primary surgery provided enough bone for the autogenous graft; harvesting from the pelvis was unnecessary. We report a new classification of rib morphology which helps in planning the site and extent of costoplasty, and in predicting the possible correction.

Aims. Spinal deformity surgery carries the risk of neurological injury. Neurophysiological monitoring allows early identification of intraoperative cord injury which enables early intervention resulting in a better prognosis. Although multimodal monitoring is the ideal, resource constraints make surgeon-directed intraoperative transcranial motor evoked potential (TcMEP) monitoring a useful compromise. Our experience using surgeon-directed TcMEP is presented in terms of viability, safety, and efficacy. Methods. We carried out a retrospective review of a single surgeon’s prospectively maintained database of cases in which TcMEP monitoring had been used between 2010 and 2017. The upper limbs were used as the control. A true alert was recorded when there was a 50% or more loss of amplitude from the lower limbs with maintained upper limb signals. Patients with true alerts were identified and their case history analyzed. Results. Of the 299 cases reviewed, 279 (93.3%) had acceptable traces throughout and awoke with normal clinical neurological function. No patient with normal traces had a postoperative clinical neurological deficit. True alerts occurred in 20 cases (6.7%). The diagnoses of the alert group included nine cases of adolescent idiopathic scoliosis (AIS) (45%) and six of congenital scoliosis (30%). The incidence of deterioration based on diagnosis was 9/153 (6%) for AIS, 6/30 (20%) for congenital scoliosis, and 2/16 (12.5%) for spinal tuberculosis. Deterioration was much more common in congenital scoliosis than in AIS (p = 0.020). Overall, 65% of alerts occurred during rod instrumentation: 15% occurred during decompression of the internal apex in vertebral column resection surgery. Four alert cases (20%) awoke with clinically detectable neurological compromise. Conclusion. Surgeon-directed TcMEP monitoring has a 100% negative predictive value and allows early identification of physiological cord distress, thereby enabling immediate intervention. In resource constrained environments, surgeon-directed TcMEP is a viable and effective method of intraoperative spinal cord monitoring. Level of evidence: III. Cite this article: Bone Joint J 2021;103-B(3):547–552

Aims. We report the use of the distal radius and ulna (DRU) classification for the prediction of peak growth (PG) and growth cessation (GC) in 777 patients with idiopathic scoliosis. We compare this classification with other commonly used parameters of maturity. Patients and Methods. The following data were extracted from the patients’ records and radiographs: chronological age, body height (BH), arm span (AS), date of menarche, Risser sign, DRU grade and status of the phalangeal and metacarpal physes. The mean rates of growth were recorded according to each parameter of maturity. PG was defined as the summit of the curve and GC as the plateau in deceleration of growth. The rates of growth at PG and GC were used for analysis using receiver operating characteristic (ROC) curves to determine the strength and cutoff values of the parameters of growth. Results. The most specific grades for PG using the DRU classification were radial grade 6 and ulnar grade 5, and for GC were radial grade 9 and ulnar grade 7. The DRU classification spanned both PG and GC, enabling better prediction of these clinically relevant stages than other methods. The rate of PG (≥ 0.7 cm/month) and GC (≤ 0.15 cm/month) was the same for girls and boys, in BH and AS measurements. Conclusion. This is the first study to note that the DRU classification can predict both PG and GC, providing evidence that it may aid the management of patients with idiopathic scoliosis. Cite this article: Bone Joint J 2016;98-B:1689–96

Aims. Significant correction of an adolescent idiopathic scoliosis in the coronal plane through a posterior approach is associated with hypokyphosis. Factors such as the magnitude of the preoperative coronal curve, the use of hooks, number of levels fused, preoperative kyphosis, screw density, and rod type have all been implicated. Maintaining the normal thoracic kyphosis is important as hypokyphosis is associated with proximal junctional failure (PJF) and early onset degeneration of the spine. The aim of this study was to determine if coronal correction per se was the most relevant factor in generating hypokyphosis. Methods. A total of 95 patients (87% female) with a median age of 14 years were included in our study. Pre- and postoperative radiographs were measured and the operative data including upper instrumented vertebra (UIV), lower instrumented vertebra (LIV), metal density, and thoracic flexibility noted. Further analysis of the post-surgical coronal outcome (group 1 < 60% correction and group 2 ≥ 60%) were studied for their association with the postoperative kyphosis in the sagittal plane using univariate and multivariate logistic regression. Results. Of the 95 patients, 71.6% (68) had a thoracic correction of > 60%. Most (97.8%) had metal density < 80%, while thoracic flexibility > 50% was found in 30.5% (29). Preoperative hypokyphosis (< 20°) was present in 25.3%. A postoperative thoracic hypokyphosis was four times more likely to occur in patients with thoracic correction ≥ 60% (odds ratio (OR) 4.08; p = 0.005), after adjusting for confounding variables. This association was not affected by metal density, thoracic flexibility, LIV, UIV, age, or sex. Conclusion. Our study supports the ‘essential lordosis’ hypothesis of Roaf and Dickson, i.e. with a greater ability to translate the apical vertebra towards the midline, there is a commensurate lengthening of the anterior column due to the vertebral wedging. Cite this article: Bone Joint J 2020;102-B(4):513–518

Aims. Intraoperative 3D navigation (ION) allows high accuracy to be achieved in spinal surgery, but poor workflow has prevented its widespread uptake. The technical demands on ION when used in patients with adolescent idiopathic scoliosis (AIS) are higher than for other more established indications. Lean principles have been applied to industry and to health care with good effects. While ensuring optimal accuracy of instrumentation and safety, the implementation of ION and its associated productivity was evaluated in this study for AIS surgery in order to enhance the workflow of this technique. The aim was to optimize the use of ION by the application of lean principles in AIS surgery. Methods. A total of 20 consecutive patients with AIS were treated with ION corrective spinal surgery. Both qualitative and quantitative analysis was performed with real-time modifications. Operating time, scan time, dose length product (measure of CT radiation exposure), use of fluoroscopy, the influence of the reference frame, blood loss, and neuromonitoring were assessed. Results. The greatest gains in productivity were in avoiding repeat intraoperative scans (a mean of 248 minutes for patients who had two scans, and a mean 180 minutes for those who had a single scan). Optimizing accuracy was the biggest factor influencing this, which was reliant on incremental changes to the operating setup and technique. Conclusion. The application of lean principles to the introduction of ION for AIS surgery helps assimilate this method into the environment of the operating theatre. Data and stakeholder analysis identified a reproducible technique for using ION for AIS surgery, reducing operating time, and radiation exposure. Cite this article: Bone Joint J. 2020;102-B(1):5–10

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.

Aims. We present the results of correcting a double or triple curve adolescent idiopathic scoliosis using a convex segmental pedicle screw technique. Patients and Methods. We reviewed 191 patients with a mean age at surgery of 15 years (11 to 23.3). Pedicle screws were placed at the convexity of each curve. Concave screws were inserted at one or two cephalad levels and two caudal levels. The mean operating time was 183 minutes (132 to 276) and the mean blood loss 0.22% of the total blood volume (0.08% to 0.4%). Multimodal monitoring remained stable throughout the operation. The mean hospital stay was 6.8 days (5 to 15). Results. The mean post-operative follow-up was 5.8 years (2.5 to 9.5). There were no neurological complications, deep wound infection, obvious nonunion or need for revision surgery. Upper thoracic scoliosis was corrected by a mean 68.2% (38% to 48%, p < 0.001). Main thoracic scoliosis was corrected by a mean 71% (43.5% to 8.9%, p < 0.001). Lumbar scoliosis was corrected by a mean 72.3% (41% to 90%, p < 0.001). No patient lost more than 3° of correction at follow-up. The thoracic kyphosis improved by 13.1° (-21° to 49°, p < 0.001); the lumbar lordosis remained unchanged (p = 0.58). Coronal imbalance was corrected by a mean 98% (0% to 100%, p < 0.001). Sagittal imbalance was corrected by a mean 96% (20% to 100%, p < 0.001). The Scoliosis Research Society Outcomes Questionnaire score improved from a mean 3.6 to 4.6 (2.4 to 4, p < 0.001); patient satisfaction was a mean 4.9 (4.8 to 5). . Conclusions. This technique carries low neurological and vascular risks because the screws are placed in the pedicles of the convex side of the curve, away from the spinal cord, cauda equina and the aorta. A low implant density (pedicle screw density 1.2, when a density of 2 represents placement of pedicle screws bilaterally at every instrumented segment) achieved satisfactory correction of the scoliosis, an improved thoracic kyphosis and normal global sagittal balance. Both patient satisfaction and functional outcomes were excellent. Cite this article: Bone Joint J 2017;99-B:1080–7

1. The natural history and prognosis of progressive infantile idiopathic scoliosis are reviewed and twenty-eight cases are reported. 2. Resolving infantile idiopathic scoliosis is described and seven cases are reported. 3. The length of the curve, the degree of rotation, the age of onset of deterioration, and the rate of progression are the important factors in determining the type and severity of the deformity. 4. Infantile idiopathic scoliosis is briefly compared with congenital scoliosis

Aims

The aim of this study was to understand the experience of mature patients who undergo a periacetabular osteotomy (PAO), a major hip-preserving surgical procedure that treats symptomatic hip dysplasia by realigning the acetabulum. Our aim was to improve our understanding of how the operation affected the lives of patients and their families, with a long-term goal of improving their experience.

Segmental vessel ligation during anterior spinal surgery has been associated with paraplegia. However, the incidence and risk factors for this devastating complication are debated. We reviewed 346 consecutive paediatric and adolescent patients ranging in age from three to 18 years who underwent surgery for anterior spinal deformity through a thoracic or thoracoabdominal approach, during which 2651 segmental vessels were ligated. There were 173 patients with idiopathic scoliosis, 80 with congenital scoliosis or kyphosis, 43 with neuromuscular and 31 with syndromic scoliosis, 12 with a scoliosis associated with intraspinal abnormalities, and seven with a kyphosis. There was only one neurological complication, which occurred in a patient with a 127° congenital thoracic scoliosis due to a unilateral unsegmented bar with contralateral hemivertebrae at the same level associated with a thoracic diastematomyelia and tethered cord. This patient was operated upon early in the series, when intra-operative spinal cord monitoring was not available. Intra-operative spinal cord monitoring with the use of somatosensory evoked potentials alone or with motor evoked potentials was performed in 331 patients. This showed no evidence of signal change after ligation of the segmental vessels. In our experience, unilateral segmental vessel ligation carries no risk of neurological damage to the spinal cord unless performed in patients with complex congenital spinal deformities occurring primarily in the thoracic spine and associated with intraspinal anomalies at the same level, where the vascular supply to the cord may be abnormal

Aims

The outcome following the development of neurological complications after corrective surgery for scoliosis varies from full recovery to a permanent deficit. This study aimed to assess the prognosis and recovery of major neurological deficits in these patients, and to determine the risk factors for non-recovery, at a minimum follow-up of two years.

Clinical, radiological, and Scoliosis Research Society-22 questionnaire data were reviewed pre-operatively and two years post-operatively for patients with thoracolumbar/lumbar adolescent idiopathic scoliosis treated by posterior spinal fusion using a unilateral convex segmental pedicle screw technique. A total of 72 patients were included (67 female, 5 male; mean age at surgery 16.7 years (13 to 23)) and divided into groups: group 1 included 53 patients who underwent fusion between the vertebrae at the limit of the curve (proximal and distal end vertebrae); group 2 included 19 patients who underwent extension of the fusion distally beyond the caudal end vertebra. A mean scoliosis correction of 80% (45% to 100%) was achieved. The mean post-operative lowest instrumented vertebra angle, apical vertebra translation and trunk shift were less than in previous studies. A total of five pre-operative radiological parameters differed significantly between the groups and correlated with the extension of the fusion distally: the size of the thoracolumbar/lumbar curve, the lowest instrumented vertebra angle, apical vertebra translation, the Cobb angle on lumbar convex bending and the size of the compensatory thoracic curve. Regression analysis allowed an equation incorporating these parameters to be developed which had a positive predictive value of 81% in determining whether the lowest instrumented vertebra should be at the caudal end vertebra or one or two levels more distal. There were no differences in the Scoliosis Research Society-22 outcome scores between the two groups (p = 0.17). In conclusion, thoracolumbar/lumbar curves in patients with adolescent idiopathic scoliosis may be effectively treated by posterior spinal fusion using a unilateral segmental pedicle screw technique. Five radiological parameters correlate with the need for distal extension of the fusion, and an equation incorporating these parameters reliably informs selection of the lowest instrumented vertebra. Cite this article: Bone Joint J 2014;96-B:1082–9

Infantile idiopathic scoliosis is a structural scoliosis seen in infants, usually boys, with the major curve to the left in almost all cases, and almost invariably in the mid-or lower thoracic region. It occasionally disappears, but in general the curve tends to increase. In the absence of any discoverable etiology it is termed "idiopathic" and it is believed not to differ in essentials from the more common adolescent scoliosis. Lumbar idiopathic scoliosis has a good prognosis as to deformity, but leads more often than any other curvature to degenerative arthritis and pain in later life

We have measured the increase in height and width of the vertebral bodies and expressed them as percentages of the total growth in children aged 10 to 17 years. The first group, 10 boys and 10 girls, each had a single thoracic adolescent idiopathic scoliosis while the second group, 10 girls, each had a single lumbar adolescent idiopathic scoliosis. No significant differences were found between the growth increments and spinal dimensions of the vertebral bodies involved in the scoliotic curve and those vertebrae outside the curve in the same patient. The vertebrae were more slender in girls than in boys

We reviewed retrospectively the role of monitoring of somatosensory spinal evoked potentials (SSEP) in 99 patients with neuromuscular scoliosis who had had operative correction with Luque-Galveston rods and sublaminar wiring. Our findings showed that SSEP monitoring was useful and that a 50% decrease in the amplitude of the trace optimised both sensitivity and specificity. The detection of true-positive results was higher than in cases of idiopathic scoliosis, but the method was less sensitive and specific and there were more false-negative results. In contrast with the findings in idiopathic scoliosis, recovery of the trace was associated with a 50% to 60% risk of neurological impairment. Only one permanent injury occurred during the use of this technique, and any temporary impairment resolved within two months

We report the results of vertebral column resection (VCR) for paediatric patients with spinal deformity. A total of 49 VCRs in paediatric patients from four university hospitals between 2005 and 2009 with a minimum two-year follow-up were retrospectively identified. After excluding single hemivertebral resections (n = 25) and VCRs performed for patients with myelomeningocele (n = 6), as well as spondylectomies performed for tumour (n = 4), there were 14 patients who had undergone full VCR at a mean age of 12.3 years (6.5 to 17.9). The aetiology was congenital scoliosis in five, neuromuscular scoliosis in three, congenital kyphosis in two, global kyphosis in two, adolescent idiopathic scoliosis in one and secondary scoliosis in one. A total of seven anteroposterior and seven posterolateral approaches were used. The mean major curve deformity was 86° (67° to 120°) pre-operatively and 37° (17° to 80°) at the two-year follow-up; correction was a mean of 54% (18% to 86%) in the anteroposterior and 60% (41% to 70%) in the posterolateral group at the two-year follow-up (p = 0.53). The mean Scoliosis Research Society-24 total scores were 100 (92 to 108) for the anteroposterior and 102 (95 to 105) for the posterolateral group. There was one paraparesis in the anteroposterior group necessitating urgent re-decompression, with a full recovery. Patients undergoing VCR are highly satisfied after a successful procedure

Preliminary costectomy before Harrington instrumentation and fusion for idiopathic scoliosis allows direct excision of the rib prominence and better correction at the second-stage operation. The excised rib fragments are used as grafts, thus avoiding the need for a separate pelvic incision. The management regime and the technique of costectomy are described. The results in 42 children, most suffering from adolescent idiopathic scoliosis and all treated by this method, have been reviewed. Respiratory function in a group of these children has been compared with that of a group treated by Harrington instrumentation alone. Costectomy produced a significantly greater reduction in total lung capacity and peak expiratory flow rate but, providing the preliminary lung function tests were reasonably normal, the cosmetic and psychological effects of costectomy were very rewarding

We reviewed 31 consecutive patients with Friedreich’s ataxia and scoliosis. There were 24 males and seven females with a mean age at presentation of 15.5 years (8.6 to 30.8) and a mean curve of 51° (13° to 140°). A total of 12 patients had thoracic curvatures, 11 had thoracolumbar and eight had double thoracic/lumbar. Two patients had long thoracolumbar collapsing scoliosis with pelvic obliquity and four had hyperkyphosis. Left-sided thoracic curves in nine patients (45%) and increased thoracic kyphosis differentiated these deformities from adolescent idiopathic scoliosis. There were 17 patients who underwent a posterior instrumented spinal fusion at mean age of 13.35 years, which achieved and maintained good correction of the deformity. Post-operative complications included one death due to cardiorespiratory failure, one revision to address nonunion and four patients with proximal junctional kyphosis who did not need extension of the fusion. There were no neurological complications and no wound infections. The rate of progression of the scoliosis in children kept under simple observation and those treated with bracing was less for lumbar curves during bracing and similar for thoracic curves. The scoliosis progressed in seven of nine children initially treated with a brace who later required surgery. Two patients presented after skeletal maturity with balanced curves not requiring correction. Three patients with severe deformities who would benefit from corrective surgery had significant cardiac co-morbidities

This is a study of children who first attended as infants with either progressive infantile idiopathic scoliosis or congenital scoliosis. All had a pattern of scoliosis in which early and damaging deterioration is inevitable. The infants were treated from before the age of three, initially by plaster casts and then a Milwaukee brace, followed at about the age of ten by correction and fusion. The cases were then observed to the end of growth or near that point. In the main study there were twelve cases, six of progressive infantile idiopathic scoliosis and six of congenital scoliosis, which were followed through this long period. Only one of the twelve had a curve worse at the end of growth compared with the initial radiograph as an infant; this one curve had increased only 16 degrees in almost as many years. Although small, the series does show that it is nearly always possible to control even the most serious scoliosis in an infant, if it is tackled early and unremittingly. There are supportive studies of children who have partially completed this regime, and interim results in a newer group of children with spina bifida and scoliosis

An attempt has been made to determine the aetiological factors in infantile idiopathic scoliosis from a clinical, genetic and epidemiological survey of 134 infants, ninety-seven of whom developed a curve in the first six months of life. Plagiocephaly was present in all cases; mental retardation occurred in 13 per cent of males with progressive scoliosis; congenital dislocation of the hip occurred in 3.5 per cent of cases and congenital heart disease in 2.5 per cent; and inguinal hernia was found in 7.4 per cent of males. Approximately 3 per cent of parents and 3 per cent of sibs had the same deformity, thirty times the general population frequency for the Edinburgh area. Other positive findings included an excess of breech presentations and of premature, low birthweight males, and a preponderance of curves developing in the winter months. Infants with progressive scoliosis tended to have older mothers and to come from poorer families. Only three children, all with resolving scoliosis, habitually lay prone in early infancy, in marked contrast to North American infants where this posture is usual. The almost complete absence of infantile idiopathic scoliosis in North America is noted and it is thought that the two facts may be related. The aetiology is likely to be multifactorial, with a genetic tendency to the deformity which is either "triggered off" or prevented by external factors