Aims. Scoliosis is a lateral curvature of the spine with associated rotation, often causing distress due to appearance. For some curves, there is good evidence to support the use of a spinal brace, worn for 20 to 24 hours a day to minimize the curve, making it as straight as possible during growth, preventing progression. Compliance can be poor due to appearance and comfort. A night-time brace, worn for eight to 12 hours, can achieve higher levels of curve correction while patients are supine, and could be preferable for patients, but evidence of efficacy is limited. This is the protocol for a randomized controlled trial of ‘full-time bracing’ versus ‘night-time bracing’ in adolescent idiopathic scoliosis (AIS). Methods. UK paediatric spine clinics will recruit 780 participants aged ten to 15 years-old with AIS, Risser stage 0, 1, or 2, and curve size (Cobb angle) 20° to 40° with apex at or below T7. Patients are randomly allocated 1:1, to either full-time or night-time bracing. A qualitative sub-study will explore communication and experiences of families in terms of bracing and research. Patient and Public Involvement & Engagement informed study design and will assist with aspects of trial delivery and dissemination. Discussion. The primary outcome is ‘treatment failure’ (Cobb angle progression to 50° or more before skeletal maturity); skeletal maturity is at Risser stage 4 in females and 5 in males, or ‘treatment success’ (Cobb angle less than 50° at skeletal maturity). The comparison is on a non-inferiority basis (non-inferiority margin 11%). Participants are followed up every six months while in brace, and at one and two years after skeletal maturity. Secondary outcomes include the Scoliosis Research Society 22 questionnaire and measures of quality of life, psychological effects of bracing, adherence, anxiety and depression, sleep, satisfaction, and educational attainment. All data will be collected through the British Spine Registry. Cite this article: Bone Jt Open 2023;4(11):873–880

Aims. To provide normative data that can assess spinal-related disability and the prevalence of back or leg pain among adults with no spinal conditions in the UK using validated questionnaires. Methods. A total of 1,000 participants with equal sex distribution were included and categorized in five age groups: 20 to 29, 30 to 39, 40 to 49, 50 to 59, and 60 to 69 years. Individuals with spinal pathologies were excluded. Participants completed the Scoliosis Research Society-22 (SRS-22r), visual analogue scale (VAS) for back/leg pain, and the EuroQol five-dimension index (EQ-5D/VAS) questionnaires, and disclosed their age, sex, and occupation. They were also categorized in five professional groups: doctors, nurses, allied health professionals, office workers, and manual workers. Results. The mean age of all participants was 43.8 years (20 to 69). There was no difference in the SRS-22r, EQ-5D, or VAS scores among male and female participants (p > 0.05). There was incremental decrease in SRS-22r total scores as the age increased. The mean EQ-5D index score (0.84) ranged little across the age groups (0.72 to 0.91) but reduced gradually with increasing age. There was difference between the SRS-22r total score (4.51), the individual domain scores, and the EQ-5D score (index: 0.94 and VAS: 89) for the doctors’ group compared to all other occupational categories (p < 0.001). Doctors had a younger mean age of participants, which may explain their improved spinal health. There was no difference in the total or sub-domain SRS-22r and EQ-5D scores between the other four occupational groups. Conclusion. This study provides the first normative data for the SRS-22r, EQ-5D, and VAS for back/leg pain questionnaires among adults in the UK. We recorded an excellent correlation between the three assessment tools with individuals who reported less back and leg pain having better quality of life and greater function. The participants’ age, rather than their sex or profession, appears to be the major determinant for spinal health and quality of life. Cite this article: Bone Jt Open 2022;3(2):130–134

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.

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

Introduction. Scoliosis surgery is a life-changing procedure, but not devoid of perioperative complications. Often patients' scoring systems do not cover their real-life needs, including return to pre-surgery activity. Return to school, physical education (PE) is an important indirect marker of recovery. Although anterior spinal fusion (ASF) may have advantages, compared to posterior spinal fusion (PSF), because of motion-saved segments, there is a paucity of literature about post-operative return to school/PE in the compared groups. Aim. To determine the recovery time for patients with scoliosis who underwent anterior spinal fusion (ASF) and posterior spinal fusion (PSF). Design. Prospective cohort. Methods. Patients undergoing Adolescent Idiopathic Scoliosis (AIS) surgery from 2018–2019, were examined. We excluded no-AIS and over 18y patients. The Scoliosis Sports Survey validated questionnaire was administered post-operatively regarding return to school, PE and other physical activities. ASF and PSF groups were subcategorised into: Selective Anterior Thoracic Fusion (SATF), Thoracolumbar Fusion (TLF), Short Posterior Spinal Fusion (SPSF) and Long Posterior Spinal Fusion (LPSF) procedures. Hospital length of stay (HLOS) and ICU LOS were recorded. Results. A total of 81 patients were contacted and 43 responded to the survey. The different procedures, return to school / PE, HLOS / ICU LOS, costoplasty are all analysed as shown below in Table 1. Conclusions. Anterior and posterior fusions had similar return to school rates at 1–3 months. The TLF and 2-stages groups returned to school the quickest, whilst the SATF had the longest return time. PSF patients returned to PE faster than ASF. Costoplasty, did not affect return time to school and PE. Further research assessing shorter fusions benefits should be conducted. For any figures or tables, please contact the authors directly

The aim of this study is to clarify the implication of ciliary pathway on the onset of the spinal curvature that occurs in Adolescent Idiopathic Scoliosis (AIS) patients through functional studies of two genes: POC5 and TTLL11. Since the genetic implication for AIS is accepted, many association and candidate gene analysis revealed the implication of ciliary genes. The characterisation of these two proteins was assessed by qPCR, WB and immunofluorescence in vitro using control cells and cells derived from AIS patients. The impact of genetic modification of these genes on the functionality of the proteins in vitro and in vivo was analysed in zebrafish model created by CRISPR/Cas9 using microCT and histologic analysis. Our study revealed that mutant cells, for both gene, were less ciliated and the primary cilia was significantly shorter compared to control cells. We also observed a default in cilia glutamylation by immunofluorescence and Western Blot. Moreover, we observed in both zebrafish model, a 3D spine curvature similar to the spinal deformation in AIS. Interestingly, our preliminary results of immunohistology showed a retinal defect, especially at the cone cell layer level. This study strongly supports the implication of the ciliary pathway in the onset of AIS and this is the first time that a mechanism is described for AIS. Indeed, we show that shorter cilia could be less sensitive to environmental factors due to lower glutamylation and result in altered signalling pathway. Identifying the biological mechanism involved is crucial for elucidating AIS pathogenesis

Adolescent Idiopathic Scoliosis (AIS) patients were subjected to four weeks of Physiotherapy Scoliosis Specific Exercise (PSSE). 124 (Exclusion = 3) Patients were enrolled and assigned to either complete their treatment in one 4-week bout (4WC) (63 patients, M. age. = 14.52), or to complete their treatment in two separate fortnightly bouts (2X2WC) (63 patients, M. age. = 14.26). Clinical exam, surface topography and Scoliometer readings were compared. The SRS-30 questionnaire before and after treatment was conducted at 6, 12, 18 and 24 months. Group 1 (4WC) showed significant improvements from baseline (Pre-3.73 – Post 3.9; p=0.026) after the course of treatment, and showed significant improvements at 12 months follow up in Mental Health (p=0.006), Aggregate score (p= 0.005) and Satisfaction score (p=0.011). Satisfaction score remained statistically significant at 18 months follow up (p=0.016). Group 2 (2X2WC) did not record a significant improvement from baseline (p=0.058); however, showed significant improvements in self-image (p=0.013). There was no statically significant difference in SRS scores with respect to follow up time. We conclude that Physiotherapy Scoliosis Specific Exercise (PSSE) is a successful non-invasive therapy for AIS. The modified Schroth technique (ScolioGold) shows significant improvement in SRS30 scores with the 4-week intensive course that are sustained at a 2 year follow up

Background. Scoliosis is described as a lateral spinal curvature exceeding ten degrees on radiograph with vertebral rotation. Approximately 80% of scoliosis presentations are adolescent idiopathic scoliosis (AIS). Current management for AIS in the UK occurs in Surgeon or Paediatrician-led clinics and can be conservative or surgical. The musculoskeletal assessment and triage of AIS appears well-suited to an advanced physiotherapist practitioner (APP) skill set. The aim of this service evaluation was to scope, develop, implement and evaluate a four-month pilot of an APP-led AIS triage pathway. Method and Results. Spinal Consultant deformity and scoliosis clinics were scoped and observed. Clinic inclusion criteria and a patient assessment form was developed. An APP AIS clinic was set up beside a consultant led clinic. All patients assessed were discussed with a spinal surgeon. Consultant and APP agreement (% of total), waiting times, surgical conversion, and patient satisfaction were reviewed. A clinical competency package was developed for training and development of APPs. A total of 49 patients were seen (20 sessions). Waiting list reduced from 10 weeks to 6 weeks. 45%(n=22) of new patients seen were diagnosed with AIS, 27% (n=6) were directly listed for surgery. Consultant/ APP percentage agreement was high for Cobb angle measurement (82%), management plans (90%), and further diagnostic requests (94%). There were no adverse events and high patient satisfaction levels (n=20), (100% Very satisfied or satisfied) were reported. Conclusion. APP-led AIS clinics can provide similar levels of management and assessment as Spinal Consultants with improved waiting times and high levels of satisfaction. Conflicts of interest: No conflicts of interest. Sources of funding: No funding obtained

There is a need for non-radiographic, objective outcome measures for children with Adolescent Idiopathic Scoliosis (AIS). Standing balance and stability is altered in children with AIS. The Margin of Stability (MoS) has been used to compare gait stability in clinical populations. Our objective was to compare the MoS in anterior-posterior (MoS. AP. ) and mediolateral (MoS. ML. ) directions in girls with AIS to Controls. Girls with AIS and healthy girls walked at three speeds on an instrumented treadmill wearing retroreflective markers, surrounded by motion capture cameras. The MoS. AP/ML. was calculated at left and right heel strike. Data was processed in Visual 3D. A two-way ANOVA was used to compare MoS. AP/ML. between group, speed and the interaction between group and speed. Pearson's correlation coefficient was used to compare the MoS to Cobb angle. Statistical significance was accepted when p > 0.05. A priori power analysis suggested 12 participants per group. Three Cases and four Controls were recruited. Girls with AIS all had right-sided main thoracic curves (Lenke type 1a, 61.3° ± 10.0°). MoS. AP. was significantly bigger for Cases compared to Controls on the left (p=0.038) and right foot (p=0.041). There was no significant difference between Cases and Controls for MoS. ML. , but there was a visual trend for a smaller MoS. ML. in Cases. There was no significant difference for speed or the interaction between group and speed for MoS. AP. or MoS. ML. In Cases, MoS. AP. increased with increasing Cobb angle on the left (r. 2. =0.687, p=0.054) and right (r. 2. =0.634, p=0.067) and MoS. ML. decreased with increasing Cobb angle on the left (r. 2. =-0.912, p=0.002). Further subjects are being recruited. Girls with Lenke type 1a AIS are more stable in the AP direction and less stable in the ML direction than Controls during treadmill walking. AP stability increases and ML stability decreases with increasing Cobb angle. This research suggests that the MoS could be used as an outcome measure for children with AIS. Continued work is required to increase the power of this study. Further work could consider these changes during walking overground, measuring an MoS or MoS-like measure using a wearable device, and in different curve types

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

To determine the current practice of scoliosis surgery in the UK. A 10 point questionnaire was constructed to identify the philosophy of surgeons on various aspects of scoliosis surgery such as choice of implant, bone graft, autologous blood transfusion (ABT), cord monitoring and computer assisted surgery. Results are compared with the current best evidence. Consultants and Fellows attending the 2009 British Scoliosis Society meeting. 50 questionnaires were completed: 45 Consultants and 5 Fellows. All pedicle screw construct favored by 25/50, hybrid 24/50 (one undecided). Posterior construct of less than 10 levels, 20/50 would not cross-link, 11/50 used one and 19/20 used two or more. More than ten levels 17/50 considered cross-links unnecessary, 4/50 used one and 29/50 used two or more. 88% preferred titanium alloy implants, while a mixture of stainless steel and cobalt chrome was used by others. For bone graft, substitutes (24), iliac crest (14), allograft (12) and demineralised bone matrix (9) in addition to local bone. 10/50 would use recombinant bone morphogenetic protein (3 for revision cases only). 39/50 routinely used intra-operative cell salvage or ABT drains and 4/50 never used autologous blood. All used cord monitoring, Sensory (19/50), Motor (2/50) and combined (29/50). None used computer-aided surgery. 26 operated alone 12 operated in pairs and 12 varied depending on type of case. This survey has brought to light interesting variations in scoliosis surgery in UK. It may reflect the conflicting evidence in the literature

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

Introduction. Although the association between osteoporosis and adolescent idiopathic scoliosis (AIS) has become widely accepted, the mechanism behind the development of osteoporosis and AIS remains unknown. To elucidate this relationship, we investigated the radiological and histological changes in a model of scoliosis in chickens, focusing on the cervical vertebrae that are not affected by scoliosis. Methods. 40 newly hatched broiler chickens were divided randomly into four equal groups: sham-operated chickens serving as control (CNT); pinealectomised chickens (PNX); and sham-operated (CNT+MLT) and pinealectomized chickens (PNX+MLT) that received intraperitoneal administration of MLT (8 mg/kg) at 2200 h daily. Pinealectomies were done at the age of 3 days. Before killing the chickens at 2 months of age, blood samples were collected at midnight and MLT concentrations were measured by radioimmunoassay. Post-mortem radiographs were examined for the presence of scoliosis, and microcomputed tomography (micro-CT) images were taken to assess the microstructure of the cervical vertebrae. Histological specimens of the scanned cervical vertebra were prepared, and a mid-sagittal section was stained with haematoxylin and eosin (HE) and tartrate-resistant acid phosphatase (TRAP) to assess the numbers of osteoblasts and osteoclasts, respectively. Results. Scoliosis developed at the thoracic spine in all chickens in the PNX group and in two of the PNX+MLT group. MLT concentrations in the PNX group were substantially reduced, whereas normal concentrations were restored in the PNX+MLT group and were normal in the CNT and CNT+MLT groups. Micro-CT data showed that chickens in the PNX group had a greater degree of generalised osteoporosis than did those in the other groups. The number of osteoblasts was significantly decreased in the PNX group, whereas we recorded no significant difference between the CNT, CNT+MLT, and PNX+MLT groups. The number of osteoclasts was similar in all groups. Conclusions. Our results suggest that MLT deficiency reduces osteoblast proliferation and leads to the development of scoliosis and osteoporosis. The restoration of MLT prevented the development of scoliosis and osteoporosis, indicating that MLT concentrations might be crucial to the development of scoliotic deformity and osteoporosis in AIS

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

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

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.

Aim:. To Determine The Effect Of Posterior Instrumented Fusion On Lung Function In Patients With Idiopathic Scoliosis Aged 8–11. Method:. Lung Function (Fvc And Fev1) Was Measured Before Surgery In 13 Patients (Aged 8 To 11) With Idiopathic Scoliosis. All Patients Had Curves Greater Than 50 And Had Undergone Posterior Instrumented Scoliosis Correction And Fusion With (3 Patients) Or Without (10 Patients) Same Day Anterior Convex Growth Arrest. Lung Function Tests Were Repeated 1–8 Years (Mean 5.3 Years) After Surgery. The Data Was Normalised To Take Into Account Standing Height And Loss Of Stature Due To Lateral Curvature, Allowing A Direct Comparison Of Percent Predicted Fev1 And Fvc Before And After Surgery. Results:. Mean Fvc Was 75% Before Surgery And 79% At Longest Follow-Up (1.9l To 2.7l). Mean Fev1 Was 73% Before Surgery And 76% At Longest Follow-Up (1.7 To 2.4). The Change In Lung Function After Surgery Did Not Correlate With Length Of Time To Longest Follow-Up. The Ratio Of Fev1 To Fvc Was 0.86 Before And After Surgery. Discussion:. In Idiopathic Scoliosis Below The Age Of 8 Years, Most Would Accept That Instrumentation Should Allow Spinal Growth To Try And Maintain Lung Function. Above The Age Of 11, Lung Function Is Probably Sufficient To Allow Instrumented Fusion With Its Reduced Complication Rate And Reduced Need For Further Surgery. Between The Ages Of 8 And 11 Controversy Exists. This Pilot Study Suggests That A Larger (Possibly Rct) Study Should Be Performed To Evaluate This Group Of Patients As Fusion May Be Appropriate As Percent Lung Function Corrected For Loss Of Height Due To Curvature Appears To Be Maintained. Conflict Of Interest Statement: No conflict of interest

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

Aim. The aim of this study is to identify the incidence of the presence of an Inverted Radial Reflex (IRR) in asymptomatic subjects with Adolescent Idiopathic Scoliosis and determine its significance. Methods. Our study group consists of Adolescent Idiopathic Scoliosis patients who presented consecutively in our institution from June to September 2010. They were either seen in outpatient clinics or as elective admissions prior to operative correction of their spinal deformity. The presence of an idiopathic scoliosis deformity and the absence of any abnormal neurological symptoms were our inclusion criteria. They were examined by two clinicians for the presence of IRR using a tendon hammer. As part of their management all such patients routinely undergo an MRI scan in our Trust to investigate for the presence of intraspinal pathology. When the IRR was present we looked at their MRI scans to identify any relevant abnormalities. Results: We identified 100 subjects. There were 72 females and 28 males with an average age of 15 years. The IRR was present in 12 cases and in 6 of them the sign was present bilaterally. There were no further associated signs or symptoms. All cases had recently undergone MRI of their whole spine and their investigations did not demonstrate any abnormalities in the lower cervical spine. Conclusion. We have found an incidence of 12% for the presence of IRR in our study group. In all cases there were no abnormal cord signal changes in MRI scans and we can therefore conclude that in asymptomatic Adolescent Idiopathic Scoliosis patients the presence of IRR is of no clinical significance

Introduction. Horizontal gaze palsy (HGP) in association with scoliosis has been reported in both orthopaedic and ophthalmological published work. Juvenile progressive scoliosis in combination with HGP is caused by a malfunction of the normal control mechanism for equilibrium related to the lower brain stem, mostly associated with ROBO3 gene mutation. The aim of this study is to establish the association of scoliosis and HGP. Methods. 13 cases (four families and three sporadic cases) with HGP and scoliosis were documented; other systemic and ocular associated findings were identified and genetic counselling was done. All patients had radiograph of the spine, cranial and spinal cord MRI, chromosome analysis, gene analysis, and full ophthalmological examination. Blood samples were tested for ROBO3 gene mutation at Engle Laboratory, USA. Results. Mean age at evaluation was 14·8 years (range 1–56). Scoliosis was of varying degree (Cobb angle range 10–65°, mean 38·4°), and horizontal pendularnystagmus of low amplitude and loss of conjugate horizontal eye movements were common in all patients. Scoliosis was early onset and progressive in all patients. Six of 11 patients underwent surgery (Cobb angle range 45–70°, mean 5·3°). There were four right thoracic, two left thoracic, three right thoracolumbar, and two left thoracolumbar curves. Two patients of the third family (cases 10 and 11) had mirror image thoracolumbar curves. Cranial and spinal MRI revealed cleft in medulla oblongata in all nine patients who underwent MRI. Two adult patients refused MRI and two infant MRI scans were suboptimal. Neurological examination was otherwise normal. Notably, the female patients of family 1 also had genital dysgenesis. This is the first report of this finding in association with HGPPS, and its significance is not yet known. Homozygous ROBO3 mutations were identified in affected members of all four families, despite no recognised consanguinity in two of the families. Two were homozygous nonsense mutations (G456X and W635X) and two families shared the same missense mutation (S1107R). Two of the three sporadic cases underwent mutation testing; one harboured a homozygous missense C901R mutation, whereas a mutation was not detected in the second (patient 4). There were no distinguishing features of patient 4, suggesting that she has a non-coding mutation or that there is a second HGPPS gene. Conclusions. Every child with HGP should be evaluated for a possibly associated scoliosis, which is a progressive condition. Our cases and other published work clearly indicate that even if scoliosis is not present at first evaluation, longitudinal follow-up will show the evolution and progression of scoliosis. ROBO3 mutation testing should be done